Proximal Interruption of the Pulmonary Artery

Abstract

Proximal interruption of the pulmonary artery (PIPA) is an uncommon developmental anomaly resulting in underdevelopment of the proximal portion of the pulmonary artery with preservation of the intrapulmonary segments. Clinical presentation ranges between an asymptomatic incidental finding to massive hemoptysis. When findings suggestive of PIPA are present radiographically, the diagnosis of PIPA can be definitively diagnosed with computed tomography or magnetic resonance pulmonary angiography. Other imaging modalities, such as nuclear perfusion scan and catheter angiography can help in the diagnosis.

Imaging of congenital lung diseases presenting in the adulthood: a pictorial review

“…It can occur on both sides, although it is more commonly located on the right side. Left-sided anomaly is generally related to other congenital cardiovascular diseases [ 48 ].…”

“…Contrast-enhanced CT images can demonstrate dilated contralateral pulmonary artery and supply of the distal pulmonary artery branches from the aortopulmonary collateral arteries, including bronchial, internal mammary, intercostal, subdiaphragmatic, or coronary arteries. Moreover, the affected lung parenchyma can show mosaic attenuation, fibrotic changes, bronchiectasis, intraparenchymal and subpleural cysts [ 3 , 10 , 48 ].

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“…Patients with PIPA can present with recurrent pneumonia and hemoptysis owing to pulmonary hypertension or enlarged collateral vascular structures, or PIPA may be seen as an incidental finding in adults. Although conservative treatment is preferred, transcatheter selective embolization of the enlarged collateral vascular structures and pneumonectomy can be performed because of clinically significant hemoptysis [ 48 , 49 ].…”

Evaluation of the bronchial arteries: normal findings, hypertrophy and embolization in patients with hemoptysis

“…Agenesis of the pulmonary artery, also called proximal interruption of the pulmonary artery, is an uncommon developmental anomaly resulting in proximal absence of the pulmonary artery, with preservation of the intrapulmonary vasculature. This variant can occur on either side, more commonly on the right side, with ipsilateral lung hypoplasia [21]. When it occurs, the distal pulmonary arterial branches are usually supplied by hypertrophied bronchial arteries, internal mammary, intercostal, subdiaphragmatic, or coronary arterial collaterals ( Fig.…”

“…4d). Clinical presentation varies from asymptomatic incidental finding to massive hemoptysis due to ruptured hypertrophied systemic collaterals, rarely requiring transcatheter embolization [21].…”