Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era

M Humbert, O Sitbon, A Chaouat, M Bertocchi… - Circulation, 2010 - Am Heart Assoc
Background—Novel therapies have recently become available for pulmonary arterial
hypertension. We conducted a study to characterize mortality in a multicenter prospective …

Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United …

Y Ling, MK Johnson, DG Kiely, R Condliffe… - American journal of …, 2012 - atsjournals.org
Rationale: Incident pulmonary arterial hypertension was underrepresented in most
pulmonary hypertension registries and may have a different disease profile to prevalent …

Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension

M Humbert, O Sitbon, A Yaïci, D Montani… - European …, 2010 - Eur Respiratory Soc
Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. We studied 674
consecutive adult patients who were prospectively enrolled in the French PAH registry (121 …

Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation

T Thenappan, SJ Shah, S Rich, L Tian… - European …, 2010 - Eur Respiratory Soc
The aim of the present study was to determine contemporary survival in pulmonary arterial
hypertension (PAH), and to investigate whether or not the National Institutes of Health (NIH) …

New predictors of outcome in idiopathic pulmonary arterial hypertension

SM Kawut, EM Horn, KK Berekashvili… - The American journal of …, 2005 - Elsevier
Idiopathic pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis.
New therapies have improved the outcome of this condition; accordingly, the factors that …

Survival of Japanese patients with idiopathic/heritable pulmonary arterial hypertension

A Ogawa, T Satoh, Y Tamura, K Fukuda… - The American journal of …, 2017 - Elsevier
Idiopathic/heritable pulmonary arterial hypertension has a poor prognosis despite the
available therapeutic options. Survival of Japanese patients with this disease entity has not …

Association between initial treatment strategy and long-term survival in pulmonary arterial hypertension

A Boucly, L Savale, X Jaïs, F Bauer… - American journal of …, 2021 - atsjournals.org
Rationale: The relationship between the initial treatment strategy and survival in pulmonary
arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term …

Pulmonary arterial hypertension

PM Hassoun - New England Journal of Medicine, 2021 - Mass Medical Soc
Pulmonary Arterial Hypertension Without effective treatment, pulmonary arterial
hypertension results in high morbidity and mortality. This review discusses the pathogenesis …

Pulmonary arterial hypertension: future directions: report of a National Heart, Lung and Blood Institute/Office of Rare Diseases workshop

JH Newman, BL Fanburg, SL Archer, DB Badesch… - Circulation, 2004 - Am Heart Assoc
likely responsible for most cases of PAH. The abnormal transduction of signals related to
BMPR2 and Alk/endoglin is unknown and needs aggressive investigation. This will involve …

Primary pulmonary arterial hypertension: a look back

AP Fishman - Journal of the American College of Cardiology, 2004 - jacc.org
For the first half of the twentieth century, the published reports on primary pulmonary
hypertension (PPH) were confined to clinical pathological correlations. In the 1950s the …