The availability of new treatments for patients with pulmonary arterial hypertension has increased awareness and interest in the medical community for pulmonary vascular diseases in general. Many uncertainties exist, however, regarding the diagnosis and treatment of patients with pulmonary arterial hypertension that are particularly pertinent for the management of patients with idiopathic pulmo...

The maximal rate of fall in right ventricular pressure (negative dp/dt) was evaluated in 34 patients. Eight had normal pulmonary arterial pressure. Seventeen had pulmonary arterial hypertension, and nine had pulmonary arterial hypertension with right ventricular failure. The right ventricular maximal negative dp/dt in patients with normal pulmonary arterial pressure was 170 +/- 20 mm Hg/sec. In...

BACKGROUND Despite the increased risk for pulmonary hypertension in children with Down syndrome, the response to treatment with targeted therapies for pulmonary hypertension in these patients is not well characterized. The Sildenafil in Treatment-naive children, Aged 1-17 years, with pulmonary arterial hypertension (STARTS-1) trial was a dose-ranging study of the short-term efficacy and safety ...

Despite the fact that nucleotides and adenosine help regulate vascular tone through purinergic signaling pathways, little is known regarding their contributions to the pathobiology of pulmonary arterial hypertension, a condition characterized by elevated pulmonary vascular resistance and remodeling. Even less is known about the potential role that alterations in CD39 (ENTPD1), the ectonucleotid...

Successful curative treatment of severe pulmonary arterial hypertension with luminal obliteration will require a thorough understanding of the mechanism underlying the development and progression of pulmonary vascular lesions. But the cells that obliterate the pulmonary arterial lumen in severe pulmonary arterial hypertension are incompletely characterized. The goal of our study was to evaluate...

Cardiovascular manifestations in hyperthyroidism occur frequently with various phenotypes. An association between hyperthyroidism and pulmonary arterial hypertension has been reported. In previously reported cases, the hemodynamic and symptomatic recovery of pulmonary arterial hypertension is usually concomitant with achievement of euthyroidism. We report a patient who had pulmonary arterial hy...

OBJECTIVES Little is known about recipient characteristics and outcomes of patients with pulmonary arterial hypertension undergoing lung transplant, particularly those with systemic sclerosis-associated disease. Here, we describe the characteristics and outcomes of patients with pulmonary arterial hypertension undergoing lung transplant, focusing on systemic sclerosis-associated disease. MATE...

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