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Exercise training in pulmonary arterial hypertension associated with connective tissue diseases. #20

- Combination therapy in pulmonary arterial hypertension. #22

An update on the use of ambrisentan in pulmonary arterial hypertension. #10
Managing pulmonary arterial hypertension and optimizing treatment options: prognosis of pulmonary artery hypertension. #23
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1. Acute Card Care. 2013 May 10. [Epub ahead of print]

A giant right-sided heart due to idiopathic pulmonary hypertension.

Ghadri JRGstrein CLüscher TFTemplin C.
Department of Cardiology, Cardiovascular Center, University Hospital Zurich , Zurich , Switzerland.
  PMID: 23663056 [PubMed - as supplied by publisher]
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2. Pulm Circ. 2013 Jan;3(1):252-66. doi: 10.4103/2045-8932.109931.

Clinical trials in neonates and children: Report of the pulmonary hypertension academic research consortium pediatric advisory committee.

Adatia IHaworth SGWegner MBarst RJIvy DStenmark KRKarkowsky ARosenzweig EAguilar C.
Stollery Children's Hospital, University of Alberta, Edmonton, Canada.


Drug trials in neonates and children with pulmonary hypertensive vascular disease pose unique but not insurmountable challenges. Childhood is defined by growth and development. Both may influence disease and outcomes of drug trials. The developing pulmonary vascular bed and airways may be subjected to maldevelopment, maladaptation, growth arrest, or dysregulation that influence the disease phenotype. Drug therapy is influenced by developmental changes in renal and hepatic blood flow, as well as in metabolic systems such as cytochrome P450. Drugs may affect children differently from adults, with different clearance, therapeutic levels and toxicities. Toxicity may not be manifested until the child reaches physical, endocrine and neurodevelopmental maturity. Adverse effects may be revealed in the next generation, should the development of ova or spermatozoa be affected. Consideration of safe, age-appropriate tablets and liquid formulations is an obvious but often neglected prerequisite to any pediatric drug trial. In designing a clinical trial, precise phenotyping and genotyping of disease is required to ensure appropriate and accurate inclusion and exclusion criteria. We need to explore physiologically based pharmacokinetic modeling and simulations together with statistical techniques to reduce sample size requirements. Clinical endpoints such as exercise capacity, using traditional classifications and testing cannot be applied routinely to children. Many lack the necessary neurodevelopmental skills and equipment may not be appropriate for use in children. Selection of endpoints appropriate to encompass the developmental spectrum from neonate to adolescent is particularly challenging. One possible solution is the development of composite outcome scores that include age and a developmentally specific functional classification, growth and development scores, exercise data, biomarkers and hemodynamics with repeated evaluation throughout the period of growth and development. In addition, although potentially costly, we recommend long-term continuation of blinded dose ranging after completion of the short-term, double-blind, placebo-controlled trial for side-effect surveillance, which should include neurodevelopmental and peripubertal monitoring. The search for robust evidence to guide safe therapy of children and neonates with pulmonary hypertensive vascular disease is a crucial and necessary goal.
PMCID: PMC3641736 Free PMC Article
  PMID: 23662203 [PubMed]
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3. Pulm Circ. 2013 Jan;3(1):245-51. doi: 10.4103/2045-8932.109922.

Clinical trials in pulmonary hypertension: Time for a consortium.

Newman JHElliott GCHaworth GSZampaglione EBrar SGibbs SJSandoval J.
Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.


Current and past clinical trials in pulmonary hypertension, while valuable, are limited by the absence of mechanistic aims, by dissatisfaction with endpoints and the inability to share data. Clinical studies in pulmonary hypertension might be enhanced by a consortium approach that utilizes the expertise of academic medicine, the treatment initiatives of the pharmaceutical industry and study design from funding agencies interested in biological mechanisms. A meeting of interested parties, the Pulmonary Hypertension Academic Research Consortium (PHARC), was held from 30 April to 1 May 2012 in Bethesda, Maryland. Members at the conference were from the USA Federal Drug Administration (FDA); pharmaceutical industry (Pfizer, Novartis, Bayer and Gilead); USA National Institutes of Health (NHLBI); the Pulmonary Vascular Research Institute (PVRI), a non-governmental organization (NGO); and research and clinical members of pulmonary hypertension programs of international scope. A recommendation to develop a clinical trials consortium was the product of the working group on academic standards in clinical trials. The working group concluded that clinical trials hold immense promise to move the field of pulmonary hypertension forward if the trials are designed by a consortium with input from multiple groups. This would result in study design, conduct and analysis determined by consortium members with a high degree of independent function. The components of a well-balanced consortium that give it scientific effectiveness are: (1) the consortium can work with multiple companies simultaneously; (2) sponsors with special interests, such as testing biological mechanisms, can add investigations to a study at lower cost than with present granting strategies; (3) data handling including archiving, analysis and future sharing would be improved; (4) ancillary studies supported by the collection and dissemination of tissues and fluids would generate a broader approach to discovery than is now possible; and (5) development of improved endpoints in consultation with regulatory agencies, industry and academia would be possible.
PMCID: PMC3641735 Free PMC Article
  PMID: 23662202 [PubMed]
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4. Pulm Circ. 2013 Jan;3(1):203-5. doi: 10.4103/2045-8932.109914.

The pulmonary hypertension academic research consortium.

Rich S.
University of Chicago, Chicago, Illinois, USA.
PMCID: PMC3641731 Free PMC Article
  PMID: 23662198 [PubMed]
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5. Pulm Circ. 2013 Jan;3(1):128-36.

Mechanics and mechanisms of pulmonary hypertension-Conference summary and translational perspectives.

Seeger WPullamsetti SS.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Department of Lung Development and Remodeling, Max-Planck-Institute for Heart and Lung Research, Bad Nauheim, Germany.
PMCID: PMC3641720 Free PMC Article
  PMID: 23662189 [PubMed]
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6. Pulm Circ. 2013 Jan;3(1):121-2. doi: 10.4103/2045-8932.109927.

Should cardiopulmonary exercise testing become a part of regular evaluation for patients with a family history of pulmonary hypertension? Regarding "Cardiopulmonary exercise testing reveals onset of disease and response to treatment in a case of heritable pulmonary arterial hypertension".

Babu ASArena RMaiya AGPadmakumar RGuazzi M.
Department of Physiotherapy, Manipal College of Allied Health Sciences, Manipal University, Manipal, Karnataka, India
PMCID: PMC3641715 Free PMC Article
  PMID: 23662184 [PubMed]
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7. Pulm Circ. 2013 Jan;3(1):100-7. doi: 10.4103/2045-8932.109923.

Supplementation of iron in pulmonary hypertension: Rationale and design of a phase II clinical trial in idiopathic pulmonary arterial hypertension.

Howard LSWatson GMWharton JRhodes CJChan KKhengar RRobbins PAKiely DGCondliffe RElliott CAPepke-Zaba JSheares KMorrell NWDavies RAshby DGibbs JS,Wilkins MR.
Department of Cardiology, National Pulmonary Hypertension Service, Hammersmith Hospital, London, United Kingdom ; National Heart and Lung Institute, London, United Kingdom.


Our aim is to assess the safety and potential clinical benefit of intravenous iron (Ferinject) infusion in iron deficient patients with idiopathic pulmonary arterial hypertension (IPAH). Iron deficiency in the absence of anemia (1) is common in patients with IPAH; (2) is associated with inappropriately raised levels of hepcidin, the key regulator of iron homeostasis; and (3) correlates with disease severity and worse clinical outcomes. Oral iron absorption may be impeded by reduced absorption due to elevated hepcidin levels. The safety and benefits of parenteral iron replacement in IPAH are unknown. Supplementation of Iron in Pulmonary Hypertension (SIPHON) is a Phase II, multicenter, double-blind, randomized, placebo-controlled, crossover clinical trial of iron in IPAH. At least 60 patients will be randomized to intravenous ferric carboxymaltose (Ferinject) or saline placebo with a crossover point after 12 weeks of treatment. The primary outcome will be the change in resting pulmonary vascular resistance from baseline at 12 weeks, measured by cardiac catheterization. Secondary measures include resting and exercise hemodynamics and exercise performance from serial bicycle incremental and endurance cardiopulmonary exercise tests. Other secondary measurements include serum iron indices, 6-Minute Walk Distance, WHO functional class, quality of life score, N-terminal pro-brain natriuretic peptide (NT-proBNP), and cardiac anatomy and function from cardiac magnetic resonance. We propose that intravenous iron replacement will improve hemodynamics and clinical outcomes in IPAH. If the data supports a potentially useful therapeutic effect and suggest this drug is safe, the study will be used to power a Phase III study to address efficacy.
PMCID: PMC3641712 Free PMC Article
  PMID: 23662181 [PubMed]
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8. Pulm Circ. 2013 Jan;3(1):20-30. doi: 10.4103/2045-8932.109911.

Nitric oxide deficiency in pulmonary hypertension: Pathobiology and implications for therapy.

Tonelli ARHaserodt SAytekin MDweik RA.
Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland, Ohio, USA.


Nitric oxide (NO) is a diffusible gas with diverse roles in human physiology and disease. Significant progress in the understanding of its biological effects has taken place in recent years. This has led to a better understanding of the pathobiology of pulmonary hypertension (PH) and the development of new therapies. This article provides an overview of the NO physiology and its role in the pathobiology of lung diseases, particularly PH. We also discuss current and emerging specific treatments that target NO signaling pathways in PH.
PMCID: PMC3641730 Free PMC Article
  PMID: 23662172 [PubMed]
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9. Thromb Haemost. 2012 Dec;108(6):1049-60. doi: 10.1160/TH11-11-0821. Epub 2012 Sep 5.

Development of prognostic tools in pulmonary arterial hypertension: lessons from modern day registries.

Benza RLGomberg-Maitland MFrost AEFrantz RPHumbert MMcGoon MD.
The Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, PA 15212, USA.


Pulmonary arterial hypertension (PAH) is characterised by increased pressure in the pulmonary arteries leading to right-sided ventricular failure, and death. Identification of factors that affect patient survival is important to improve patient management and outcomes. The first registry to evaluate survival and develop a prognostic model was the National Institutes of Health (NIH) registry in 1981. Importantly this prognostic model is based on data collected prior to availability of PAH-targeted therapies and does not reflect survival rates for treated patients. Since the 1980s, however, four modern registries of PAH now exist which compensate for the NIH equations shortcomings and include the French National registry, Pulmonary Hypertension Connection registry, the Mayo registry, and the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL). The similarities and difference in these registries are highlighted in this review and although similar in many respects, the four registries vary in patient population, including the numbers of newly and previously diagnosed patients, as well as the era of observation, period of survival, and timing of assessment of potential predictive factors. Despite this, the predictive factors identified in each registry and described in detail within the body of this manuscript share surprising homology in that disease aetiology, patient gender and factors reflective of right heart failure are integral in depicting survival. Future modifications of modern prognostic equations should be an ongoing goal of the PAH community in order to provide increased accuracy with identification of novel risk factors and prediction of disease course.
  PMID: 22955290 [PubMed - indexed for MEDLINE]
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10. Ther Adv Respir Dis. 2012 Dec;6(6):331-43. doi: 10.1177/1753465812458014. Epub 2012 Aug 29.

An update on the use of ambrisentan in pulmonary arterial hypertension.

D'Alto M.
Department of Cardiology, Second University of Naples, Monaldi Hospital, Piazzale E. Ruggieri, Naples, Italy.


The development of effective oral treatments that are capable of modulating the activity of endothelin receptor 1 (ET-1) represents a significant milestone in the field of pulmonary arterial hypertension (PAH). Randomized clinical trials confirm that endothelin receptor antagonist (ERA) treatments confer significant improvements on important clinical endpoints, such as exercise capacity, functional class, quality of life and pulmonary hemodynamics. Moreover, ERAs may prevent or delay clinical worsening and retard disease progression. Ambrisentan is a propanoic acid-based ERA, showing preferential affinity for the type A ET-1 over the type B receptor. It provides another valuable, effective treatment option in PAH. Two large, randomized-placebo controlled trials demonstrated the efficacy of ambrisentan in PAH at improving exercise tolerance as measured by the 6 min walk distance. Additional secondary measures of improvement including time to clinical worsening, survival, functional class, quality of life and hemodynamic variables have been reported in clinical trials. A favorably low incidence of aminotransferase elevation indicating lower hepatic toxicity than other ERAs has been observed. Ambrisentan can be safely administered with warfarin or sildenafil without the need for dose adjustment of either therapy. A once daily oral medication with relatively few side effects is an attractive option, especially as the use of therapies in combination continues to increase. Long-term data and hemodynamic data confirm the benefits can be compared with other ERAs with fewer drug-drug interactions and a better liver safety profile.
  PMID: 22933513 [PubMed - indexed for MEDLINE]
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11. Congenit Heart Dis. 2012 Nov-Dec;7(6):551-8. doi: 10.1111/j.1747-0803.2012.00674.x. Epub 2012 May 22.

Cardiac medical conditions have become the leading cause of death in children with heart disease.

Schlingmann TRThiagarajan RRGauvreau KLofgren KCZaplin MConnor JAdel Nido PJLock JEJenkins KJ.
Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Mass, USA.



Mortality among children with congenital and acquired heart disease has decreased significantly over the past decades. We sought to determine whether the underlying problems leading to death in these patients had changed over the past decade.


We reviewed medical records for 100 deaths of cardiac patients in 2004-2005 and 100 deaths in 1995-1996. Demographic, clinical, and procedural data as well as circumstances of death were collected. A consensus committee reviewed each case and sought to identify the condition leading to death. These conditions were classified as predominantly surgical or medical.


General patient characteristics (age, gender, cardiac history, comorbidities, proportion of surgical patients) did not change significantly between the two time periods. However, in 1995-1996, 64% of deceased surgical patients had died within 30 days of surgery. This rate was nearly halved to only 38% by 2004-2005 (P= .003). Furthermore, the conditions leading to death changed significantly: 51% of patient deaths in 1995-1996 resulted from a surgical problem, 29% from a medical condition. This ratio was reversed in 2004-2005: Only 31% of patient deaths were due to a surgical problem, while 50% of deaths resulted from a medical condition (P= .005). The most common medical conditions resulting in death were pulmonary vein stenosis, pulmonary arterial hypertension, and primary myocardial failure.


The proportion of deaths within 30 days of cardiac surgery decreased significantly over the past decade. While surgical causes accounted for the majority of these deaths in 1995-1996, most patient deaths in 2004-2005 resulted from cardiac medical causes.
© 2012 Wiley Periodicals, Inc.
  PMID: 22612795 [PubMed - indexed for MEDLINE]
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12. Am J Respir Crit Care Med. 2013 May 15;187(10):1139-40. doi: 10.1164/rccm.201301-0109LE.

Reply: the Renin-Angiotensin system in pulmonary hypertension.

de Man FSVonk Noordegraaf AHumbert MGuignabert C.
VU University Medical Center Amsterdam Amsterdam, The Netherlands.
  PMID: 23675720 [PubMed - in process]
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13. Am J Respir Crit Care Med. 2013 May 15;187(10):1138-9. doi: 10.1164/rccm.201210-1872LE.

The Renin-Angiotensin system in pulmonary hypertension.

Morrell NWStenmark KR.
University of Cambridge School of Clinical Medicine Cambridge, United Kingdom.
  PMID: 23675718 [PubMed - in process]
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14. Chest. 2013 May 9. doi: 10.1378/chest.12-2659. [Epub ahead of print]


Sztrymf BGünther SArtaud-Macari ESavale LJaïs XSitbon OSimonneau GHumbert MChemla D.



Novel non-invasive tools may improve the management of patients with pulmonary arterial hypertension (PAH) experiencing heart failure. Major right ventricle overload leads to decreased stroke volume, which shortens left ventricular ejection time (LVET). Our arterial tonometry study tested the hypothesis that LVET carries prognostic value in PAH patients with heart failure.


Clinical, biological and radial artery tonometry variables were prospectively obtained at admission and at day 3-5 in 53 consecutive PAH patients admitted in our ICU for clinical deterioration. LVET was measured from the reconstructed aortic pressure curve.


Overall ICU mortality (median stay 5 days) was 17% and 28%. At admission, the LVET was shorter in patients with unfavourable outcome (median 228 ms (212-278) vs 257 ms (237-277), p=0.032), while other tonometric indices were similar. The LVET at entry (237 ms) had 73% sensitivity and 89% specificity for identifying death in the ICU. Other prognostic factors at admission were higher serum levels of brain natriuretic peptide (BNP) and creatinine, and lower natremia. Dobutamine requirement, higher furosemide dose and higher oxygen flow were associated with unfavourable outcome. At the second evaluation, higher serum level of creatinine and BNP, higher furosemide dose and oxygen flow, and dobutamine or norepinephrine requirement were associated with poor outcome. The change in LVET between admission and follow-up measurement was not associated with outcome. The 90-day mortality was 28%.


Shortened LVET at ICU admission was a prognostic factor in PAH with heart failure. Previously documented prognostic factors were also confirmed in this cohort.
  PMID: 23670726 [PubMed - as supplied by publisher]
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15. Am J Med Sci. 2013 May 17. [Epub ahead of print]

Risk of Death and Need for Transplantation in Patients With Chronic Pulmonary Hypertension.

López-Candales A.
Division of Cardiovascular Diseases, University of Cincinnati, Cincinnati, Ohio.


BACKGROUND:: Echo-Doppler parameters that exemplify right ventricular (RV) outflow dynamics and measures of annular tissue Doppler imaging to assess left ventricular (LV) and RV diastolic function, known to be affected in chronic pulmonary hypertension (cPH), have never been studied to determine if they could be predictive of mortality or need for transplantation 1-year after follow-up. METHODS:: Numerous echo-Doppler parameters of RV and LV performance were recorded from 120 patients. This patient population was divided into 3 groups. Group I had no PH, group II had cPH but no documented death or need for either lung or heart transplantation, at 1-year follow-up after their initial echocardiogram whereas group III had cPH and patients had either died or required heart and/or lung transplantation during the same time period. RESULTS:: Analysis of variance was first used to identify which echo-Doppler variables were significant among the studied groups. A logistic regression analysis was then performed to identify predictive variables of the occurrence death and need for transplantation. Finally, a multiple regression analysis was used between groups II and III to identify which echo-Doppler variables were most useful in identifying severe cPH patients at risk of the prespecified events. CONCLUSIONS:: Even though older patients with worse RV fractional area change might be considered at risk of worse prognosis in patients with severe cPH, only a low mitral annular early diastolic velocity was useful in identifying which of those individuals were at highest risk of death or in need of transplantation.
  PMID: 23689051 [PubMed - as supplied by publisher]
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16. J Pediatr (Rio J). 2013 Apr 26. pii: S0021-7557(13)00059-4. doi: 10.1016/j.jped.2012.11.009. [Epub ahead of print]

Persistent pulmonary hypertension of the newborn: recent advances in pathophysiology and treatment.

Cabral JEBelik J.
Neonatologist, Hospital São Luiz, São Paulo, SP, Brazil. Electronic address:



Although recognized for decades, little is known about the etiology, physiopathology, and prevention of persistent pulmonary hypertension of the newborn (PPHN). and its treatment remains a major challenge for neonatologists. In this review, the clinical features and physiopathology of the syndrome will be addressed, as well as its general and specific treatments.


A review was carried out in PubMed, Cochrane Library, and MRei consult databases, searching for articles related to the syndrome and published between 1995 and 2011.


Risk factors and the physiopathological mechanisms of the syndrome are discussed. The clinical picture depends on the different factors involved, which are probably related to the etiology and physiopathological mechanisms. In addition to the measures used to allow the decrease in pulmonary vascular resistance after birth, some cases will require pulmonary vasodilators. Although nitric oxide has proved effective, other vasodilators have been recently used, but clinical evidence is still lacking to demonstrate their benefits in the treatment of PPHN.


Despite recent technological advances and new physiopathological knowledge, mortality associated with PPHN remains at 10%. More clinical research and evidence-based experimental results are needed to prevent, treat, and reduce the morbidity/mortality associated with this neonatal syndrome.
Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.
Free Article
  PMID: 23684454 [PubMed - as supplied by publisher]
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17. Lung. 2013 May 17. [Epub ahead of print]

Exercise Capacity Affects Quality of Life in Patients with Pulmonary Hypertension.

Halank MEinsle FLehman SBremer HEwert RWilkens HMeyer FJGrünig ESeyfarth HJKolditz MWieder GHöffken GKöllner V.
Department of Internal Medicine I, Carl Gustav Carus University Hospital, Technical University of Dresden, Fetscherstrasse 74, 01309, Dresden, Germany,



The objective of this prospective study was to evaluate the impact of exercise capacity, mental disorders, and hemodynamics on quality-of-life (QoL) parameters in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).


Sixty-three patients with invasively diagnosed PAH (n = 48) or CTEPH (n = 15) underwent a broad panel of assessments, including cardiopulmonary exercise testing (CPET), 6-minute walking distance (6-MWD), World Health Organization functional class (WHO-FC), and assessment of hemodynamics. QoL was evaluated by the 36-item Medical Outcome Study Short Form Health Survey Questionnaire (SF-36). Exercise capacity, hemodynamics, age, gender, and mental disorders (anxiety and depression) were assessed for association with QoL subscores by uni- and multivariate regression analyses.


Exercise capacity, WHO-FC, oxygen therapy, symptoms of right heart failure, right atrial pressure, and mental disorders were significantly associated with QoL (p < 0.05). In the stepwise backward selection multivariate analysis, depression remained an independent parameter in seven of eight subscales of the SF-36. Furthermore, peak oxygen uptake (peakVO2) during CPET, 6-MWD, anxiety, long-term oxygen therapy, right heart failure, and age remained independent factors for QoL. Hemodynamic parameters at rest did not independently correlate with any domain of the SF-36 QoL subscores.


Mental disorders, exercise capacity, long-term oxygen therapy, right heart failure, and age play important role in the quality of life in patients with PAH and CTEPH.
  PMID: 23681593 [PubMed - as supplied by publisher]
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18. Arq Bras Cardiol. 2012 Nov;99(5):1049-55. Epub 2012 Nov 9.

Study of breathing pattern and thoracoabdominal movement in mitral valve disease.

[Article in English, Portuguese]
Franco SSBardi PNGrinberg MFeltrim MI.
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Brasil.



patients with mitral valve disease can progress to having pulmonary congestion, which increases the work the respiratory muscles. This overload can change the breathing pattern with a predominance of rib cage displacement or presence of paradoxical movements.


a) to study the breathing pattern and thoracoabdominal movement of patients with mitral valve disease; b) to study the effect of body position on breathing parameters; and c) to correlate pulmonary hypertension with lack of coordination of thoracoabdominal movement.


the breathing pattern and thoracoabdominal movement of patients with mitral valve disease were assessed using respiratory inductive plethysmography during quiet breathing in the dorsal decubitus and sitting positions for two minutes. The variables assessed were tidal volume, breathing time and thoracoabdominal movement.


of the 65 patients selected, 10 were excluded, 29 were in the mitral stenosis group and 26 in the mitral regurgitation group. Tidal volume, pulmonary ventilation and mean inspiratory flow significantly increased in the sitting position, with no difference between the groups. The thoracoabdominal movement remained coordinated in all groups and positions; except for five patients in the dorsal decubitus position, who lacked coordination (three in the mitral stenosis group; two in the mitral regurgitation group). A significant correlation with pulmonary artery pressure values was observed (r = 0.992; p = 0.007).


No difference in breathing pattern or thoracoabdominal movement was found between patients with mitral stenosis and regurgitation. The sitting position increased tidal volume without altering breathing times. The lack of coordination of the thoracoabdominal movement in the dorsal decubitus position was associated with pulmonary hypertension.
Free Article
  PMID: 23138669 [PubMed - indexed for MEDLINE]
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19. Lung. 2012 Dec;190(6):645-9. doi: 10.1007/s00408-012-9425-5. Epub 2012 Oct 11.

Pulmonary arterial hypertension in the elderly-clinical characteristics and long-term survival.

Shimony AFox BDAfilalo JRudski LGHirsch ALangleben D.
Center for Pulmonary Vascular Disease and Divisions of Cardiology and Respirology, Lady Davis Institute for Medical Research, Jewish General Hospital, McGill University, Montreal, QC, Canada.



Recent registries describe a significant prevalence of pulmonary arterial hypertension (PAH) in the elderly, but little is known of their characteristics. We aimed to examine the features and prognostic factors of long-term survival in elderly (≥65 years) PAH patients.


Clinical, echocardiographic, angiographic, hemodynamic, treatments, and survival data were reviewed in consecutive patients over the course of 20 years. Elderly PAH patients (n = 47) were compared to younger PAH patients (n = 107).


At presentation, elderly patients were more likely to have hypertension, diabetes, dyslipidemia, coronary disease, and PAH associated with scleroderma (42.6 vs. 24.3 %; p = 0.02) than younger patients. Prior to PAH therapy, elderly patients had better right ventricular myocardial performance index (RV-MPI; 0.48 ± 0.20 vs. 0.62 ± 0.23, p = 0.006) and lower mean pulmonary arterial pressure (PAP; 45.0 ± 11.1 vs. 49.2 ± 11.8 mmHg, p = 0.04). Elderly patients were treated less often with epoprostenol (8.5 vs. 29 %, p = 0.006) or trepostinil (8.5 vs. 23.4 %, p = 0.04). The 1, 3, and 5 year survival rates of elderly patients were estimated to be 76.4, 50.5, and 37.6 %, respectively. In comparison, younger patients had survival estimates of 92.2, 74.2 and 64.0 % (p = 0.002). Baseline right atrial pressure, mean PAP, cardiac index, and RV-MPI were associated with survival in elderly patients; however in these patients, survival was not affected by any PAH subgroup or age (per year) by itself.


The diagnosis of PAH in elderly patients is associated with poorer survival which is in part explained by a greater vulnerability to the hemodynamic disturbances of PAH.
  PMID: 23064491 [PubMed - indexed for MEDLINE]
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20. Arthritis Res Ther. 2012 Jun 18;14(3):R148. doi: 10.1186/ar3883.

Exercise training in pulmonary arterial hypertension associated with connective tissue diseases.

Grünig EMaier FEhlken NFischer CLichtblau MBlank NFiehn CStöckl FPrange FStaehler GReichenberger FTiede HHalank MSeyfarth HJWagner SNagel C.
Centre for Pulmonary Hypertension at Thoraxclinic Heidelberg, Thoraxclinic at the University Hospital Heidelberg, Amalienstrasse 5, Heidelberg, 69126, Germany.



The objective of this prospective study was to assess short- and long-term efficacy of exercise training (ET) as add-on to medical therapy in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH).


Patients with invasively confirmed CTD-APAH received ET in-hospital for 3 weeks and continued at home for 12 weeks. Efficacy parameters have been evaluated at baseline and after 15 weeks by blinded-observers. Survival rate has been evaluated in a follow-up period of 2.9 ± 1.9 years.


Twenty-one consecutive patients were included and assessed at baseline, and after 3 weeks, 14 after 15 weeks. Patients significantly improved the mean distance walked in 6 minutes compared to baseline by 67 ± 52 meters after 3 weeks (p < 0.001) and by 71 ± 35 meters after 15 weeks (p = 0.003), scores of quality of life (p < 0.05), heart rate at rest, peak oxygen consumption, oxygen saturation and maximal workload. Systolic pulmonary artery pressure and diastolic systemic blood pressure improved significantly after 3 weeks of ET. The 1- and 2-year overall-survival rates were 100%, the 3-year survival 73%. In one patient lung transplantation was performed 6 months after ET.


ET as add-on to medical therapy is highly effective in patients with CTD-APAH to improve work capacity, quality of life and further prognostic relevant parameters and possibly improves the 1-, 2- and 3-year survival rate. Further randomized controlled studies are needed to confirm these results.

PMCID: PMC3446533 Free PMC Article
  PMID: 22709477 [PubMed - indexed for MEDLINE]
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21. Am J Cardiol. 2013 Apr 16;111(8 Suppl):21C-4C. doi: 10.1016/j.amjcard.2013.01.321.

When to initiate intravenous therapy and/or refer.

Champion HC.
Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pennsylvania Medical Center Montefiore Hospital, Vascular Medicine Institute, University of Pittsburgh/UPMC, Pittsburgh, Pennsylvania, USA.


Intravenous (IV) prostacyclin (epoprostanol) and its analogs (iloprost and treprostinil) are effective in treating pulmonary artery hypertension (PAH). Although prostacyclins are available for inhaled and subcutaneous delivery, IV administration of prostacyclins, sometimes in combination with other agents, such as bosentan or sildenafil, is considered the most aggressive method to manage PAH. This report attempts to help clinicians determine when to initiate IV treatment of PAH and when to refer a patient with PAH to a center for treatment. IV prostacyclin therapy initiation is suggested when patients exhibit World Health Organization functional class IV symptoms. The Registry to EValuate Early And Long-term Pulmonary Arterial Hypertension disease management (REVEAL) risk calculator can help determine a patient's 1-year mortality with PAH and characterize the clinical course, treatment, and predictors of outcomes in patients with PAH. Referring physicians can screen their patients for PAH and refer even before the diagnosis has been confirmed so that the center can facilitate the diagnostic process and provide suggestions for initial therapy selection and provide other collaborative and supportive services. Alternatively, the physician can diagnose and initiate early therapy with a plan to involve the pulmonary hypertension center at the need for IV therapy or consideration for transplantation, working closely with the patient to ensure stability. Physicians and pulmonary centers must develop good methods of communication to ensure effective diagnosis and management.
Copyright © 2013. Published by Elsevier Inc.
  PMID: 23558027 [PubMed - indexed for MEDLINE]
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22. Am J Cardiol. 2013 Apr 16;111(8 Suppl):16C-20C. doi: 10.1016/j.amjcard.2013.01.320.

Combination therapy in pulmonary arterial hypertension.

Channick RN.
Pulmonary Hypertension and Thromboendarterectomy Program, Massachusetts General Hospital, Department of Medicine, Harvard Medical School, Boston, Massachusetts, USA.


Many potential therapeutic options are now available for patients with pulmonary arterial hypertension. Interest has emerged in using therapies in various combinations. Retrospective experience has suggested that this approach is common and can be efficacious. Data are emerging supporting the benefit of combination therapy; however, limitations and questions remain about this strategy. This report reviewed the rationale for combination therapy and summarized the results from clinical trials.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 23558025 [PubMed - indexed for MEDLINE]
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23. Am J Cardiol. 2013 Apr 16;111(8 Suppl):10C-5C. doi: 10.1016/j.amjcard.2013.01.319.

Managing pulmonary arterial hypertension and optimizing treatment options: prognosis of pulmonary artery hypertension.

McLaughlin V.
Department of Internal Medicine, University of Michigan Cardiovascular Center, Ann Arbor, MI, USA.


Survival in patients with pulmonary artery hypertension has improved, but outcomes are still suboptimal. Therapeutic focus must shift from short-term functional changes to improvements in long-term outcomes. Several outcome predictors, both at baseline and on therapy, offer guidance for clinicians treating pulmonary artery hypertension.
Copyright © 2013 Elsevier Inc. All rights reserved.
  PMID: 23558024 [PubMed - indexed for MEDLINE]
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24. Am J Physiol Heart Circ Physiol. 2013 May 17. [Epub ahead of print]


Mackenzie Ross RVToshner MRSoon ENaeije RPepke-Zaba J.
1Papworth Hospital.


This study analysed the relationship between pulmonary vascular resistance (PVR) and compliance (Ca) in patients with idiopathic pulmonary arterial hypertension (IPAH) and proximal chronic thromboembolic pulmonary hypertension (CTEPH). It has recently been shown that the time constant of the pulmonary circulation (RC-time), or PVR x Ca, remains unaltered in various forms and severities of PH, with the exception of left heart failure. We reasoned that increased wave reflection in proximal CTEPH would be another cause of decreased RC-time. We conducted a retrospective analysis of invasive pulmonary hemodynamic measurements in IPAH (n=78), proximal CTEPH (n=91) before and after pulmonary endarterectomy (PEA) and distal CTEPH (n=53). Proximal CTEPH was defined by a postoperative mean pulmonary artery pressure (mPAP) ≤ 25 mmHg. Outcome measures were the RC-time, PVR, Ca and the relationship between systolic and mean pulmonary artery pressures. The RC time for Pre-PEA CTEPH was 0.49 ± 0.11s compared with Post PEA-CTEPH 0.37 ± 0.11s (p<0.0001), IPAH 0.63 ± 0.14s (p<0.001) and Distal CTEPH 0.55 ± 0.12s (p<0.05). A shorter RC-time was associated with a disproportionate decrease in systolic PAP with respect to mPAP. We concluded that the pulmonary RC-time is decreased in proximal CTEPH compared to IPAH, before and after PEA, which may be explained by increased wave reflection but also importantly by persistent structural changes after removal of proximal obstructions. A reduced RC-time in CTEPH is in accord with a wider pulse pressure and hence greater RV work for a given mean PA pressure.
  PMID: 23686712 [PubMed - as supplied by publisher]
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