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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.
Highlights
Pulmonary arterial hypertension (PAH) is a rare, heterogeneous disease of the pulmonary vasculature, haemodynamically defined by a mean pulmonary arterial pressure >20 mmHg, a normal pulmonary artery wedge pressure ≤15 mmHg and elevated pulmonary vascular resistance ≥3 Wood units [1]
Congenital heart disease (CHD) is frequently complicated by PAH, including four individual groups with shared features; Eisenmenger syndrome (ES), congenital systemic to pulmonary shunts, PAH associated with coincidental or small defects, and PAH encountered in patients with repaired congenital defects [2]
Its incidence is estimated as 3.1% to 5.5% [5,6] in PAH-CHD patients, whereas it is markedly elevated in patients with ES, accounting for 6% to 49% of cases [7,8,9,10,11,12]
Summary
Pulmonary arterial hypertension (PAH) is a rare, heterogeneous disease of the pulmonary vasculature, haemodynamically defined by a mean pulmonary arterial pressure (mPAP) >20 mmHg, a normal pulmonary artery wedge pressure ≤15 mmHg and elevated pulmonary vascular resistance ≥3 Wood units [1]. Its incidence is estimated as 3.1% to 5.5% [5,6] in PAH-CHD patients, whereas it is markedly elevated in patients with ES, accounting for 6% to 49% of cases [7,8,9,10,11,12] It is an alarming symptom for patients and physicians, haemoptysis seems to be a relatively infrequent cause of death, accounting only for 3% of deaths in a recent international, multi-centre, retrospective study of patients with ES [12]. The current review exhibits the pathophysiology and clinical manifestations of haemoptysis in patients with PAH-CHD, presents current diagnostic strategies, discusses therapeutic management options and highlights areas of uncertainty and gaps in current evidence
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