Unilateral Absence of the Left Pulmonary Artery With an Associated Vascular Anomaly in Adulthood

Abstract

Left-sided pulmonary artery agenesis is a rare malformation that commonly requires childhood intervention secondary to associated congenital cardiovascular anomalies. We present an uncommon case of left-sided agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung. Additionally, there is radiographic evidence of emphysema and pulmonary artery hypertension. Pulmonary artery agenesis is not a common entity, but should be considered in adult patients presenting with recurrent pneumonias and radiographic evidence suggestive of pulmonary hypoplasia. A prompt diagnosis is beneficial for affected individuals who may be candidates for a revascularization procedure or embolization of collaterals. Earlier diagnosis also allows for proper management and follow-up care, considering pulmonary artery hypertension is a severe complication of pulmonary artery agenesis.

An elderly patient and an adult patients with isolated unilateral pulmonary arterial hypoplasia: Two cases reports and literature review of the literature

“…Other causes of unilateral hyperlucent lung, such as pneumothorax, idiopathic giant bullae, congenital lobar emphysema, and bronchial obstruction due to a foreign body or endobronchial malignancy, should be considered. Other chest X-ray or CT findings include absence of the pulmonary artery shadow with enlargement of the contralateral pulmonary artery shadow, volume loss of the affected side, and ipsilateral mediastinal shift [5] . Three-dimensional reconstruction of thorax CT can be helpful for outlining abnormalities of the bronchi and associated vascular structures.…”

“…The treatment decision is based upon on the age of patients, symptoms, and the presence of cardiac abnormalities. Asymptomatic patients with no evidence of cardiac diseases do not usually need treatment [1] , [5] . Pneumonectomy, lobectomy, embolization of collateral arteries, and revascularization are considered for patients with PHT, recurrent pulmonary infections or hemoptysis [1] , [7] , [8] .…”

Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

“…The treatment for UPAA depends on age at presentation, symptoms of the patient, associated cardiovascular anomalies, and PAH. 3,8 The options include revascularization of the distal affected pulmonary artery, considering that intrapulmonary arteries are still formed during embryological development. 1,3,8 In older patients, because the intrapulmonary arteries can be severely narrowed or even completely obstructed by fibrosis, revascularization is not encouraged or even feasible.…”

“…3,8 The options include revascularization of the distal affected pulmonary artery, considering that intrapulmonary arteries are still formed during embryological development. 1,3,8 In older patients, because the intrapulmonary arteries can be severely narrowed or even completely obstructed by fibrosis, revascularization is not encouraged or even feasible. 13,14 In our patient, late presentation and severe PAH precluded any surgical intervention.…”

A Late Presentation of an Uncommon Congenital Vascular Anomaly

“…Most common symptoms are recurrent pulmonary infections, hemoptysis and shortness of breath [1]. Most patients who have no associated cardiac anomalies may have no symptoms and survive into adulthood, which is rare.…”