Unilateral pulmonary artery atresia in an adult: A case report

Abstract

IntroUnilateral pulmonary artery atresia (UPAA), while encountered frequently in the congenital cardiac anomaly cohort, is occasionally diagnosed in adulthood after typical symptoms of hemoptysis, pulmonary infection, or as an incidental finding on contrast CT scan. Due to its rarity, a brief discussion of UPAA and its treatment is warranted.Case reportA 35 year old male presented with three days of hemoptysis. After diagnosis of right UPAA, he underwent angioembolization of 6 large systemic collaterals supplying his right lung, followed by right pneumonectomy. He was discharged on post-operative day 3, and at follow up 6 weeks later was doing well with minimal residual incisional pain and excellent pulmonary reserve.ConclusionsUPAA presents classically with hemoptysis, but also with pneumonia, pulmonary hypertension, or incidentally. Management includes selective collateral embolization, pneumonectomy, or medical management directed towards decreasing pulmonary hypertension in patients unable to tolerate pneumonectomy due to comorbidities. Pneumonectomy in these patients is characterized by dense and hypervascular adhesions, with large volume blood loss expected during adhesiolysis, which can be decreased with pre-operative embolization. Outcomes are typically excellent in otherwise healthy patients.

Isolated unilateral pulmonary artery atresia in an adult presenting with cor pulmonale

“…It occurs due to the failure of rotation and migration of the primitive sixth aortic arch and the persistence of connection between the intrapulmonary segment of the pulmonary artery and the distal sixth arch. UPAA is most often seen as a defect in the right pulmonary trunk and presents as an isolated finding in adulthood; left-sided UPAA as its counterpart is often diagnosed in childhood and is associated with cardiac anomalies, thus indicating a bleaker prognosis 3 6…”

“…The collaterals are exposed to significantly elevated pressures, hence prone to rupture, leading to acute presentations of moderate-severe haemoptysis. Collateral bleeds in documented cases of UPAA have been commonly noted from bronchial (70%), phrenic (47%), internal thoracic (44%), intercostal (44%) and subclavian (34%) vessels 6 10. Apart from arterial haemoptysis, venous overloading could also present as mild haemoptysis 9.…”

“…Post-stabilisation, selective embolisation of systemic collaterals could help prevent further recurrent episodes 11 20. For control of haemoptysis, bronchial artery embolisation is less invasive as compared with pneumonectomy; however, due to the lack of technical expertise and a higher risk of recurrence, pneumonectomy stands as a better treatment choice in long-term care 6. In cases of severe and recurrent infections, a lobectomy or pneumonectomy can also be performed 11.…”

Exertional dyspnea and hemoptysis in an adolescent: is it tuberculosis only?

“…because of large collateral circulations in the pulmonary venous system [4]. The haemoptysis in these patients can be mild and self-limiting or sometimes even massive.…”

“…Most commonly it is detected in childhood with only a few cases remaining asymptomatic till adulthood and these usually present when they develop complications [3]. In adults, it presents most commonly with haemoptysis, dyspnoea on exertion and recurrent chest infections [4]. Few cases of pulmonary tuberculosis (TB) in a patient with unilateral pulmonary artery atresia have been reported globally, with no such case being reported from India.…”