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.

Protective effects of calcyclin-binding protein against pulmonary vascular remodeling in flow-associated pulmonary arterial hypertension

“…Pulmonary arterial hypertension (PAH) is a relatively common complication of congenital heart disease (CHD), which manifests as an abnormal elevation in pulmonary arterial pressure and increased vascular resistance, leading to heart failure and eventually death [ 1 – 3 ]. Typically, the pathophysiology of PAH is characterized by progressive remodeling of small pulmonary arteries and muscularization of precapillary arterioles attributed to a neoplastic-like proliferative and pro-migratory phenotype of pulmonary smooth muscle cells (PASMCs) [ 4 , 5 ].…”

“…Pulmonary arterial hypertension (PAH) is a relatively common complication of congenital heart disease (CHD), which manifests as an abnormal elevation in pulmonary arterial pressure and increased vascular resistance, leading to heart failure and eventually death [1][2][3].…”

The landscape of coagulation cascade in pulmonary arterial hypertension

“…Moreover, hypocoagulable state and multiple haemostatic defects have been demonstrated in patients with PAH 12–14 . Converse to thrombosis, the hemorrhagic events, such as hemoptysis were frequently complicated in patients with PAH, particularly to PAH associated with CHD and connective tissue disease 15–17 . In light of the high prevalence of thrombosis in the distal pulmonary arterioles, anticoagulant therapy would be beneficial for PAH patients.…”

Pulmonary Hypertension: Current Diagnosis, Approach and Treatment at the Dawn of the New European Guidelines

“…An important issue, that is a comprehensive approach of the hemoptysis occurring in PAH patients secondary to congenital heart disease is presented by Baroutidou et al [ 9 ]. It appears that the pulmonary vascular pathobiology that accompanies this disorder predisposes patients to hemoptysis.…”

“…The authors report that despite the major advances of the recent past, hemoptysis is always associated with increased morbidity and mortality in these high-risk patients. In addition to the related pathophysiology, diagnostic approaches, therapeutic options and future directions are also discussed [ 9 ].…”