Unilateral absence of pulmonary artery: a radiographically occult cause of life-threatening hemoptysis

Abstract

Unilateral absence or agenesis of pulmonary artery (UAPA) is a rare congenital abnormality with an estimated prevelance of 1 in 2,00,000 adults. The entity occurs commonly in association with other congenital heart diseases like septal defects or patent ductus arteriosus. The condition usually runs a benign clinical course with patients usually presenting clinically in adulthood with history of recurrent respiratory tract infections. Two such patients presented with recurrent respiratory tract infections, breathlessness and hemoptysis. The chest radiograph of first patient was reported as normal in the referring hospital, while that of second patient showed volume loss in left lung. CT Pulmonary Angiography (CTPA) was then performed which demonstrated the absence of right and left pulmonary arteries respectively in the first and second patients. Pulmonary artery branches were reformed distally by multiple collaterals arising from systemic arteries. The entire spectrum, including embryology, imaging features and management of UAPA are discussed. UAPA remains a potential cause for life-threatening hemoptysis, due to extensive collateralization associated with the condition. It is important for radiologists to be aware of this uncommon entity in order to suspect it on a routine chest radiograph, diagnose it and map associated collaterals on CTPA and also embolize the bleeding collaterals.

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

“…Pulmonary angiography is the gold standard investigation for the diagnosis of pulmonary artery agenesis and estimation of collateral circulation [6]. However, being an invasive procedure, it is better advised when a patient is planned for arterial embolization [6,7].…”

“…An early diagnosis is extremely important because prognosis depends on the presence of many complications such as pulmonary infections, pulmonary hemorrhage, and, especially, PHT [3,4,6,7]. The overall mortality rate in UAPA is 7% [3,6,7].…”

“…An early diagnosis is extremely important because prognosis depends on the presence of many complications such as pulmonary infections, pulmonary hemorrhage, and, especially, PHT [3,4,6,7]. The overall mortality rate in UAPA is 7% [3,6,7]. Common causes of mortality include right heart failure, respiratory failure, massive hemoptysis, and high-altitude pulmonary edema [6,7].…”

“…The overall mortality rate in UAPA is 7% [3,6,7]. Common causes of mortality include right heart failure, respiratory failure, massive hemoptysis, and high-altitude pulmonary edema [6,7]. There are no guidelines or consensus regarding treatment [3,6].…”

“…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].…”