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- Pulmonary hypertension in systemic lupus erythematosus: pulmonary thromboembolism is the leading cause. (#55)

- World health organization group 5 pulmonary hypertension. (#53)

Vasopressin as an adjunct therapy for pulmonary hypertension: a case report. (#2)

- And 55 other publications
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1. Circ Heart Fail. 2013 Dec 2. [Epub ahead of print]

High Prevalence of Occult Pulmonary Venous Hypertension Revealed by Fluid Challenge in Pulmonary Hypertension.

Robbins IMHemnes ARPugh MEBrittain EZhao DXPiana RNFong PPNewman JH.
1Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN.

Abstract

BACKGROUND:

-Determining the cause for pulmonary hypertension (PH) is difficult in many patients. Pulmonary arterial hypertension (PAH) is differentiated from pulmonary venous hypertension (PVH) by a wedge pressure (PWP) >15 mmHg in PVH. Patients undergoing RHC for evaluation of PH may be dehydrated and have reduced intravascular volume, potentially leading to a falsely low measurement of PWP and an erroneous diagnosis of PAH. We hypothesized that a fluid challenge during RHC would identify occult pulmonary venous hypertension (OPVH).

METHODS AND RESULTS:

-We reviewed the results of patients undergoing fluid challenge in our PH database from 2004-2011. Baseline hemodynamics were obtained and repeated following infusion of 0.5 liters of normal saline over 5-10 minutes. Patients were categorized as OPVH if PWP increased to >15 mm Hg after fluid challenge. Baseline hemodynamics in 207 patients met criteria for PAH. Following fluid challenge, 46 patients (22.2%) developed a PWP >15 mm Hg and were re-classified as OPVH. OPVH patients had a greater increase in PWP compared to PAH patients, p<0.001, and their demographics and comorbid illnesses were similar to PVH patients. There were no adverse events related to fluid challenge.

CONCLUSIONS:

-Fluid challenge at the time of RHC is easily performed, safe, and identifies a large group of patients diagnosed initially with PAH, but for whom OPVH contributes to PH. These results have implications for therapeutic trials in PAH and support the routine use of fluid challenge during RHC in patients with risk factors for PVH.
  PMID: 24297689 [PubMed - as supplied by publisher]
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2. Eur J Pediatr. 2013 Dec 3. [Epub ahead of print]

Vasopressin as an adjunct therapy for pulmonary hypertension: a case report.

Malikiwi ASasi ATan KSehgal A.
Monash Newborn, Monash Children's Hospital, Melbourne, Australia.

Abstract

Vasopressin is emerging as a therapeutic adjunct option towards treatment of shock states in the pediatric population. Its effects on pulmonary vasculature are less well understood. This report describes a 5-month-old infant with nitric oxide-unresponsive pulmonary hypertension, oxygenation failure, and systemic hypotension. Vasopressin therapy improved oxygenation and blood pressure and biventricular function, allowing weaning of nitric oxide and inotropic support. No decrease in coronary flow was noted. Conclusions: Vasopressin could be considered as an adjunct option in infants with pulmonary hypertension and systemic hypotension. Echocardiographic monitoring during treatment is recommended.
  PMID: 24297672 [PubMed - as supplied by publisher]
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3. Pharmacology. 2013 Nov 29;92(5-6):281-285. [Epub ahead of print]

Effect of Bosentan on Pulmonary Hypertension Secondary to Systolic Heart Failure.

Padeletti MCaputo MZacà VTsioulpas CBernazzali SMondillo SMaccherini MJelic S.
Departments of Cardiovascular Diseases, University of Siena, Siena, Italy.

Abstract

Objectives: The dual endothelin receptor antagonist bosentan improves pulmonary vascular resistance (PVR) in patients with primary pulmonary hypertension (PH). The effects of bosentan on secondary PH due to systolic heart failure (HF) are not well defined. This study evaluates the effect and tolerability of bosentan in patients with PH secondary to HF. Methods: Seventeen adult HF patients with PH and New York Heart Association class III-IV symptoms were treated with bosentan, 62.5 mg twice daily, for 1 month, which was gradually increased to 125 mg twice daily thereafter. Right heart catheterization (RHC), a clinical evaluation and echocardiographic measurements were performed at baseline and at 4 ± 3 (mean ± SD) months of follow-up. Response to bosentan was defined as an improvement in clinical, echocardiographic and RHC parameters. Results: Six patients did not complete the study (therapy was discontinued due to hypotension, elevated liver enzymes or acute decompensation of HF), 11 patients completed the follow-up; 9 patients responded to therapy. Systemic arterial pressures, pulmonary pressures, PVR and the transpulmonary gradient significantly decreased compared with baseline levels in 9 responders (p = 0.05, 0.05, 0.01 and 0.004, respectively), and 4 became eligible for heart transplantation and 3 for left ventricular assist device implantation. Conclusions: Bosentan decreased pulmonary pressures and PVR in the majority of patients with PH secondary to systolic HF, thereby allowing them to be considered candidates for heart transplantation. © 2013 S. Karger AG, Basel.
  PMID: 24296902 [PubMed - as supplied by publisher]
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4. Clin Exp Rheumatol. 2013 Dec 2. [Epub ahead of print]

Recognition of pulmonary hypertension in the rheumatology community: lessons from a Quality Enhancement Research Initiative.

Khanna DTan MFurst DEHill NSMcLaughlin VVSilver RMSteen VDLanger ASeibold JR.
Department of Internal Medicine, University of Michigan Scleroderma Program, Ann Arbor, MI, USA. khannad@med.umich.edu.

Abstract

OBJECTIVES:

The aim of this study was to utilise the Quality Enhancement Research Initiative in Systemic Sclerosis (QuERI-SSc) to measure and reduce a perceived gap in the diagnosis of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc).

METHODS:

Rheumatologists enrolled patients with SSc (aged ≥18 years) and provided data on a panel of diagnostic tests over 3 years. Pulmonary function testing, echocardiography, 6-minute walk distance, N-terminal pro-brain natriuretic peptide assays, high-resolution computed tomography of the lungs, and ventilation/perfusion scan plus right heart catheterisation (RHC; when appropriate) were emphasised. Exclusion criteria included previously documented PAH, interstitial lung disease, and SSc overlapping with other connective tissue disease.

RESULTS:

Participating rheumatologists enrolled 207 patients with SSc (90% female; 80% white), with a median age of 57 years and median disease duration of 5 years. A total of 82% of patients were classified as New York Heart Association functional class I and II; of these patients, 177 had an echocardiogram at enrolment and 191 at any time during the study. Of those who met study-specified criteria for RHC at enrolment, only 3 of 7 patients underwent RHC.

CONCLUSIONS:

The screening algorithm was successful in identifying patients with mild impairment. Although specific tools were recommended for screening PAH in patients with SSc, results indicate that significant diagnostic care gaps still exist in the general rheumatology community. Better understanding and adherence to guidelines could improve the care and, ideally, outcomes of these high-risk patients.
  PMID: 24295227 [PubMed - as supplied by publisher]
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5. Exp Clin Cardiol. 2013 Winter;18(1):e40-e43.

Atorvastatin improves endothelial progenitor cell function and reduces pulmonary hypertension in patients with chronic pulmonary heart disease.

Liu HFQi XWMa LLYao DKWang L.
Department of Health, Liaocheng People's Hospital of Taishan Medical University, Shandong Province, China;

Abstract

OBJECTIVE:

To explore the effects of atorvastatin on the migration and adhesion of endothelial progenitor cells (EPCs) and on pulmonary artery pressure (PAP) in patients with chronic pulmonary heart disease.

METHODS:

A total of 68 patients with chronic pulmonary heart disease were randomly assigned to either a control group (n=35) or a treatment group (n=33). In addition, 30 healthy volunteers (17 male, 13 female) were enrolled as healthy controls. Atorvastatin (20 mg per day) was administered to the treatment group. The migration and adhesion activities of EPCs in peripheral blood were assessed before and six months after the treatment. PAP was measured using echocardiography before and after the treatment.

RESULTS:

EPC number, migration ability and adhesion activity in the peripheral blood of patients in the control and treatment groups were lower than in patients in the healthy control group at baseline (all P<0.05). After six months of atorvastatin therapy, the number of EPCs in the treatment group was greater than in the control group (P<0.05). Migration and adhesion functions of EPCs in the treatment group were greater than in the control group (all P<0.05). The reduction in PAP in the treatment group was greater than in the untreated control group following six months of therapy (P<0.05).

CONCLUSION:

Atorvastatin therapy increased the migration and adhesion activities of EPCs in patients with chronic pulmonary heart disease. Atorvastatin treatment was also associated with a reduction in PAP in these patients.
PMCID: PMC3716501 Free PMC Article
  PMID: 24294047 [PubMed - as supplied by publisher]
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6. Exp Clin Cardiol. 2013 Winter;18(1):e37-e39.

Relationship between serum uric acid levels and ventricular function in patients with idiopathic pulmonary hypertension.

Zhang CYMa LLWang LX.
Department of Cardiac Surgery, Liaocheng People's Hospital of Taishan Medical University, Liaocheng, Shandong, People's Republic of China;

Abstract

OBJECTIVE:

To investigate the relationship between serum uric acid levels and pulmonary hypertension in patients with idiopathic pulmonary artery hypertension (IPAH).

METHODS:

Serum uric acid levels were measured in 86 patients (mean [± SD] age 35.2±12.3 years; 36 men) with IPAH. Pulmonary arterial pressure and ventricular function were assessed using echocardiography. Serum uric acid levels were also measured in 40 healthy subjects (35.9±11.6 years of age; 15 men).

RESULTS:

Serum uric acid levels in IPAH patients were higher compared with control subjects (405±130 μmol/L versus 344±96 μmol/L; P<0.05). Fifty-two (60.4%) of the 86 patients with IPAH had elevated serum uric acid levels. The pulmonary systolic pressure and mean pulmonary pressure in the high uric acid group were higher than in the normal uric acid group (P<0.05). The left and right ventricular ejection fractions were lower in the high uric acid group compared with the normal uric acid group (P<0.05). Serum uric acid levels were correlated with the mean pulmonary arterial pressure (r=0.387; P<0.01) and New York Heart Association class (r=0.41; P<0.01). There was also an inverse correlation between uric acid levels and the left (r=-0.550; P<0.01) and right ventricular ejection fractions (r=-0.481; P<0.05).

CONCLUSION:

Serum uric acid levels are associated with IPAH severity and the severity of ventricular dysfunction.
PMCID: PMC3716500 Free PMC Article
  PMID: 24294046 [PubMed - as supplied by publisher]
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7. Exp Clin Cardiol. 2013 Winter;18(1):13-16.

Effect of prostaglandin E1 inhalation on pulmonary hypertension following corrective surgery for congenital heart disease.

Zhang CYMa ZSMa LLWang LX.
Department of Cardiac Surgery, Charles Sturt University, Wagga Wagga, New South Wales, Australia;

Abstract

BACKGROUND:

Intravenous infusion of prostaglandin E1 (PGE1) has been used to treat pulmonary arterial hypertension (PAH); however, the efficacy and safety of inhaled PGE1 is unclear.

OBJECTIVES:

To investigate the effect of inhaled PGE1 on PAH following corrective surgery for congenital heart disease.

METHODS:

Sixty patients with postoperative residual PAH following corrective surgery for congenital heart disease were randomly assigned to a control group, a PGE1 infusion group (intravenous PGE1 infusion; 30 ng/kg/min daily for 10 days) or a PGE1 inhalation group (100 μg nebulized PGE1 every 8 h for 10 days). Systolic blood pressure, mean pulmonary arterial pressure, arterial oxygen pressure, oxygen saturation and serum endothelin-1 level were measured before and after the treatment.

RESULTS:

At the end of the study, the mean pulmonary arterial pressure in the two PGE1 groups were lower than in the control group (P<0.01), whereas the mean arterial oxygen pressure was higher (P<0.01). Compared with the PGE1 infusion group, the mean pulmonary arterial pressure in the PGE1 inhalation group was lower (P<0.01) whereas the arterial oxygen pressure was higher (P<0.01). The mean endothelin-1 levels in the two PGE1 groups were lower than in the control group (P<0.01), but there was no statistically significant difference in endothelin-1 levels between the PGE1 inhalation and infusion groups (P>0.05).

CONCLUSIONS:

In pediatric patients with PAH, PGE1 inhalation was associated with a reduction in pulmonary arterial pressure and improvement in arterial blood oxygen levels.
PMCID: PMC3716482 Free PMC Article
  PMID: 24294030 [PubMed - as supplied by publisher]
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8. Nat Genet. 2013 Dec 1. doi: 10.1038/ng.2844. [Epub ahead of print]

EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension.

Eyries MMontani DGirerd BPerret CLeroy ALonjou CChelghoum NCoulet FBonnet DDorfmüller PFadel ESitbon OSimonneau GTregouët DAHumbert MSoubrier F.
1] Unité Mixte de Recherche en Santé (UMR_S 956), Université Pierre and Marie Curie Université Paris 06 (UPMC) and Institut National de la Santé et de la Recherche Médicale (INSERM), Paris, France. [2] Genetics Department, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. [3] Institute for Cardiometabolism and Nutrition (ICAN), Paris, France.

Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare and devastating cause of pulmonary hypertension that is characterized histologically by widespread fibrous intimal proliferation of septal veins and preseptal venules and is frequently associated with pulmonary capillary dilatation and proliferation. PVOD is categorized into a separate pulmonary arterial hypertension-related group in the current classification of pulmonary hypertension. PVOD presents either sporadically or as familial cases with a seemingly recessive mode of transmission. Using whole-exome sequencing, we detected recessive mutations in EIF2AK4 (also called GCN2) that cosegregated with PVOD in all 13 families studied. We also found biallelic EIF2AK4 mutations in 5 of 20 histologically confirmed sporadic cases of PVOD. All mutations, either in a homozygous or compound-heterozygous state, disrupted the function of the gene. These findings point to EIF2AK4 as the major gene that is linked to PVOD development and contribute toward an understanding of the complex genetic architecture of pulmonary hypertension.
  PMID: 24292273 [PubMed - as supplied by publisher]
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9. Yakugaku Zasshi. 2013;133(12):1351-9.

Enhanced Ca(2+)-sensing Receptor Function in Pulmonary Hypertension.

Yamamura AYamamura HYuan JX.
Kinjo Gakuin University School of Pharmacy.

Abstract

  Pulmonary arterial hypertension (PAH) is a rare, progressive, and fetal disease. The five-year survival rate after diagnosis is ~50%. In Japan, PAH is listed in the Specified Rare and Intractable Diseases. Pulmonary vascular remodeling and sustained pulmonary vasoconstriction are the major causes for the elevated pulmonary vascular resistance (PVR) in PAH. The pathogenic mechanisms involved in the pulmonary vascular abnormalities in PAH remain unclear. Sustained vasoconstriction and vascular remodeling owing to proliferation of pulmonary arterial smooth muscle cells (PASMCs) are key pathogenic events that lead to early morbidity and mortality. These events have been closely linked to Ca(2+) mobilization and signaling in PASMCs. An increase in cytosolic Ca(2+) concentration ([Ca(2+)]cyt) in PASMCs is an important stimulus for pulmonary vasoconstriction and cell proliferation which subsequently cause pulmonary vascular wall thickening followed by the increase in PVR. Increased resting [Ca(2+)]cyt and enhanced Ca(2+) influx have been implicated in PASMCs from PAH patients, but precise therapeutic targets to interrupt these signal pathways have not been identified. We recently found that the extracellular Ca(2+)-sensing receptor (CaSR), a G protein-coupled receptor (GPCR), is upregulated in PASMCs from patients with idiopathic pulmonary arterial hypertension (IPAH). In addition, blockage of the CaSR with an antagonist (NPS2143) prevents the development of pulmonary hypertension and right ventricular hypertrophy in animal models of pulmonary hypertension. The functionally upregulated CaSR in PASMCs is a novel pathogenic mechanism contributing to the augmented Ca(2+) signaling and excessive cell proliferation in IPAH. Targeting CaSR in PASMCs may help develop novel therapeutic approach for PAH.
Free Article
  PMID: 24292183 [PubMed - in process]
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10. Respir Med. 2013 Nov 19. pii: S0954-6111(13)00447-2. doi: 10.1016/j.rmed.2013.11.006. [Epub ahead of print]

Clinical characteristics and prediction of pulmonary hypertension in severe emphysema.

Minai OAFessler HStoller JKCriner GJScharf SMMeli YNutter BDecamp MMNETT Research Group.
Department of Pulmonary, Allergy, and Critical Care, Cleveland Clinic, USA. Electronic address: minaio@ccf.org.

Abstract

BACKGROUND:

We explored the prevalence, clinical and physiologic correlates of pulmonary hypertension (PH), and screening strategies in patients with severe emphysema evaluated for the National Emphysema Treatment Trial (NETT).

METHODS:

Patients undergoing Doppler echocardiography (DE) and right heart catheterization were included. Patients with mean pulmonary arterial pressure ≥25 mmHg (PH Group) were compared to the remainder (non-PH Group).

RESULTS:

Of 797 patients, 302 (38%) had PH and 18 (2.2%) had severe PH. Compared to the non-PH Group, patients with PH had lower % predicted FEV1 (p < 0.001), % predicted diffusion capacity for carbon monoxide (p = 0.006), and resting room air PaO2 (p < 0.001). By multivariate analysis, elevated right ventricular systolic pressure, reduced resting room air PaO2, reduced post-bronchodilator % predicted FEV1, and enlarged pulmonary arteries on computed tomographic scan were the best predictors of PH. A strategy using % predicted FEV1, % predicted DLCO, PaO2, and RVSP was predictive of the presence of pre-capillary PH and was highly predictive of its absence.

CONCLUSIONS:

Mildly elevated pulmonary artery pressures are found in a significant proportion of patients with severe emphysema. However, severe PH is uncommon in the absence of co-morbidities. Simple non-invasive tests may be helpful in screening patients for pre-capillary PH in severe emphysema but none is reliably predictive of its presence.
Copyright © 2013. Published by Elsevier Ltd.
  PMID: 24290900 [PubMed - as supplied by publisher]
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11. J Saudi Heart Assoc. 2013 Jul;25(3):219-23. doi: 10.1016/j.jsha.2012.12.001. Epub 2013 Jan 5.

Primary pulmonary hypertension during pregnancy: A case report.

Albackr HBAldakhil LOAhamd A.
King Fahad Cardiac Center, King Khalid University Hospital College of Medicine, King Saud University, Saudi Arabia.

Abstract

We describe a case of a 25-year-old pregnant woman who presented with severe primary pulmonary hypertension (PPH). Her echocardiogram showed severe right ventricular hypertrophy with dilatation and Moderate right ventricular systolic dysfunction. Right ventricle systolic pressure (RVSP) was estimated to be 125 mmHg. She had an elective caesarean section under general anaesthesia at 32 weeks of gestation. Pulmonary artery pressures measured by a pulmonary artery catheter before anaesthesia were 102 mmHg and pulmonary vascular resistance was 429. Intraoperative nitric oxide was used to reduce pulmonary artery systolic pressure (PASP). After the delivery of a healthy infant, PASP was controlled with nebulized iloprost and silandifil. Five days later she was transferred from intensive care unit after she was started on silandifil 50 mg three times daily and a small dose of warfarin.
PMCID: PMC3809475 Free PMC Article
  PMID: 24174863 [PubMed]
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12. Am J Health Syst Pharm. 2013 Nov 15;70(22):1960. doi: 10.2146/news130073.

Riociguat approved for pulmonary hypertension.

Traynor K.
  PMID: 24173000 [PubMed - in process]
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13. EuroIntervention. 2013 Oct 30. pii: 20130414-01. [Epub ahead of print]

Decrease of pulmonary hypertension impacts on prognosis after transcatheter aortic valve replacement.

Sinning JMHammerstingl CChin DGhanem ASchueler RSedaghat ABence JSpyt TWerner N,Kovac JGrube ENickenig GVasa-Nicotera M.
Heart Center Bonn, Department of Medicine II, University Hospital Bonn, Bonn, Germany.

Abstract

Aims: Transcatheter aortic valve replacement (TAVR) is established as a treatment strategy for patients with end-stage aortic stenosis, many of whom are suffering from severe pulmonary hypertension (PH). In cardiac surgery patients, PH is associated with less symptomatic improvement and increased late mortality. This study elucidates the impact of PH on outcome after TAVR. Methods and results: Pre and 90 days post TAVR, pulmonary artery systolic pressure (PASP) was determined non-invasively by echocardiography in 353 patients undergoing TAVR. PH was classified as absent (<30 mmHg), mild-to-moderate (30-60 mmHg), and severe (>60 mmHg). Three hundred and fifty-three patients at high surgical risk, indicated by a logistic EuroSCORE of 26.6±16.5%, underwent TAVR. The severity of PH before TAVR was related to outcome with two-year mortality rates of 13.9%, 27.3%, and 48.4% for PASP <30 mmHg, 30-60 mmHg, and >60 mmHg, respectively (p=0.001). In patients with baseline PASP >60 mmHg, PASP decreased from 65.6±7.6 mmHg to 49.5±14.0 mmHg (p<0.001) at 90 days after TAVR. Patients with persistent severe PH had a worse prognosis than patients with a decrease of PASP below 60 mmHg (two-year mortality rate: 50.0% vs. 18.6%; p=0.001). Conclusions: Severe pulmonary hypertension predicts adverse outcome after TAVR. Reduction of PASP after the procedure is associated with favourable prognosis.
  PMID: 24168931 [PubMed - as supplied by publisher]
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14. Eur Heart J. 2013 Oct 29. [Epub ahead of print]

Pulmonary hypertension related to congenital heart disease: a call for action.

Dimopoulos KWort SJGatzoulis MA.
Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, NIHR Cardiovascular BRU, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, Sydney Street, London SW3 6NP, UK.

Abstract

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH). Despite this, little emphasis has been given to this group of patients until recently, when compared with idiopathic PAH. This is largely because of the complexity and the wide range of underlying cardiac anatomy and physiology, with a multitude of adaptive mechanisms not fully understood. Pulmonary arterial hypertension related to congenital heart disease is, therefore, best diagnosed and managed in centres specializing in both CHD and PAH, to avoid common pitfalls and old practices and to provide state-of-the-art care. We discuss the optimal management of PAH-CHD patients in a series of actions to be taken in order to optimize short- and long-term outcome, based on current knowledge of the condition and the advent of targeted advanced therapies.
  PMID: 24168793 [PubMed - as supplied by publisher]
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15. Eur J Pediatr Surg. 2013 Oct 25. [Epub ahead of print]

Intravenous Sildenafil in the Management of Pulmonary Hypertension Associated with Congenital Diaphragmatic Hernia.

Bialkowski AMoenkemeyer FPatel N.
Newborn Intensive Care, Royal Children's Hospital, Melbourne, Victoria, Australia.

Abstract

Background Pulmonary artery hypertension (PAH) is a significant cause of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). The phosphodiesterase-5 inhibitor sildenafil may be beneficial as a pulmonary vasodilator in CDH. Use of oral preparations of sildenafil may be restricted by feeding delays and intolerance. This study assessed the cardiorespiratory effects of a newly available intravenous (IV) preparation of sildenafil in CDH.Objectives The objective of the article is to assess the acute effects of IV sildenafil infusion on myocardial function, pulmonary artery pressure (PAP), and oxygenation in infants with CDH.Methods Retrospective case review of infants with CDH who received continuous IV sildenafil. Physiological and echocardiographic data were reviewed to obtain oxygenation index (OI), PAP, patent ductus arteriosus (PDA) flow, myocardial tissue Doppler velocities, and right ventricular output (RVO) at 48 hours presildenafil, and at 24 to 48 hours and 72 to 96 hours after commencing IV sildenafil.Results A total of nine infants received IV sildenafil at a dose of 100 to 290 μg/kg/h after CDH repair but before enteral feeding. Pre-IV sildenafil PAP was ≥ systemic blood pressure in all infants, systolic and diastolic right ventricular myocardial velocities were impaired. After 72 to 96 hours of IV sildenafil, OI and Fio2 were significantly reduced. Ratio of right-to-left to left-to-right PDA flow was > 1 pre-IV sildenafil and < 1 post-IV sildenafil.Conclusions IV sildenafil infusion was associated with improved oxygenation. Prospective trials of IV sildenafil are required to determine effects on longer term outcome.
Georg Thieme Verlag KG Stuttgart · New York.
  PMID: 24163194 [PubMed - as supplied by publisher]
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16. Clin Exp Rheumatol. 2013 Jul-Aug;31(4):657-8. Epub 2013 Apr 22.

Rituximab treatment for pulmonary arterial hypertension in adult-onset Still's disease.

Padilla-Ibarra JSanchez-Ortiz ASandoval-Castro CRamos-Remus C.
  PMID: 23622421 [PubMed - indexed for MEDLINE]
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17. Am J Cardiol. 2013 Sep 1;112(5):720-5. doi: 10.1016/j.amjcard.2013.04.051. Epub 2013 Jun 7.

Effectiveness of spironolactone plus ambrisentan for treatment of pulmonary arterial hypertension (from the [ARIES] study 1 and 2 trials).

Maron BAWaxman ABOpotowsky ARGillies HBlair CAghamohammadzadeh RLoscalzo J,Leopold JA.
Veterans Affairs Boston Healthcare System, Department of Cardiology, 1400 VFW, Parkway, Boston, MA, USA. bmaron@partners.org

Abstract

In translational models of pulmonary arterial hypertension (PAH), spironolactone improves cardiopulmonary hemodynamics by attenuating the adverse effects of hyperaldosteronism on endothelin type-B receptor function in pulmonary endothelial cells. This observation suggests that coupling spironolactone with inhibition of endothelin type-A receptor-mediated pulmonary vasoconstriction may be a useful treatment strategy for patients with PAH. We examined clinical data from patients randomized to placebo or the selective endothelin type-A receptor antagonist ambrisentan (10 mg/day) and in whom spironolactone use was reported during ARIES-1 and -2, which were randomized, double-blind, placebo-controlled trials assessing the effect of ambrisentan for 12 weeks on clinical outcome in PAH. From patients randomized to placebo (n = 132) or ambrisentan (n = 67), we identified concurrent spironolactone use in 21 (15.9%) and 10 (14.9%) patients, respectively. Compared with patients treated with ambrisentan alone (n = 57), therapy with ambrisentan + spironolactone improved change in 6-minute walk distance by 94% at week 12 (mean ± SE, +38.2 ± 8.1 vs +74.2 ± 27.4 m, p = 0.11), improved plasma B-type natriuretic peptide concentration by 1.7-fold (p = 0.08), and resulted in a 90% relative increase in the number of patients improving ≥1 World Health Organization functional class (p = 0.08). Progressive illness, PAH-associated hospitalizations, or death occurred as an end point for 5.3% of ambrisentan-treated patients; however, no patient treated with ambrisentan + spironolactone reached any of these end points. In conclusion, these pilot data suggest that coupling spironolactone and endothelin type-A receptor antagonism may be clinically beneficial in PAH. Prospective clinical trials are required to further characterize our findings.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 23751938 [PubMed - indexed for MEDLINE]
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18. Am J Cardiol. 2013 Sep 15;112(6):873-82. doi: 10.1016/j.amjcard.2013.05.016. Epub 2013 Jun 1.

A simple echocardiographic method to estimate pulmonary vascular resistance.

Opotowsky ARClair MAfilalo JLandzberg MJWaxman ABMoko LMaron BAVaidya AForfia PR.
Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.sasha.opo@gmail.com

Abstract

Pulmonary hypertension includes heterogeneous diagnoses with distinct hemodynamic pathophysiologic features. Identifying elevated pulmonary vascular resistance (PVR) is critical for appropriate treatment. We reviewed data from patients seen at referral pulmonary hypertension clinics who had undergone echocardiography and right-side cardiac catheterization within 1 year. We derived equations to estimate PVR using the ratio of estimated pulmonary artery (PA) systolic pressure (PASPDoppler) to right ventricular outflow tract velocity time integral (VTI). We validated these equations in a separate sample and compared them with a published model based on the ratio of the transtricuspid flow velocity to right ventricular outflow tract VTI (model 1, Abbas et al 2003). The derived models were as follows: PVR = 1.2 × (PASP/right ventricular outflow tract VTI) (model 2) and PVR = (PASP/right ventricular outflow tract VTI) + 3 if notch present (model 3). The cohort included 217 patients with mean PA pressure of 45.3 ± 11.9 mm Hg, PVR of 7.3 ± 5.0 WU, and PA wedge pressure of 14.8 ± 8.1 mm Hg. Just >1/3 had a PA wedge pressure >15 mm Hg (35.5%) and 82.0% had PVR >3 WU. Model 1 systematically underestimated catheterization estimated PVR, especially for those with high PVR. The derived models demonstrated no systematic bias. Model 3 correlated best with PVR (r = 0.80 vs r = 0.73 and r = 0.77 for models 1 and 2, respectively). Model 3 had superior discriminatory power for PVR >3 WU (area under the curve 0.946) and PVR >5 WU (area under the curve 0.924), although all models discriminated well. Model 3-estimated PVR >3 was 98.3% sensitive and 61.1% specific for PVR >3 WU (positive predictive value 93%; negative predictive value 88%). In conclusion, we present an equation to estimate the PVR, using the ratio of PASPDoppler to right ventricular outflow tract VTI and a constant designating presence of right ventricular outflow tract VTI midsystolic notching, which provides superior agreement with catheterization estimates of PVR across a wide range of values.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 23735649 [PubMed - indexed for MEDLINE]
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19. Am J Cardiol. 2013 Aug 15;112(4):580-4. doi: 10.1016/j.amjcard.2013.04.022. Epub 2013 May 16.

Effect of elevated pulmonary vascular resistance on outcomes after percutaneous mitral valvuloplasty.

Cruz-Gonzalez ISemigram MJInglessis-Azuaje ISanchez-Ledesma MMartin-Moreiras JJneid H,Rengifo-Moreno PCubeddu RJMaree AOSanchez PLPalacios IF.
Cardiology Division, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.cruzgonzalez.ignacio@gmail.com

Abstract

Patients with mitral stenosis with severe pulmonary hypertension constitute a high-risk subset for surgical commissurotomy or valve replacement. The aim of the present study was to examine the effect of elevated pulmonary vascular resistance (PVR) on percutaneous mitral valvuloplasty (PMV) procedural success, short- and long-term clinical outcomes (i.e., mortality, mitral valve surgery, and redo PMV) in 926 patients. Of the 926 patients, 263 (28.4%) had PVR ≥4 Woods units (WU) and 663 (71.6%) had PVR <4 WU. Patients with PVR ≥4 WU were older and more symptomatic and had worse valve morphology for PMV. The patients with PVR ≥4 WU also had lower PMV procedural success than those with PVR <4 WU (78.2% vs 85.6%, p = 0.006). However, after multivariate adjustment, PVR was no longer an independent predictor of PMV success nor an independent predictor of the combined end point at a median follow-up of 3.2 years. In conclusion, elevated PVR at PMV is not an independent predictor of procedural success or long-term outcomes. Therefore, appropriately selected patients with rheumatic mitral stenosis might benefit from PMV, even in the presence of elevated preprocedural PVR.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 23683954 [PubMed - indexed for MEDLINE]
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20. J Heart Lung Transplant. 2013 Apr;32(4):381-7. doi: 10.1016/j.healun.2013.01.1049. Epub 2013 Feb 14.

Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience.

Esch JJShah PBCockrill BAFarber HWLandzberg MJMehra MRMullen MPOpotowsky AR,Waxman ABLock JEMarshall AC.
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.

Comment in

Transcatheter Potts shunt: an innovative IDEA.Ventura HO. J Heart Lung Transplant. 2013 Apr; 32(4):388-9.
Radiofrequency perforation may increase the safety of transcatheter Potts shunt creation.Sayadpour Zanjani K. J Heart Lung Transplant. 2013 Sep; 32(9):938. Epub 2013 Jun 6.

Abstract

BACKGROUND:

Patients with severe pulmonary arterial hypertension (PAH) face significant morbidity and death as a consequence of progressive right heart failure. Surgical shunt placement between the left PA and descending aorta (Potts shunt) appears promising for PAH palliation in children; however, surgical mortality is likely to be unacceptably high in adults with PAH.

METHODS:

We describe a technique for transcatheter Potts shunt (TPS) creation by fluoroscopically guided retrograde needle perforation of the descending aorta at the site of apposition to the left PA to create a tract for deployment of a covered stent between these vessels. This covered stent-anchored by the vessel walls and surrounding tissue-serves as the shunt.

RESULTS:

TPS creation was considered in 7 patients and performed in 4. The procedure was technically successful in 3 patients; 1 patient died during the procedure as a result of uncontrolled hemothorax. One acute survivor, critically ill at the time of TPS creation, later died of comorbidities. The 2 mid-term survivors (follow-up of 10 and 4 months) are well at home, with symptomatic improvement and no late complications. The 3 candidate patients in whom the procedure was not performed died within 1 month of consideration, underscoring the tenuous nature of this population.

CONCLUSIONS:

TPS creation is feasible and may offer symptomatic relief to select patients with refractory PAH. Further study of this innovative approach is warranted.
Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.
  PMID: 23415728 [PubMed - indexed for MEDLINE]
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21. Radiol Med. 2013 Nov 15. [Epub ahead of print]

Rare causes of pulmonary hypertension: spectrum of radiological findings and review of the literature.

Rossi AZompatori MTchouante Tchouanhou PAmadori MPalazzini MConficoni EGaliè NPoletti VGavelli G.
I.R.C.C.S Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (I.R.S.T.), via P. Maroncelli 40/42, Meldola, FC, Italy.

Abstract

Following a brief introduction covering the clinical signs and symptoms of pulmonary hypertension (PH), its most recent classification into six groups, and the computed tomography (CT) features common to all forms of PH, this paper illustrates the typical patterns that can be found on chest radiography and CT in rare causes of PH. We present and compare with the existing literature our personal series of cases of rare forms of PH, found in the following diseases: veno-occlusive disease, pulmonary capillary haemangiomatosis, non-thrombotic pulmonary embolism (tumour embolism and carcinomatous lymphangitis, talcosis, hydatid disease), pulmonary artery sarcoma, neurofibromatosis, sarcoidosis, and Langerhans cell histiocytosis. Rare forms of PH show low incidence and prevalence, and are, therefore, poorly recognised. Their diagnosis is a challenge for clinicians, pathologists, and radiologists, and any additional knowledge about the CT findings may help the diagnosis in the case of patients affected by PH of unknown origin.
  PMID: 24234182 [PubMed - as supplied by publisher]
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22. Rejuvenation Res. 2013 Nov 14. [Epub ahead of print]

Role of Vasoactive Intestinal Peptide in COPD with Pulmonary Hypertension.

Lacedonia DValerio GPalladino GPCarpagnano GECorreale Mdi Biase MFoschino Barbaro MP.
University of Foggia, Viale degli Aviatori, Foggi, Italy, 70121 ; donatolacedonia@gmail.com.

Abstract

Background: The aim of this study was to define the involvement of some biomarkers in patients with COPD and Pulmonary Hypertension (PH), with particular attention to subgroups with a PH that is "out of proportion". Materials and Methods: Patients with COPD without PH, with PH and marked airways obstruction and with PH and mild airways obstruction were compared. Assays for human IL-6, Leukotriene B4 (LTB4), VIP and Endotelin 1 (ET-1) were performed on the blood samples taken during RHC in a pulmonary artery. Results: 83 patients were enrolled and divided into three groups: 37 simple COPD (mPAP <25 mmHg); 46 COPD with PH (mPAP ≥25 mmHg) and, among the latter, those who had an mPAP ≥35mmHg and FEV1≥50% were classified as "out of proportion" (OP) 7 patients. Patients with PH were older and had a BMI higher than the other groups; moreover, they had lower FEV1 and DLCO. A lower level of PaO2 was observed in the group of OP patients. The levels of ET-1, IL-6 and LTB4 were similar in each group; VIP was higher in the OP patients than in simple COPD and it was related to PAP . Conclusions: In the patients with COPD and PH and in particular in the group of out-of proportion PH, VIP is significantly increased, probably in order to correct the imbalance between vasoconstrictor and vasodilatator mediators.
  PMID: 24228853 [PubMed - as supplied by publisher]
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23. Biomed Res Int. 2013;2013:489574. doi: 10.1155/2013/489574. Epub 2013 Oct 21.

Pulmonary hypertension in portugal: first data from a nationwide registry.

Baptista RMeireles JAgapito ACastro GMarinho da Silva AShiang TGonçalves FRobalo-Martins SNunes-Diogo AReis A.
Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal ; Institute for Biomedical Imaging and Life Sciences, Faculty of Medicine of University of Coimbra, Azinhaga de Santa Comba, 3000 Coimbra, Portugal.

Abstract

Introduction. Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical. Methods. We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH). Results. Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min(-1) ·m(-2)); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015). Conclusions. We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.
PMCID: PMC3818811 Free PMC Article
  PMID: 24228252 [PubMed - in process]
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24. Indian Heart J. 2013 Sep-Oct;65(5):644-9. doi: 10.1016/j.ihj.2013.08.010. Epub 2013 Sep 2.

Contributions of pulmonary hypertension to HIV-related cardiac dysfunction.

Isiguzo GCOkeahialam BNDanbauchi SSOdili ANIroezindu MOPlacid U.
Internal Medicine Department, Federal Teaching Hospital Abakaliki, Ebonyi State, Nigeria. Electronic address: isiguzogodsent@yahoo.com.

Abstract

BACKGROUND/AIM:

To evaluate the prevalence of pulmonary hypertension among patients living with HIV/AIDS and to determine its contribution to cardiac dysfunction.

METHOD:

A hospital based cross sectional study was carried out over a 6-month period at the Jos University Teaching Hospital. The subjects were 200 confirmed HIV positive patients, ≥18 years of age who consented to the study. Physical examination, laboratory investigations, 2 dimensional and Doppler echocardiography were conducted on the subjects.

RESULTS:

The mean age of the patients was 38 ± 9 years, and there were 142 females (71%). Females were younger, mean age 36 ± 8 years versus 41 ± 10 years for males (p-value <0.01). The median CD4 cell count was 312 cells/μl, there were no homosexual or intravenous drug user among the subjects. Eight of the subjects had pulmonary hypertension, with a case prevalence of 4%, and this had no relationship to CD4 cell count. Both systolic and diastolic functions were worse in subjects with pulmonary hypertension, with a negative correlation between mean pulmonary arterial systolic pressure (mPASP) and parameters like ejection fraction (r = -0.28, p-value 0.0003), fractional shortening (r = -0.21, p-value 0.003), deceleration time (r = -0.13. p-value 0.09).

CONCLUSION:

Immune-suppression affects the cardiac function adversely and coexisting pulmonary hypertension contributes to poor systolic and diastolic function in affected patients. The subtle nature of presentation of pulmonary hypertension and other cardiac dysfunctions in HIV/AIDS patients demand a high-index of suspicion and early intervention if detected, to ensure better care for these emerging threats to our patients.
Copyright © 2013 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.
  PMID: 24206894 [PubMed - in process]
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25. Echocardiography. 2013 Nov 7. doi: 10.1111/echo.12393. [Epub ahead of print]

Pulmonary Hypertension in Hypertensive Patients: Association with Diastolic Dysfunction and Increased Pulmonary Vascular Resistance.

Mukherjee MMehta NKConnolly JJDusaj RSChoi BGKatz RJLewis JF.
Division of Cardiology, Johns Hopkins, University School of Medicine, Baltimore, Maryland.

Abstract

BACKGROUND:

Pulmonary hypertension (PH) in patients with systemic hypertension and preserved ejection fraction (PEF) has been described. However, the pathophysiology and consequences are not entirely clear. We sought to distinguish the clinical and anatomic features among hypertensive patients with or without coexistent PH.

METHODS:

Echocardiograms and records of hypertensive patients with left ventricular (LV) hypertrophy and PEF from January 2009 to January 2011 were reviewed. We identified 174 patients, including 36 with PH (calculated pulmonary artery systolic pressure [PASP] ≥ 35 mmHg), and 138 with normal pulmonary pressures.

RESULTS:

Hypertensive patients with PH were older (76 ± 13 vs. 65 ± 13 years, P < 0.0001), more often female (91, 70%), had lower estimated glomerular filtration rate (eGFR) (63 ± 44 vs. 88 ± 48 mL/min, P = 0.002), and higher pro-BNP levels (3141 ± 4253 vs. 1219 ± 1900 pg/mL, P = 0.003). PH patients also had larger left atrial areas (23.7 ± 3.8 vs. 20.8 ± 4.6 cm2 , P = 0.002), evidence of diastolic dysfunction (i.e., septal E/e' 17.6 ± 8.6 vs. 12.7 ± 4.4, P = 0.0005), and higher calculated peripheral vascular resistance (PVR) (2.3 ± 1.1 vs. 1.6 ± 0.4, P < 0.0001). Both PVR and septal E/e' showed strong linear correlation with PASP (P < 0.0001 and P < 0.0001, respectively).

CONCLUSIONS:

Hypertension in elderly patients is frequently complicated by LV diastolic dysfunction and secondary PH. These hypertensive patients tended to have reduced renal function and higher pro-BNP. Because of the known morbidity and mortality associated with PH, these observations have potentially important implications for target medical therapy.
© 2013, Wiley Periodicals, Inc.
  PMID: 24199601 [PubMed - as supplied by publisher]
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26. Med Glas (Zenica). 2013 Feb;10(1):191-3.

Ocular findings in patient with primary familial pulmonary hypertension.

Jovanović SOros ALješević LPeković SVučićević-Trobok JGvozdenović L.

Abstract

Exudative retinal detachment as subretinal fluid accumulation occurs due to chorioretinal eye disease (inflammation, infection, tumor, vascular abnormalities) and in systemic diseases too. The aim of this case report was to emphasize connection between the ophthalmological pathology and other systemic conditions. This is a case report of a 45-year old woman who suffered from pulmonary hypertension caused by many recurrent thromboembolic events. During one of eight previous hospitalizations she complained of blurred vision. Bilateral exudative detachment is diagnosed by an ophthalmologist. Parenteral steroids and acetazolamide provided quick recovery.
  PMID: 24199273 [PubMed - in process]
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27. Transplantation. 2013 Aug 27;96(4):421-5. doi: 10.1097/TP.0b013e31829853ac.

Lung transplantation after lung volume reduction surgery.

Shigemura NGilbert SBhama JKCrespo MMZaldonis DPilewski JMBermudez CA.
Division of Cardiothoracic Transplantation, Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, 200 Lothrop Street, Pittsburgh, PA 15213, USA. shigemuran@upmc.edu

Abstract

BACKGROUND:

Lung volume reduction surgery (LVRS) as a bridge to lung transplantation was first advocated in 1995 and published studies have supported the concept but with limited data. The risk-benefit tradeoffs of the combined procedure have not been thoroughly examined, although substantial information regarding LVRS has emerged.

METHODS:

Of 177 patients who underwent lung transplantation for end-stage emphysema between 2002 and 2009 at our center, 25 had prior LVRS (22 bilateral and 3 unilateral). Lung transplantation was performed 22.9±15.9 months after LVRS. We compared in-hospital morbidity, functional capacity, and long-term outcomes of patients who underwent LVRS before lung transplantation with a matched cohort of patients without prior LVRS to assess the influence of LVRS on posttransplantation morbidity and mortality.

RESULTS:

The incidence of postoperative bleeding requiring reexploration and the incidence of renal dysfunction requiring dialysis were higher in patients with LVRS before lung transplantation. Posttransplantation peak forced expiratory volume in 1 s was worse in patients with LVRS before lung transplantation (56.7% vs. 78.8%; P<0.05). Five-year survival was not significantly different (59.7% in patients with LVRS before lung transplantation vs. 66.2% in patients with lung transplantation alone). In multivariate analysis, age more than 65 years, prolonged cardiopulmonary bypass time, and severe pulmonary hypertension were significant predictors for mortality (P<0.05).

CONCLUSIONS:

Although LVRS remains a viable option as a bridge to lung transplantation in appropriately selected patients, LVRS before lung transplantation can impart substantial morbidity and compromised functional capacity after lung transplantation. LVRS should not be easily considered as a bridge to transplantation for all lung transplant candidates.
  PMID: 23736352 [PubMed - indexed for MEDLINE]
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28. J Am Coll Cardiol. 2013 Sep 17;62(12):1101-2. doi: 10.1016/j.jacc.2013.06.032. Epub 2013 Jul 10.

New treatment strategies for pulmonary arterial hypertension: hopes or hypes?

Galiè NManes A.
Department of Experimental, Diagnostic and Specialty Medicine-DIMES, Bologna University Hospital, Bologna, Italy. Electronic address: nazzareno.galie@unibo.it.

Comment on

Pulmonary artery denervation to treat pulmonary arterial hypertension: the single-center, prospective, first-in-man PADN-1 study (first-in-man pulmonary artery denervation for treatment of pulmonary artery hypertension).Chen SL, Zhang FF, Xu J, Xie DJ, Zhou L, Nguyen T, Stone GW. J Am Coll Cardiol. 2013 Sep 17; 62(12):1092-100. Epub 2013 Jul 10.
  PMID: 23850924 [PubMed - indexed for MEDLINE]
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29. J Am Coll Cardiol. 2013 Sep 17;62(12):1092-100. doi: 10.1016/j.jacc.2013.05.075. Epub 2013 Jul 10.

Pulmonary artery denervation to treat pulmonary arterial hypertension: the single-center, prospective, first-in-man PADN-1 study (first-in-man pulmonary artery denervation for treatment of pulmonary artery hypertension).

Chen SLZhang FFXu JXie DJZhou LNguyen TStone GW.
Cardiological Department, Nanjing First Hospital, Nanjing Medical University, Nanjing, China; Cardiological Department, Nanjing Heart Center, Nanjing, China. Electronic address: chmengx@126.com.

Comment in

New treatment strategies for pulmonary arterial hypertension: hopes or hypes?Galiè N, Manes A. J Am Coll Cardiol. 2013 Sep 17; 62(12):1101-2. Epub 2013 Jul 10.

Abstract

OBJECTIVES:

This study was designed to test the safety and efficacy of pulmonary artery (PA) denervation (PADN) for patients with idiopathic PA hypertension (IPAH) not responding optimally to medical therapy.

BACKGROUND:

Baroreceptors and sympathetic nerve fibers are localized in or near the bifurcation area of the main PA. We previously demonstrated that PADN completely abolished the experimentally elevated PA pressure responses to occlusion of the left interlobar PA.

METHODS:

Of a total of 21 patients with IPAH, 13 patients received the PADN procedure, and the other 8 patients who refused the PADN procedure were assigned to the control group. PADN was performed at the bifurcation of the main PA, and at the ostial right and left PA. Serial echocardiography, right heart catheterization, and a 6-min walk test (6MWT) were performed. The primary endpoints were the change of PA pressure (PAP), tricuspid excursion (Tei) index, and 6MWT at 3 months follow-up.

RESULTS:

Compared with the control group, at 3 months follow-up, the patients who underwent the PADN procedure showed significant reduction of mean PAP (from 55 ± 5 mm Hg to 36 ± 5 mm Hg, p < 0.01), and significant improvement of the 6MWT (from 324 ± 21 m to 491 ± 38 m, p < 0.006) and of the Tei index (from 0.7 ± 0.04 to 0.50 ± 0.04, p < 0.001).

CONCLUSIONS:

We report for the first time the effect of PADN on functional capacity and hemodynamics in patients with IPAH not responding optimally to medical therapy. Further randomized study is required to confirm the efficacy of PADN. (First-in-Man Pulmonary Artery Denervation for Treatment of Pulmonary Artery Hypertension [PADN-1] study; chiCTR-ONC-12002085).
Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
  PMID: 23850902 [PubMed - indexed for MEDLINE]
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30. Thorax. 2013 Nov 12. doi: 10.1136/thoraxjnl-2013-204664. [Epub ahead of print]

Riociguat, a novel therapy for pulmonary hypertension?

Bailey SJ.
  PMID: 24222173 [PubMed - as supplied by publisher]
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31. PLoS One. 2013 Nov 6;8(11):e78569. doi: 10.1371/journal.pone.0078569.

Changes in large pulmonary arterial viscoelasticity in chronic pulmonary hypertension.

Wang ZLakes RSGolob MEickhoff JCChesler NC.
Department of Biomedical Engineering, University of Wisconsin - Madison, Madison, Wisconsin, United States of America.

Abstract

Conduit pulmonary artery (PA) stiffening is characteristic of pulmonary arterial hypertension (PAH) and is an excellent predictor of mortality due to right ventricular (RV) overload. To better understand the impact of conduit PA stiffening on RV afterload, it is critical to examine the arterial viscoelastic properties, which require measurements of elasticity (energy storage behavior) and viscosity (energy dissipation behavior). Here we hypothesize that PAH leads to frequency-dependent changes in arterial stiffness (related to elasticity) and damping ratio (related to viscosity) in large PAs. To test our hypothesis, PAH was induced by the combination of chronic hypoxia and an antiangiogenic compound (SU5416) treatment in mice. Static and sinusoidal pressure-inflation tests were performed on isolated conduit PAs at various frequencies (0.01-20 Hz) to obtain the mechanical properties in the absence of smooth muscle contraction. Static mechanical tests showed significant stiffening of large PAs with PAH, as expected. In dynamic mechanical tests, structural stiffness (κ) increased and damping ratio (D) decreased at a physiologically relevant frequency (10 Hz) in hypertensive PAs. The dynamic elastic modulus (E), a material stiffness, did not increase significantly with PAH. All dynamic mechanical properties were strong functions of frequency. In particular, κ, E and D increased with increasing frequency in control PAs. While this behavior remained for D in hypertensive PAs, it reversed for κ and E. Since these novel dynamic mechanical property changes were found in the absence of changes in smooth muscle cell content or contraction, changes in collagen and proteoglycans and their interactions are likely critical to arterial viscoelasticity in a way that has not been previously described. The impact of these changes in PA viscoelasticity on RV afterload in PAH awaits further investigation.
PMCID: PMC3819365 Free PMC Article
  PMID: 24223157 [PubMed - in process]
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32. PLoS One. 2013 Oct 18;8(10):e78001. doi: 10.1371/journal.pone.0078001.

Relevance of Partitioning DLCO to Detect Pulmonary Hypertension in Systemic Sclerosis.

Sivova NLaunay DWémeau-Stervinou LDe Groote PRemy-Jardin MDenis GLambert M,Lamblin NMorell-Dubois SFertin MLefevre GSobanski VLe Rouzic OHatron PYWallaert B,Hachulla EPerez T.
Service de Médecine Interne, Centre de Référence des Maladies Autoimmunes et Systémiques Rares (Sclérodermie), Centre de Compétence de l'Hypertension Artérielle Pulmonaire Sévère, Université Lille Nord de France, Hôpital Claude-Huriez, CHRU Lille, Lille, France.

Abstract

We investigated whether partitioning DLCO into membrane conductance for CO (DmCO) and pulmonary capillary blood volume (Vcap) was helpful in suspecting precapillary pulmonary (arterial) hypertension (P(A)H) in systemic sclerosis (SSc) patients with or without interstitial lung disease (ILD). We included 63 SSc patients with isolated PAH (n=6), isolated ILD (n=19), association of both (n=12) or without PAH and ILD (n=26). Partitioning of DLCO was performed by the combined DLNO/DLCO method. DLCO, DmCO and Vcap were equally reduced in patients with isolated PAH and patients with isolated ILD but Vcap/alveolar volume (VA) ratio was significantly lower in the isolated PAH group. In patients without ILD, DLCO, DmCO, Vcap and Vcap/VA ratio were reduced in patients with isolated PAH when compared to patients without PAH and both Vcap/VA and DLCO had the highest AUC to detect PAH. In patients with ILD, Vcap had the highest AUC and performed better than DLCO to detect PH in this subgroup. In conclusion, Vcap/VA was lower in PAH than in ILD in SSC whereas DLCO was not different. Vcap/VA ratio and DLCO had similar high performance to detect PAH in patients without ILD. Vcap had better AUC than DLCO, DmCO and FVC/DLCO ratio to detect PH in SSC patients with ILD. These results suggest that partitioning of DLCO might be of interest to detect P(A)H in SSC patients with or without ILD.
PMCID: PMC3799734 Free PMC Article
  PMID: 24205063 [PubMed - in process]
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33. Cardiovasc Drugs Ther. 2013 Nov 6. [Epub ahead of print]

Combination of Sildenafil and Bosentan for Pulmonary Hypertension in a Human Ex Vivo Model.

Ried MPotzger TNeu RSziklavari ZSzöke TLiebold AHofmann HSHoenicka M.
Department of Thoracic Surgery, University Medical Center Regensburg, Franz-Josef-Strauss-Allee 11, 93053, Regensburg, Germany, micha.ried@t-online.de.

Abstract

PURPOSE:

Both sildenafil and bosentan have been used clinically to treat pulmonary arterial hypertension. As these substances target different pathways to modulate vasoconstriction, we investigated the combined effects of both drug classes in isolated human pulmonary vessels.

METHODS:

Segments of pulmonary arteries (PA) and veins (PV) were harvested from 51 patients undergoing lobectomy. Contractile force was determined isometrically in an organ bath. Vessels were constricted with norepinephrine (NE) to determine effects of sildenafil. They were constricted with ET-1 to assess effects of bosentan, and with NE and ET-1 to evaluate the combination of both substances.

RESULTS:

Sildenafil (1E-5 M) significantly reduced maximum constriction by NE of both PA (13.0 ± 11.1 vs. 34.9 ± 7.6 % relative to KCl induced constriction; n = 6; p < 0.001) and PV (81.2 ± 34.2 vs 121.6 ± 20.8 %; n = 6; p < 0.01) but did not affect basal tones. Bosentan (1E-5 M) significantly reduced maximum constriction of PV (56.6 ± 21.5 vs. 172.1 ± 30.0 %; n = 6; p < 0.01) by ET-1 and led to a small but insignificant decrease of basal tone (p = 0.07). Bosentan almost completely abolished constriction of PA (1.0 ± 0.9 vs. 74.7 ± 25.7 %; n = 6; p < 0.001) by ET-1, but did not affect basal tone. Bosentan (1E-7 M) significantly attenuated combined ET-1/NE dose-response curves in PA (93.1 ± 47.4 vs. 125.3 ± 41.0 %; n = 12; p < 0.001) whereas the effect of sildenafil (1E-5 M) was less pronounced (103.6 ± 20.2 %; p < 0.05). Simultaneous administration of both substances showed a significantly greater reduction of maximum constriction in PA compared to individual administration (64.6 ± 26.3 %; p < 0.001).

CONCLUSIONS:

Sildenafil only at its highest concentration was effective in suppressing NE induced pulmonary vessel contraction. Bosentan was able to completely suppress ET-1 induced contraction of PA and strongly attenuated contraction of PV. The present data suggest a benefit of sildenafil/bosentan combination therapy as they affect different pathways and may allow lower dosages.
  PMID: 24193244 [PubMed - as supplied by publisher]
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34. Minerva Anestesiol. 2013 Oct 31. [Epub ahead of print]

What is the clinical significance of pulmonary hypertension in acute respiratory distress syndrome? A review.

Lai PMita CThompson B.
Pulmonary and Critical Care Unit, Department of Medicine, Massachusetts General Hospital, Boston, MA USA - tthompson1@partners.org.

Abstract

Elevated pulmonary arterial pressures appear to be a prominent feature of the acute respiratory distress syndrome (ARDS). Current clinical guidelines for the management of ARDS do not specifically address treatment of pulmonary hypertension or associated right ventricular dysfunction because the clinical significance of this entity remains unclear. Interpretation of elevated pulmonary arterial pressures, pulmonary vascular resistance, and transpulmonary gradient as well as signs of right ventricular dysfunction is confounded by the effects of positive pressure ventilation. There does not appear to be a consistent relationship between the diagnosis of pulmonary hypertension or right ventricular failure and mortality in patients with ARDS, but it is unclear if right ventricular failure contributes to the mortality risk per se or if the underlying cause of pulmonary hypertension, including intravascular micro and macro thrombosis, are simply markers for systemic dysregulation of coagulation and fibrinolysis that may lead to multiorgan failure in ARDS. While studies of pulmonary vasodilator therapies have not shown a mortality benefit in ARDS, such trials have targeted improved oxygenation rather than improved pulmonary hemodynamics so that the possible contribution of improved right ventricular function to better outcomes has not been directly tested in large trials. Future studies are needed to determine if treatment of pulmonary hypertension and associated right ventricular dysfunction will affect mortality in patients with ARDS.
  PMID: 24193181 [PubMed - as supplied by publisher]
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35. Pediatrics. 2013 Nov 4. [Epub ahead of print]

Pulmonary Hypertension Associated With Scurvy and Vitamin Deficiencies in an Autistic Child.

Duvall MGPikman YKantor DBAriagno KSummers LSectish TCMullen MP.
Division of Critical Care Medicine, Department of Anesthesia.

Abstract

Restricted dietary intake is common among children with behavioral issues. Here we report a case of a severely autistic child who presented initially with limp but who soon developed cough, tachypnea, hypoxia, and tachycardia. An echocardiogram revealed evidence of pulmonary hypertension (PH) with severely dilated right ventricle and elevated right-sided pressures. The etiology of his PH was unclear but further laboratory evaluation demonstrated severe nutritional deficiencies, in particular an undetectable ascorbic acid (vitamin C) level as well as deficient levels of thiamine (vitamin B1), pyridoxine (vitamin B6), cobalamin (vitamin B12), and vitamin D. Repletion of these vitamins was associated with resolution of his PH and his musculoskeletal complaints. We report this case and a review of the relevant literature as a clinical lesson to expand the differential diagnosis of limp in children who may be difficult to assess as well as to report on an unusual association between severe vitamin deficiencies and PH.
  PMID: 24190688 [PubMed - as supplied by publisher]
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36. Thromb Res. 2013 Dec;132(6):729-34. doi: 10.1016/j.thromres.2013.09.024. Epub 2013 Sep 26.

Chronic thromboembolic pulmonary hypertension-associated dysfibrinogenemias exhibit disorganized fibrin structure.

Marsh JJChiles PGLiang NCMorris TA.
Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California, San Diego, CA, USA.

Abstract

INTRODUCTION:

Mechanisms contributing to the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) are poorly understood. This disorder is characterized by incomplete resolution of pulmonary perfusion defects resulting from acute venous thromboembolism. We previously identified several dysfibrinogenemias in some patients with CTEPH. The purpose of this study was to determine whether fibrin clot architecture might be implicated in the thrombolytic resistance in patients with these CTEPH-associated dysfibrinogenemias.

MATERIALS AND METHODS:

Purified fibrinogen from patients and healthy controls was clotted with thrombin in the presence of calcium. Clot turbidity, porosity, and susceptibility to fibrinolysis were evaluated by spectrophotometric and permeation analyses. Fibrin network structure was assessed by laser-scanning confocal microscopy.

RESULTS:

Compared to normal fibrinogen, CTEPH-associated dysfibrinogenemias exhibited low clot turbidity, decreased porosity, and fibrinolytic resistance. In addition, the dysfibrinogenemias exhibited a more disorganized fibrin network structure characterized by thinner fibers, greater network dispersal and more extensive fiber branching.

CONCLUSIONS:

Abnormal clot architecture and fibrinolytic resistance may contribute to incomplete clot resolution following acute venous thromboembolism in patients with CTEPH-associated dyfibrinogenemia.
© 2013. Published by Elsevier Ltd. All rights reserved.
  PMID: 24182551 [PubMed - in process]
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37. J Clin Diagn Res. 2013 Sep;7(9):2027-8. doi: 10.7860/JCDR/2013/5627.3396. Epub 2013 Sep 10.

Brain abscess as a complication of chronic thromboembolic pulmonary hypertension - a rare case report.

K LR SS C.
Assistant Professor, Department of Microbiology, Sree Balaji Medical College and Hospital , Chennai, India .

Abstract

A brain abscess is a life threatening condition which can occur as a complication of various clinical conditions. An intra-cerebral abscess which occurs as a complication of pulmonary arterial hypertension is extremely rare. The present report has described such an uncommon case of an intra-cerebral abscess in a chronic pulmonary thrombo embolism patient with pulmonary hypertension. A-34-year old male who was a known case of chronic pulmonary thrombo embolism with pulmonary artery hypertension, who was diagnosed 6 months back, presented to the Out Patients Department (OPD) with headache and vomiting. He had right homonymous hemianopia. Contrast MRI (Magnetic Resonance Imaging) of brain showed a peripherally enhancing lesion in the left temporo-occipital lobe, with oedema and a mass effect. Left parieto-occipital craniotomy and excision of the abscess was done. Staphylococcus aureus was isolated from the aspirated pus.
PMCID: PMC3809673 Free PMC Article
  PMID: 24179934 [PubMed]
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38. Br J Neurosurg. 2013 Nov 1. [Epub ahead of print]

Causative role of infection in chronic non-thromboembolic pulmonary hypertension following ventriculo-atrial shunt.

Amelot ABouazza SGeorge BBresson D.
Department of Neurosurgery, Hopital Lariboisière, APHP , Paris , France.

Abstract

A severe complication of ventriculo-atrial (VA) shunt placement for treatment of hydrocephalus is chronic thromboembolic pulmonary hypertension (CTEPH). We report here a patient with a VA shunt for treatment of hydrocephalus who presented two consecutive episodes of VA shunt-induced infection by Staphylococcus epidermidis and who rapidly developed chronic PH. Extensive radiological investigations and normal ventilation/perfusion lung scan allowed us to rule out CTEPH. To our knowledge, no other case of chronic pulmonary hypertension (PH) related to VA shunt insertion has been reported so far. PH in this patient with VA shunt is clinically distinct from CTEPH and has been caused by VA shunt-induced S. epidermidis infection per se.
  PMID: 24175581 [PubMed - as supplied by publisher]
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39. Can Respir J. 2013 Mar-Apr;20(2):111-5.

Directing therapy in pulmonary arterial hypertension using a target 6 min walk distance.

Porhownik NRMyers RBshouty Z.
Section of Respiratory Medicine, University of Manitoba, Winnipeg. nporhownik@hsc.mb.ca

Abstract

BACKGROUND:

The most effective approaches to escalating advanced therapies in pulmonary arterial hypertension (PAH) are controversial.

OBJECTIVE:

To compare outcomes before and after introducing a target 6 min walk distance (6MWD) treatment strategy in PAH using registry data.

METHODS:

From 2001 to 2005, WHO class II to IV patients were treated with bosentan or prostanoids. In July 2005, a target 6MWD strategy was adopted. Monotherapy continued if 6MWD remained >350 m. For patients in whom 6MWD was ≤350 m, sildenafil was added. If 6MWD remained <350 m, prostanoids were considered. Changes in 6MWD, WHO class and survival rate were compared between periods.

RESULTS:

Before using the 6MWD strategy, there was a statistically significant improvement in mean WHO class at six, nine and 12 months (2.5±0.8 [P<0.015]; 2.5±0.8 [P<0.005]; and 2.5±0.9 [P<0.03], respectively) compared with baseline (2.9±0.9). There was a statistically significant increase in mean 6MWD at three, six, nine and 12 months (383±113 m [P<0.005]; 401±102 m [P<0.006]; 400±109 m [P<0.001]; and 399±110 m [P<0.004], respectively) compared with baseline (321±119 m). The survival rate was 95% at one and two years. From 2005 to 2009, there was a statistically significant improvement in mean WHO class at three, six, nine and 12 months (2.6±0.8 [P<0.05]; 2.3±0.9 [P<0.0001]; 2.3±0.9 [P<0.0001]; and 2.3±1.0 [P<0.0005], respectively) compared with baseline (2.8±0.7). There was statistically significant improvement in 6MWD at six months (381±126 m [P<0.05]), followed by a decline toward baseline (354±117 m). One- and two-year survival rates in the 6MWD target era were 95% and 80%, respectively.

CONCLUSION:

Based on registry data, adoption of this strategy did not affect survival rates, nor cause a sustained improvement in 6MWD by 12 months. WHO class improved similarly in both treatment groups.
PMCID: PMC3630045 [Available on 2014/3/1]
  PMID: 23616968 [PubMed - indexed for MEDLINE]
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40. Respiration. 2013;85(5):391-9. doi: 10.1159/000339423. Epub 2012 Aug 3.

Spleen size in idiopathic and heritable pulmonary arterial hypertension.

Tonelli ARYadav RGupta AArrossi AVHeresi GADweik RA.
Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland, Ohio, USA. tonella@ccf.org

Abstract

BACKGROUND:

It is unknown whether the spleen size correlates with disease severity and outcome in patients with idiopathic and heritable pulmonary arterial hypertension (PAH). Objectives: To determine the prevalence of splenomegaly in PAH and assess whether it correlates with severity of disease and outcome.

METHODS:

We identified subjects with either heritable or idiopathic PAH who had Doppler echocardiography, right-heart catheterization and computed tomography (CT) of the chest and/or abdomen that included the spleen.

RESULTS:

We included 62 subjects with a mean age (±SD) of 49 (±15) years; 82% were women. Spleen dimensions were 10 (±3), 6 (±2) and 9 (±2) cm for the craniocaudal length, thickness and width measurements, respectively. The median [interquartile range (IQR)] spleen volume was 344 (225-533) cm3. Splenomegaly was observed in 52-63% of the patients, depending on the formula used. The spleen volume was not associated with clinical, echocardiographic or hemodynamic variables. Spleen volume was not associated with adjusted mortality. We studied the characteristics of the spleen during autopsy in 9 patients with idiopathic PAH who died of right-heart failure. The mean (IQR) spleen weight was 220 (151-325) g. We observed early congestion in all but 2 patients who had chronic congestion.

CONCLUSIONS:

Splenomegaly of predominantly mild degree is common in idiopathic and heritable PAH. However, spleen size was not associated with clinical, echocardiographic, hemodynamic and survival data in these patients.
Copyright © 2012 S. Karger AG, Basel.
PMCID: PMC3707306 [Available on 2014/1/1]
  PMID: 22869505 [PubMed - indexed for MEDLINE]
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41. Am J Respir Crit Care Med. 2013 Sep 15;188(6):756-9. doi: 10.1164/rccm.201303-0467LE.

A proof of concept for the detection and classification of pulmonary arterial hypertension through breath analysis with a sensor array.

Cohen-Kaminsky SNakhleh MPerros FMontani DGirerd BGarcia GSimonneau GHaick H,Humbert M.
1 Université Paris-Sud, Faculté de Médecine Le Kremlin-Bicêtre, France INSERM UMR-S 999, LabEx LERMIT Le Plessis-Robinson, France Centre Chirurgical Marie Lannelongue Le Plessis-Robinson, France AP-HP, DHU Thorax Innovation, Service de Pneumologie, Hôpital Bicêtre Le Kremlin-Bicêtre, France and International Associated Laboratory INSERM-TECHNION Le Plessis-Robinson, France.
  PMID: 24032390 [PubMed - indexed for MEDLINE]
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42. Chest. 2013 Oct 31. doi: 10.1378/chest.13-1766. [Epub ahead of print]

Bosentan for sarcoidosis associated pulmonary hypertension: A double-blind placebo controlled randomized trial.

Baughman RPCulver DACordova FCPadilla MGibson KFLower EEEngel PJ.

Abstract

ABSTRACT BACKGROUND:

Sarcoidosis associated pulmonary hypertension (SAPH) is a common problem in persistently dyspneic sarcoidosis patients.

OBJECTIVE:

Determine the effect of bosentan therapy on pulmonary arterial hemodynamics in SAPH patients.

DESIGN:

Double blind, placebo controlled trial of 16 weeks of either bosentan or placebo in SAPH patients confirmed by right heart catheterization.

SETTING:

Multiple academic centers specializing in sarcoidosis care.

PATIENTS:

On stable treatment for their sarcoidosis therapy with confirmed pulmonary hypertension and were receiving no therapy for their pulmonary hypertension.

INTERVENTION:

Randomized 2:1 to receive bosentan at maximal dose of 125 mg twice a day or placebo for sixteen weeks MEASUREMENTS: Pulmonary function studies, six minute walk test, and right heart hemodynamics including pulmonary artery mean (PAm) and pulmonary vascular resistance (PVR) before and after 16 weeks of therapy.

RESULTS:

Thirty-five patients completed 16 weeks of therapy (23 treated with bosentan, 12 with placebo). For those treated with bosentan, repeat hemodynamic studies at 16 weeks demonstrated a significant fall in PAm of -4 (6.6) (Mean (SD)) mm Hg (p=0.0105) and PVR -1.7 (2.75) Wood's units (p=0.0104). For the placebo treated patients, there was no significant change in either PAm (1 (3.7) mm Hg, p&gt;0.05) or PVR (0.1 (1.42) Wood's units, p&gt;0.05). There was no significant change in six minute walk distance for either group. Two bosentan treated patients required an increase of supplemental oxygen by more than two liters after 16 weeks of therapy.

CONCLUSIONS:

This study demonstrated that bosentan was able to significantly improve pulmonary hemodynamics in SAPH patientsStudy was registered at ClinTrials NCT00581607.
  PMID: 24177203 [PubMed - as supplied by publisher]
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43. Heart. 2013 Nov 1. doi: 10.1136/heartjnl-2013-304254. [Epub ahead of print]

Pulmonary hypertension leads to a loss of gravity dependent redistribution of regional lung perfusion: a SPECT/CT study.

Lau EMBailey DLBailey EATorzillo PJRoach PJSchembri GPCorte TJCelermajer DS.
Department of Medicine, Sydney Medical School, University of Sydney, , Camperdown, Australia.

Abstract

OBJECTIVE:

Pre-capillary pulmonary hypertension (PHT) is characterised by progressive pulmonary vascular obliteration and loss of vascular reserves. In health, regional lung perfusion redistributes under the influence of gravity due to the presence of recruitable vessels. We investigated a combined single photon emission computed tomography/CT (SPECT/CT) method for assessing the pulmonary circulation by quantifying the gravity dependent redistribution of lung perfusion.

DESIGN:

Characterisation of patients versus healthy controls.

PATIENTS:

15 patients with pre-capillary PHT and 11 healthy controls.

SETTING:

University hospital clinic.

INTERVENTION:

Regional lung perfusion was measured using SPECT/CT in two different postures (supine vs upright). A perfusion redistribution index (PRI) was used to quantify the cranial-caudal shift in regional lung perfusion resulting from gravitational (postural) change.

MAIN OUTCOME MEASURE:

PRI was compared between cases and controls, and correlated with markers of disease severity in cases.

RESULTS:

Patients with pre-capillary PHT had notably reduced PRI compared to controls (0.02±0.06 vs 0.28±0.15 normalised perfusion/cm, p<0.0001). PRI was significantly associated with prognostic parameters such as 6 min walk distance (r=0.60, p=0.018), functional class (p=0.008), and tricuspid annular plane systolic excursion (r=0.58, p=0.022). The receiver operating characteristic curve showed that PRI differentiated patients with pre-capillary PHT from controls with AUC=0.94 (p<0.001).

CONCLUSIONS:

With SPECT/CT, gravity dependent redistribution of lung perfusion can be quantified using the PRI derived from supine and upright perfusion analysis. The potential utility of PRI for the non-invasive detection of PHT and assessment of disease severity warrants further study.
  PMID: 24186566 [PubMed - as supplied by publisher]
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44. J Thorac Cardiovasc Surg. 2013 Oct 28. pii: S0022-5223(13)01070-2. doi: 10.1016/j.jtcvs.2013.08.073. [Epub ahead of print]

Predicting early left ventricular dysfunction after mitral valve reconstruction: The effect of atrial fibrillation and pulmonary hypertension.

Varghese RItagaki SAnyanwu ACMilla FAdams DH.
Department of Cardiothoracic Surgery, Icahn School of Medicine, Mount Sinai Medical Center, New York, NY. Electronic address: robin.varghese@mountsinai.org.

Abstract

OBJECTIVE:

The preoperative ejection fraction (EF) and left ventricular (LV) end-systolic dimension are known predictors of postoperative LV dysfunction after mitral valve repair. We investigated the effect of a preoperative history of atrial fibrillation and moderate pulmonary hypertension (defined as pulmonary artery systolic pressure >50 mm Hg) on early postoperative LV dysfunction.

METHODS:

From 2003 to 2010, 632 patients who had undergone successful mitral valve repair surgery for degenerative disease were included in the present study. The preoperative and postoperative echocardiographic data and postoperative outcomes were collected retrospectively. We analyzed the demographic, hemodynamic, and echocardiographic parameters to assess the predictors of early postoperative LV dysfunction, defined as an LVEF <50%.

RESULTS:

The mean age of the cohort was 57 ± 13 years. All patients had less than mild mitral regurgitation on postoperative echocardiography. After mitral valve repair, a significant decrease in the LVEF (60% ± 8% to 54% ± 9%), LV end-systolic diameter (36 ± 7 mm to 33 ± 7 mm), and LV end-diastolic dimension (56 ± 8 mm to 48 ± 7 mm) was observed at early postoperative echocardiography (P < .001). On multivariate regression analysis, preoperative atrial fibrillation, pulmonary hypertension, and LV end-systolic dimension were independent predictors of the postoperative LVEF (P = .035 and P < .001, respectively). Preoperative atrial fibrillation (odds ratio, 1.97; 95% confidence interval, 1.28-3.02; P = .002) and pulmonary artery systolic pressure >50 mm Hg (odds ratio, 1.82; 95% confidence interval, 1.11-2.97; P = .017) increased the risk of postoperative LV dysfunction by almost twofold.

CONCLUSIONS:

In addition to the established predictors of postoperative LV dysfunction, the presence of preoperative pulmonary hypertension and a history of atrial fibrillation in patients undergoing mitral valve repair surgery increased the risk of early postoperative LV dysfunction by almost twofold.
Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.
  PMID: 24176266 [PubMed - as supplied by publisher]
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45. Exp Ther Med. 2013 Dec;6(6):1350-1358. Epub 2013 Sep 30.

Non-invasive evaluation of hemodynamics in pulmonary hypertension by a Septal angle measured by computed tomography pulmonary angiography: Comparison with right-heart catheterization and association with N-terminal pro-B-type natriuretic peptide.

Tang QLiu MMa ZGuo XKuang TYang Y.
Department of Cardiology, Shougang Hospital, Peking University, Beijing 100144, P.R. China.

Abstract

The septal angle, an angle between the interventricular septum and the line connecting the sternum midpoint and thoracic vertebral spinous process, as measured by computed tomographic pulmonary angiography (CTPA), has been observed to be increased in patients with pulmonary hypertension (PH), but its meaning remains unclear. The aim of this study was to investigate the potential role of the septal angle in evaluating hemodynamics and its association with N-terminal pro-B-type natriuretic peptide (NT-proBNP) in patients with PH. Patients with PH (n=106), including 76 with chronic thromboembolic pulmonary hypertension (CTEPH) and 30 with pulmonary artery hypertension (PAH), were retrospectively reviewed. The patients underwent CTPA prior to right-heart catheterization. The septal angle was measured on transversal CTPA images. Hemodynamic parameters were evaluated by right-heart catheterization. The level of plasma NT-proBNP was measured by enzyme-linked sandwich immunoassay. The septal angle had a moderate correlation with cardiac output (CO; r=-0.535, P=0.000) and a high correlation with pulmonary vascular resistance (PVR; r=0.642, P=0.000). The mean level of NT-proBNP in PH was 1,716.09±1,498.30 pg/ml, which correlated with the septal angle (r=0.693, P=0.000). In a stepwise forward regression analysis, the Septal angle was entered into the final equation for predicting PVR, leading to the following equation: PVR = 28.256 × Septal angle - 728.72. In CTEPH, the Septal angle strongly correlated with NT-proBNP (r=0.668, P=0.000) and PVR (r=0.676, P=0.000). In PAH, the Septal angle strongly correlated with NT-proBNP (r=0.616, P=0.003) and PVR (r=0.623, P=0.000). The CTPA-derived Septal angle is a superior predictor for evaluating and monitoring the level of NT-proBNP and PVR in patients with PH.
PMCID: PMC3829756 Free PMC Article
  PMID: 24255661 [PubMed]
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46. Curr Cardiol Rev. 2013 Nov 17. [Epub ahead of print]

Pulmonary Hypertension: Types and Treatments.

Rose-Jones LJMcLaughlin V.
UNC Center for Heart & Vascular Care, 160 Dental Circle, CB 7075, Chapel Hill, NC 27599-7075 USA.lisa_rose-jones@med.unc.edu.

Abstract

Pulmonary arterial hypertension (PAH) is a panvasculopathy that affects the distal pulmonary arteries and leads to restricted blood flow. This increased afterload leads to adaptive mechanisms of the right ventricle, with eventual failure once it can no longer compensate. Pulmonary hypertension from associated conditions, most importantly left heart disease,i.e. heart failure, can also lead to the same sequela. Patients often experience early vague symptoms of dyspnea and exercise intolerance, and thus PH can elude clinicians until right heart failure symptoms predominate. Evidence-based treatment options with pulmonary vasodilators are available for those with PAH and should be employed early. It is essential that patients be accurately categorized by their etiology of PH, as treatment strategies differ, and can potentially be dangerous if employed in the wrong clinical scenario.
  PMID: 24251459 [PubMed - as supplied by publisher]
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47. Best Pract Res Clin Rheumatol. 2013 Jun;27(3):425-34. doi: 10.1016/j.berh.2013.07.011.

Pulmonary hypertension in systemic lupus erythematosus.

Schreiber BEConnolly MJCoghlan JG.
National Pulmonary Hypertension Service, Royal Free Hospital NHS Foundation Trust, UK. Electronic address: benjamin.schreiber@nhs.net.

Abstract

Systemic lupus erythematosus is associated with several forms of pulmonary hypertension. It can cause pulmonary hypertension through pulmonary thromboembolic disease, left heart disease and lung disease as well as causing an isolated pulmonary vasculopathy called pulmonary arterial hypertension. The true prevalence of pulmonary arterial hypertension in patients with lupus is not known but probably is no more than 1%. Currently, treatment for lupus-associated pulmonary arterial hypertension is with pulmonary vasodilators including phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostacyclin analogues, as it is for other causes of pulmonary arterial hypertension. Case series suggest there may be a special role for immunosuppression in this rare group of patients. We present two brief case histories and summarise our experience over 15 years. Prognosis is better in lupus-associated pulmonary arterial hypertension than in systemic sclerosis-associated pulmonary arterial hypertension, but unfortunately it remains a fatal condition in most patients.
Copyright © 2013 Elsevier Ltd. All rights reserved.
  PMID: 24238697 [PubMed - in process]
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48. Circ Heart Fail. 2013 Sep 1;6(5):953-63.

Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension.

Tedford RJMudd JOGirgis REMathai SCZaiman ALHousten-Harris TBoyce DKelemen BW,Bacher ACShah AAHummers LKWigley FMRussell SDSaggar RSaggar RMaughan WL,Hassoun PMKass DA.

Abstract

BACKGROUND:

Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function.

METHODS AND RESULTS:

We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis–related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure–volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0.8±0.3 versus 2.3±1.1, P<0.01; Msw=21±11 versus 45±16, P=0.01), with differential RV-PA uncoupling (Ees/Ea=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH).

CONCLUSIONS:

RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.
PMCID: PMC3815697 [Available on 2014/9/1]
  PMID: 23797369 [PubMed - indexed for MEDLINE]
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49. Circ J. 2013 Nov 27. [Epub ahead of print]

Histopathological Examination by Lung Biopsy for the Evaluation of Operability and Postoperative Prognosis in Patients With Chronic Thromboembolic Pulmonary Hypertension.

Yamaki SAndo MFukumoto YHiguchi YKaneko KMaeda KShimokawa H.
Japanese Research Institute of Pulmonary Vasculature.

Abstract

Background: To evaluate the prognosis after pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH), a lung biopsy was performed in 34 patients with central CTEPH and in 7 patients with peripheral CTEPH during PTE. Methods and Results: Postoperative prognosis was classified from A to E based on the postoperative hemodynamic parameters and clinical condition, and was compared with the index of occlusion (IOCTEPH), which indicates the degree of occlusion in the small pulmonary arteries. Criteria of (A-E) were established only for central CTEPH. Category (A) corresponded to an IOCTEPH from 1.0 to 1.4, (B) from 1.5 to 1.7, (C) from 1.8 to 2.0, and (D) from 2.1 to 2.4. One patient with an index of 3.0 was rated as (E). This patient had collateral vessels around the obstructed small pulmonary arteries and died postoperatively. In all 12 patients who underwent PTE after the criteria were established, postoperative hemodynamic parameters and clinical conditions were consistent with the IOCTEPH. One patient with a high degree of medial atrophy in their small pulmonary arteries died after PTE. Conclusions: These results indicate that a lung biopsy during PTE is useful for prognostication in patients with CTEPH.
Free Article
  PMID: 24284920 [PubMed - as supplied by publisher]
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50. Multidiscip Respir Med. 2013 Nov 26;8(1):72. [Epub ahead of print]

Clinical variability of respiratory pulmonary hypertension: implications for diagnosis and management.

Casali LCarratù PSofia M.

Abstract

Pulmonary Hypertension (PH) associated to chronic respiratory diseases is currently classified in the 3rd group, as a mild to moderate form of pre-capillary PH that progressively complicates the prognosis of associated pulmonary disease. In clinical practice, however, some unresolved issues in the respiratory PH should be considered: 1) the PH heterogeneity in some respiratory diseases, such as Chronic Obstructive Pulmonary Disease (COPD), where the prevalence of unrecognized left heart disease, or chronic pulmonary thromboembolism may change the clinical classification; 2) the combining form of severe PH which often is not correlated to chronic ventilator impairment, while outcome is strictly related to pulmonary haemodynamics. The recognition of out of proportion respiratory PH in several chronic respiratory diseases which include COPD, Idiopathic Pulmonary Fibrosis (IPF), Combined Pulmonary Fibrosis and Emphysema, Obstructive Sleep Apnea (OSA), Obesity Hypoventilation Syndrome (OHS) may be important for a comprehensive clinical classification of severe respiratory PH, as well as, for the inclusion of these patients in randomized clinical trials on PH targeted therapy.
Free Article
  PMID: 24280232 [PubMed - as supplied by publisher]
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51. JACC Cardiovasc Imaging. 2013 Oct 17. pii: S1936-878X(13)00664-5. doi: 10.1016/j.jcmg.2013.08.007. [Epub ahead of print]

RV Contractility and Exercise-Induced Pulmonary Hypertension in Chronic Mountain Sickness: A Stress Echocardiographic and Tissue Doppler Imaging Study.

Pratali LAllemann YRimoldi SFFaita FHutter DRexhaj EBrenner RBailey DMSartori CSalmon CSVillena MScherrer UPicano ESicari R.
Institute of Clinical Physiology, CNR, Pisa, Italy. Electronic address: lorenza@ifc.cnr.it.

Abstract

OBJECTIVES:

The aim of this study was to evaluate right ventricular (RV) and left ventricular function and pulmonary circulation in chronic mountain sickness (CMS) patients with rest and stress echocardiography compared with healthy high-altitude (HA) dwellers.

BACKGROUND:

CMS or Monge's disease is defined by excessive erythrocytosis (hemoglobin >21 g/dl in males, 19 g/dl in females) and severe hypoxemia. In some cases, a moderate or severe increase in pulmonary pressure is present, suggesting a similar pathogenesis of pulmonary hypertension.

METHODS:

In La Paz (Bolivia, 3,600 m sea level), 46 CMS patients and 40 HA dwellers of similar age were evaluated at rest and during semisupine bicycle exercise. Pulmonary artery pressure (PAP), pulmonary vascular resistance, and cardiac function were estimated by Doppler echocardiography.

RESULTS:

Compared with HA dwellers, CMS patients showed RV dilation at rest (RV mid diameter: 36 ± 5 mm vs. 32 ± 4 mm, CMS vs. HA, p = 0.001) and reduced RV fractional area change both at rest (35 ± 9% vs. 43 ± 9%, p = 0.002) and during exercise (36 ± 9% vs. 43 ± 8%, CMS vs. HA, p = 0.005). The RV systolic longitudinal function (RV-S') decreased in CMS patients, whereas it increased in the control patients (p < 0.0001) at peak stress. The RV end-systolic pressure-area relationship, a load independent surrogate of RV contractility, was similar in CMS patients and HA dwellers with a significant increase in systolic PAP and pulmonary vascular resistance in CMS patients (systolic PAP: 50 ± 12 mm Hg vs. 38 ± 8 mm Hg, CMS vs. HA, p < 0.0001; pulmonary vascular resistance: 2.9 ± 1 mm Hg/min/l vs. 2.2 ± 1 mm Hg/min/l, p = 0.03). Both groups showed comparable systolic and diastolic left ventricular function both at rest and during stress.

CONCLUSIONS:

Comparable RV contractile reserve in CMS and HA suggests that the lower resting values of RV function in CMS may represent a physiological adaptation to chronic hypoxic conditions rather than impaired RV function. (Chronic Mountain Sickness, Systemic Vascular Function [CMS]; NCT01182792).
Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
  PMID: 24269266 [PubMed - as supplied by publisher]
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52. Clin Chest Med. 2013 Dec;34(4):779-97. doi: 10.1016/j.ccm.2013.08.012. Epub 2013 Oct 20.

Chronic thromboembolic pulmonary hypertension.

Marshall PSKerr KMAuger WR.
Yale University School of Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, 15 York Street, LCI 101, New Haven, CT 06510, USA. Electronic address: peter.marshall@yale.edu.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease with high mortality and few treatment options. This article reviews the epidemiology of CTEPH and identifies risk factors for its development. The pathobiology and the progression from thromboembolic events to chronically increased right-sided pressures are discussed. The diagnosis and assessment of CTEPH requires several modalities and the role of these is detailed. The pre-operative evaluation assesses peri-operative risk and determines the likelihood of benefit from PTE. Pulmonary thromboendarterectomy (PTE) remains the treatment of choice in appropriate patients. Nonsurgical therapies for CTEPH may provide benefit in patients who cannot be offered surgery.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 24267304 [PubMed - in process]
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53. Clin Chest Med. 2013 Dec;34(4):753-78. doi: 10.1016/j.ccm.2013.08.005. Epub 2013 Oct 18.

World health organization group 5 pulmonary hypertension.

Lahm TChakinala MM.
Division of Pulmonary, Allergy, Critical Care, Occupational and Sleep Medicine, Department of Medicine, Richard L. Roudebush VA Medical Center, Center for Immunobiology, Indiana University School of Medicine, 980 West Walnut Street, Room C400, Indianapolis, IN 46202, USA. Electronic address:tlahm@iu.edu.

Abstract

World Health Organization (WHO) group 5 pulmonary hypertension (PH) entails a heterogeneous group of disorders that may cause PH by unclear and/or multiple mechanisms. In particular, group 5 includes PH caused by hematologic disorders, systemic diseases, metabolic disorders, chronic renal failure, and disorders leading to pulmonary vascular occlusion or compression. This article discusses common pathogenic mechanisms leading to group 5 PH, followed by a detailed overview of epidemiology, pathogenesis, and disease-specific management of the individual group 5 conditions. Off-label use of vasomodulatory therapies, typically indicated for pulmonary arterial hypertension (WHO group 1 PH), in group 5 conditions is also discussed.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 24267303 [PubMed - in process]
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54. Clin Chest Med. 2013 Dec;34(4):683-94. doi: 10.1016/j.ccm.2013.09.004. Epub 2013 Oct 17.

Pulmonary hypertension owing to left heart disease.

Mathier MA.
Pulmonary Hypertension Program, University of Pittsburgh Medical Center, University of Pittsburgh School of Medicine, Scaife Hall S559, 200 Lothrop Street, Pittsburgh, PA 15213, USA. Electronic address:mathierm@upmc.edu.

Abstract

The relationship between pulmonary hypertension and left heart disease is complex. When initial assessment suggests the presence of pulmonary hypertension, it is critical to determine its precise nature: pulmonary arterial hypertension versus pulmonary hypertension owing to left heart disease. Clues to diagnosis can be found in the history, physical examination, initial laboratory testing, and imaging studies. Treatment requires optimal therapy of the underlying left heart disease. It is uncertain whether therapies for pulmonary arterial hypertension have any role in treating pulmonary hypertension owing to left heart disease, and there are safety concerns with these agents in this population.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 24267298 [PubMed - in process]
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55. J Clin Rheumatol. 2013 Dec;19(8):421-5. doi: 10.1097/RHU.0000000000000037.

Pulmonary hypertension in systemic lupus erythematosus: pulmonary thromboembolism is the leading cause.

Akdogan AKilic LDogan IOkutucu SEr EKaya BCoplu LCalguneri MTokgozoglu LErtenli I.
From the *Division of Rheumatology, Department of Internal Medicine, †Departments of Cardiology, ‡Internal Medicine, and §Chest Diseases, Hacettepe University Hospital, Ankara, Turkey.

Abstract

BACKGROUND:

Pulmonary hypertension (PH) is a life-threatening complication of systemic lupus erythematosus (SLE). Pulmonary hypertension in SLE has a variety of causes. Diagnosing early and defining the cause of PH accurately can provide better clinical outcome in SLE. We investigated the causes and characteristics of PH in patients with SLE.

METHODS:

One hundred twenty-one patients with SLE who had a visit in a 6-month period were assessed retrospectively. Patients who ever had a systolic pulmonary arterial pressure of 40 mm Hg or greater by Doppler echocardiography were considered to have PH.

RESULTS:

Among 122 patients, 65 had echocardiography for some reason, and 10 (8.2%) were diagnosed as having PH by echocardiographic examination. This number reduced to 9 (7.4%) when we excluded the patient with normal pulmonary artery pressure at right heart catheterization. Causes of PH were as follows: thromboembolic events in 4 patients (44.4%) (2 of them had chronic thromboembolic PH), left-sided heart disease in 2 patients (22.2%), pulmonary arterial hypertension in 1 patient (11.1%), high cardiac output state in 1 patient (11.1%), and transient elevation of systolic pulmonary artery pressure in 1 patient (11.1%) who had a history of venous thromboembolism. Venous thromboembolic disease was significantly higher in patients with SLE with PH in comparison to patients with SLE without PH (7 patients [6.3%] vs 5 patients [50.0%]; P = 0.001). All patients improved clinically during their short-term follow-up.

CONCLUSIONS:

Patients with SLE are at increased risk for PH. This study highlights the complexity of the differential diagnosis of PH in patients with SLE once again and emphasizes the importance of pulmonary thromboembolism as a cause of PH. One should investigate patients with SLE with unexplained symptoms and/or signs related to PH for possible treatable causes.
  PMID: 24263143 [PubMed - in process]
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56. J Card Fail. 2013 Nov;19(11):753-5. doi: 10.1016/j.cardfail.2013.10.006. Epub 2013 Oct 25.

Dynamic pulmonary hypertension in decompensated heart failure with preserved ejection fraction: is functional mitral regurgitation the driver?

Campbell P.
Advanced Heart Disease Section, University of Calgary, Calgary, Canada. Electronic address:drpmcampbell@gmail.com.
  PMID: 24263119 [PubMed - in process]
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57. JACC Cardiovasc Interv. 2013 Nov;6(11):1212-3. doi: 10.1016/j.jcin.2013.03.025.

Percutaneous transluminal pulmonary angioplasty for central-type chronic thromboembolic pulmonary hypertension.

Ishiguro HKataoka MInami TYanagisawa RShimura NTaguchi HKohshoh HYoshino HSatoh T.
Division of Cardiology, Second Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.
  PMID: 24262622 [PubMed - in process]
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