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.