A full-term female newborn with a prenatal diagnosis of pulmonary atresia with intact interventricular septum (PA-IVS) was delivered via caesarean section. There was no immediate cardiorespiratory compromise; treatment with intravenous prostaglandin was started.

On the sixth day of life, a low-dose cardiac computed tomography (CT) was performed to evaluate ductal anatomy and pulmonary artery hypoplasia (Figure 1 and Video 1; total dose length product 1 mGy cm). The cardiac CT showed a hypoplastic right ventricle and intact interventricular septum (Panel A), pulmonary atresia (yellow arrowhead in Panels B and C) and a large-caliber apical ventriculo-coronary arterial communication (VCAC) (orange arrowhead in Panels D and E) between the right ventricle and the left anterior descending coronary artery (white arrowhead in Panels D and E).

Figure 1.

Ultra-fast, low dose high-pitch (FLASH) cardiac CT scan with systolic synchronization in a 6-day-old neonate with pulmonary atresia with intact interventricular septum and a ventriculo-coronary arterial communication. (A) Four-chamber view. (B) Axial oblique MIP-reformat. (C) Coronal oblique MIP-reformat. (D) Coronal oblique MIP-reformat. (E) Coronal cinematic volume rendering. Ao: aorta; LA: left atrium; PDA: patent ductus arteriosus; RA: right atrium; RV: right ventricle; *: atrial septal defect.

Cardiac catheterization revealed an unobstructed VCAC, no evidence of coronary atresia or stenosis, and no indication of right ventricle-dependent coronary circulation (RVDCC).

In the subsequent days a ductal stent was placed. At four-months of life, a modified Blalock-Taussig shunt was created and at one-year the infant was palliated with a bidirectional Glenn procedure and experienced good evolution had a good clinical course.

PA-IVS is a rare cyanotic congenital heart disease in which obstruction of the right ventricle may lead to increased pressures and large-calibre VCAC.1,2 Very few cases of VCAC have been documented on CT, but their recognition is crucial as the coronary circulation may depend on the right ventricle and surgical decompression could trigger myocardial infarction.3 Although CT imaging offers precise anatomical detail, cardiac catheterization remains essential for assessing the physiology/dependency of VCAC. In this case, however, CT visualization of an unobstructed left anterior descending coronary artery suggested the absence of RVDCC. Despite the absence of RVDCC, the patient underwent staged single-ventricle palliation due to significant hypoplasia of the right ventricle, tricuspid valve and pulmonary artery, and is expected to complete total cavopulmonary connection (Fontan operation) at three to five years of age.

Modern CT technology enables non-invasive, high-resolution, and low-radiation cardiac imaging in neonates, with vendor-specific applications further optimizing scan parameters to minimize radiation exposure while maintaining diagnostic quality. The use of CT in congenital heart disease is expected to expand further, offering comprehensive three-dimensional anatomical views that improve diagnostic accuracy and surgical planning.