Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management

Abstract

Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to increased hemorrhagic diathesis, resulting in mild to massive and life-threatening episodes of haemoptysis. Despite major advances in PAH targeted treatment strategies, haemoptysis is still correlated with substantial morbidity and impaired quality of life, requiring a multidisciplinary approach by adult CHD experts in tertiary centres. Technological innovations in the field of diagnostic and interventional radiology enabled the application of bronchial artery embolization (BAE), a valuable tool to efficiently control haemoptysis in modern clinical practice. However, bleeding recurrences are still prevalent, implying that the optimum management of haemoptysis and its implications remain obscure. Moreover, regarding the use of oral anticoagulation in patients with haemoptysis, current guidelines do not provide a clear therapeutic strategy due to the lack of evidence. This review aims to discuss the main pathophysiological mechanisms of haemoptysis in PAH-CHD, present the clinical spectrum and the available diagnostic tools, summarize current therapeutic challenges, and propose directions for future research in this group of patients.

Keywords: Eisenmenger syndrome; congenital heart disease; haemoptysis; pulmonary arterial hypertension.

Figure 1

Pathophysiology of haemoptysis in patients with PAH-CHD. CHD: Congenital heart disease, PAH: Pulmonary arterial hypertension, PA: Pulmonary artery.

Figure 2

Proposed diagnostic and management algorithm of haemoptysis in patients with PAH-CHD. This algorithm has not been validated by a prospective study. # Cardiac magnetic resonance imaging may be additionally implemented to evaluate the intracardiac anatomy, abnormalities of the pulmonary vascular bed and the ventricular volumes. BAE: Bronchial artery embolization, CHD: Congenital heart disease, CTPA: Computerized tomography pulmonary angiography, ICU: Intensive care unit, PAH: Pulmonary arterial hypertension, TA: Tranexamic acid.

Figure 3

A 40-year-old female patient with Eisenmenger syndrome on the background of a large secundum ASD presenting with recurrent episodes of haemoptysis. (A) Sagittal view of thorax computed tomography after intravenous contrast medium injection, showing aneurysmatic dilatation of the left pulmonary artery with intraluminal thrombus and adjacent post-bleeding infiltrations in the lung parenchyma (red arrow). (B) Catheterization of the bronchial arteries with a 5 Fr Cobra catheter, showing the tortuous origin of the left bronchial artery (red arrow). (C) Angiographic image in a delayed parenchymal phase, showing abnormal imaging of the left posterior lung parenchyma adjacent to the descending aorta, corresponding to the area of bleeding (red arrow). (D) Angiographic image after embolization with 100–300 μ particles through a 2.6 Fr microcatheter. The left bronchial artery could not be selectively catheterized, so injection of particles was performed in both bronchial arteries until stasis was achieved, especially in the left side (red arrow).