Pulmonary Atresia with IVS and Completely Absent Coronary Arteries

Background: Pulmonary atresia with intact ventricular septum accounts for approximately 0.3% of all congenital heart defects. Aplasia of the coronary arteries has been documented in 0.3% of cases.

Method: In this case study, we report two patients born with pulmonary atresia with IVS where the typical ostia of the coronary arteries were missing. After failing to detect coronary arteries in the echocardiography, CT scans were performed to confirm the diagnosis. The CT scan of the first patient was judged to be unnecessary as coronary arteries were believed to be seen in the echocardiography study. Thus, surgery was planned for an aortopulmonary (AP) shunt. Intraoperatively, aplasia of the coronary arteries was detected right at the start of the surgery and no further invasive measures were undertaken. It was decided to transfer the patient to the intensive care unit for palliative care, where he died within a few hours. In the second patient complete aplasia of both coronary ostia detected in the echocardiography study was confirmed in the CT scan and even in cardiac catheterization and the parents were advised about palliative care. This patient died on the third day of life due to impaired myocardial perfusion.

Conclusion: Few cases have been reported in the literature describing heart transplantation as a possible curative therapeutic option when coronary aplasia is detected. However, both patients in this study would not have survived long enough to have the chance for being listed for heart transplantation. Other studies have demonstrated that revascularization by coronary artery occlusion is possible. However, here at least the existence of coronary ostia is a prerequisite, which was not the case in our patients. Still, surgery for the first patient could have been avoided and this could have led to more time for compassionate care.

Publication History

Article published online:
28 January 2023