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-  Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. #9

- Perioperative Management of the Patient With Pulmonary Hypertension. #25

- Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. #32

- And 35 other recent PHT publications.
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1. Jpn J Radiol. 2014 Apr 24. [Epub ahead of print]

Organized thrombus in pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension; imaging with cone beam computed tomography.

Sugiyama M1Fukuda TSanda YMorita YHigashi MOgo TTsuji ADemachi JNakanishi NNaito H.
Author information: 
1Department of Radiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan, musugijp@c3-net.ne.jp.

Abstract

PURPOSE:

To assess the usefulness of cone-beam CT (CBCT) during pulmonary angiography for the evaluation of organized thrombus at segmental or subsegmental arteries in patients with chronic thromboembolic pulmonary hypertension (CTEPH).

MATERIALS AND METHODS:

The segmental and/or subsegmental pulmonary arteries of 13 patients with CTEPH were evaluated by CBCT. We classified representative forms of organized thrombus into 4 types (type 1: webs, type 2: web and slits, type 3: slits, and type 4: narrowing or complete occlusion), and the distribution and frequency of the organized thrombus were evaluated. The relative detectability of these lesions using CBCT was compared with that in contrast-enhanced CT pulmonary angiography (CTPA).

RESULTS:

Type 1 lesions were most frequently observed in both segmental (30/65 = 46 %) and subsegmental branches (72/156 = 46 %). Type 2 lesions were relatively less frequent than type 1, but subsegmental branches were frequently involved (29/156 = 19 %). Type 3 lesions observed as a thin flap in 9/156 subsegmental branches (6 %). Comparing with CTPA, all 40 lesions in segmental branches were detectable in CTPA, whereas only 62 lesions among 90 lesions (69 %) in subsegmental branches could be observed by CTPA.

CONCLUSION:

CBCT is found to be useful for the treatment planning of balloon pulmonary angioplasty distal to segmental arteries.
  PMID: 24760203 [PubMed - as supplied by publisher]
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2. Rofo. 2014 Apr 22. [Epub ahead of print]

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) - Potential Role of Multidetector-Row CT (MD-CT) and MR Imaging in the Diagnosis and Differential Diagnosis of the Disease.

Wirth G1Brüggemann K1Bostel T1Mayer E2Düber C1Kreitner KF1.
Author information: 
1Department of Radiology, Universitätsmedizin Mainz.
2Department of Thoracic Surgery, Kerckhoff Hospital, Bad Nauheim.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as pulmonary hypertension (resting mean pulmonary arterial pressure of 25 mm Hg or more determined at right heart catheterization) with persistent pulmonary perfusion defects. It is a rare, but underdiagnosed disease with estimated incidences ranging from 0.5 % to 3.8 % of patients after an acute pulmonary embolism (PE), and in up to 10 % of those with a history of recurrent PE. CTEPH is the only form of pulmonary hypertension that can be surgically treated leading to normalization of pulmonary hemodynamics and exercise capacity in the vast majority of patients. The challenges for imaging in patients with suspected CTEPH are fourfold: the imaging modality should have a high diagnostic accuracy with regard to the presence of CTEPH and allow for differential diagnosis. It should enable detection of patients suitable for PEA with great certainty, and allow for quantification of PH by measuring pulmonary hemodynamics (mPAP and PVR), and finally, it can be used for therapy monitoring. This overview tries to elucidate the potential role of ECG-gated multidetector CT pulmonary angiography (MD-CTPA) and MR imaging, and summarizes the most important results that have been achieved so far. Generally speaking, ECG-gated MD-CTPA is superior to MR in the assessment of parenchymal and vascular pathologies of the lung, and allows for the assessment of cardiac structures. The implementation of iodine maps as a surrogate for lung perfusion enables functional assessment of lung perfusion by CT. MR imaging is the reference standard for the assessment of right heart function and lung perfusion, the latter delineating typical wedge-shaped perfusion defects in patients with CTEPH. New developments show that with MR techniques, an estimation of hemodynamic parameters like mean pulmonary arterial pressure and pulmonary vascular resistance will be possible. CT and MR imaging should be considered as complementary investigations providing comprehensive information in patients with CTEPH. Citation Format: • Wirth G, Brüggemann K, Bostel T et al. Chronic Thromboembolic Pulmonary Hypertension (CTEPH) - Potential Role of Multidetector-Row CT (MD-CT) and MR Imaging in the Diagnosis and Differential Diagnosis of the Disease. Fortschr Röntgenstr 2014; DOI: 10.1055/s-0034-1366425.
© Georg Thieme Verlag KG Stuttgart · New York.
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  PMID: 24756429 [PubMed - as supplied by publisher]
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3. Ann Am Thorac Soc. 2014 Apr;11 Suppl 3:S178-85. doi: 10.1513/AnnalsATS.201312-443LD.

Pulmonary Hypertension: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.

Austin ED1Kawut SMGladwin MTAbman SH.
Author information: 
11 Department of Pediatrics, Vanderbilt University, Nashville, Tennessee.

Abstract

Pulmonary vascular dysfunction (PVD) precedes the onset of clinical signs and symptoms of pulmonary arterial hypertension (PAH). PAH is defined by the elevation of pulmonary arterial pressure, which often progresses to right ventricular (RV) dysfunction and failure. PAH affects subjects of all ages, and is associated with diverse medical conditions, most of which are rare. Several factors pose immediate challenges to the development of strategies for primary prevention of PAH, including: (1) the idiopathic or primary form of the disease is extremely rare, limiting screening practicality; (2) methods for the detection of preclinical PVD are currently not established; (3) the understanding of determinants of pulmonary vascular growth, structure, and function in normal and PAH states is insufficient; (4) relatively small numbers of "at-risk" subjects are available for long-term studies to accurately assess disease development; and (5) preventative therapies for PVD are lacking. Despite these limitations, leveraging known at-risk patient populations for study, as well as growing progress across multiple disciplines, ranging from systems biology to advanced and more sensitive functional imaging modalities, may facilitate the opportunity to significantly improve primary prevention research and implementation over the next decade.
  PMID: 24754827 [PubMed - in process]
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4. Drug Discov Today. 2014 Apr 18. pii: S1359-6446(14)00132-9. doi: 10.1016/j.drudis.2014.04.007. [Epub ahead of print]

Inflammation in pulmonary hypertension: what we know and what we could logically and safely target first.

Cohen-Kaminsky S1Hautefort A1Price L2Humbert M3Perros F4.
Author information: 
1Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France.
2Pulmonary Hypertension Service, Royal Brompton Hospital, SW3 6NP, UK.
3Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France; AP-HP, DHU TORINO, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Bicêtre, Le Kremlin-Bicêtre, F94270, France.
4Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, F-94276, France; INSERM UMR-S 999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, LabEx LERMIT, Le Plessis-Robinson, F-92350, France; Centre Chirurgical Marie Lannelongue, Département de recherche Médicale, Le Plessis-Robinson, F-92350, France. Electronic address: frederic.perros@gmail.com.

Abstract

Inflammation is important for the initiation and the maintenance of vascular remodeling in most of the animal models of pulmonary arterial hypertension (PAH), and therapeutic targeting of inflammation in these models blocks PAH development. In humans, pulmonary vascular lesions of PAH are the source of cytokine and chemokine production, related to inflammatory cell recruitment and lymphoid neogenesis. Circulating autoantibodies to endothelial cells and to fibroblasts have been reported in 10-40% of patients with idiopathic PAH, suggesting a possible role for autoimmunity in the pathogenesis of pulmonary vascular lesions. Current specific PAH treatments have immunomodulatory properties, and some studies have demonstrated a correlation between levels of circulating inflammatory mediators and patient survival. New immunopathological approaches to PAH should enable the development of innovative treatments for this severe condition.
Copyright © 2014. Published by Elsevier Ltd.
  PMID: 24747559 [PubMed - as supplied by publisher]
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5. Ann Thorac Cardiovasc Surg. 2014 Apr 18. [Epub ahead of print]

The Impact of Preoperative and Postoperative Pulmonary Hypertension on Long-Term Surgical Outcome after Mitral Valve Repair for Degenerative Mitral Regurgitation.

Murashita T1Okada YKanemitsu HFukunaga NKonishi YNakamura KKoyama T.
Author information: 
1Department of Cardiovascular Surgery, Kobe City Medical Center General Hospital, Kobe, Hyogo, Japan.

Abstract

Purpose: The aim of this study is to elucidate the impact of preoperative and postoperative pulmonary hypertension (PH) on long-term clinical outcomes after mitral valve repair for degenerative mitral regurgitation.Methods: A total of 654 patients who underwent mitral valve repair for degenerative mitral regurgitation between 1991 and 2010 were retrospectively reviewed. Patients were divided into PH(+) group (137 patients) and PH(-) group (517 patients). Follow-up was complete in 99.0%. The median follow-up duration was 7.5 years.Results: Patients in PH(+) group were older, more symptomatic and had higher tricuspid regurgitation grade. Thirty-day mortality was not different between 2 groups (p = 0.975). Long-term survival rate was lower in PH(+) group; 10-year survival rate after the operation was 85.2% ± 4.0% in PH(+) group and 89.7% ± 1.8% in PH(-) group (Log-rank, p = 0.019). The incidence of late cardiac events were not different between groups, however, the recurrence of PH was more frequent in PH(+) group. The recurrence of PH had an adverse impact on survival rate, late cardiac events and symptoms. Univariate analysis showed age and preoperative tricuspid regurgitation grade were the predictors of PH recurrence.Conclusion: Early surgical indication should be advocated for degenerative mitral regurgitation before the progression of pulmonary hypertension and tricuspid regurgitation.
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  PMID: 24747547 [PubMed - as supplied by publisher]
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6. Arch Cardiovasc Dis. 2014 Apr 16. pii: S1875-2136(14)00050-3. doi: 10.1016/j.acvd.2014.02.005. [Epub ahead of print]

Imaging in pulmonary hypertension: Focus on the role of echocardiography.

Moceri P1Baudouy D2Chiche O2Cerboni P2Bouvier P2Chaussade C2Ferrari E2.
Author information: 
1Service de cardiologie, hôpital Pasteur, CHU de Nice, 30, avenue de la Voie-Romaine, BP69, CS 51069, 06001 Nice cedex 1, France. Electronic address: moceri.p@chu-nice.fr.
2Service de cardiologie, hôpital Pasteur, CHU de Nice, 30, avenue de la Voie-Romaine, BP69, CS 51069, 06001 Nice cedex 1, France.

Abstract

Patients with pulmonary hypertension must be evaluated using a multimodality approach to ensure a correct diagnosis and basal evaluation as well as a prognostic assessment. Beyond the assessment of pulmonary pressures, the echocardiographical examination allows the evaluation of right ventricular adaptation to elevated afterload. Numbers of variables are commonly used in the assessment of the pulmonary hypertension patient in order to detect changes in right heart geometry, right-to-left interaction and right ventricular dysfunction. Whereas an isolated change in one echocardiographical variable is not meaningful, multiple echocardiographical variable modifications together provide accurate information. In this review, we will link pulmonary hypertension pathophysiological changes with echocardiographical indices and describe the clinical implications of echocardiographical findings.
Copyright © 2014 Elsevier Masson SAS. All rights reserved.
  PMID: 24746538 [PubMed - as supplied by publisher]
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7. Expert Rev Respir Med. 2014 Apr 18. [Epub ahead of print]

KCNK3: new gene target for pulmonary hypertension?

Girerd B1Perros FAntigny FHumbert MMontani D.
Author information: 
1Université Paris-Sud, Le Kremlin-Bicêtre, France.

Abstract

Recently, KCNK3 has been identified as a new predisposing gene for pulmonary arterial hypertension (PAH) by whole-exome sequencing. Mutation in KCNK3 gene is responsible for the first channelopathy identified in PAH. PAH due to KCNK3 mutations is an autosomal dominant disease with an incomplete penetrance as previously described in PAH due to BMPR2 mutations. This discovery represents an important advance for genetic counselling, allowing identification of high risk relatives for PAH and possible screening for PAH in KCNK3 mutation carriers.
  PMID: 24742047 [PubMed - as supplied by publisher]
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8. Life Sci. 2014 Mar 7;98(1):39-43. doi: 10.1016/j.lfs.2013.12.208. Epub 2014 Jan 8.

Activation of PPAR-γ ameliorates pulmonary arterial hypertension via inducing heme oxygenase-1 and p21(WAF1): an in vivo study in rats.

Zhang D1Wang G1Han D1Zhang Y1Xu J1Lu J1Li S1Xie X1Liu L1Dong L1Li M2.
Author information: 
1Department of Respiratory Medicine, The Second Affiliated Hospital of Medical College, Xi'an Jiaotong University, China.
2Department of Respiratory Medicine, The Second Affiliated Hospital of Medical College, Xi'an Jiaotong University, China. Electronic address: manxiangli@hotmail.com.

Abstract

AIMS:

Our previous study has indicated that activation of PPAR-γ inhibits the proliferation of rat pulmonary artery smooth muscle cells (PASMCs) in vitro through inducing the expression of heme oxygenase-1 (HO-1), which in turn up-regulates the p21(WAF1) expression. In the present study, we intended to determine whether similar mechanisms have been involved in activation of PPAR-γ inhibition of development of rat PAH model.

MATERIAL AND METHODS:

Rat pulmonary arterial hypertension (PAH) model was established by subcutaneous injection of monocrotaline (MCT). Rosiglitazone was administered to activate PPAR-γ. Zinc protoporphyria IX (ZnPP-IX), was used to confirm the role of HO-1 in mediating PPAR-γ function. Parameters including the right ventricle systolic pressure (RVSP), the right ventricular hypertrophy (RVH) and the percentage of medial wall thickness were used to evaluate the development of PAH. Immunoblotting was used to determine the expression of HO-1 and p21(WAF1).

KEY FINDINGS:

Rosiglitazone significantly decreased the RVSP and inhibited the RVH in MCT-induced rat PAH model, and partially inhibited the pulmonary vascular remodeling. These effects were coupled with the sequential increase of HO-1 and p21(WAF1) expressions by rosiglitazone.

SIGNIFICANCE:

Activation of PPAR-γ benefits PAH by inhibiting proliferation of PASMCs and reducing pulmonary vascular remodeling. The present study suggests that enhancing PPAR-γ activity might have potential value in clinical treatment of PAH.
Copyright © 2014 Elsevier Inc. All rights reserved.
  PMID: 24412385 [PubMed - indexed for MEDLINE]
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9. Arthritis Care Res (Hoboken). 2014 Mar;66(3):489-95. doi: 10.1002/acr.22121.

Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry.

Chung LDomsic RTLingala BAlkassab FBolster MCsuka MEDerk CFischer AFrech TFurst DEGomberg-Maitland MHinchcliff MHsu VHummers LKKhanna DMedsger TA JrMolitor JAPreston IRSchiopu EShapiro L,Silver RSimms RVarga JGordon JKSteen VD.

Abstract

OBJECTIVE:

To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US.

METHODS:

The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure >25 mm Hg and pulmonary capillary wedge pressure <15 mm Hg without significant interstitial lung disease) were included in these analyses.

RESULTS:

In total, 131 SSc patients with incident PAH were followed for a mean ± SD of 2.0 ± 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1- 8.4), male sex (HR 3.9, 95% CI 1.1-13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8 -22.8), and diffusing capacity for carbon monoxide (DLCO) <39% predicted (HR 4.2, 95% CI 1.3-13.8) were significant predictors of mortality.

CONCLUSION:

This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLCO and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.
  PMID: 23983198 [PubMed - indexed for MEDLINE]
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10. Am J Respir Cell Mol Biol. 2014 Jan;50(1):74-86. doi: 10.1165/rcmb.2012-0506OC.

Skeletal muscle dysfunction in idiopathic pulmonary arterial hypertension.

Batt J1Ahmed SSCorrea JBain AGranton J.
Author information: 
11 Keenan Research Center, Li Ka Shing Knowledge Institute, St. Michael's Hospital, Toronto, Ontario, Canada; and.

Abstract

Despite improvements in survival with disease-targeted therapies, the majority of patients with pulmonary arterial hypertension (PAH) have persistent exercise intolerance that results from impaired cardiac function and skeletal muscle dysfunction. Our intent was to understand the molecular mechanisms mediating skeletal muscle dysfunction in PAH. A total of 12 patients with PAH and 10 matched control subjects were assessed. Patients with PAH demonstrated diminished exercise capacity (lower oxygen uptake max, lower anaerobic threshold and higher minute ventilation/CO2) compared with control subjects. Quadriceps muscle cross-sectional area was significantly smaller in patients with PAH. The vastus lateralis muscle was biopsied to enable muscle fiber morphometric assessment and to determine expression levels/activation of proteins regulating (1) muscle mass, (2) mitochondria biogenesis and shaping machinery, and (3) excitation-contraction coupling. Patients with PAH demonstrated a decreased type I/type II muscle fiber ratio, with a smaller cross-sectional area in the type I fibers. Diminished AKT and p70S6 kinase phosphorylation, with increased atrogin-1 and muscle RING-finger protein-1 transcript levels, were evident in the PAH muscle, suggesting engagement of cellular signaling networks stimulating ubiquitin-proteasome-mediated proteolysis of muscle, with concurrent depression of networks mediating muscle hypertrophy. Although there were no differences in expression/activation of proteins associated with mitochondrial biogenesis or fission (MTCO2 [cytochrome C oxidase subunit II]/succinate dehydrogenase flavoprotein subunit A, mitochondrial transcription factor A, nuclear respiratory factor-1/dynamin-related protein 1 phosphorylation), protein levels of a positive regulator of mitochondrial fusion, Mitofusin2, were significantly lower in patients with PAH. Patients with PAH demonstrated increased phosphorylation of ryanodine receptor 1 receptors, suggesting that altered sarcoplasmic reticulum Ca(++) sequestration may impair excitation-contraction coupling in the PAH muscle. These data suggest that muscle dysfunction in PAH results from a combination of muscle atrophy and intrinsically impaired contractility.
  PMID: 23972212 [PubMed - indexed for MEDLINE]
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11. Chest. 2014 Mar 1;145(3):551-8. doi: 10.1378/chest.13-1363.

Breath analysis in pulmonary arterial hypertension.

Cikach FS JrTonelli ARBarnes JPaschke KNewman JGrove DDababneh LWang SDweik RA.

Abstract

BACKGROUND:

Pulmonary arterial hypertension (PAH) is a progressive and devastating condition characterized by vascular cell proliferation and is associated with several metabolic derangements. We hypothesized that metabolic derangements in PAH can be detected by measuring metabolic by-products in exhaled breath.

METHODS:

We collected breath and blood samples from patients with PAH at the time of right-sided heart catheterization (n=31) and from healthy control subjects (n=34). Breath was analyzed by selected ion flow tube-mass spectrometry in predetermined training and validation cohorts.

RESULTS:

Patients with PAH were 51.5±14 years old, and 27 were women (85%). Control subjects were 38±13 years old, and 22 were women (65%). Discriminant analysis in the training set identified three ion peaks (H3O+29+, NO+56+, and O2+98+) and the variable age that correctly classified 88.9% of the individuals. In an independent validation cohort, 82.8% of the individuals were classified correctly. The concentrations of the volatile organic compounds 2-propanol, acetaldehyde, ammonia, ethanol, pentane, 1-decene, 1-octene, and 2-nonene were different in patients with PAH compared with control subjects. Exhaled ammonia was higher in patients with PAH (median [interquartile range]: 94.7 parts per billion (ppb) [70-129 ppb] vs 60.9 ppb [46-77 ppb], P<.001) and was associated with right atrial pressure (ρ=0.57, P<.001), mean pulmonary artery pressure (ρ=0.43, P=.015), cardiac index by thermodilution (ρ=-0.39, P=.03), pulmonary vascular resistance (ρ=0.40, P=.04), mixed venous oxygen (ρ=-0.59, P<.001), and right ventricular dilation (ρ=0.42, P=.03).

CONCLUSIONS:

Breathprint is different between patients with PAH and healthy control subjects. Several specific compounds, including ammonia, were elevated in the breath of patients with PAH. Exhaled ammonia levels correlated with severity of disease.
PMCID: PMC3941248 [Available on 2015/3/1]
  PMID: 24091389 [PubMed - indexed for MEDLINE]
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12. Circulation. 2014 Feb 18;129(7):786-97. doi: 10.1161/CIRCULATIONAHA.113.006167. Epub 2013 Nov 22.

Role for DNA damage signaling in pulmonary arterial hypertension.

Meloche J1Pflieger AVaillancourt MPaulin RPotus FZervopoulos SGraydon CCourboulin ABreuils-Bonnet STremblay ECouture CMichelakis EDProvencher SBonnet S.
Author information: 
1Department of Medicine, Laval University, Pulmonary Hypertension Research Group, IUCPQ Research Centre, Québec, Canada (J.M., A.P., M.V., F.P., C.G., A.C., S.B.-B., E.T., C.C., S.P., S.B.); and Vascular Biology Research Group, Department of Medicine, University of Alberta, Edmonton, Canada (R.P., S.Z., E.D.M.).

Abstract

BACKGROUND:

Pulmonary arterial hypertension (PAH) is associated with sustained inflammation known to promote DNA damage. Despite these unfavorable environmental conditions, PAH pulmonary arterial smooth muscle cells (PASMCs) exhibit, in contrast to healthy PASMCs, a pro-proliferative and anti-apoptotic phenotype, sustained in time by the activation of miR-204, nuclear factor of activated T cells, and hypoxia-inducible factor 1-α. We hypothesized that PAH-PASMCs have increased the activation of poly(ADP-ribose) polymerase-1 (PARP-1), a critical enzyme implicated in DNA repair, allowing proliferation despite the presence of DNA-damaging insults, eventually leading to PAH.

METHODS AND RESULTS:

Human PAH distal pulmonary arteries and cultured PAH-PASMCs exhibit increased DNA damage markers (53BP1 and γ-H2AX) and an overexpression of PARP-1 (immunoblot and activity assay), in comparison with healthy tissues/cells. Healthy PASMCs treated with a clinically relevant dose of tumor necrosis factor-α harbored a similar phenotype, suggesting that inflammation induces DNA damage and PARP-1 activation in PAH. We also showed that PARP-1 activation accounts for miR-204 downregulation (quantitative reverse transcription polymerase chain reaction) and the subsequent activation of the transcription factors nuclear factor of activated T cells and hypoxia-inducible factor 1-α in PAH-PASMCs, previously shown to be critical for PAH in several models. These effects resulted in PASMC proliferation (Ki67, proliferating cell nuclear antigen, and WST1 assays) and resistance to apoptosis (terminal deoxynucleotidyl transferase dUTP nick end labeling and Annexin V assays). In vivo, the clinically available PARP inhibitor ABT-888 reversed PAH in 2 experimental rat models (Sugen/hypoxia and monocrotaline).

CONCLUSIONS:

These results show for the first time that the DNA damage/PARP-1 signaling pathway is important for PAH development and provide a new therapeutic target for this deadly disease with high translational potential.
  PMID: 24270264 [PubMed - indexed for MEDLINE]
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13. Eur Respir J. 2014 Apr 17. [Epub ahead of print]

A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis.

Oliveira RK1Pereira CARamos RPFerreira EVMessina CMKuranishi LTGimenez ACampos OSilva CMOta-Arakaki JS.
Author information: 
1Dept of Medicine, Federal University of São Paulo (UNIFESP), Brazil.

Abstract

Chronic hypersensitivity pneumonitis is a common fibrotic interstitial lung disease. The prevalence of pulmonary hypertension diagnosed by right heart catheterisation and its cardiopulmonary function findings in patients with chronic hypersensitivity pneumonitis are unknown.Consecutive symptomatic patients with chronic hypersensitivity pneumonitis were prospectively evaluated. All patients were submitted to right heart catheterisation, pulmonary function testing, a 6-min walk test, echocardiography, blood gas determination and N-terminal pro-brain natriuretic peptide analyses. Nonhypoxaemic patients also underwent incremental cardiopulmonary exercise testing.50 patients underwent right heart catheterisation; 25 (50%) of these had pulmonary hypertension and 22 (44%) had a pre-capillary haemodynamic pattern. The patients with pre-capillary pulmonary hypertension had lower forced vital capacity (mean±sd 50±17% versus 69±22% predicted, p<0.01), carbon monoxide diffusing capacity (37±12% versus 47±14% predicted, p<0.01), arterial oxygen tension (median (interquartile range) 59.0 (47.8-69.3) versus 73.0 (62.2-78.5) mmHg, p<0.01) and saturation after the 6-min walk test (78±8% versus 86±7%, p<0.01). In pre-capillary pulmonary hypertension, oxygen uptake was also lower at the anaerobic threshold (41±11% versus 50±8% predicted, p = 0.04) and at peak exercise (12.8±1.6 versus 15.0±2.5 mL·kg-1·min-1, p = 0.02).Pre-capillary pulmonary hypertension is common in symptomatic chronic hypersensitivity pneumonitis and is related to interstitial lung disease severity. Additionally, pulmonary hypertension is more prevalent in hypoxaemic patients with impaired lung function and exercise capacity.
  PMID: 24743965 [PubMed - as supplied by publisher]
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14. Heart Vessels. 2014 May 18. [Epub ahead of print]

Autoimmunity and pulmonary hypertension in patients with Graves' disease.

Sugiura T1Yamanaka STakeuchi HMorimoto NKamioka MMatsumura Y.
Author information: 
1Department of Laboratory Medicine, Kochi Medical School, Kohasu Oko-cho, Nankoku City, Kochi, 783-8505, Japan.

Abstract

A link between hyperthyroidism and pulmonary hypertension has been reported, but the underlying mechanisms of these two conditions have not been clearly identified. The aim of this study was to determine the clinical correlates of pulmonary hypertension in patients with Graves' disease. Among 50 consecutive patients with Graves' disease referred for echocardiography, 18 patients (36 %) had pulmonary hypertension measured by continuous-wave Doppler echocardiography (pulmonary artery systolic pressure >35 mmHg). The patients with pulmonary hypertension had significantly higher pulmonary vascular resistance (PVR), cardiac output and thyroid-stimulating hormone receptor antibody (TRAb) compared to those without (p < 0.001, p = 0.028 and p < 0.001, respectively). Pulmonary artery systolic pressure had a good correlation with TRAb (r = 0.74, p < 0.001), but was not related to free T4 (r = 0.12, p = 0.419) and free T3 (r = 0.22, p = 0.126). To determine the important variables present in patients with Graves' disease that may be related to pulmonary artery systolic pressure, 4 variables (PVR, cardiac output, TRAb and free T3) were used in the multivariate analysis. In addition to PVR (standard regression coefficient = 0.831, p < 0.001) and cardiac output (standard regression coefficient = 0.592, p < 0.001), TRAb (standard regression coefficient = 0.178, p < 0.001) emerged as a significant variable related to pulmonary artery systolic pressure. Thus, in addition to the effect of thyroid hormone on the cardiovascular system, autoimmune-mediated pulmonary vascular remodeling may play a role in Graves' disease-linked elevated pulmonary artery systolic pressure.
  PMID: 24838983 [PubMed - as supplied by publisher]
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15. BMJ Open. 2014 May 16;4(5):e004735. doi: 10.1136/bmjopen-2013-004735.

Living with pulmonary hypertension: unique insights from an international ethnographic study.

Kingman M1Hinzmann B2Sweet O3Vachiéry JL4.
Author information: 
1Pulmonary Hypertension Program, UT Southwestern Medical Center, Dallas, Texas, USA.
2Department of Global Market Research, Market Research Cardiology, Bayer HealthCare Pharmaceuticals, Berlin, Germany.
3Ethnography Centre of Excellence, Ipsos MORI, London, UK.
4Département de Cardiologie, Cliniques Universitaires de Bruxelles, Hôpital Erasme, Brussels, Belgium.

Abstract

OBJECTIVES:

To better understand the patient's perspective of pulmonary hypertension (PH), including the impact of living with PH, disease management and treatment.

DESIGN:

This qualitative ethnographic study collected observational video footage, supplemented by field notes and patient diaries to assess the impact of PH on the patient's life.

SETTING:

Patients were observed and filmed in their home for up to 6 h, capturing the environment, interactions and activities of everyday life.

PARTICIPANTS:

Patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic PH who were receiving PAH-specific medication were recruited through healthcare professionals (HCPs) and patient associations in seven countries across four continents. Sampling was purposive and subgroup analysis was not intended.

RESULTS:

Overall, 39 patients with PH were enrolled. Many patients had a poor understanding of PH and found their 'invisible' disease difficult to explain to others. An important finding was the secrecy surrounding PH. Feelings of insecurity and isolation were regularly reported, and many patients admitted to hiding their symptoms. The marked improvement in symptoms after therapy initiation made assessment of disease progression more difficult as patients compared their quality of life (QoL) against pretreatment levels. Extensive planning and adherence to daily routines were required in patients' everyday life.

CONCLUSIONS:

Ethnography was used for the first time, in several countries, to evaluate the patient's perception of living with PH. This approach revealed key findings that would not typically be uncovered using other qualitative techniques, including the secrecy surrounding PH, the difficulties in describing the disease and the challenges in assessing disease progression. A more tailored dissemination of information from HCPs and development of a simple and understandable PH definition may be beneficial in alleviating the secrecy reported by patients. A greater appreciation of how patients perceive their disease and QoL has the potential to improve PH management.
Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
PMCID: PMC4024598 Free PMC Article
  PMID: 24838724 [PubMed]
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16. Clin Sci (Lond). 2014 Jul;127(2):65-76. doi: 10.1042/CS20130296.

Lung capillary injury and repair in left heart disease: a new target for therapy?

Azarbar S1Dupuis J.
Author information: 
1*Department of Medicine, Montreal Heart Institute, Université de Montréal, 5000 Belanger Street, Montreal, Quebec, Canada, H1T 1C8.

Abstract

The lungs are the primary organs affected in LHD (left heart disease). Increased left atrial pressure leads to pulmonary alveolar-capillary stress failure, resulting in cycles of alveolar wall injury and repair. The reparative process causes the proliferation of MYFs (myofibroblasts) with fibrosis and extracellular matrix deposition, resulting in thickening of the alveolar wall. Although the resultant reduction in vascular permeability is initially protective against pulmonary oedema, the process becomes maladaptive causing a restrictive lung syndrome with impaired gas exchange. This pathological process may also contribute to PH (pulmonary hypertension) due to LHD. Few clinical trials have specifically evaluated lung structural remodelling and the effect of related therapies in LHD. Currently approved treatment for chronic HF (heart failure) may have direct beneficial effects on lung structural remodelling. In the future, novel therapies specifically targeting the remodelling processes may potentially be utilized. In the present review, we summarize data supporting the clinical importance and pathophysiological mechanisms of lung structural remodelling in LHD and propose that this pathophysiological process should be explored further in pre-clinical studies and future therapeutic trials.
  PMID: 24678967 [PubMed - indexed for MEDLINE]
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17. Can J Cardiol. 2014 Apr;30(4):465.e1-2. doi: 10.1016/j.cjca.2013.11.017. Epub 2013 Nov 21.

Left atrial myxoma: a rare cause of dyspnea.

Pourdjabbar A1Hibbert B1Maze R1Lai C2Le May MR3.
Author information: 
1Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada.
2Division of Pathology, Ottawa Hospital, Ottawa, Ontario, Canada.
3Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada. Electronic address: mlemay@ottawaheart.ca.
  PMID: 24507974 [PubMed - indexed for MEDLINE]
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18. J Comput Assist Tomogr. 2014 Mar-Apr;38(2):163-8. doi: 10.1097/RCT.0b013e3182aa7fc5.

Relationship of main pulmonary artery diameter to pulmonary arterial pressure in scleroderma patients with and without interstitial fibrosis.

McCall RK1Ravenel JGNietert PJGranath ASilver RM.
Author information: 
1From the *Division of Rheumatology, Department of Medicine, †Department of Radiology and Radiologic Sciences, and ‡Division of Biostatistics and Epidemiology, Department of Medicine, Medical University of South Carolina, Charleston, SC.

Abstract

OBJECTIVE:

This study aimed to determine the relationship between main pulmonary artery diameter (MPAD) and pulmonary hypertension (PH) in scleroderma patients with and without interstitial lung disease.

METHODS:

We retrospectively reviewed 48 subjects with scleroderma who underwent a chest computed tomography (CT) and right heart catheterization with 6 months of each other. Patients were divided into 2 groups based on the absence or presence of interstitial lung disease on chest CT. Subset analysis was performed based on available pulmonary function tests and divided into groups by forced vital capacity (FVC). Computed tomographic scans were scored for extent of fibrosis and ground glass opacity. Pulmonary artery and ascending aorta measurements were obtained by 2 independent observers. Univariate and multivariate models were used to evaluate the correlation between MPAD and mean pulmonary artery pressure (mPAP) measured by right heart catheterization. Receiver operating characteristic analysis was performed for diagnostic accuracy of the MPAD measurement in predicting PH.

RESULTS:

Strong correlations between mPAP and MPAD were found in this study regardless of the presence or absence of mild to moderate interstitial fibrosis on chest CT. When dividing patients based on FVC, the correlation between mPAP and MPAD was substantially attenuated. An MPAD value of 30.8 mm yielded the highest sensitivity and specificity at 81.3% and 87.5%, respectively.

CONCLUSIONS:

In scleroderma patients, an enlarged main pulmonary artery (>30 mm) predicts PH even in the presence of mild to moderate fibrosis although the relationship may be attenuated in the presence of a low % FVC.
PMCID: PMC3959588 [Available on 2015/3/1]
  PMID: 24448503 [PubMed - indexed for MEDLINE]
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19. Am J Cardiol. 2013 Dec 1;112(11):1834-9. doi: 10.1016/j.amjcard.2013.08.003. Epub 2013 Sep 5.

Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study).

Cha KS1Cho KISeo JSChoi JHPark YHYang DHHong GRKim DS.
Author information: 
1Department of Cardiology, Pusan National University Hospital, Busan, South Korea; Medical Research Institute, Pusan National University Hospital, Busan, South Korea.

Abstract

There are limited data on the effect of iloprost therapy in patients with Eisenmenger syndrome (ES). The aim of our study was to evaluate the effect of inhaled iloprost therapy on exercise capacity, quality of life (QoL), cardiac function, and hemodynamics in patients with ES. Eighteen consecutive patients with ES and exertional dyspnea according to the World Health Organization functional class III or IV were prospectively recruited. Exercise capacity was assessed by a 6-minute walk test, and QoL was measured on a 12-Item Short-Form Health Survey. Echocardiographic measurements included peak systolic and mean pulmonary arterial pressures, pulmonary vascular resistance, and myocardial performance index of the right ventricle (RV). All patients underwent comprehensive evaluation at baseline and after 24 weeks of treatment. Of the 18 patients with ES, 13 were included for analysis. After 24 weeks of iloprost therapy, 6-minute walk test distance significantly increased (289.1 ± 76.9 to 369.5 ± 93.4 m, p = 0.032) in addition to concomitant improvements in the 12-Item Short-Form Health Survey physical and mental component summaries (20.6 ± 19.3 to 52.6 ± 28.0, p <0.05; 33.9 ± 19.7 to 54.9 ± 21.3, p <0.05, respectively). RV myocardial performance index improved significantly after treatment (0.80 ± 0.31 to 0.59 ± 0.12, p = 0.042). Pulmonary arterial pressure and pulmonary vascular resistance did not improve with iloprost therapy. This study showed that 24 weeks of inhaled iloprost therapy in patients with ES led to significant improvements in exercise capacity, QoL, and RV function. These results likely explain the symptomatic relief reported by patients with ES receiving iloprost therapy.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 24012036 [PubMed - indexed for MEDLINE]
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20. Am J Physiol Lung Cell Mol Physiol. 2014 Apr 1;306(7):L661-71. doi: 10.1152/ajplung.00244.2013. Epub 2014 Feb 7.

High proliferative potential endothelial colony-forming cells contribute to hypoxia-induced pulmonary artery vasa vasorum neovascularization.

Nijmeh H1Balasubramaniam VBurns NAhmad AStenmark KRGerasimovskaya EV.
Author information: 
1Univ. of Colorado Denver, Pediatric Critical Care Medicine, Box B131, Research 2, Rm. 6119, 12700 E. 19th Ave., Aurora, CO 80045. evgenia.gerasimovskaya@ucdenver.edu.

Abstract

Angiogenic expansion of the vasa vasorum (VV) is an important contributor to pulmonary vascular remodeling in the pathogenesis of pulmonary hypertension (PH). High proliferative potential endothelial progenitor-like cells have been described in vascular remodeling and angiogenesis in both systemic and pulmonary circulations. However, their role in hypoxia-induced pulmonary artery (PA) VV expansion in PH is not known. We hypothesized that profound PA VV neovascularization observed in a neonatal calf model of hypoxia-induced PH is due to increased numbers of subsets of high proliferative cells within the PA adventitial VV endothelial cells (VVEC). Using a single cell clonogenic assay, we found that high proliferative potential colony-forming cells (HPP-CFC) comprise a markedly higher percentage in VVEC populations isolated from the PA of hypoxic (VVEC-Hx) compared with control (VVEC-Co) calves. VVEC-Hx populations that comprised higher numbers of HPP-CFC also demonstrated markedly higher expression levels of CD31, CD105, and c-kit than VVEC-Co. In addition, significantly higher expression of CD31, CD105, and c-kit was observed in HPP-CFC vs. the VVEC of the control but not of hypoxic animals. HPP-CFC exhibited migratory and tube formation capabilities, two important attributes of angiogenic phenotype. Furthermore, HPP-CFC-Co and some HPP-CFC-Hx exhibited elevated telomerase activity, consistent with their high replicative potential, whereas a number of HPP-CFC-Hx exhibited impaired telomerase activity, suggestive of their senescence state. In conclusion, our data suggest that hypoxia-induced VV expansion involves an emergence of HPP-CFC populations of a distinct phenotype with increased angiogenic capabilities. These cells may serve as a potential target for regulating VVEC neovascularization.
PMCID: PMC3962625 [Available on 2015/4/1]
  PMID: 24508729 [PubMed - indexed for MEDLINE]
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21. PLoS One. 2014 May 16;9(5):e96720. doi: 10.1371/journal.pone.0096720. eCollection 2014.

Recombinant human interferon alpha 2b prevents and reverses experimental pulmonary hypertension.

Bauer EM1Zheng H2Lotze MT1Bauer PM3.
Author information: 
1Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America; University of Pittsburgh Cancer Institute, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America.
2Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America.
3Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America; Department of Pharmacology and Chemical Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America; Vascular Medicine Institute, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America.

Abstract

Pulmonary hypertension (PH) is a progressive and fatal disease with no cure. Vascular remodeling in PH involves intraluminal growth of endothelial and smooth muscle cells, leading to obliterative vascular lesions. Cell growth in these lesions is quasi-neoplastic, with evidence of monoclonality, apoptosis resistance and cancer-like metabolic derangements. Herein we tested the effect of human interferon alpha 2b (IFNα), a pleiotropic cytokine and anti-cancer therapeutic, on the development and progression of PH in the rat SU5416/hypoxia (SUH) model and mouse hypoxia model of the disease. In both models IFNα attenuated the development of PH and reversed established PH as assessed by measuring right ventricular systolic pressure and right ventricular hypertrophy. The effect of IFNα was dependent on the type I interferon receptor (IFNAR) since mice lacking a subunit of the IFNAR were not protected by IFNα. Morphometric analysis of pulmonary aterioles from hypoxic mice or SUH rats showed that IFNα inhibited pulmonary vascular remodeling in both models and that IFNα reversed remodeling in SUH rats with established disease. Immunohistochemical staining revealed that IFNα decreased the number of PCNA and Tunel positive cells in the wall of pulmonary arterioles. In vitro, IFNα inhibited proliferation of human pulmonary artery smooth muscle cells and as well as human pulmonary artery endothelial cell proliferation and apoptosis. Together these findings demonstrate that IFNα reverses established experimental PH and provide a rationale for further exploration of the use of IFNα and other immunotherpies in PH.
PMCID: PMC4024039 Free PMC Article
  PMID: 24837600 [PubMed - in process]
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22. Respirology. 2014 May 15. doi: 10.1111/resp.12321. [Epub ahead of print]

Non-invasive markers of pulmonary hypertension in interstitial lung disease: Is cardiopulmonary exercise testing the Holy Grail?

Boutou AK1Pitsiou GGArgyropoulou P.
Author information: 
1Respiratory Failure Unit, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Free Article
  PMID: 24831928 [PubMed - as supplied by publisher]
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23. Biomarkers. 2014 May 15:1-6. [Epub ahead of print]

Pulmonary hypertension in patients with advanced heart failure is associated with increased levels of interleukin-6.

Dolenc J1Sebeštjen MVrtovec BKoželj MHaddad F.
Author information: 
1Department of Cardiology, University Medical Centre Ljubljana , Ljubljana , Slovenia .

Abstract

Abstract Context: Inflammatory, endothelial and neurohormonal biomarkers are involved in heart failure (HF) and pulmonary hypertension (PH) pathogenesis. Objective: To study these biomarkers in PH due to advanced HF. Materials and methods: Thirty adults with HF were included. Interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), high-sensitivity C-reactive protein (hsCRP), endothelin-1 and N-terminal prohormone of brain natriuretic peptide (NT-proBNP) were measured in peripheral vein and pulmonary artery during right heart catheterisation. Results: IL-6, TNF-α, hsCRP and NT-proBNP correlated with pulmonary pressures independent of ventricular function, HF etiology and vascular bed. IL-6 was independent predictor of systolic pulmonary artery pressure (sPAP). Discussion and conclusion: Inflammatory biomarkers correlate to PH severity. IL-6 predicts sPAP in advanced HF.
  PMID: 24831174 [PubMed - as supplied by publisher]
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24. Ann Am Thorac Soc. 2014 May;11(4):653-4. doi: 10.1513/AnnalsATS.201401-046OR.

"You have pulmonary hypertension until proven otherwise".

Hudak BB.
Author information: 
Pediatric Pulmonology, Nemours Children's Clinic, Jacksonville, Florida.
  PMID: 24828804 [PubMed - in process]
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25. Semin Cardiothorac Vasc Anesth. 2014 May 13. pii: 1089253214534780. [Epub ahead of print]

Perioperative Management of the Patient With Pulmonary Hypertension.

Fox DL1Stream AR2Bull T2.
Author information: 
1University of Colorado, Aurora, CO, USA Daniel.L.Fox@ucdenver.edu.
2University of Colorado Health Sciences Center, Aurora, CO, USA.

Abstract

Patients with pulmonary hypertension are at increased risk for perioperative morbidity and mortality. Elective surgery is generally discouraged in this patient population; however, there are times when surgery is deemed necessary. Currently, there are no guidelines for the preoperative risk assessment or perioperative management of subjects with pulmonary hypertension. The majority of the literature evaluating perioperative risk factors and mortality rates is observational and includes subjects with multiple etiologies of pulmonary hypertension. Subjects with pulmonary arterial hypertension, also referred to as World Health Organization group I pulmonary hypertension, and particularly those receiving pulmonary arterial hypertension-specific therapy may be at increased risk. Perioperative management of these patients requires a solid understanding and careful consideration of the hemodynamic effects of anesthetic agents, positive pressure ventilation and volume shifts associated with surgery in order to prevent acute right ventricular failure. We reviewed the most recent data regarding perioperative morbidity and mortality for subjects with pulmonary hypertension in an effort to better guide preoperative risk assessment and perioperative management by a multidisciplinary team.
© The Author(s) 2014.
  PMID: 24828282 [PubMed - as supplied by publisher]
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26. Anadolu Kardiyol Derg. 2014 Apr 16. doi: 10.5152/akd.2013.4650. [Epub ahead of print]

Combined effect of aerosolized iloprost and oxygen on assessment of pulmonary vasoreactivity in children with pulmonary hypertension.

Elkıran O1Karakurt CKoçak G.
Author information: 
1Department of Pediatric Cardiology, Faculty of Medicine, İnönü University; Malatya-Turkey. ozlemelkiran@yahoo.com.

Abstract

OBJECTIVE:

The evaluation of pulmonary vascular reactivity plays a significant role in the management of patients with pulmonary hypertension. Inhaled nitric oxide in combination with oxygen (O2) has become widely used as an agent for pulmonary vasodilator testing. However, inhaled nitric oxide is not available in many developing countries. Recently, aerosolized iloprost was suggested as an alternative to nitric oxide for this purpose. In the present study, aerosolized iloprost was used together with O2 in the pulmonary vasoreactivity test of children with severe pulmonary hypertension. Thus, the synergistic effect of both vasodilators was utilized without extending the duration of cardiac catheterization.

METHODS:

The prospective cohort study registered a total of 16 children with severe pulmonary hypertension whose median age was 4.5 years. Hemodynamic parameters were quantified before and after the vasoreactivity test. Increased left-to-right shunt, pulmonary vascular resistance of <6 Woods units (WU). /m2 and a pulmonary-systemic resistance ratio of <0.3, as well as a decrease >10% in the pulmonary vascular resistance and pulmonary-systemic vascular resistance ratio after the vasoreactivity test were accepted as a positive response. The data were analyzed using Wilcoxon signed-rank and the Mann-Whitney U tests.

RESULTS:

Eleven children gave a positive response to the vasoreactivity test, while 5 children did not respond. Pulmonary vascular resistance dropped from 9.98±1.39 WU./m2 to 5.08±1.05 WU./m2 (p=0.013) and the pulmonary-systemic vascular resistance ratio fell from 0.68±0.08 to 0.32±0.05 (p=0.003) in the children who were responsive. No side effects were observed related to iloprost administration.

CONCLUSION:

Administration of inhaled iloprost in combination with O2 for pulmonary vasoreactivity testing can be useful for correctly identifying pulmonary vasoreactivity without extending the duration of cardiac catheterization.
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  PMID: 24818629 [PubMed - as supplied by publisher]
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27. Lancet Respir Med. 2014 May;2(5):348-50. doi: 10.1016/S2213-2600(14)70090-6.

Pulmonary hypertension: smaller kids, smaller steps.

Berger RM.
Author information: 
Center for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen 9713GZ, Netherlands. Electronic address: r.m.f.berger@umcg.nl.
  PMID: 24815801 [PubMed - in process]
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28. J Rheumatol. 2013 Oct;40(10):1706-11. doi: 10.3899/jrheum.130400. Epub 2013 Aug 15.

Combination of echocardiographic and pulmonary function test measures improves sensitivity for diagnosis of systemic sclerosis-associated pulmonary arterial hypertension: analysis of 2 cohorts.

Gladue H1Steen VAllanore YSaggar RSaggar RMaranian PBerrocal VJAvouac JMeune CTrivedi MKhanna D.
Author information: 
1From the University of Michigan Scleroderma Program; Division of Rheumatology, Department of Medicine, Georgetown University, Washington, DC, USA; Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; St. Joseph's Hospital and Medical Center, Division of Pulmonary, Department of Medicine, Phoenix, AZ; David Geffen School of Medicine at UCLA, Division of Pulmonary, Department of Medicine, Los Angeles, CA; Arizona State University, Biodesign Institute, Tempe, AZ; Department of Biostatistics, University of Michigan, School of Public Health, Ann Arbor, MI, USA; and Paris XIII University, Cardiology Department, Avicenne Hospital, Bobigny, France.

Abstract

OBJECTIVE:

To evaluate routinely collected non-invasive tests from 2 systemic sclerosis (SSc) cohorts to determine their predictive value alone and in combination versus right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH).

METHODS:

We evaluated 2 cohorts of patients who were at risk or with incident PAH: (1) The Pulmonary Hypertension Assessment and Recognition Outcomes in Scleroderma (PHAROS) cohort and (2) an inception SSc cohort at Cochin Hospital, Paris, France. Estimated right ventricular systolic pressure (eRVSP) as determined by transthoracic echocardiogram (TTE) and pulmonary function test (PFT) measures was evaluated, and the predictive values determined. We then evaluated patients with PAH missed on TTE cutoffs that were subsequently identified by a PFT measure.

RESULTS:

In the PHAROS cohort (n = 206), 59 (29%) had RHC-defined PAH. An eRVSP threshold of 35-50 mm Hg failed to diagnose PAH in 7% to 31% of patients, 50% to 70% of which (n = 2-13) were captured by PFT measures. In the Cochin cohort (n = 141), 10 (7%) patients had RHC confirmed PAH. An eRVSP threshold of 35-50 mm Hg missed 0% to 70% (n = 0-7) of patients, of which 0% to 68% (n = 0-6) were met by PFT measures. The combination of TTE and PFT improved the negative predictive value for diagnosing PAH.

CONCLUSION:

In 2 large SSc cohorts, screening with TTE and PFT captured a majority of patients with PAH. TTE and PFT complement each other for the diagnosis of PAH.
PMCID: PMC3798032 [Available on 2014/10/1]
  PMID: 23950183 [PubMed - indexed for MEDLINE]
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29. Am J Cardiol. 2014 Apr 18. pii: S0002-9149(14)00973-4. doi: 10.1016/j.amjcard.2014.04.016. [Epub ahead of print]

Effect of Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension on Stroke Volume Response to Exercise.

Surie S1van der Plas MN2Marcus JT3Kind T4Kloek JJ5Vonk-Noordegraaf A4Bresser P6.
Author information: 
1Department of Respiratory Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. Electronic address: s.surie@umcg.nl.
2Department of Respiratory Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands; Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands.
3Department of Physics and Medical Technology, Institute for Cardiovascular Research, Vrije Universiteit Medical Center, Amsterdam, The Netherlands.
4Department of Pulmonary Medicine, Institute for Cardiovascular Research, Vrije Universiteit Medical Center, Amsterdam, The Netherlands.
5Department of Cardiothoracic Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
6Department of Respiratory Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands; Department of Respiratory Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands; Department of Cardiothoracic Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

Abstract

In pulmonary hypertension, exercise is limited by an impaired right ventricular (RV) stroke volume response. We hypothesized that improvement in exercise capacity after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) is paralleled by an improved RV stroke volume response. We studied the extent of PEA-induced restoration of RV stroke volume index (SVI) response to exercise using cardiac magnetic resonance imaging (cMRI). Patients with CTEPH (n = 18) and 7 healthy volunteers were included. Cardiopulmonary exercise testing and cMRI were performed before and 1 year after PEA. For cMRI studies, pre- and post-operatively, all patients exercised at 40% of their preoperative cardiopulmonary exercise testing-assessed maximal workload. Post-PEA patients (n = 13) also exercised at 40% of their postoperative maximal workload. Control subjects exercised at 40% of their predicted maximal workload. Preoperatively, SVI (n = 18) decreased during exercise from 35.9 ± 7.4 to 33.0 ± 9.0 ml·m2 (p = 0.023); in the control subjects, SVI increased (46.6 ± 7.6 vs 57.9 ± 11.8 ml·m-2, p = 0.001). After PEA, the SVI response (ΔSVI) improved from -2.8 ± 4.6 to 4.0 ± 4.6 ml·m2 (p <0.001; n = 17). On exercise at 40% of the postoperative maximal workload, SVI did not increase further and was still significantly lower compared with controls. Moreover, 4 patients retained a negative SVI response, despite (near) normalization of their pulmonary hemodynamics. The improvement in SVI response was accompanied by an increased exercise tolerance and restoration of RV remodeling. In conclusion, in CTEPH, exercise is limited by an impaired stroke volume response. PEA induces a restoration of SVI response to exercise that appears, however, incomplete and not evident in all patients.
Copyright © 2014 Elsevier Inc. All rights reserved.
  PMID: 24819907 [PubMed - as supplied by publisher]
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30. Am J Physiol Lung Cell Mol Physiol. 2014 May 9. [Epub ahead of print]

Progress In Solving The Sex Hormone Paradox In Pulmonary Hypertension.

Lahm T1Tuder RMPetrache I.
Author information: 
11Indiana University School of Medicine.

Abstract

Pulmonary arterial hypertension (PAH) is a devastating and progressive disease with marked morbidity and mortality. Even though female gender represents one of the most powerful risk factors for PAH, multiple questions about the underlying mechanisms remain, and two "estrogen paradoxes" in PAH exist. First, it is puzzling why estrogens have been found to be protective in various animal models of PAH, whereas PAH registries uniformly demonstrate a female susceptibility to the disease. Second, despite the pronounced tendency for the disease to develop in women, female PAH patients exhibit better survival than men. Recent mechanistic studies in classical as well as in novel animal models of PAH, as well as recent studies in PAH patients have significantly advanced the field. In particular, it is now accepted that estrogen metabolism and receptor signaling, as well as estrogen interactions with key pathways in PAH development, appear to be potent disease modifiers. A better understanding of these interactions may lead to novel PAH therapies. It is the purpose of this review to 1) review sex hormone synthesis, metabolism, and receptor physiology, 2) assess the content in which sex hormones affect PAH pathogenesis, 3) provide a potential explanation for the observed estrogen paradoxes and gender differences in PAH, and 4) identify knowledge gaps and future research opportunities. As the majority of published studies investigated 17beta-estradiol and/or its metabolites, this review will primarily focus on estrogen effects on the pulmonary vasculature and right ventricle. Data for other sex hormones will be discussed very briefly.
  PMID: 24816487 [PubMed - as supplied by publisher]
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31. Eur J Cardiothorac Surg. 2014 May 12. [Epub ahead of print]

Successful staged operation for acute aortic dissection and chronic thromboembolic pulmonary hypertension.

Ishida K1Masuda MIshizaka TMatsumiya G.
Author information: 
1Department of Cardiovascular Surgery, Chiba University Graduate School of Medicine, Chiba, Japan.

Abstract

We describe surgical treatment for a patient with chronic thromboembolic pulmonary hypertension who developed acute type A aortic dissection. Acute aortic dissection is a life-threatening disease and must be operated emergently, and chronic thromboembolic pulmonary hypertension can be treated only by pulmonary endarterectomy. We performed a staged procedure consisting of hemiarch replacement with antegrade cerebral perfusion first and pulmonary endareterectomy with periods of deep hypothermic circulatory arrest a week later. We used extracorporeal membrane oxygenation after aortic surgery as a bridge to pulmonary endarterectomy. Our strategy was useful for patients with chronic thromboembolic pulmonary hypertension who require complicated aortic surgery.
  PMID: 24819361 [PubMed - as supplied by publisher]
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32. Heart. 2014 May 14. pii: heartjnl-2014-305574. doi: 10.1136/heartjnl-2014-305574. [Epub ahead of print]

Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies.

D'Alto M1Diller GP2.
Author information: 
1Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy.
2Department of Cardiology and Angiology, Adult Congenital and Valvular Heart Disease Centre, University of Münster, Muenster, Germany.

Abstract

The presence of pulmonary arterial hypertension (PAH) increases morbidity and reduces survival in patients with congenital heart disease (CHD). PAH-CHD is a heterogeneous condition, depending on the type of the underlying defect and previous repair strategies. There is growing evidence of the benefits of PAH-specific therapy in the PAH-CHD population, but despite recent advances mortality rates remain relatively high. In the last years, an increasing focus has been placed on patients with PAH-CHD and net left-to-right shunt. Currently, there are limited data to guide the management of these patients and uncertainty on the cut-off values for eventual defect closure. Pregnancy conveys significant risks in PAH-CHD patients: appropriate counselling and care, including psychological support and a multidisciplinary team, should be part of the routine management of women with PAH-CHD of reproductive age. Some subgroups, such as patients with Down's syndrome, Fontan circulation and 'segmental' pulmonary hypertension, present particular challenges in terms of management and therapy. The current review focuses on contemporary treatment strategies in PAH-CHD patients with particular emphasis on challenging patient groups and conditions.
Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
  PMID: 24829371 [PubMed - as supplied by publisher]
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33. Chest. 2014 Apr 3. doi: 10.1378/chest.14-0527. [Epub ahead of print]

Pulmonary Hypertension Surveillance - United States, 2001-2010.

George MGSchieb LJAyala CTalwalkar ALevant S.

Abstract

ABSTRACT BACKGROUND:

Pulmonary hypertension (PH) is an uncommon but progressive condition. Much of what we know about PH comes from specialized disease registries. With expanding research into the diagnosis and treatment of PH, it is important to provide updated surveillance on the impact of this disease on hospitalizations and mortality. This study builds on previous PH surveillance of mortality and hospitalization.

METHODS:

This study analyzed mortality data from the National Vital Statistics System and data from the National Hospital Discharge Survey between 2001 and 2010. Pulmonary hypertension deaths were identified using ICD-10 codes I27.0, I27.2, I27.8, or I27.9 as any contributing cause of death on the death certificate. Hospital discharges associated with PH were identified using ICD-9-CM codes 416.0, 416.8, or 416.9 as one of up to seven listed medical diagnoses.

RESULTS:

The decline in death rates associated with pulmonary hypertension among males from 1980 to 2005 has reversed and now shows a significant increasing trend. Similarly, the death rates for women with pulmonary hypertension have continued to significantly increase during the past decade. Pulmonary hypertension-associated mortality rates for those aged 85 and older have accelerated compared to rates for younger age groups. There have been significant declines in pulmonary hypertension-associated mortality rates for those with pulmonary embolism and emphysema. Rates of hospitalization for pulmonary hypertension have significantly increased for both men and women during the past decade. For those aged 85 and older, hospitalization rates have nearly doubled.

CONCLUSIONS:

Continued surveillance helps us understand and address evolving trends in hospitalization and mortality associated with pulmonary hypertension and for pulmonary hypertension-associated conditions, especially for sex, age, and race/ethnicity disparities.
  PMID: 24700091 [PubMed - as supplied by publisher]
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34. Respiration. 2014;87(5):379-87. doi: 10.1159/000358565. Epub 2014 Apr 11.

Cardiopulmonary exercise testing to detect chronic thromboembolic pulmonary hypertension in patients with normal echocardiography.

Held M1Grün MHoll RHübner GKaiser RKarl SKolb MSchäfers HJWilkens HJany B.
Author information: 
1Department of Internal Medicine, Medical Mission Hospital, Academic Teaching Hospital, Julius Maximilian University of Würzburg, Würzburg, Germany.

Abstract

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious complication of pulmonary embolism (PE). Taking into account the reported incidence of CTEPH after acute PE, the number of patients with undiagnosed CTEPH may be high. Objectives: We aimed to determine if cardiopulmonary exercise testing (CPET) could serve as complementary tool in the diagnosis of CTEPH and can detect CTEPH in patients with normal echocardiography. Methods: At diagnosis, we analyzed the data of CPET parameters in 42 patients with proven CTEPH and 51 controls, and evaluated the performance of two scores. Results: VE/VCO2 slope, EQO2, EQCO2, P(A-a)O2, end-tidal partial pressure of CO2 at anaerobic threshold (PETCO2) and capillary to end-tidal carbon dioxide gradient [P(c-ET)CO2] were significantly different between patients with CTEPH and controls (p < 0.001). P(c-ET)CO2 was the single parameter with the highest sensitivity (85.7%) and specificity (88.2%). A score combining VE/VCO2 slope, P(A-a)O2, P(c-ET)CO2, PETCO2 [4-parameter-CPET (4-P-CPET) score] reached a sensitivity of 83.3% and a specificity of 92.2% after cross-validation. In 42 patients with CTEPH, echocardiography identified PH in 29 patients (69%), but it was normal in 13 patients (31%). All patients with normal or unmeasurable right ventricular systolic pressure had a pathological CPET. Twelve of the 13 patients (92%) were detected by both CPET scores. Conclusion: CPET is a useful noninvasive diagnostic tool for the detection of CTEPH in patients with suspected PH but normal echocardiography. The 4-P-CPET score provides a high sensitivity with the highest specificity. © 2014 S. Karger AG, Basel.
  PMID: 24732343 [PubMed - in process]
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35. Nurs Health Sci. 2014 Apr 15. doi: 10.1111/nhs.12138. [Epub ahead of print]

Impact of living with pulmonary hypertension: A qualitative exploration.

Yorke J1Armstrong IBundock S.
Author information: 
1University of Manchester, Manchester, UK.

Abstract

Little is known about the impact of living with pulmonary hypertension. This paper reports data exploring the experience of living with pulmonary hypertension. Qualitative, semistructured, one-to-one interviews were conducted in participants' homes to understand their experiences of living with pulmonary hypertension. Thematic analysis was used to identify codes and generate themes from the interview data. The identification of initial codes was conducted independently by the first author, and checked by the second. Thirty patients recruited through the pulmonary hypertension descriptions of living with pulmonary hypertension are presented under five themes that center on the invisibility of pulmonary hypertension, and its complex treatment are presented: (i) living with a hidden illness; (ii) being on a symptomology rollercoaster; (iii) expectations from treatments; (iv) treatment burden; and (v) awareness of financial burden of treatments. Key findings included daily challenges of living with a rare condition that is largely "hidden" and its related complex treatment regimes. People with pulmonary hypertension would benefit if more healthcare professionals, as well as family and friends, would validate their condition and provide them with appropriate support.
© 2014 Wiley Publishing Asia Pty Ltd.
  PMID: 24730424 [PubMed - as supplied by publisher]
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36. Arthritis Rheumatol. 2014 Apr 11. doi: 10.1002/art.38623. [Epub ahead of print]

Improved transplant-free survival in patients with systemic sclerosis-associated pulmonary hypertension and interstitial lung disease.

Volkmann ER1Saggar RKhanna DTorres BFlora AYoder LClements PJElashoff RMRoss DJAgrawal HBorazan NFurst DESaggar R.
Author information: 
1Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, U.S.A.

Abstract

Objective: Survival in patients with systemic sclerosis (SSc) associated pulmonary hypertension (PH) and interstitial lung disease (ILD) [SSc-PH-ILD] is poor. Evidence supporting the efficacy of aggressive PAH-targeted therapy in this population is limited. This study investigates transplant-free survival in patients with isolated SSc pulmonary arterial hypertension (SSc-PAH) and SSc-PH-ILD treated with aggressive PAH-targeted therapy. Methods: SSc patients with right heart catheterization (RHC)-diagnosed precapillary PH (mean pulmonary artery pressure ≥25 mmHg, pulmonary capillary wedge pressure ≤15 mmHg, and pulmonary vascular resistance ≥240 dynesxsecond/cm5 ) were included. Patients were classified as having ILD based on review of high-resolution computed tomography (HRCT) chest imaging and spirometry. Kaplan-Meier and Cox proportional hazards models were constructed to analyze survival and identify predictive variables. Results: Of 99 patients with SSc-precapillary PH, 28% had SSc-PAH and 72% had SSc-PH-ILD. The 1- and 2-year survival estimates were 72% and 59% versus 82% and 66% for the SSc-PH-ILD and SSc-PAH groups, respectively (p=0.5). Within 6 months of the diagnostic RHC, 24% of all patients started prostanoid therapy; an additional 24% started prostanoid therapy after 6 months. In the multivariate model, male gender (HR 0.6; p=0.008) and prostanoid therapy initiation within 6 months of the RHC (HR 1.4; p=0.007) were the only factors significantly associated with transplant-free survival, after accounting for the presence of ILD and severity of PH. Conclusion: Survival of patients with SSc-PH-ILD has modestly improved relative to historical series. While these findings may not be generalizable, improved survival may partly be due to aggressive PAH-targeted therapy. © 2014 American College of Rheumatology.
Copyright © 2014 American College of Rheumatology.
  PMID: 24729406 [PubMed - as supplied by publisher]
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37. J Reprod Med. 2014 Mar-Apr;59(3-4):181-4.

Pulmonary artery dissection leading to cardiac tamponade as a cause of maternal death in a woman with pulmonary hypertension: a case report.

Barrett JPRamlall AKirsch TArtiles-Martinez D.

Abstract

BACKGROUND:

Severe pulmonary hypertension in pregnancy is known to carry a 40% risk of death for the mother. The most common cause of death in cases of pulmonary hypertension is heart failure.

CASE:

We present a case of maternal death due to dissection of the pulmonary artery resulting in cardiac tamponade.

CONCLUSION:

The sudden onset of severe chest pain radiating to the back should alert the clinician to the possibility of pulmonary artery dissection in pregnant patients with pulmonary hypertension. Severe chest pain may not be accompanied by changes in vital signs or oxygen saturation. Immediate delivery should be considered. However, delivery may worsen the mother's condition due to postpartum cardiovascular changes.
  PMID: 24724229 [PubMed - indexed for MEDLINE]
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38. PLoS One. 2014 Apr 11;9(4):e94587. doi: 10.1371/journal.pone.0094587. eCollection 2014.

A new era of therapeutic strategies for chronic thromboembolic pulmonary hypertension by two different interventional therapies; pulmonary endarterectomy and percutaneous transluminal pulmonary angioplasty.

Inami T1Kataoka M2Ando M3Fukuda K4Yoshino H1Satoh T1.
Author information: 
1Division of Cardiology, Second Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.
2Division of Cardiology, Second Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan; Department of Cardiology, Keio University School of Medicine, Tokyo, Japan.
3Department of Cardiovascular Surgery, Fujita Health University, Aichi, Japan.
4Department of Cardiology, Keio University School of Medicine, Tokyo, Japan.

Abstract

BACKGROUND:

Pulmonary endarterectomy (PEA) is established for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Recently, percutaneous transluminal pulmonary angioplasty (PTPA) has been added for peripheral-type CTEPH, whose lesions exist in segmental, subsegmental, and more distal pulmonary arteries. A shift in clinical practice of interventional therapies occurred in 2009 (first mainly PEA, later PTPA). We examined the latest clinical outcomes of patients with CTEPH.

METHODS AND RESULTS:

This study retrospectively included 136 patients with CTEPH. Twenty-nine were treated only with drug (Drug-group), and the other 107 underwent interventional therapies (Interventions-group) (39 underwent PEA [PEA-group] and 68 underwent PTPA [PTPA-group]). Total 213 PTPA sessions (failures, 0%; mortality rate, 1.47%) was performed in the PTPA-group (complications: reperfusion pulmonary edema, 7.0%; hemosputum or hemoptysis, 5.6%; vessel dissection, 2.3%; wiring perforation, 0.9%). Although baseline hemodynamic parameters were significantly more severe in the Interventions-group, the outcome after the diagnosis was much better in the Interventions-group than in the Drug-group (98% vs. 64% 5-year survival, p<0.0001). Hemodynamic improvement in the PEA-group was a 46% decrease in mean pulmonary arterial pressure (PAP) and a 49% decrease in total pulmonary resistance (TPR) (follow-up period; 74.7±32.3 months), while those in the PTPA-group were a 40% decrease in mean PAP and a 49% decrease in TPR (follow-up period; 17.4±9.3 months). The 2-year survival rate in the Drug-group was 82.0%, and the 2-year survival rate, occurrence of right heart failure, and re-vascularization rate in the PEA-group were 97.4%, 2.6%, and 2.8%, and those in the PTPA-group were 98.5%, 2.9%, and 2.9%, respectively.

CONCLUSION:

The patients who underwent interventional therapies had better results than those treated only with drugs. The availability of both of these operative and catheter-based interventional therapies leads us to expect the dawn of a new era of therapeutic strategies for CTEPH.
PMCID: PMC3984177 Free PMC Article
  PMID: 24728482 [PubMed - in process]
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