Abstract
IntroductionWhen hemoptysis complicates pulmonary arterial hypertension (PAH), it is assumed to result from bronchial artery hypertrophy. In heritable PAH, the most common mutation is in the BMPR2 gene, which regulates growth, differentiation and apoptosis of mesenchymal cells. The aim of this study is to determine the relationship in PAH between the occurrence of hemoptysis, and disease progression, bronchial artery hypertrophy, pulmonary artery dilation and BMPR2 mutations.Methods129 IPAH patients underwent baseline pulmonary imaging (CT angio or MRI) and repeated right-sided heart catheterization. Gene mutations were assessed in a subset of patients.ResultsHemoptysis was associated with a greater presence of hypertrophic bronchial arteries and more rapid hemodynamic deterioration. The presence of a BMPR2 mutation did not predispose to the development of hemoptysis, but was associated with a greater number of hypertrophic bronchial arteries and a worse baseline hemodynamic profile.ConclusionHemoptysis in PAH is associated with bronchial artery hypertrophy and faster disease progression. Although the presence of a BMPR2 mutation did not correlate with a greater incidence of hemoptysis in our patient cohort, its association with worse hemodynamics and a trend of greater bronchial arterial hypertrophy may increase the risk of hemoptysis.
Highlights
When hemoptysis complicates pulmonary arterial hypertension (PAH), it is assumed to result from bronchial artery hypertrophy
Our aims were 1) To determine whether PAH patients who have suffered from hemoptysis exhibit a worse hemodynamic profile, more bronchial artery hypertrophy or more severe dilation of the main pulmonary artery; and 2) To explore whether a bone morphogenetic protein type 2 receptor gene (BMPR2) mutation puts PAH patients at a greater risk to develop bronchial hypertrophy or pulmonary artery dilation and subsequent hemoptysis
We show that the development of hemoptysis in PAH is associated with worse hemodynamics, more bronchial artery hypertrophy, a trend towards a greater pulmonary artery diameter and more rapid disease progression
Summary
When hemoptysis complicates pulmonary arterial hypertension (PAH), it is assumed to result from bronchial artery hypertrophy. The aim of this study is to determine the relationship in PAH between the occurrence of hemoptysis, and disease progression, bronchial artery hypertrophy, pulmonary artery dilation and BMPR2 mutations. Pulmonary arterial hypertension (PAH) is a rare yet severe disease characterized by a progressive increase in pulmonary vascular resistance, right ventricular failure and premature death. It is assumed that the presence of bronchial artery hypertrophy puts patients at risk for the subsequent development of hemoptysis. Other than from hypertrophic bronchial arteries, hemoptysis in PAH may originate from pulmonary artery rupture. PAH is associated with progressive dilation of pulmonary arteries [9,10] and together with an increased intraluminal pressure, pulmonary artery dilation may put patients at an increased risk of pulmonary artery rupture and subsequent hemoptysis
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