Isolated left-sided pulmonary artery agenesis with left lung hypoplasia

Abstract

Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA) is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI. There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

A Rare Case of Isolated Unilateral Atresia of Pulmonary Artery in an Elderly Female

“…UAPA was first described in 1868 by Frantzel O. Angeborener, and later it was proven angiographically by Madoff and his colleagues in 1953 [4]. It has been estimated that the prevalence of this unique disorder is about one in 200,000 [5]. Involution of the proximal sixth aortic arch of the affected side is the main embryological deficit, which leads to an absence of the proximal pulmonary artery.…”

“…Presenting symptoms in adult patients can be variable such as exertional dyspnea, limited exercise intolerance, hemoptysis, recurrent pulmonary infections and pulmonary hypertension, which is by far the most common presentation among adult population [7]. It has been proposed that alveolar hypocapnia can cause bronchoconstriction, while impaired mucociliary clearance and diminished delivery of inflammatory cells may contribute to the high incidence of respiratory infections [5]. Blood flow to the affected lung is supplied by collaterals arising from bronchial, subclavian, subdiaphragmatic and intercostal arteries; therefore, patients with UAPA may present with hemoptysis [8].…”

Right Pulmonary Artery Agenesis and Right Pulmonary Hypoplasia, presenting with Bronchiolitis Symptoms

“…Unilateral pulmoner arter agenezisi her iki cinsi de etkileyen bir durumdur ve genellikle aortik arkın karşısında görülür yani sağ akciğeri daha sık etkiler. Sol tarafın etkilendiği UPAA olguları infantil yaşta daha sık görülür, bu hastalarda erken dönemde pulmoner hipertansiyon geliştiğinden erişkin yaşa erişemezler (8). Sağ tarafın etkilendiği UPAA olguları, olgumuzda olduğu gibi yetişkin yaşa ulaşabilirler ve asemptomatik olduğundan daha geç tanı alırlar.…”

A Rare Etiology of Unilateral Pseudo-Pulmonary Fibrosis in a Puerto Rican Child

“…Due to embryologic relationships, pulmonary artery agenesis commonly occurs on the side of the chest opposite to the aortic arch [5]. The distal intrapulmonary branches of the affected artery usually remain intact and receive collateral supply from bronchial, intercostal, internal mammary, subdiaphragmatic, subclavian, and even the coronary arteries [6]. As a result of diminished blood supply, the lung on the affected side is usually small and hypoplastic [6].…”

“…The distal intrapulmonary branches of the affected artery usually remain intact and receive collateral supply from bronchial, intercostal, internal mammary, subdiaphragmatic, subclavian, and even the coronary arteries [6]. As a result of diminished blood supply, the lung on the affected side is usually small and hypoplastic [6]. An appearance termed "pseudofibrosis" is sometimes seen in the affected lung apex due to the formation of transpleural collateral vessels between peripheral pulmonary arterial branches and systemic arteries [7].…”