Case Report
The patient’s mother was referred to our hospital after being suspected of a fetal cardiac malformation detected with a fetal screening at 24 weeks of gestation. Fetal echocardiography (Voluson E8; GE Healthcare) revealed a perimembranous VSD, 4 mm in diameter, the aorta riding over the interventricular septum with an overriding rate of 40-50% on the 5-chamber view, central pulmonary artery (PA), and major aortopulmonary collateral artery (MAPCA) arising from the descending aorta (dAo). However, the pulmonary valve and pulmonary forward blood flow from the right ventricle were not clearly defined on the right ventricular outflow tract view at 28 weeks of gestation (Fig 1, movie S1A, S1B). Follow-up fetal echocardiography detected a CPAF communicating to the central PA without ductus arteriosus (DA) at 35 weeks of gestation (Fig 2, movie S2). According to these results, we diagnosed CPAF with PA/VSD and talked the possible therapeutic strategies after birth and the potential risk of coronary steal to the parents. Finally, a male infant was delivered via cesarean section at 41 weeks of gestation. On transthoracic echocardiography (TTE) performed immediately after birth, we observed subaortic VSD with the overriding aorta, pulmonary valve atresia, confluent PA, right side aortic arch, and normal right and left coronary sinus without DA, which were completely consistent with previous fetal echocardiographic findings. The notable finding was CPAF arising from the proximal RCA connected to the central PA. To observe the appearance of CPAF in detail immediately after birth, cardiac angiography and ECG-gated 320-row CT (Aquilion ONE GENESIS Edition; Toshiba Medical Systems) was performed on day 0 and 3 respectively, and the images of CPAF were consistent with fetal echocardiography findings. CPAF originating from the proximal right coronary artery (RCA) and the right ventricular branch was connected to the central PA. The left coronary artery originated from the left coronary sinus. Two MAPCAs derived from the descending aorta supplied blood to the bilateral lung lobes, indicating a dual supply of pulmonary blood from the central PA and MAPCAs (Fig 3, movie S3). The patient had tachypnea (100/min) and required high flow nasal ventilation and nitrogen inhalation therapy from 11 days of age; however, no abnormal electrocardiography findings, such as ST-T alteration, were observed. We performed a central shunt operation (left innominate artery to central PA) using a 3.5 mm Gore-Tex graft, division of CPAF, and ligation of MAPCAs at 16 days after birth (Fig 4). Thereafter, palliative right ventricular outflow tract reconstruction (RVOTR) using a 12 mm Gore-Tex conduit with tricuspid valve was performed at five months of age, and intracardiac repair with RVOTR using a 16 mm Gore-Tex conduit at 21 months of age. The patient demonstrated good growth and neurological development and showed no complications at 36 months of age.