Case report
A neonate with prenatal diagnosis of CPAM, born from cesarean section at
35 weeks of gestation and 2.5 kg, was led in neonatal intensive care for
respiratory failure at birth. He was intubated and mechanically assisted
with high-frequency oscillatory ventilation (HFOV). At that time, first
urgent cardiac ultrasound (CUS), with poor acoustic window, showed
normal origin and course of the coronary branches and systemic right
ventricular pressures (Fig 1a, b). At day2 for worsening clinical
conditions he was put on veno-arterial extracorporeal membrane
oxygenation (VA-ECMO). At day7 he underwent right upper and medium
lobectomy for removal of CPAM III and at day 16 he was weaned-off ECMO.
Serial CUS showed progressive reduction of pulmonary arterial pressure
(PAP). At day52 CUS showed the presence of a small network of multiple
coronary vessels in the field of the right coronary artery (RCA), a
systo-diastolic jet of flow into the main pulmonary artery (MPA) at
color-mapping and an inverted retrograde flow into the RCA (Fig 2a, b –
video 1, 2 and 3). A more detailed examination showed a suspected
abnormal origin of the RCA from the main pulmonary artery (ARCAPA) (Fig
2c – video 4). At day59 CT scan confirmed the echocardiographic
diagnosis of ARCAPA origination from the medial wall of the MPA (Fig 2d
and 3) and dilation of the main epicardial coronary network due to the
presence of a multiple coronary collateral connections. After recovery
from thoracic surgery and multidisciplinary review of the case, the
patient was discharged at home with strict follow-up and future
evaluation for elective coronary surgery.