Pulmonary arterial hypertension (PAH) is a rare dyspnea-fatigue syndrome caused by increased pulmonary vascular resistance (PVR) and eventual right heart failure. Several conditions and some definite gene mutations can be associated with PAH. A small number of patients with a diagnosis of pulmonary hypertension (PH) in relation to genes mutations associated with congenital erythrocytosis, and in particular with von Hippel-Lindau (VHL) gene mutation, have been reported. However, these reports only mentioned an increased pulmonary artery pressure (PAP), without reporting complete clinical and hemodynamic data, so whether the patients really suffered from PAH is uncertain.

(2016). Pulmonary arterial hypertension associated with a von Hippel-Lindau gene mutation [journal article - articolo]. In THE JOURNAL OF HEART AND LUNG TRANSPLANTATION. Retrieved from http://hdl.handle.net/10446/188880

Pulmonary arterial hypertension associated with a von Hippel-Lindau gene mutation

Caravita, Sergio;
2016-01-01

Abstract

Pulmonary arterial hypertension (PAH) is a rare dyspnea-fatigue syndrome caused by increased pulmonary vascular resistance (PVR) and eventual right heart failure. Several conditions and some definite gene mutations can be associated with PAH. A small number of patients with a diagnosis of pulmonary hypertension (PH) in relation to genes mutations associated with congenital erythrocytosis, and in particular with von Hippel-Lindau (VHL) gene mutation, have been reported. However, these reports only mentioned an increased pulmonary artery pressure (PAP), without reporting complete clinical and hemodynamic data, so whether the patients really suffered from PAH is uncertain.
articolo
2016
Caravita, Sergio; Deboeck, Gael; Vachiery, Jean-Luc; Naeije, Robert
(2016). Pulmonary arterial hypertension associated with a von Hippel-Lindau gene mutation [journal article - articolo]. In THE JOURNAL OF HEART AND LUNG TRANSPLANTATION. Retrieved from http://hdl.handle.net/10446/188880
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10446/188880
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