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- World Health Organization Pulmonary Hypertension Group 2: Pulmonary hypertension due to left heart disease in the adult. (39)

Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients. (37)

Renal Replacement Therapy in Patients with Severe Precapillary Pulmonary Hypertension with Acute Right Heart Failure. (3)

- Possible anti-metastatic effect of iloprost in a patient with systemic sclerosis with lung cancer: a case study. (11)

- 35 other PHT publications including multiple PHT treatment specific publications (Ambrisentan, Bosentan, Sildenafil, Tadalafil, Iloprost)

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1. Circ Cardiovasc Imaging. 2012 Aug 22. [Epub ahead of print]
A Simple Echocardiographic Prediction Rule for Hemodynamics in Pulmonary Hypertension.
Opotowsky AR, Ojeda J, Rogers F, Prasanna V, Clair M, Moko L, Vaidya A, Afilalo J, Forfia PR.
Source
1 Hospital of the Univ of Penn, Philadelphia, PA, Boston Children's Hospital and BWH, Boston, MA;
Abstract
BACKGROUND:
-Pulmonary hypertension (PH) has diverse causes with heterogeneous physiology compelling distinct management. Differentiating patients with primarily elevated pulmonary vascular resistance (PVR) from those with PH predominantly due to elevated left sided filling pressure is critical.
METHODS AND RESULTS:
-We reviewed hemodynamics, echocardiography, and clinical data for 108 patients seen at a referral PH clinic with transthoracic echocardiogram and right heart catheterization within 1 year. We derived a simple echocardiographic prediction rule to allow hemodynamic differentiation of PH due to pulmonary vascular disease (PH(PVD), defined as pulmonary artery wedge pressure (PAWP) ≤15mmHg and PVR >3WU). Age averaged 61.3±14.8 years, μPAWP and PVR were 16.4±7.1mmHg and 6.3±4.0WU respectively, and 52 (48.1%) patients fulfilled PH(PVD) hemodynamic criteria. The derived prediction rule ranged from -2 to +2 with higher scores suggesting higher probability of PH(PVD): +1 point for left atrial AP dimension<3.2cm; +1 for presence of a mid-systolic notch or acceleration time<80msec; -1 for lateral mitral E:e'>10; -1 for left atrial AP dimension>4.2cm. PVR increased stepwise with score (for -2, 0 and +2, μPVR were 2.5, 4.5, and 8.1WU) while the inverse was true for PAWP (corresponding μPAWP were 21.5, 16.5 and 10.4mmHg). Among subjects with complete data, the score had an AUC of 0.921 for PH(PVD). A score ≥0 had 100% sensitivity and 69.3% positive predictive value for PH(PVD), with 62.3% specificity. No patients with a negative score had PH(PVD). Patients with a negative score and acceleration time >100msec had normal PVR (μPVR=1.8WU, range=0.7-3.2WU).
CONCLUSIONS:
-We present a simple echocardiographic prediction rule that accurately defines PH hemodynamics facilitates improved screening and focused clinical investigation for PH diagnosis and management.
  PMID: 22914595 [PubMed - as supplied by publisher]
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2. Invest Radiol. 2012 Aug 21. [Epub ahead of print]
Pulmonary Artery Relative Area Change Detects Mild Elevations in Pulmonary Vascular Resistance and Predicts Adverse Outcome in Pulmonary Hypertension.
Swift AJ, Rajaram S, Condliffe R, Capener D, Hurdman J, Elliot C, Kiely DG, Wild JM.
Source
From the *Unit of Academic Radiology, University of Sheffield; †National Institutes of Health Research, Cardiovascular Biomedical Research Unit; and ‡Sheffield Pulmonary Vascular Clinic, Sheffield Teaching Hospitals Trust, Sheffield, UK.
Abstract
OBJECTIVE:
The aim of this study was to evaluate the clinical use of magnetic resonance imaging measurements related to pulmonary artery stiffness in the evaluation of pulmonary hypertension (PH).
MATERIALS AND METHODS:
A total of 134 patients with suspected PH underwent right heart catheterization (RHC) and magnetic resonance imaging on a 1.5-T scanner within 2 days. Phase contrast imaging at the pulmonary artery trunk and cine cardiac views were acquired. Pulmonary artery area change (AC), relative AC (RAC), compliance (AC/pulse pressure from RHC), distensibility (RAC/pulse pressure from RHC), right ventricular functional indices, and right ventricular mass were all derived. Regression curve fitting identified the statistical model of best fit between RHC measurements and pulmonary artery stiffness indices. The diagnostic accuracy and prognostic value of noninvasive AC and RAC were also assessed.
RESULTS:
The relationship between pulmonary vascular resistance and pulmonary artery RAC was best reflected by an inverse linear model. Patients with mild elevation in pulmonary vascular resistance (<4 Woods units) demonstrated reduced RAC (P = 0.02) and increased right ventricular mass index (P < 0.0001) without significant loss of right ventricular function (P = 0.17). At follow-up of 0 to 40 months, 18 patients with PH had died (16%). Analysis of Kaplan-Meier plots showed that both AC and RAC predicted mortality (log-rank test, P = 0.046 and P = 0.012, respectively). Area change and RAC were also predictors of mortality using univariate Cox proportional hazards regression analysis (P = 0.046 and P = 0.03, respectively).
CONCLUSIONS:
Noninvasive assessment of pulmonary artery RAC is a marker sensitive to early increased vascular resistance in PH and is a predictor of adverse outcome.
  PMID: 22914228 [PubMed - as supplied by publisher]
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3. Respiration. 2012 Aug 15. [Epub ahead of print]
Renal Replacement Therapy in Patients with Severe Precapillary Pulmonary Hypertension with Acute Right Heart Failure.
Sztrymf B, Prat D, Jacobs FM, Brivet FG, O'Callaghan DS, Price LC, Jais X, Sitbon O, Simonneau G, Humbert M.
Source
Réanimation Médicale, Hôpital Antoine Béclère, AP-HP, Clamart, France.
Abstract
Background: Renal replacement therapy has been suggested as a therapeutic option in the setting of acute right ventricular failure in patients with severe precapillary pulmonary hypertension. However, there are few data supporting this strategy. Objectives: To describe the clinical course and the prognosis of pulmonary hypertensive patients undergoing renal replacement therapy in the setting of acute right heart failure. Methods: This was a single-center retrospective study over an 11-year period. Data were collected from all patients with chronic precapillary pulmonary hypertension requiring catecholamine infusions for clinical worsening and acute kidney injury that necessitated renal replacement therapy. Results: Fourteen patients were included. At admission, patients had a blood urea of 28.2 mmol/l (22.3-41.2), a creatinine level of 496 µmol/l (304-590), and a mean urine output in the 24 h preceding hospitalization of 200 ml (0-650). Sixty-eight renal replacement therapy sessions were performed, 36 of which were continuous and 32 of which were intermittent. Systemic hypotension occurred in 16/32 intermittent and 16/36 continuous sessions (p = 0.9). Two patients died during a continuous session. The intensive care unit-related, 1-, and 3-month mortality was 46.7, 66.7, and 73.3%, respectively. Conclusion: Renal replacement therapy is feasible in the setting of acute right ventricular failure in patients with severe precapillary pulmonary hypertension but is associated with a poor prognosis. The best modality and timing in this population remain to be defined.
Copyright © 2012 S. Karger AG, Basel.
  PMID: 22906846 [PubMed - as supplied by publisher]
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4. J Magn Reson Imaging. 2012 Sep;36(3):spcone. doi: 10.1002/jmri.23554.
Contrast-enhanced multidetector-row computed tomography vs. time-resolved magnetic resonance angiography vs. contrast-enhanced perfusion MRI: Assessment of treatment response by patients with inoperable chronic thromboembolic pulmonary hypertension.
Ohno Y, Koyama H, Yoshikawa T, Nishio M, Matsumoto S, Matsumoto K, Aoyama N, Nogami M, Murase K, Sugimura K.
Source
Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan; Center for Radiology and Radiation Oncology, Kobe University Hospital, Kobe, Hyogo, Japan. yosirad@kobe-u.ac.jp.
Abstract
PURPOSE:
To compare therapeutic effect assessment capability of multidetector-row computed tomography (MDCT), magnetic resonance angiography (MRA), and dynamic perfusion MRI for chronic thromboembolic pulmonary hypertension (CTEPH) patients.
MATERIALS AND METHODS:
Twenty-four consecutive CTEPH patients treated with conventional therapy underwent pre- and posttherapeutic MDCT, MRA, dynamic perfusion MRI, 6-minute walk distance (6-MWD), cardiac ultrasound (US), and right heart catheterization. According to therapeutic results, all patients were divided into response (n = 13) and nonresponse (n = 11) groups. CTEPH indexes for MDCT (CTEPH(CT) ) and MRA (CTEPH(MRA) ) were calculated on the basis of embolic burden. Pulmonary perfusion parameter maps were generated from all perfusion MR data, followed by determination of improvements in mean perfusion parameter at regions of interest (ROIs) for each patient. Receiver operating characteristic (ROC)-based positive tests were performed to determine the feasible threshold values for distinguishing two groups. Finally, diagnostic capabilities were compared by means of McNemar's test.
RESULTS:
When feasible threshold values adapted, specificity (90.9 〈10/11〉%, P < 0.05) and accuracy (95.8 〈23/24〉%, P < 0.05) for improvement in pulmonary blood flow were significantly higher than those for CTEPH(CTA) (specificity: 36.4 〈4/11〉%, accuracy: 70.8 〈17/24〉%).
CONCLUSION:
Dynamic perfusion MRI has better capability for assessment of therapeutic effect on CTEPH patients than does MDCT. J. Magn. Reson. Imaging 2012;36:612-623. © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
  PMID: 22903656 [PubMed - in process]
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5. Intern Emerg Med. 2012 Aug 18. [Epub ahead of print]
Pulmonary hypertension: have we learned enough yet?
Cattano D, Doursout MF.
Source
Department of Anesthesiology, The University of Texas, Medical School at Houston, Houston, TX, USA, davide.cattano@uth.tmc.edu.
  PMID: 22903538 [PubMed - as supplied by publisher]
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6. Zhongguo Fei Ai Za Zhi. 2012 Aug 20;15(8):471-5.
[The Assessment of Right Ventricular Function and Pulmonary Artery
Hemodynamics in Patients with Pulmonary Hypertension by 1.5T MRI].
[Article in Chinese]
Han Y, Yang Z, Yu T, Yu J, Xue M, Zhang Z, Li D.
Source
Department of Radiology, Tianjin Medical University General Hospital, Tianjin 300052, China.
Abstract
BACKGROUND:
Pulmonary hypertension (PH) is characterized by rising pulmonary arterial pressure, decreasing right ventricular (RV) function, and ultimately, RV failure. Therefore, it is important to monitor RV function and pulmonary artery hemodynamics accurately and noninvasively. This study evaluates cardiac magnetic resonance imaging (CMRI) in assessing RV function and pulmonary artery hemodynamics in patients with PH.
METHODS:
Cine-MRI and phase-contrast MRI (PC-MRI) were performed in 25 PH patients and 30 healthy volunteers. Cine-MRI images were post-processed on Report Card software and the following parameters were obtained: RV end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF), and myocardial mass (MM). Except for EF, all of the above parameters were normalized to body surface area (BSA). PC-MRI images were post-processed on Report Card software, peak velocity and distensibility of main pulmonary artery (MPA) could also be obtained. Student t test was employed for statistical assessment.
RESULTS:
Compared with controls, RV EDV, ESV and MM index in PH patients were significantly increased (P<0.01), EF was significantly impaired (P<0.01), peak velocity and distensibility of MPA were significantly lower (P<0.01). SV index between the two groups had no significant difference (P>0.05).
CONCLUSIONS:
Cardiac MRI is of great value in the assessment of RV function and MPA hemodynamic parameters in patients with PH.
Free Article
  PMID: 22901995 [PubMed - in process]
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7. J Surg Res. 2012 Aug 10. [Epub ahead of print]
The use of ECMO for persistent pulmonary hypertension of the newborn: a decade of experience.
Lazar DA, Cass DL, Olutoye OO, Welty SE, Fernandes CJ, Rycus PT, Lee TC.
Source
Texas Children's Fetal Center and the Michael E DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
Abstract
PURPOSE:
Despite improvements in the management of persistent pulmonary hypertension of the newborn (PPHN), a number of infants with inadequate gas exchange are treated with extracorporeal membrane oxygenation (ECMO). The objectives of this study were to use the Extracorporeal Life Support Organization Registry to review the outcomes of neonates with PPHN receiving ECMO, and to identify pre-ECMO variables that may be associated with increased mortality.
METHODS:
The study is a retrospective analysis of all patients with PPHN supported with ECMO and reported to the Extracorporeal Life Support Organization registry from 2000 to 2010. We defined prematurity as <37 wk gestation. We performed univariate analysis using Student's t-test or Fisher's exact test. Variables found to be statistically significant underwent multivariate analysis by logistic regression. We generated Kaplan-Meier survival curves to analyze the relationship between duration of ECMO support and patient survival.
RESULTS:
A total of 1569 neonates with PPHN received ECMO support during the study period, at an average age of 3.1 d of life and for a duration of 6.9 d. Survival among neonates with PPHN receiving ECMO support was 81%, and those receiving support for 7, 10, 14, and 21 d survived at rates of 88%, 78%, 55%, and 25%, respectively. By logistic regression, prematurity (P < 0.01), pre-ECMO pH ≤7.2 (P = 0.02), pre-ECMO SaO(2) ≤65% (P = 0.01), and duration of ECMO ≥7 d (P < 0.001) were independent predictors of death in this group. An average of 2.2 complications occurred per patient, with cardiovascular, mechanical, and renal complications being the most common.
CONCLUSIONS:
Neonates with PPHN have high survival rates with ECMO support. Prematurity, acidosis, and profound hypoxemia are independently associated with increased mortality. Furthermore, prolonged ECMO support (>7 d) is associated with a higher risk of mortality in this cohort than in patients supported for <1 wk.
Copyright © 2012 Elsevier Inc. All rights reserved.
  PMID: 22901797 [PubMed - as supplied by publisher]
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8. Arq Bras Cardiol. 2012 Aug 16:0. [Epub ahead of print]
Sildenafil vs. sodium before nitroprusside for the pulmonary hypertension reversibility test before cardiac transplantation.
[Article in English, Portuguese]
Freitas Jr AF, Bacal F, Oliveira Júnior JD, Fiorelli AI, Santos RH, Moreira LF, Silva CP, Mangini S, Tsutsui JM, Bocchi EA.
Source
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Abstract
BACKGROUND:
Pulmonary hypertension is associated with a worse prognosis after cardiac transplantation. The pulmonary hypertension reversibility test with sodium nitroprusside (SNP) is associated with a high rate of systemic arterial hypotension, ventricular dysfunction of the transplanted graft and high rates of disqualification from transplantation.
OBJECTIVE:
This study was aimed at comparing the effects of sildenafil (SIL) and SNP on hemodynamic, neurohormonal and echocardiographic variables during the pulmonary reversibility test.
METHODS:
The patients underwent simultaneously right cardiac catheterization, echocardiography, BNP measurement, and venous blood gas analysis before and after receiving either SNP (1 - 2 µg/kg/min) or SIL (100 mg, single dose).
RESULTS:
Both drugs reduced pulmonary hypertension, but SNP caused a significant systemic hypotension (mean blood pressure - MBP: 85.2 vs. 69.8 mm Hg; p < 0.001). Both drugs reduced cardiac dimensions and improved left cardiac function (SNP: 23.5 vs. 24.8%, p = 0.02; SIL: 23.8 vs. 26%, p < 0.001) and right cardiac function (SIL: 6.57 ± 2.08 vs. 8.11 ± 1.81 cm/s, p = 0.002; SNP: 6.64 ± 1.51 vs. 7.72 ± 1.44 cm/s, p = 0.003), measured through left ventricular ejection fraction and tissue Doppler, respectively. Sildenafil, contrary to SNP, improved venous oxygen saturation, measured on venous blood gas analysis.
CONCLUSION:
Sildenafil and SNP are vasodilators that significantly reduce pulmonary hypertension and cardiac geometry, in addition to improving biventricular function. Sodium nitroprusside, contrary to SIL, was associated with systemic arterial hypotension and worsening of venous oxygen saturation.
Free Article
  PMID: 22898992 [PubMed - as supplied by publisher]
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9. Circ J. 2012 Apr 25;76(5):1245-52. Epub 2012 Feb 14.
Impact of first-line sildenafil monotreatment for pulmonary arterial hypertension.
Yanagisawa R, Kataoka M, Taguchi H, Kawakami T, Tamura Y, Fukuda K, Yoshino H, Satoh T.
Source
Second Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo 181-8611, Japan.
Comment in
•       How long can we leave patients with pulmonary arterial hypertension on oral drug monotreatment? [Circ J. 2012]
How long can we leave patients with pulmonary arterial hypertension on oral drug monotreatment?Ogawa A, Matsubara H. Circ J. 2012 Apr 25; 76(5):1089-90. Epub 2012 Mar 30.
Abstract
BACKGROUND:
Sildenafil has been demonstrated as effective for the treatment of pulmonary arterial hypertension (PAH). The purpose of this study was to investigate the occurrence of clinical events after sildenafil monotreatment as a first-line therapy in patients with PAH over a long-term observation period.
METHODS AND RESULTS:
Sildenafil was administered as a first-line drug to 46 patients with PAH (including 24 patients with idiopathic PAH) during 2003-2010. We investigated subsequent clinical events such as the addition of epoprostenol, hospitalization for right-side heart failure, and death. All the hemodynamic parameters and the 6-min walk distance improved significantly in the enrolled patients as a whole receiving sildenafil treatment; 15 (33%) of the 46 patients required the addition of epoprostenol during follow-up. Kaplan-Meier analysis demonstrated that more than 60% of the patients receiving first-line sildenafil treatment did not require the addition of epoprostenol for a 5-year period. Furthermore, the 5-year survival rate after first-line sildenafil treatment was 81%. Finally, more than 75% of the enrolled patients did not reach the composite endpoint of hospitalization for right-side heart failure and death for a 5-year period.
CONCLUSIONS:
This study describes the long-term outcome of patients with PAH receiving sildenafil monotreatment as a first-line therapy and suggests that it is a promising therapeutic strategy.
Free Article
  PMID: 22333215 [PubMed - indexed for MEDLINE]
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10. Curr Opin Cardiol. 2012 May;27(3):296-300.
Right heart failure after left ventricular assist device implantation: early and late.
MacGowan GA, Schueler S.
Source
Departments of Cardiology, Freeman Hospital, Newcastle upon Tyne, UK. guy.macgowan@nuth.nhs.uk
Abstract
PURPOSE OF REVIEW:
To review recent publications on right heart failure developing early and late after implantation of left ventricular assist devices (LVAD).
RECENT FINDINGS:
Whereas use of the newer continuous flow devices is associated with improved outcomes, right heart failure remains a considerable challenge. Recent studies have provided good evidence about predicting right heart failure early after LVADs, though how to predict right heart failure late after LVAD is still unclear as most studies in this area are small single centre experience studies. With respect to medical therapies for treating right heart failure, there is evidence for the use of both nitric oxide and the phosphodiesterase type 5 inhibitor sildenafil in carefully selected patients with secondary pulmonary hypertension who undergo implantation of LVADs.
SUMMARY:
Despite improved outcomes, right heart failure remains a significant challenge to selecting and managing patients with LVADs, though we do have clearer evidence now for predicting early right heart failure, and treating it in those patients with secondary pulmonary hypertension.
  PMID: 22327288 [PubMed - indexed for MEDLINE]
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11. Rheumatol Int. 2012 May;32(5):1437-41. Epub 2011 Mar 30.
Possible anti-metastatic effect of iloprost in a patient with systemic sclerosis with lung cancer: a case study.
Pehlivan Y, Turkbeyler IH, Balakan O, Sevinc A, Yilmaz M, Bakir K, Onat AM.
Source
School of Medicine, Department of Rheumatologyn, Sahinbey Medical Center, Gaziantep University, TR-27310, Gaziantep, Turkey. drpehlivan@hotmail.com
Abstract
Systemic sclerosis (SSc) is a chronic disease of unknown etiology which affects the vascular system and connective tissue. A wide series of studies showed an increased prevalence of cancer in patients with SSc than the normal population. Prostacyclin (PGI2) is an endogenously produced element that is basically synthesized by arachiodonic acid through prostacyclin synthesis in vascular system endothelial cells. Iloprost is a stable analogue of PGI2 which is used in the treatment of pulmonary arterial hypertension (PAH). In a limited number of animal models, the anti-metastatic activity of PGI2 is observed. Herein, we report iloprost treatment of a 60-year-old-woman with SSc, who lately developed PAH as a complication of her disease and lung adenocarcinoma as a co-incidence simultaneously. These two mortal complications were both treated successfully with inhaled iloprost until her death due to gastrointestinal complications of SSc.
  PMID: 21448647 [PubMed - indexed for MEDLINE]
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12. Eur Respir J. 2012 Aug 16. [Epub ahead of print]
Vascular and right ventricular remodeling in chronic thromboembolic pulmonary hypertension.
Delcroix M, Vonk-Noordegraaf A, Fadel E, Lang I, Simonneau G, Naeije R.
Source
University Hospitals of Leuven, Leuven, Belgium.
Abstract
In chronic thromboembolic pulmonary hypertension (CTEPH) increased pulmonary vascular resistance is caused by fibrotic organization of unresolved thromboemboli. CTEPH mainly differs from pulmonary arterial hypertension (PAH) by the proximal location of pulmonary artery obliteration, although distal arteriopathy can be observed as a consequence of non-occluded area overperfusion. Accordingly, there is proportionally more wave reflection in CTEPH, impacting on pressure and flow wave morphology. However, the time constant, that is resistance times compliance, is not different in CTEPH and PAH, indicating only trivial effects of proximal wave reflection on hydraulic right ventricular load. More discriminative is the analysis of the pressure decay after pulmonary arterial occlusion, which is more rapid in the absence of significant distal arteriopathy. Structure and function of the right ventricle show a similar pattern of right ventricular hypertrophy, namely dilatation and wall thickening as well as loss of function in CTEPH as in PAH. This is probably related to similar loading conditions. Hyperventilation with hypocapnia is characteristic of both PAH and CTEPH. Ventilatory equivalents for carbon dioxide, as a function of arterial PCO2, conform to the alveolar ventilation equation in both conditions, indicating a predominant role of increased chemosensitivity. However, a slight increase in the arterial to end-tidal PCO2 gradient in CTEPH shows a contribution of increased dead space ventilation.
  PMID: 22903956 [PubMed - as supplied by publisher]
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13. PLoS One. 2012;7(8):e43793. Epub 2012 Aug 20.
Angiostatic factors in the pulmonary endarterectomy material from chronic thromboembolic pulmonary hypertension patients cause endothelial dysfunction.
Zabini D, Nagaraj C, Stacher E, Lang IM, Nierlich P, Klepetko W, Heinemann A, Olschewski H, Bálint Z, Olschewski A.
Source
Experimental Anesthesiology, Department of Anesthesia and Intensive Care Medicine, Medical University of Graz, Graz, Austria.
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease with persistent thrombotic occlusion or stenosis of the large pulmonary arteries resulting in pulmonary hypertension. Surgical removal of the neointimal layer of these vessels together with the non-resolved thrombus consisting of organized collagen-rich fibrotic areas with partly recanalized regions is the treatment of choice (pulmonary endarterectomy, PEA). The present study investigates endothelial cells isolated from such material as well as factors present in the surgical PEA material, which may contribute to impairment of recanalization and thrombus non-resolution. We observed muscularized vessels and non-muscularized vessels in the PEA material. The isolated endothelial cells from the PEA material showed significantly different calcium homeostasis as compared to pulmonary artery endothelial cells (hPAECs) from normal controls. In the supernatant (ELISA) as well as on the tissue level (histochemical staining) of the PEA material, platelet factor 4 (PF4), collagen type I and interferon-gamma-inducible 10 kD protein (IP-10) were detected. CXCR3, the receptor for PF4 and IP-10, was particularly elevated in the distal parts of the PEA material as compared to human control lung (RT-PCR). PF4, collagen type I and IP-10 caused significant changes in calcium homeostasis and affected the cell proliferation, migration and vessel formation in hPAECs. The presence of angiostatic factors like PF4, collagen type I and IP-10, as recovered from the surgical PEA material from CTEPH patients, may lead to changes in calcium homeostasis and endothelial dysfunction.
Free PMC Article
  PMID: 22916307 [PubMed - in process]
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14. PLoS One. 2012;7(8):e43433. Epub 2012 Aug 17.
The Soluble Guanylate Cyclase Stimulator Riociguat Ameliorates Pulmonary Hypertension Induced by Hypoxia and SU5416 in Rats.
Lang M, Kojonazarov B, Tian X, Kalymbetov A, Weissmann N, Grimminger F, Kretschmer A, Stasch JP, Seeger W, Ghofrani HA, Schermuly RT.
Source
University of Giessen and Marburg Lung Center, Giessen, Germany.
Abstract
BACKGROUND:
The nitric oxide (NO)-soluble guanylate cyclase (sGC)-cyclic guanosine monophosphate (cGMP) signal-transduction pathway is impaired in many cardiovascular diseases, including pulmonary arterial hypertension (PAH). Riociguat (BAY 63-2521) is a stimulator of sGC that works both in synergy with and independently of NO to increase levels of cGMP. The aims of this study were to investigate the role of NO-sGC-cGMP signaling in a model of severe PAH and to evaluate the effects of sGC stimulation by riociguat and PDE5 inhibition by sildenafil on pulmonary hemodynamics and vascular remodeling in severe experimental PAH.
METHODS AND RESULTS:
Severe angioproliferative PAH was induced in rats by combined exposure to the vascular endothelial growth factor receptor antagonist SU5416 and hypoxia (SUHx). Twenty-one days thereafter rats were randomized to receive either riociguat (10 mg/kg/day), sildenafil (50 mg/kg/day) or vehicle by oral gavage, for 14 days until the day of the terminal hemodynamic measurements. Administration of riociguat or sildenafil significantly decreased right ventricular systolic pressure (RVSP). Riociguat significantly decreased RV hypertrophy (RVH) (0.55±0.02, p<0.05), increased cardiac output (60.8±.8 mL/minute, p<0.05) and decreased total pulmonary resistance (4.03±0.3 mmHg min(-1) ml(-1) 100 g BW, p<0.05), compared with sildenafil and vehicle. Both compounds significantly decreased the RV collagen content and improved RV function, but the effects of riociguat on tricuspid annular plane systolic excursion and RV myocardial performance were significantly better than those of sildenafil (p<0.05). The proportion of occluded arteries was significantly lower in animals receiving riociguat than in those receiving vehicle (p<0.05); furthermore, the neointima/media ratio was significantly lower in those receiving riociguat than in those receiving sildenafil or vehicle (p<0.05).
CONCLUSION:
Riociguat and sildenafil significantly reduced RVSP and RVH, and improved RV function compared with vehicle. Riociguat had a greater effect on hemodynamics and RVH than sildenafil.
PMCID: PMC3422306 Free PMC Article
  PMID: 22912874 [PubMed - in process]
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15. Circ Cardiovasc Imaging. 2012 Aug 8. [Epub ahead of print]
Prognostic Value of Right Ventricular Longitudinal Peak Systolic Strain in Patients with Pulmonary Hypertension.
Haeck ML, Scherptong RW, Ajmone Marsan N, Holman ER, Schalij MJ, Bax JJ, Vliegen HW, Delgado V.
Source
Leiden University Medical Center, Leiden, Netherlands.
Abstract
BACKGROUND:
-Right ventricular (RV) function is an important prognostic marker in patients with pulmonary hypertension. The present evaluation assessed the prognostic value of RV longitudinal peak systolic strain (LPSS) in patients with pulmonary hypertension.
METHODS AND RESULTS:
-A total of 150 patients with pulmonary hypertension of different etiologies (mean age 59±15 years, 37.3% male) were evaluated. RV fractional area change and tricuspid annular plane systolic excursion (TAPSE) index were evaluated with 2-dimensional echocardiography. RV LPSS was assessed with speckle tracking echocardiography. The patient population was categorized according to a RV LPSS value of -19%. Among several clinical and echocardiographic parameters, the significant determinants of all-cause mortality were evaluated. There were no significant differences in age, sex, pulmonary hypertension etiology and left ventricular ejection fraction between patients with RV LPSS <-19% and patients with RV LPSS ≥-19%. However, patients with RV LPSS ≥-19% had significantly worse NYHA functional class (2.7±0.6 vs. 2.3±0.8, p=0.003) and lower TAPSE (16±4 mm vs. 18±3 mm, p<0.001) than their counterparts. During a median follow-up of 2.6 years, 37 patients died. RV LPSS was a significant determinant of all-cause mortality (HR 3.40, 95% CI 1.19-9.72, p=0.02).
CONCLUSIONS:
-In patients with pulmonary hypertension, RV LPSS is significantly associated with all-cause mortality. RV LPSS may be a valuable parameter for risk stratification of these patients. Future studies are needed to confirm these results in the pulmonary hypertension subgroups.
  PMID: 22875884 [PubMed - as supplied by publisher]
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16. Hum Exp Toxicol. 2012 Aug 7. [Epub ahead of print]
Tadalafil in pulmonary hypertension: May be more than seen?
Karasu-Minareci E.
Source
Department of Pharmacology, Faculty of Medicine, Akdeniz University, Antalya, Turkey.
  PMID: 22872608 [PubMed - as supplied by publisher]
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17. Curr Heart Fail Rep. 2012 Aug 3. [Epub ahead of print]
Right Ventricular Pulmonary Hypertension.
Guazzi M, Castelvecchio S, Bandera F, Menicanti L.
Source
Heart Failure Unit, I.R.C.C.S., Policlinico San Donato, University of Milan, Milan, Italy, marco.guazzi@unimi.it.
Abstract
In heart failure (HF) syndrome, the development of pulmonary hypertension (PH), right ventricular (RV) dysfunction and failure are ominous prognostic signs. Pathophysiology, clinical interest and targeted therapeutic approaches for left-sided PH and its consequences on RV function have been traditionally confined to advanced HF stages. Community- and population-based studies have clearly indicated that PH is frequent even in HF patients with preserved ejection fraction, and may carry important prognostic implications in normal ageing as well. HF guidelines are inconclusive on both preventive and curative strategies for left-sided PH and its consequences on RV function. The search for new therapeutic opportunities targeted on pulmonary vascular and right heart remodeling are an important challenge for the future.
  PMID: 22864855 [PubMed - as supplied by publisher]
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18. Expert Opin Drug Saf. 2012 Aug 4. [Epub ahead of print]
Safety and efficacy evaluation of ambrisentan in pulmonary hypertension.
Vizza CD, Fedele F, Pezzuto B, Rubin LJ.
Source
"Sapienza" University of Rome, Department of Cardiovascular and Respiratory Disease , Rome , Italy.
Abstract
Introduction: Pulmonary arterial hypertension (PAH) is characterized by an increase in pulmonary vascular resistance, which can lead to right heart failure and death. Endothelin-1 binding ETA and ETB receptors seem to play a critical role in the pathogenesis and progression of the disease, and oral endothelin receptor antagonists (ERAs) have been shown to be an effective treatment. Bosentan and ETA-selective ambrisentan are the ERAs currently available for PAH treatment. Areas covered: On the basis of the analysis of the literature, this paper addresses the efficacy and safety of ambrisentan in the treatment for PAH. Expert opinion: Ambrisentan has shown an efficacy comparable with other ERAs. Compared with bosentan, ambrisentan seems to have a better safety profile with regards to hepatic safety and drug-drug interactions. On the other hand, ambrisentan shows a higher rate of other adverse events, such as nasal congestion and peripheral edema. Ambrisentan is a viable option for PAH treatment. However, there is still a need for more robust data about long-term mortality, treatment in non-PAH pulmonary hypertension (PH) (such as PH due to left heart disease and PH due to chronic hypoxic lung diseases) and combination therapy.
  PMID: 22861496 [PubMed - as supplied by publisher]
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19. Clin Transplant. 2012 Aug 2. doi: 10.1111/j.1399-0012.2012.01689.x. [Epub ahead of print]
Bosentan in heart transplantation candidates with severe pulmonary hypertension: efficacy, safety and outcome after transplantation.
Perez-Villa F, Farrero M, Cardona M, Castel MA, Tatjer I, Penela D, Vallejos I.
Source
Heart Failure and Heart Transplantation Unit, Hospital Clinic, IDIBAPS, Barcelona, Spain.
Abstract
BACKGROUND:
Increased pulmonary vascular resistance (PVR) is associated with increased right ventricular failure and mortality after heart transplantation.
METHODS:
In this prospective study, 22 patients considered high-risk candidates for heart transplantation because of severe pulmonary hypertension (PVR = 6 ± 2 Wood units; transpulmonary gradient 22 ± 7 mmHg), received bosentan 125 mg bid. Right heart catheterization was repeated after four months (n = 22) and 12 months (n = 9). Eleven patients who declined participation in the study were considered as control group.
RESULTS:
After four months, PVR decreased by 38% in patients receiving bosentan (n = 22), while it increased by 25% in the control group (p = 0.001). Those patients who received bosentan for 12 months (n = 9), experienced a 60% reduction in PVR compared to baseline (p = 0.003). Only three patients (14%) had no hemodynamic improvement with bosentan. After bosentan therapy, 14 patients (64%) underwent heart transplantation. Patients with high PVR who received bosentan showed a trend toward better one-yr survival after transplantation than patients with PVR ≤ 2.5 Wood units transplanted in the same period of time (93% vs. 83%).
CONCLUSIONS:
In patients considered high-risk candidates for heart transplantation because of high PVR, therapy with bosentan is associated with a significant reduction in PVR and a good outcome after transplantation.
© 2012 John Wiley & Sons A/S.
  PMID: 22861120 [PubMed - as supplied by publisher]
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20. J Vet Intern Med. 2012 Aug 3. doi: 10.1111/j.1939-1676.2012.00981.x. [Epub ahead of print]
Tricuspid Annular Plane Systolic Excursion (TAPSE) in Dogs: Reference Values and Impact of Pulmonary Hypertension.
Pariaut R, Saelinger C, Strickland KN, Beaufrère H, Reynolds CA, Vila J.
Source
Department of Veterinary Clinical Sciences, LSU School of Veterinary Medicine, Baton Rouge, LA.
Abstract
BACKGROUND:
The impact of pulmonary hypertension (PH) on right ventricular systolic function is difficult to assess. Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement of right ventricular systolic function and a strong predictor of outcome in human PH patients.
HYPOTHESIS/OBJECTIVES:
Determine a reference range for TAPSE in healthy dogs, and quantify TAPSE in dogs with PH. It is hypothesized that TAPSE is lower in dogs with PH compared with a reference group, and decreases as PH worsens.
ANIMALS:
Fifty normal dogs and 30 dogs with PH.
METHODS:
TAPSE was measured by 2-dimensional echocardiography-guided M-mode from the left apical 4-chamber view. Peak systolic tricuspid valve regurgitation jet velocity was measured by continuous-wave Doppler to estimate right ventricular-to-right atrial pressure gradient. PH was subjectively classified as mild, moderate, and severe.
RESULTS:
There was a curvilinear correlation between TAPSE and body weight. The upper and lower limits of the 95% reference interval were determined by quantile regression. Interobserver and intraobserver agreement was adequate with a coefficient of variation <10%. There were significant differences when comparing dogs with PH and the healthy group, as well as between the PH subgroups (P < .01), except between dogs with mild and moderate PH (P = .99). Only dogs in the severe PH group had TAPSE values that were mostly below the lower limit of the reference interval.
CONCLUSIONS AND CLINICAL IMPORTANCE:
TAPSE is easily obtainable with acceptable inter and intraobserver agreement. TAPSE is decreased in PH and below the reference interval in most dogs with severe PH.
Copyright © 2012 by the American College of Veterinary Internal Medicine.
  PMID: 22860631 [PubMed - as supplied by publisher]
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21. Int J Med Sci. 2012;9(6):406-12. Epub 2012 Jul 21.
Frequency of Pulmonary Hypertension in Patients with COPD due to Biomass Smoke and Tobacco Smoke.
Sertogullarindan B, Gumrukcuoglu HA, Sezgi C, Akil MA.
Source
1. Department of Pulmonary and Critical Care, Yuzuncu Yil University Medical Faculty, Turkey;
Abstract
Objectives; Pulmonary hypertension (PH) is a common and well established complication of chronic obstructive pulmonary disease (COPD). Its presence is associated with decreased survival. This study was designed to investigate the PH frequency and its relations in hospitalized tobacco and biomass related COPD patients. Methods and Results; The study was a retrospective review of inpatients with COPD defined as a history of tobacco or biomass smoking, Pulmonary function tests (PFT) within stable status, an echocardiogram within stable status. PH was defined as systolic pulmonary artery pressure (sPAP) >35 mmHg. Of the 694 individuals, 600 had suitable aspects for inclusion of study. All Females were biomass exposer and males were tobacco smoker. The Prevalence of PH was found more frequent in females than males. It was more prominent in moderate level COPD cases (56,2% and 37,5%, P<0,002). Both groups had airflow limitation, hypercapnia and hypoxemia, but no differences were found in terms of PaCO(2) and PaO2. However, FEV1 % was lower in males than females (p<0,005). On the other hand, FVC % was lower in the females compared with the males (p < 0.02). When analyzing the influence of PFT and demographic parameters on PH in separate COPD level groups, the results a bit varied among the groups. Conclusion; Our study demonstrated that PH frequency is higher in female COPD cases due to biomass smoke than in male COPD cases due to tobacco smoke. The influence of FVC % on the risk of a person having PH increased with increasing COPD level.
PMCID: PMC3410359 Free PMC Article
  PMID: 22859900 [PubMed - in process]
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22. Joint Bone Spine. 2012 Mar;79(2):205-6. Epub 2012 Jan 28.
Pulmonary arterial hypertension during systemic lupus.
Cinquetti G, Sordet C, Chatelus E, Ronde-Oustau C, Javier RM, Gottenberg JE, Sibilia J.
  PMID: 22284612 [PubMed - indexed for MEDLINE]
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23. Lung. 2012 Apr;190(2):155-60. Epub 2011 Dec 3.
Pulmonary arterial hypertension in the setting of pregnancy: a case series and standard treatment approach.
Smith JS, Mueller J, Daniels CJ.
Source
Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Louisville Medical Center, Louisville, KY, USA. JShaun.Smith@louisville.edu
Abstract
Pregnancy in patients with pulmonary arterial hypertension (PAH) is associated with a maternal mortality of 30-50% despite modern treatment modalities. The majority of maternal deaths in PAH patients occur either during labor and delivery or within 1 month postpartum. Cardiovascular collapse is attributed to a mismatch between the physiologic limitations of PAH and the changes that occur with pregnancy and delivery. In the Unites States, there is no consensus on the management of PAH in pregnancy. Several case reports have been published describing improved maternal-fetal outcomes, likely due to new advanced PH therapies, earlier diagnosis of PAH, and an adoption of a multidisciplinary treatment approach. We present five cases of gravid PAH patients successfully managed at our institution with a description of our standardized multidisciplinary treatment approach.
  PMID: 22139549 [PubMed - indexed for MEDLINE]
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24. J Clin Pharmacol. 2012 Mar;52(3):425-31. Epub 2011 Mar 17.
Oral sildenafil therapy for Chinese patients with pulmonary arterial hypertension: a multicenter study.
Xiong CM, Lu XL, Shan GL, Wu BX, Zhu XY, Wu GH, Zeng XF, Guo T, Liu ZH, Ni XH, Cheng XS, Gu Q, Zhao ZH, Tian HY, Li WM, Zhang DZ, Zhang C, Li MT, Liu HM, Guo YJ, Shen JY, Zhang WJ, Liu S, Zhou DX, Bao CD, Huang SA, Chen JY, Wu WF, Huang K, Li CL, Wang LH, He B, He JG, Li JJ.
Source
Center for Diagnosis and Management of Pulmonary Vascular Diseases, Department of Cardiology, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Abstract
The impact of sildenafil on pulmonary arterial hypertension (PAH) in Chinese patients has been less investigated. A prospective, open-label, uncontrolled and multicenter study, therefore, was carried out to address this issue. Ninety patients with multicause-induced PAH received oral sildenafil (75 mg/day) for 12 weeks. The 6-minute walk test (SMWT) and cardiac catheterization were performed at the beginning and the end of the 12 weeks. The primary endpoint was the changes in exercise capacity assessed by the SMWT; the secondary endpoint included assessment of functional class, evaluation of cardiopulmonary hemodynamics, and clinical worsening. Drug safety and tolerability were also examined. The results showed that there was a significant improvement in SMWT distances (342 ± 93 m vs 403 ± 88 m, P < .0001), Borg dyspnea score (2.9 ± 2.6 vs 2.4 ± 2.0, P = .0046), World Health Organization functional class, and cardiopulmonary hemodynamics (mean pulmonary artery pressure, P < .0001; cardiac index, P < .0001; pulmonary vascular resistance, P < .0001) after 12 weeks of oral sidenafil therapy. Almost all enrolled patients did not experience significant clinical worsening. This study confirms and extends the findings of previous studies relating to effects of sildenafil on PAH, suggesting that oral sildenafil is safe and effective for the treatment of adult patients with PAH in the Chinese population.
  PMID: 21415281 [PubMed - indexed for MEDLINE]
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25. Eur Respir J. 2012 Apr;39(4):927-38. Epub 2011 Aug 4.
Bosentan inhibits cigarette smoke-induced endothelin receptor expression in pulmonary arteries.
Milara J, Gabarda E, Juan G, Ortiz JL, Guijarro R, Martorell M, Morcillo EJ, Cortijo J.
Source
Clinic Research Unit, University General Hospital Consortium, Valencia, Spain. xmilara@hotmail.com
Abstract
The endothelin (ET) system contributes to lung vascular tension and remodelling in smokers and chronic obstructive pulmonary disease (COPD) patients. This study examined the effect of cigarette smoke (CS) on ET receptor A (ET(A)) and B (ET(B)) expression in human pulmonary artery smooth muscle cells (HPASMCs) and human small intrapulmonary arteries, as well as their functional consequences. CS extract (CSE) increased ET(A) and ET(B) expression in HPASMCs and small intrapulmonary arteries, which was attenuated by bosentan, the ET(A) antagonist BQ123 and the ET(B) antagonist BQ788, and by blocking ET-1 with a monoclonal antibody against ET-1, suggesting a feed-forward mechanism mediated by ET-1 release. ET receptor (ETR) antagonism attenuated the CSE-induced HPASMC proliferation. Furthermore, CSE exposure increased the acute ET-1-induced small intrapulmonary artery contraction, which was attenuated by bosentan, BQ123 and BQ788. Pulmonary arteries from smokers and COPD patients showed a higher expression of ET(A) and ET(B) than those of nonsmoker patients. These results show a novel mechanism by which ETR blockade attenuates CS-induced ETR overexpression and, subsequently, small intrapulmonary artery tension. These data may be of potential value to explain therapeutic effects of bosentan in some forms of disproportionate pulmonary hypertension in COPD patients.
  PMID: 21828025 [PubMed - indexed for MEDLINE]
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26. J Am Coll Cardiol. 2012 Jun 12;59(24):2193-202.
Electrophysiologic remodeling of the left ventricle in pressure overload-induced right ventricular failure.
Hardziyenka M, Campian ME, Verkerk AO, Surie S, van Ginneken AC, Hakim S, Linnenbank AC, de Bruin-Bon HA, Beekman L, van der Plas MN, Remme CA, van Veen TA, Bresser P, de Bakker JM, Tan HL.
Source
Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Meibergdreef 9, Amsterdam, the Netherlands.
Comment in
•       Pulmonary hypertension: a stage for ventricular interdependence? [J Am Coll Cardiol. 2012]
Pulmonary hypertension: a stage for ventricular interdependence?Hsia HH, Haddad F. J Am Coll Cardiol. 2012 Jun 12; 59(24):2203-5.
Abstract
OBJECTIVES:
The purpose of this study was to analyze the electrophysiologic remodeling of the atrophic left ventricle (LV) in right ventricular (RV) failure (RVF) after RV pressure overload.
BACKGROUND:
The LV in pressure-induced RVF develops dysfunction, reduction in mass, and altered gene expression, due to atrophic remodeling. LV atrophy is associated with electrophysiologic remodeling.
METHODS:
We conducted epicardial mapping in Langendorff-perfused hearts, patch-clamp studies, gene expression studies, and protein level studies of the LV in rats with pressure-induced RVF (monocrotaline [MCT] injection, n = 25; controls with saline injection, n = 18). We also performed epicardial mapping of the LV in patients with RVF after chronic thromboembolic pulmonary hypertension (CTEPH) (RVF, n = 10; no RVF, n = 16).
RESULTS:
The LV of rats with MCT-induced RVF exhibited electrophysiologic remodeling: longer action potentials (APs) at 90% repolarization and effective refractory periods (ERPs) (60 ± 1 ms vs. 44 ± 1 ms; p < 0.001), and slower longitudinal conduction velocity (62 ± 2 cm/s vs. 70 ± 1 cm/s; p = 0.003). AP/ERP prolongation agreed with reduced Kcnip2 expression, which encodes the repolarizing potassium channel subunit KChIP2 (0.07 ± 0.01 vs. 0.11 ± 0.02; p < 0.05). Conduction slowing was not explained by impaired impulse formation, as AP maximum upstroke velocity, whole-cell sodium current magnitude/properties, and mRNA levels of Scn5a were unaltered. Instead, impulse transmission in RVF was hampered by reduction in cell length (111.6 ± 0.7 μm vs. 122.0 ± 0.4 μm; p = 0.02) and width (21.9 ± 0.2 μm vs. 25.3 ± 0.3 μm; p = 0.002), and impaired cell-to-cell impulse transmission (24% reduction in Connexin-43 levels). The LV of patients with CTEPH with RVF also exhibited ERP prolongation (306 ± 8 ms vs. 268 ± 5 ms; p = 0.001) and conduction slowing (53 ± 3 cm/s vs. 64 ± 3 cm/s; p = 0.005).
CONCLUSIONS:
Pressure-induced RVF is associated with electrophysiologic remodeling of the atrophic LV.
Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
  PMID: 22676940 [PubMed - indexed for MEDLINE]
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27. Hepatobiliary Pancreat Dis Int. 2012 Aug;11(4):434-7.
Terlipressin improves pulmonary pressures in cirrhotic patients with pulmonary hypertension and variceal bleeding or hepatorenal syndrome.
Kalambokis GN, Pappas K, Tsianos EV.
Source
1st Division of Internal Medicine and Hepato-Gastroenterology Unit, University Hospital of Ioannina, Ioannina 45500, Greece. etsianos@uoi.gr.
Abstract
Terlipressin has been shown to improve both pulmonary and systemic hemodynamics in stable cirrhotic patients with pulmonary hypertension, whereas other vasoconstrictors may cause pulmonary pressures to deteriorate. We investigated the pulmonary and systemic hemodynamic effects of the first terlipressin dose (2 mg) in 7 cirrhotic patients with PH presenting with variceal bleeding (n=4) or hepatorenal syndrome (n=3). Terlipressin decreased pulmonary vascular resistance (158.8+/-8.9 vs 186.5+/-13.9 dynes · sec · cm-5; P=0.003) together with an increase in systemic vascular resistance (2143+/-126 vs 1643+/-126 dynes · sec · cm-5; P<0.001). Terlipressin should be the vasoconstrictor treatment of choice when patients present with variceal bleeding or HRS.
  PMID: 22893473 [PubMed - in process]
   
 
28. Can Respir J. 2012 Jul;19(4):261-6.
Thermodilution and Fick cardiac outputs differ: Impact on pulmonary hypertension evaluation.
Fares WH, Blanchard SK, Stouffer GA, Chang PP, Rosamond WD, Ford HJ, Aris RM.
Abstract
BACKGROUND:
The relationship between thermodilution and indirect Fick cardiac output determination methods has not been well described.
OBJECTIVE:
To describe the relationship between these two cardiac output determination methods in patients evaluated for pulmonary hypertension and to highlight potential clinical implications.
METHODS:
A retrospective review of charts of all adult patients who underwent a right heart catheterization (RHC) between January 1, 2007 and November 10, 2010, and participated in the pulmonary hypertension program of the pulmonary division at an academic institution was conducted. For validation, the charts of all patients who underwent RHC during the same period within the cardiology division were reviewed.
RESULTS:
A total of 198 patients underwent 213 RHCs, 79 (40%) of whom had pulmonary arterial hypertension, were included. Forty-three per cent of patients had >20% difference between thermodilution and Fick. The average difference (thermodilution - Fick ±SD) was -0.39±2.03 L⁄min (n=213; P=0.006). There was no significant difference in bias or variability between thermodilution and Fick among patients with tricuspid regurgitant jet velocity (TRJ) of <3 m⁄s versus those with TRJ >3 m⁄s (-0.41±2.10 L⁄min versus -0.36±1.93 L⁄min, respectively; P=0.87). In a multivariable analysis, the thermodilution-Fick difference increased with age (P=0.001).
DISCUSSION:
The presence of such discrepancy in 36% of patients evaluated for heart failure and⁄or heart transplant validated the results. In total, 37% of the 1315 procedures (213 performed by pulmonologists and 1102 performed by cardiologists) had a difference of >20% between thermodilution and Fick.
CONCLUSION:
Significant discrepancy exists between thermodilution and indirect Fick methods. This discrepancy potentially impacts pulmonary arterial hypertension prognostication and diagnosis, and is independent of TRJ.
  PMID: 22891186 [PubMed - in process]
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29. Respiration. 2012 Aug 9. [Epub ahead of print]
Out-of-Proportion Pulmonary Hypertension and Heart Failure with Preserved Ejection Function.
Adir Y, Humbert M, Sitbon O, Wolf R, Lador F, Jaïs X, Simonneau G, Amir O.
Source
Pulmonary Division, Lady Davis Carmel Medical Center, Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Abstract
Backgrond: A subset of patients with heart failure with preserved ejection fraction (HFpEF) will have a marked increase in pulmonary artery pressure (PAP). Objective: To evaluate the clinical and hemodynamic characteristics of these patients in comparison to patients with idiopathic pulmonary arterial hypertension (IPAH). Methods: We reviewed the clinical and hemodynamic data of patients with HFpEF with out-of-proportion pulmonary hypertension (HFpEF-PH) and compared it to the corresponding data of age-matched patients with IPAH. Results: Twenty consecutive patients with HFpEF-PH and 20 patients with IPAH were included in the study. The mean age (±SD) was 71.3 ± 7.8 and 70.2 ± 6.7 years, respectively. The majority of the HFpEF-PH patients were postmenopausal females with at least two features of the metabolic syndrome and atrial fibrillation. Although HFpEF-PH patients fulfilled the criteria for out-of-proportion PH, with transpulmonary gradient (TPG) >12 mm Hg, the difference between the diastolic PAP and the pulmonary capillary wedge pressure (PCWP) was significantly lower compared to IPAH (6.3 ± 6.2 vs. 27.5 ± 4.8, p < 0.00001). Conclusions: Our results suggest that a diagnosis of HFpEF-PH should be suspected when severe PH occurs in an elderly postmenopausal female with one or more features of the metabolic syndrome and atrial fibrillation. Interestingly, these patients had significantly lower differences between diastolic PAP and PCWP, suggesting that the increase in TPG is mainly caused by an elevated systolic PAP, possibly as a result of increased pulmonary vascular stiffness, and not pulmonary vascular remodeling.
Copyright © 2012 S. Karger AG, Basel.
  PMID: 22890046 [PubMed - as supplied by publisher]
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30. Exp Lung Res. 2012 Sep;38(7):333-43.
Atorvastatin prevents dehydromonocrotaline-induced pulmonary hypertension in beagles.
Chen D, Zhou D, Qian J, Chen F, Guan L, Dong L, Ge J.
Source
Department of Cardiology, Zhongshan Hospital of Fudan University, Shanghai Cardiovascular Disease Institute, Shanghai, China.
Abstract
ABSTRACT Background: Pulmonary arterial hypertension is a life-threatening disease characterized by marked and sustained elevation of blood pressure in the lungs. Statins, 3-hydroxy-3-methylglutaryl CoA (HMG-CoA) reductase inhibitors, have been shown to attenuate the effects of pulmonary hypertension resulting from hypoxia, Monocrotaline exposure, or Monocrotaline exposure in the setting of pneumonectomy. In particular, the effects of Simvastatin have been well studied. Whether other statins, such as Atorvastatin, are capable of preventing dehydromonocrotaline-induced pulmonary hypertension in beagles has not been explored. Methods: We used eighteen 3-month-old beagles of both genders, weighing 10.3 ± 3.2 kg. The experimental animals were randomized into one of 3 groups: the control group (n = 6), the dehydromonocrotaline (DHMC) + vehicle group (n = 5), and the DHMC + Atorvastatin group (n = 7). The beagles were injected with DHMC (n = 12) on day 1, and from day 5 to day 65 they received Atorvastatin (2 mg/kg, daily by gavage) or vehicle (0.9% saline, daily by gavage) treatment. We used the thermodilution method of hemodynamic measurements at baseline and at day 65 of treatment. At day 65, pulmonary tissue was sampled for morphometry and real-time quantitative PCR. Results: After 65 days, DHMC increased mean pulmonary arterial pressure (mPAP), and this increase was prevented with Atorvastatin treatment (32 ± 11 mmHg vs. 15 ± 3 mmHg, P < .05). Hematoxylin and eosin staining demonstrated less pulmonary endothelium destruction and smooth muscle cell proliferation in the Atorvastatin-treated beagles, compared with the DHMC group. The eNOS mRNA expression was increased in the DHMC group, and this increase was prevented in the Atorvastatin-treated group. In addition, IL-1β, prepro-ET-1, TNF-α, and VEGF (vascular endothelial growth factor) mRNA expression levels were increased in the lungs of the DHMC group, and these increases were reduced toward normal levels in the Atorvastatin-treated group. Conclusion: Atorvastatin prevents the effects of monocrotaline-induced pulmonary hypertension in beagles.
  PMID: 22888849 [PubMed - in process]
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31. Eur Radiol. 2012 Aug 12. [Epub ahead of print]
Magnetic resonance imaging to assess the effect of exercise training on pulmonary perfusion and blood flow in patients with pulmonary hypertension.
Ley S, Fink C, Risse F, Ehlken N, Fischer C, Ley-Zaporozhan J, Kauczor HU, Klose H, Gruenig E.
Source
Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 430, 69120, Heidelberg, Germany, ley@gmx.de.
Abstract
OBJECTIVES:
To evaluate whether careful exercise training improves pulmonary perfusion and blood flow in patients with pulmonary hypertension (PH), as assessed by magnetic resonance imaging (MR).
METHODS:
Twenty patients with pulmonary arterial hypertension or inoperable chronic thromboembolic PH on stable medication were randomly assigned to control (n = 10) or training groups (n = 10). Training group patients received in-hospital exercise training; patients of the sedentary control group received conventional rehabilitation. Medication remained unchanged during the study period. Changes of 6-min walking distance (6MWD), MR pulmonary flow (peak velocity) and MR perfusion (pulmonary blood volume) were assessed from baseline to week 3.
RESULTS:
After 3 weeks of training, increases in mean 6MWD (P = 0.004) and mean MR flow peak velocity (P = 0.012) were significantly greater in the training group. Training group patients had significantly improved 6MWD (P = 0.008), MR flow (peak velocity -9.7 ± 8.6 cm/s, P = 0.007) and MR perfusion (pulmonary blood volume +2.2 ± 2.7 mL/100 mL, P = 0.017), whereas the control group showed no significant changes.
CONCLUSION:
The study indicates that respiratory and physical exercise may improve pulmonary perfusion in patients with PH. Measurement of MR parameters of pulmonary perfusion might be an interesting new method to assess therapy effects in PH. The results of this initial study should be confirmed in a larger study group. KEY POINTS : • Quantification of magnetic resonance perfusion is feasible in patients with pulmonary hypertension. • Quantified magnetic resonance perfusion may become useful for non-invasive monitoring of treatment. • Quantification of lung perfusion allows new insights into lung (patho-)physiology of PH. • Careful exercise training improves pulmonary perfusion and blood flow in patients with PH.
  PMID: 22886553 [PubMed - as supplied by publisher]
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32. Blood Coagul Fibrinolysis. 2012 Aug 8. [Epub ahead of print]
Soluble CD163 in young sickle cell disease patients and their trait siblings: a biomarker for pulmonary hypertension and vaso-occlusive complications.
Tantawy AA, Adly AA, Ismail EA.
Source
aPediatrics Department bClinical Pathology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Abstract
CD163 is expressed on cells of monocyte-macrophage lineage and is the main hemoglobin-haptoglobin receptor. Inflammation and monocyte activation are predisposing factors to vaso-occlusion and pulmonary hypertension, which are serious complications in sickle cell disease (SCD). Siblings of SCD patients may have the same pathophysiology without displaying symptoms. We assessed soluble CD163 (sCD163) levels in 60 children with SCD and 30 sickle cell trait (SCT) siblings compared with 30 healthy controls as a potential marker for disease severity and treatment response. Patients were studied stressing on the presence of pulmonary hypertension by Dopplar-Echocardiography, sickling crisis, transfusion requirements, hydroxyurea response, hematological profile, high sensitivity C-reactive protein (hs-CRP) and serum sCD163. sCD163 was significantly elevated in SCD patients and SCT siblings compared with controls and the highest levels were in untreated SCD patients (P < 0.001). sCD163 was higher in patients with pulmonary hypertension, acute chest syndrome or stroke as well as in patients who developed sickling crisis during the study period (P < 0.05). Hydroxyurea-treated patients had lower sCD163 compared with untreated patients (P < 0.001). sCD163 was positively correlated to leukocyte count, HbS, hs-CRP, pulmonary artery pressure and tricuspid regurgitant velocity whereas inversely correlated to hemoglobin and HbF levels. The cut-off value of sCD163 at 1400 ng/ml could be considered a predictor for vaso-occlusive crisis in SCD with a sensitivity of 92.3% and specificity of 94.1%. sCD163 can be considered a biomarker for pulmonary hypertension, early crisis prediction and monitoring hydroxyurea response in SCD patients. Elevated sCD163 in trait siblings could reflect increased risk of sickling in challenging situations.
  PMID: 22885767 [PubMed - as supplied by publisher]
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33. Heart Lung Circ. 2012 Aug 7. [Epub ahead of print]
Pulmonary Artery Haemodynamic Properties in Patients with Pulmonary Hypertension Secondary to Rheumatic Mitral Stenosis.
Yan T, Zhang GX, Li BL, Zhong K, Xu ZY, Han L.
Source
Department of Cardiothoracic Surgery, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.
Abstract
We sought to explore the pulmonary haemodynamic changes in rheumatic mitral stenosis patients with secondary pulmonary hypertension. The pulmonary artery resistance and compliance of 35 patients with rheumatic mitral stenosis and 12 controls without cardiopulmonary vascular disease were evaluated by using an improved method, which is based on making calculations with parameters obtained from right heart catheterisation. The results are as follows: (1) pulmonary artery compliance in patients with secondary pulmonary hypertension was significantly lower than that of the control group (P<0.01); (2) linear correlation analyses showed that preoperative mean pulmonary artery pressure (mPAP) closely correlated with zero-pressure compliance in the mitral stenosis group (r=-0.745, P<0.05); (3) PAP and pulmonary vascular resistance decreased significantly in both groups with mitral stenosis after infusing 0.5μgkg(-1)min(-1) of sodium nitroprusside (P<0.01). The pulmonary zero pressure compliance and mean pressure compliance increased significantly in the group with mild pulmonary hypertension; whereas in the severe group, the mean compliance changed with significance as the mPAP decreased (1.51±0.59 vs 1.81±0.77ml/mmHg), however no significant change occurred in the pulmonary zero pressure compliance (2.35±1.24ml/mmHg vs. 2.24±1.53ml/mmHg, P>0.05) The walls of pulmonary artery vessels in patients with pulmonary hypertension secondary to rheumatic mitral stenosis appeared to be remodelled by varying degrees as indicated by their haemodynamic properties. Structural remodelling may be a factor affecting preoperative pulmonary artery pressure. Mitral stenosis patients with severe pulmonary hypertension have significantly lower responses to sodium nitroprusside possibly due to aggradation and deposition of collagen in the artery walls, decreasing constriction and dilation, or atrophy of smooth muscle cells.
Copyright © 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
  PMID: 22883627 [PubMed - as supplied by publisher]
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34. Aviat Space Environ Med. 2012 Jul;83(7):673-6.
Pulmonary artery pressure increases during commercial air travel in healthy passengers.
Smith TG, Talbot NP, Chang RW, Wilkinson E, Nickol AH, Newman DG, Robbins PA, Dorrington KL.
Source
Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford OX1 3PT, UK. thomas.smith@dpag.ox.ac.uk
Abstract
BACKGROUND: It is not known whether the mild hypoxia experienced by passengers during commercial air travel triggers hypoxic pulmonary vasoconstriction and increases pulmonary artery pressure in flight. Insidious pulmonary hypertensive responses could endanger susceptible passengers who have cardiopulmonary disease or increased hypoxic pulmonary vascular sensitivity. Understanding these effects may improve pre-flight assessment of fitness-to-fly and reduce in-flight morbidity and mortality. Methods: Eight healthy volunteers were studied during a scheduled commercial airline flight from London, UK, to Denver, CO. The aircraft was a Boeing 777 and the duration of the flight was 9 h. Systolic pulmonary artery pressure (sPAP) was assessed by portable Doppler echocardiography during the flight and over the following week in Denver, where the altitude (5280 ft/1610 m) simulates a commercial airliner environment. Results: Cruising cabin altitude ranged between 5840 and 7170 ft (1780 to 2185 m), and mean arterial oxygen saturation was 95 +/- 0.6% during the flight. Mean sPAP increased significantly in flight by 6 +/- 1 mmHg to 33 +/- 1 mmHg, an increase of approximately 20%. After landing in Denver, sPAP was still 3 +/- 1 mmHg higher than baseline and remained elevated at 30 +/- 1 mmHg for a further 12 h. Conclusions: Pulmonary artery pressure increases during commercial air travel in healthy passengers, raising the possibility that hypoxic pulmonary hypertension could develop in susceptible individuals. A hypoxia altitude simulation test with simultaneous echocardiography ('HAST-echo') may be beneficial in assessing fitness to fly in vulnerable patients.
  PMID: 22779310 [PubMed - indexed for MEDLINE]
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35. Radiology. 2012 Jun;263(3):678-87. Epub 2012 Apr 16.
Pulmonary arterial hypertension: MR imaging-derived first-pass bolus kinetic parameters are biomarkers for pulmonary hemodynamics, cardiac function, and ventricular remodeling.
Skrok J, Shehata ML, Mathai S, Girgis RE, Zaiman A, Mudd JO, Boyce D, Lechtzin N, Lima JA, Bluemke DA, Hassoun PM, Vogel-Claussen J.
Source
Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Nelson Basement MRI 110, 600 N Wolfe St, Baltimore, MD 21287, USA.
Abstract
PURPOSE:
To prospectively compare contrast material-enhanced (CE) magnetic resonance (MR) imaging-derived right-to-left ventricle pulmonary transit time (PTT), left ventricular (LV) full width at half maximum (FWHM), and LV time to peak (TTP) between patients with pulmonary arterial hypertension (PAH) and healthy volunteers and to correlate these measurements with survival markers in patients with PAH.
MATERIALS AND METHODS:
This HIPAA-compliant study received institutional review board approval. Written informed consent was obtained from all participants. Forty-three patients (32 with PAH [29 women; median age, 55.4 years], 11 with scleroderma but not PAH [seven women; median age, 58.9 years]) underwent right-sided heart catheterization and 3-T CE cardiac MR imaging. Eighteen age- and sex-matched healthy control subjects (12 women; median age, 51.7 years) underwent only CE MR imaging. A short-axis saturation-recovery gradient-echo section was acquired in the basal third of both ventricles, and right-to-left-ventricle PTT, LV FWHM, and LV TTP were calculated. Statistical analysis included Kruskal-Wallis test, Wilcoxon rank sum test, Spearman correlation coefficient, multiple linear regression analysis, and Lin correlation coefficient analysis.
RESULTS:
Patients had significantly longer PTT (median, 8.2 seconds; 25th-75th percentile, 6.9-9.9 seconds), FWHM (median, 8.2 seconds; 25th-75th percentile, 5.7-11.4 seconds), and TTP (median, 4.8 seconds; 25th-75th percentile, 3.9-6.5 seconds) than did control subjects (median, 6.4 seconds; 25th-75th percentile, 5.7-7.1 seconds; median, 5.2 seconds; 25th-75th percentile, 4.1-6.1 seconds; median, 3.2 seconds; 25th-75th percentile, 2.8-3.8 seconds, respectively; P < .01 for each) and subjects with scleroderma but not PAH (median, 6.5 seconds; 25th-75th percentile, 5.6-7.0 seconds; median, 5.0 seconds; 25th-75th percentile, 4.0-7.3 seconds; median, 3.6 seconds; 25th-75th percentile, 2.7-4.0 seconds, respectively; P < .02 for each). PTT, LV FWHM, and LV TTP correlated with pulmonary vascular resistance index (P < .01), right ventricular stroke volume index (P ≤ .01), and pulmonary artery capacitance (P ≤ .02). In multiple linear regression models, PTT, FWHM, and TTP were associated with mean pulmonary arterial pressure and cardiac index.
CONCLUSION:
CE MR-derived PTT, LV FWHM, and LV TTP are noninvasive compound markers of pulmonary hemodynamics and cardiac function in patients with PAH. Their predictive value for patient outcome warrants further investigation.
PMCID: PMC3359516 [Available on 2013/6/1]
  PMID: 22509050 [PubMed - indexed for MEDLINE]
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36. Curr Cardiol Rep. 2012 Jun;14(3):366-73.
Redefining the role of cardiovascular imaging in patients with pulmonary arterial hypertension.
Freed BH, Patel AR, Lang RM.
Source
Section of Cardiology, Department of Medicine, University of Chicago Medical Center, Chicago, IL 60637, USA. benjamin.freed@uchospitals.edu
Abstract
While pulmonary arterial hypertension is a disease primarily affecting the pulmonary vasculature, the right ventricle plays an integral part in the disease process. Although widely used, two-dimensional echocardiography is limited in visualizing the right ventricle and, therefore, assessment of its structure and function has been largely subjective or invasive. Advanced imaging modalities such as real-time three-dimensional echocardiography and cardiovascular magnetic resonance overcome many challenges of two-dimensional echocardiography and have provided further insight into the pathophysiology of pulmonary arterial hypertension. Indices of right ventricular function obtained from these noninvasive techniques are being assessed for their prognostic capabilities as well as their ability to monitor response to pulmonary arterial hypertension-specific therapies. Future research is needed to compare the accuracy, reproducibility, and prognostic value of each of these parameters to definitively establish the role of cardiovascular imaging in the management of patients with pulmonary arterial hypertension.
  PMID: 22327930 [PubMed - indexed for MEDLINE]
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37. Ann Rheum Dis. 2012 Aug 11. [Epub ahead of print]
Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients.
Shah AA, Chung SE, Wigley FM, Wise RA, Hummers LK.
Source
1Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Abstract
OBJECTIVES:
Annual echocardiography screening is widely used in scleroderma, but the utility of longitudinal assessment is unknown. We evaluated whether change in right ventricular systolic pressure (RVSP) was a risk factor for mortality and development of pulmonary arterial hypertension (PAH) in a cohort of scleroderma patients.
METHODS:
The study population consisted of scleroderma patients who had at least three echocardiograms and pulmonary function tests (PFTs) over ≥1 year as part of routine care. The annual rate of change in RVSP was determined for each subject. Cox proportional hazards regression was performed to assess the association between PAH and mortality and change in RVSP/year, adjusted for relevant covariates.
RESULTS:
613 scleroderma patients with 3244 echocardiograms were studied. The adjusted relative hazards of PAH and mortality were 1.08 (95% CI 1.05-1.11) and 1.12 (95% CI 1.08-1.15) per 1 mm Hg increase in RVSP/year, respectively. Compared with patients with a stable RVSP, the relative hazards for the development of PAH were 1.90 (95% CI 0.91-3.96), 5.09 (95% CI 2.53-10.26) and 6.15 (95% CI 3.58-10.56) for subjects whose RVSP increased at rates of 1-1.99, 2-2.99 and 3+ mm Hg/year. Compared with the same reference group, the relative hazards for death were 0.92 (95% CI 0.48-1.73), 2.16 (95% CI 1.16-4.01) and 5.05 (95% CI 3.47-7.34) for subjects whose RVSP increased at rates of 1-1.99, 2-2.99 and 3+ mm Hg/year.
CONCLUSIONS:
In a population of scleroderma patients, the rate of increase in RVSP is a risk factor for mortality and PAH even after adjustment for clinical characteristics and longitudinal PFT data.
  PMID: 22887850 [PubMed - as supplied by publisher]
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38. Chest. 2012 Aug 1. doi: 10.1378/chest.11-3124. [Epub ahead of print]
HIGH OCCURRENCE OF HYPOXEMIC SLEEP RESPIRATORY DISORDERS IN PRECAPILLARY PULMONARY HYPERTENSION AND MECHANISMS.
Jilwan FN, Escourrou P, Garcia G, Jaïs X, Humbert M, Roisman G.
Abstract
ABSTRACT BACKGROUND The occurrence and mechanisms of nocturnal hypoxemia (NH) in precapillary pulmonary hypertension (PH) are not clearly defined.
METHODS:
In an observational prospective and transversal design, we studied 46 clinically stable PH patients with BMI &lt; 35 kg/m2, FEV1 &gt; 60% of predicted and diagnosis of idiopathic pulmonary arterial hypertension (IPAH, n=29) or chronic thrombo-embolic PH (CTEPH, n=17). They underwent nocturnal polysomnography with transcutaneous capnography.
RESULTS:
Most patients were in NYHA functional class II (69.6%). Mean pulmonary arterial pressure was 44 ± 13 mmHg and cardiac index 3.2 ± 0.6 L/min/m2. The time spent below a saturation of 90% (D90%) was 48.9 ± 35.9% of sleep time and 38 out of 46 (82.6%) patients had NH. Mean apnea hypopnea index (AHI) was 24.9 ± 22.1/ hour and 41 patients (89%) had sleep apnea. The major mechanism of NH was a ventilation/perfusion mismatch alone or associated with obstructive apneic events. Multivariate logistic regression identified both FEV25-75 (odds ratio, 0.9519; 95% CI, 0.9089 to 0.9968; P = 0,036) and mean pulmonary arterial pressure (odds ratio, 1.1068; 95% CI, 1.0062 to 1.2175; P = 0,037) as significant predictors of NH. Clinical symptoms were not predictive of NH.
CONCLUSIONS:
The occurrence of NH is high in PH and should be systematically screened for. Further studies are needed to determine the impact of NH on the outcome of patients with PH.ClinicalTrials.gov Identifier: NCT013716691-Université Paris-Sud, Faculté de médecine, Le Kremlin-Bicêtre, F-94276, France;2-AP-HP, Unité de Médecine du Sommeil, Hôpital Antoine Béclère, Clamart, F-92140, France;3-AP-HP, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, F-92140, France;4-INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Le Plessis-Robinson, F-92350, France;5-Université Paris-Sud, EA3544, Faculté de Pharmacie, Châtenay-Malabry, F-92290, France;Corresponding author and reprint requests: Gabriel Roisman, MD, PhD, Unité de Médecine du Sommeil, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France, Email: gabriel.roisman@abc.aphp.fr.
  PMID: 22878784 [PubMed - as supplied by publisher]
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39. J Heart Lung Transplant. 2012 Sep;31(9):913-33.
World Health Organization Pulmonary Hypertension Group 2: Pulmonary hypertension due to left heart disease in the adult-a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation.
Fang JC, Demarco T, Givertz MM, Borlaug BA, Lewis GD, Rame JE, Gomberg-Maitland M, Murali S, Frantz RP, McGlothlin D, Horn EM, Benza RL.
Source
Division of Cardiovascular Medicine, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center, Cleveland, Ohio.
Abstract
Pulmonary hypertension associated with left heart disease is the most common form of pulmonary hypertension encountered in clinical practice today. Although frequently a target of therapy, its pathophysiology remains poorly understood and its treatment remains undefined. Pulmonary hypertension in the context of left heart disease is a marker of worse prognosis and disease severity, but whether its primary treatment is beneficial or harmful is unknown. An important step to the future study of this important clinical problem will be to standardize definitions across disciplines to facilitate an evidence base that is interpretable and applicable to clinical practice. In this current statement, we provide an extensive review and interpretation of the current available literature to guide current practice and future investigation. At the request of the Pulmonary Hypertension (PH) Council of the International Society for Heart and Lung Transplantation (ISHLT), a writing group was assembled and tasked to put forth this document as described above. The review process was facilitated through the peer review process of the Journal of Heart and Lung Transplantation and ultimately endorsed by the leadership of the ISHLT PH Council.
Copyright © 2012 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.
  PMID: 22884380 [PubMed - in process]
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