A Combination of Absent Right Pulmonary Artery and Left Pulmonary Veins Stenoses Fostering a Proclivity to Pulmonary Hypertension and Congestive Heart Failure: A Case Report

Meng-Luen Lee ； Ming-Chih Lin ； Ming-Che Chang

《The Changhua Journal of Medicine》 21卷2期 (2023/06) Pp. 63-68

https://doi.org/10.6501/CJM.202306_21(2).0004

摘要

We report an 80-day-old boy presenting with tachypnea, wheezing, subcostal and suprasternal retraction, and lip cyanosis. Chest radiogram showed interstitial pulmonary edema. Echocardiography with Doppler showed disconnection between the main pulmonary artery and right pulmonary artery and severe tricuspid regurgitation. Cardiac catheterization confirmed supra-systemic pulmonary hypertension (PHT). Multidetector computerized tomography showed congenital absent right pulmonary artery (ARPA) and pulmonary vein stenoses involving the left superior pulmonary veins and left inferior pulmonary veins, congenital intra-parenchymal right superior-segmental and right inferior-segmental pulmonary arteries stenoses, stenosis of the left main bronchus, and compression of the lower trachea. Despite intensive treatment, the patient died of intractable PHT and congestive heart failure (CHF) before surgical revascularization. We highlight that a rare constellation of congenital ARPA and congenital left pulmonary vein stenoses may foster with time a spatiotemporal proclivity to PHT, CHF, and airway compression.

關鍵字

absent right pulmonary artery ； cardiac catheterization ； congestive heart failure ； left pulmonary vein stenosis ； pulmonary hypertension