One-time pulmonary artery reconstruction to repair infant unilateral absence of pulmonary artery: A case report

摘要

INTRODUCTION Unilateral Absence of Pulmonary Artery(UAPA)is a rare complicated congenital heart disease firstly described by Frentzel in 1868.An absent pulmonary artery is caused by the involution of the proximal sixth aortic arch and persistence of the connection of the intrapulmonary pulmonary artery to the distal sixth aortic arch in embryonic development period.Patent ductus arteriosus which connects the pulmonary arteries with systemic circulation artery might get atresia gradually after birth with lack of blood flow for this side of pulmonary arteries and pulmonary hypertension in the other side.There are no evidences showing the development of the pulmonary vessels especially in the position of the hilus of the lung and the distribution of the pulmonary arteries on the regular examination,such as echocardiogram and CT scan.Our team has taken enhanced CT scan and three-dimensional reconstruction to confirm the diagnosis and to assess the surgical indications.We adopted utwosegment”technique to reconstruct the absent pulmonary artery for a 3-month-old infant followed by significant alleviation of pulmonary hypertension and excellent rehabilitation.