Pulmonary Arterial Hypertension in France

Humbert, Marc; Sitbon, Olivier; Chaouat, Ari; Bertocchi, M.; Habib, Gilbert; Gressin, Virginie; Yaïci, Azzedine; Weitzenblum, E; Cordier, Jean-François; Chabot, F.; Dromer, C.; Pison, Christophe; Reynaud‐Gaubert, Martine; Haloun, A.; Laurent, Marcel; Hachulla, É.; Simonneau, Gérald

doi:10.1164/rccm.200510-1668oc

Cited by 1,799 publications

(1,557 citation statements)

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“…All consecutive patients with PAH aged ⩾18 years were prospectively included in the French pulmonary hypertension (PH) registry [11]. Data from a single centre (Louis Pradel Hospital, Lyon, France) collected between June 1995 and September 2011 were extracted from the registry and patients satisfying the inclusion criteria were included in the present study.…”

Section: Methodsmentioning

confidence: 99%

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

et al. 2014

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Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy.In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78). After a median follow-up of 4.1 years, 41 deaths occurred, including 35 from cardiovascular causes. Patients with a (median) baseline RVEF >25% had better survival than those with a RVEF <25% using Kaplan-Meier analysis ( p=0.010). RVEF at baseline was an independent predictor of all-cause and cardiovascular mortality in adjusted Cox regression model ( p=0.002 and p=0.007, respectively; HR 0.93 for both). Patients with stable or increased RVEF at 3-6 months had a trend for improved all-cause survival (HR 2.43, p=0.086) and had less cardiovascular mortality (HR 3.25, p=0.034) than those in whom RVEF decreased despite therapy.RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change in RVEF 3-6 months after therapy initiation independently predict outcomes in patients with pulmonary arterial hypertension. @ERSpublications RVEF assessed with CPERA at baseline, and its changes on therapy, independently predict outcome in patients with PAH http://ow.ly/DsCS4

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Section: Methodsmentioning

confidence: 99%

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

et al. 2014

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“…Pulmonary arterial hypertension (PAH) is a lethal cardiovascular disorder with a 1‐year mortality rate of 15% to 20% and a median survival of 5 to 7 years after diagnosis 1, 2, 3. In PAH, obstructive remodeling of the pulmonary vasculature and reduced PA compliance increase pulmonary arterial pressures and right ventricular (RV) workload 4, 5.…”

Section: Introductionmentioning

confidence: 99%

Colchicine Depolymerizes Microtubules, Increases Junctophilin‐2, and Improves Right Ventricular Function in Experimental Pulmonary Arterial Hypertension

Prins

Tian

et al. 2017

JAHA

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BackgroundPulmonary arterial hypertension (PAH) is a lethal disease characterized by obstructive pulmonary vascular remodeling and right ventricular (RV) dysfunction. Although RV function predicts outcomes in PAH, mechanisms of RV dysfunction are poorly understood, and RV‐targeted therapies are lacking. We hypothesized that in PAH, abnormal microtubular structure in RV cardiomyocytes impairs RV function by reducing junctophilin‐2 (JPH2) expression, resulting in t‐tubule derangements. Conversely, we assessed whether colchicine, a microtubule‐depolymerizing agent, could increase JPH2 expression and enhance RV function in monocrotaline‐induced PAH.Methods and ResultsImmunoblots, confocal microscopy, echocardiography, cardiac catheterization, and treadmill testing were used to examine colchicine's (0.5 mg/kg 3 times/week) effects on pulmonary hemodynamics, RV function, and functional capacity. Rats were treated with saline (n=28) or colchicine (n=24) for 3 weeks, beginning 1 week after monocrotaline (60 mg/kg, subcutaneous). In the monocrotaline RV, but not the left ventricle, microtubule density is increased, and JPH2 expression is reduced, with loss of t‐tubule localization and t‐tubule disarray. Colchicine reduces microtubule density, increases JPH2 expression, and improves t‐tubule morphology in RV cardiomyocytes. Colchicine therapy diminishes RV hypertrophy, improves RV function, and enhances RV–pulmonary artery coupling. Colchicine reduces small pulmonary arteriolar thickness and improves pulmonary hemodynamics. Finally, colchicine increases exercise capacity.ConclusionsMonocrotaline‐induced PAH causes RV‐specific derangement of microtubules marked by reduction in JPH2 and t‐tubule disarray. Colchicine reduces microtubule density, increases JPH2 expression, and improves both t‐tubule architecture and RV function. Colchicine also reduces adverse pulmonary vascular remodeling. These results provide biological plausibility for a clinical trial to repurpose colchicine as a RV‐directed therapy for PAH.

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“…The average duration between symptom and diagnosis of PAH is >2 years. 3 It was further confirmed by the National Institute of Health Registry, where the mean interval from symptoms onset to diagnosis was 2 years, it was shorter in cases with family history of pulmonary artery hypertension (PAH). 7 The familial PAH shows an autosomal inheritance with 10-20% penetrance.…”

Section: Epidemiologymentioning

confidence: 89%

“…3,4 Primary or iPAH is a rare disorder, the frequency is estimated at 1-2 cases/million people. 5 A study from Scotland, however, cited the incidence as 2.5 cases/million/yr in men and 4.0 cases/million/yr in women.…”

Section: Epidemiologymentioning

confidence: 99%

Pulmonary hypertension—“state of the art” management in 2012

Saxena

2012

Indian Heart Journal

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A B S T R A C TPulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not change the natural history of the disease. However, over the years, improvement in understanding the pathogenesis has resulted in the development of targeted approaches to the treatment of PAH. Survival advantage has also been shown with some of the pharmacologic agents. This review article discusses the current management strategy for PAH with special emphasis on an idiopathic variety, in an Indian context.

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Pulmonary Arterial Hypertension in France

Abstract: This contemporary registry highlights current practice and shows that PAH is detected late in the course of the disease, with a majority of patients displaying severe functional and hemodynamic compromise.

Cited by 1,799 publications

References 28 publications

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

Colchicine Depolymerizes Microtubules, Increases Junctophilin‐2, and Improves Right Ventricular Function in Experimental Pulmonary Arterial Hypertension

Pulmonary hypertension—“state of the art” management in 2012

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