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. 2013 Oct 23;8(10):e78132.
doi: 10.1371/journal.pone.0078132. eCollection 2013.

Risk factors for hemoptysis in idiopathic and hereditary pulmonary arterial hypertension

Darryl Tio  1 , Edward Leter, Bart Boerrigter, Anco Boonstra, Anton Vonk-Noordegraaf, Harm Jan Bogaard
Affiliations

Risk factors for hemoptysis in idiopathic and hereditary pulmonary arterial hypertension

Darryl Tio et al. PLoS One. .

Abstract

Introduction: When 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.

Methods: 129 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.

Results: Hemoptysis 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.

Conclusion: Hemoptysis 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.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. PAH Study cohort.
Studies performed within 12 months of the original diagnosis were considered baseline data. Pulmonary imaging consisted of CT angiography and/or thoracic MRI.
Figure 2
Figure 2. Shown is a CT angiography scan of a 23 year old iPAH patient with presence of bronchial artery hypertrophy.
(upper arrow: left bronchial artery; lower arrow: right bronchial artery).
Figure 3
Figure 3. The pulmonary artery diameter was measured at the trunk of the pulmonary artery, on axial CT angiography slices using electronic callipers.
Figure 4
Figure 4. CT angiography from a 23 year old iPAH patient who died from massive hemoptysis.
Panel A is a transverse section showing central pulmonary artery dilatation and small saccular aneurysms in more peripheral vessels (small arrows). Note the irregular borders of the central vessels. Panel B shows a coronal reconstruction with aneurysmatic changes to the main pulmonary artery (large arrow) and peripheral vessels (small arrows). Panel C is a 3 D reconstruction.
See this image and copyright information in PMC

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