Changes in hemodynamic classification over time are common in systemic sclerosis-associated pulmonary hypertension: insights from the PHAROS cohort

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Changes in hemodynamic classification over time are common in systemic sclerosis-associated pulmonary hypertension: insights from the PHAROS cohort

Group classification of pulmonary hypertension (PH) is based on pulmonary artery wedge pressure (PAWP) on right heart catheterization (RHC). How hemodynamics, particularly PAWP, change over time in systemic sclerosis (SSc)-PH patients is unknown. SSc-PH patients enrolled in the prospective observational PHAROS registry who had > 1 RHC (n = 120) were included in this analysis. Patients were cons...

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Pulmonary hypertension in systemic sclerosis.

A woman born in 1912 (Case 8 in the necropsy series) had an unremarkable family history. In 1940, she first noted blanching and cyanosis of the fingers on exposure to cold. Since 1960, small, hard, subcutaneous nodules developed at different locations on her body. Early in 1968, progressive effort dypsnoea and anginal chest pains started insidiously, and treatment with digitalis, furosemide, an...

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Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension.

BACKGROUND Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related pulmonary hypertension with interstitial lung disease imposes a greater pulmo...

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Outcome measures in pulmonary arterial hypertension associated with systemic sclerosis.

SSc is complicated in approximately 10% of the patients by pulmonary arterial hypertension (PAH), a rare dyspnoea-fatigue syndrome caused by an increase in pulmonary vascular resistance. The prognosis of SSc-PAH is particularly poor, with estimated survival rates of approximately 50% at 2 yrs without pulmonary circulation-targeted therapies. Prostacyclins, endothelin receptor antagonists and ph...

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Sex disparities in systemic sclerosis-associated pulmonary arterial hypertension: a cohort study

BACKGROUND The impact of male sex as a determinant of health outcomes in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is controversial. The primary objective of this study was to evaluate the effect of sex on survival in patients with SSc-PAH. The secondary objectives were to evaluate the effect of sex on age of PAH diagnosis, time from SSc diagnosis to PAH diagnosis,...

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ژورنال

عنوان ژورنال: Pulmonary Circulation

سال: 2018

ISSN: 2045-8940,2045-8940

DOI: 10.1177/2045893218757404