The Outcome of Surgical Repair for Congenital Unilateral Absence of the Proximal Pulmonary Artery Contralateral to the Aortic Arch

Satoshi Okugi, Akihisa Furuta, Goki Matsumura, Hiroshi Niinami

Tokyo women medical university, Tokyo, Japan

Date, time and location: 2018.05.25 15:30, Press Hall, 2F

Abstract

OBJECTIVES

Congenital unilateral absence of the proximal pulmonary artery contralateral to the aortic arch (UAPPA) is a rare anomaly. The purpose of this retrospective study is to present our institutional surgical experiences for seven patients with UAPPA and to evaluate the affected PA growth in a diameter and the flow distribution of the PA in both lungs

METHODS

From 1984 to 2017, seven patients with UAPPA presented at our institution. There were three females and four males and their average age at diagnosis was 6.4±12.8 months. The diameter of the PA was measured by using catheter angiography. Paired t test was used to compare the diameter of affected PA between at the time of initial diagnosis and after intracardiac repair. The flow distribution of PA after intracardiac repair was evaluated with lung perfusion scintigraphy.

RESULTS

Four patients had the palliations to grow the affected PA before the intracardiac repair. The average age at palliation was 16.75±15.1 months (median 15 months). The average age at intracardiac repair was 61.6±94 months (median 10 months). One patient with truncus arteriosus died early after the intracardiac repair due to heart failure. The average diameter of the affected PA was 44.5±20.0 % of normal value at the time of the diagnosis and slightly increased to 57.6±34.2% of normal value after intracardiac repair. However, there was no significant difference. The flow distribution of ipsilateral lung to the affected PA was 21.3±23.3 % of the total lung perfusion after intracardiac repair.

CONCLUSION

Congenital UAPPA might have a possibility of having affected pulmonary artery developed poorly even after the surgical repair. The average 6 months at initial presentation seems to be somewhat late to develop the closed proximal PA.