INTRODUCTION
Adults with congenital heart disease represent an expanding patient
population requiring life-long tertiary medical care. Approximately 5%
to 10% of them develop pulmonary arterial hypertension (PAH) of
variable severity [1]. In the anomalous origin of the pulmonary
artery, the pathological branch originates from the ascending aorta,
with an incidence of about 0.12% [2], hence often being a
misdiagnosis or a missed diagnosis. Left-to-right shunting caused by
patent ductus arteriosus is more pronounced in the presence of an
additional shunt-like anomalous origin of the pulmonary artery (AOPA).
AOPA coupled with PDA could present with lethal complications like
hemoptysis in the clinical course of disabling pulmonary hypertension
[3]. The incidence of hemoptysis is estimated at 3.1% to 5.5%
[4] in PAH-congenital heart disease (CHD) patients and is thus a
rare clinical picture. Follow-up is key for these patients to promptly
detect new or misdiagnosed pathologic findings [3]. CHD-PAH patients
have been found to have a survival advantage compared to those with
other types of PAH presenting with hemoptysis and hence require prompt
recognition and multi-centered treatment strategies [5]. Numerous
strategies employed in the past include supportive care, surgical
resection, and lung transplants. Presently, the most commonly used
strategy is bronchial artery embolization (BAE) [5]. BAE is
minimally invasive and has proved to be an immense success, resulting in
rapid cessation of hemoptysis with low complication rates [4].
Recent studies have shown the effectiveness of tranexamic acid in
reducing the volume and duration of hemoptysis [6]. Despite
milestone advances in PAH-targeted treatment strategies and techniques,
the morbidity burden of hemoptysis remains high, causing impaired
quality of life with a global impact on healthcare systems, hence
requiring a case-specific approach and close interdisciplinary
management. This includes collaboration among adult CHD experts,
pulmonologists, radiologists, and thoracic surgeons in tertiary centers
[3, 4].