Pulmonary hypertension in hereditary hemorrhagic telangiectasia is associated with multiple clinical conditions

Introduction: Pulmonary hypertension (PH) is a rare complication of hereditary hemorrhagic telangiectasia (HHT). This is usually a post-capillary PH (group 2), due to high cardiac output and leading to left-heart failure. More rarely, it can result from pulmonary vascular remodeling (pre-capillary PH). We investigated prevalence, hemodynamic profiles and associated clinical conditions of pre-capillary PH associated with HHT.Methods: We conducted a retrospective monocentric study in the French national reference center (Lyon, France) among patients with confirmed HHT who underwent right heart catheterization (RHC). Patients were classified according current hemodynamic definitions as pre-capillary PH, isolated post-capillary PH, combined pre- and post-capillary PH.Results: Out of 901 patients with HHT, 30 underwent RHC. Pre-capillary PH was found in 0.7% (n=6) and combined PH in 0.9% (n=8) of patients.Various conditions that might have contributed to pulmonary vascular remodeling were found: genetic mutations (ALK1 n= 12, ENG n=2), portal hypertension (n=1), chronic lung diseases with FEV1
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research

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Source: Preventive Medicine - Category: International Medicine & Public Health Source Type: research
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