Right Coronary Artery Aneurysms in Pulmonary Atresia with Intact Ventricular Septum

Aneurysmal dilatation of the coronary arteries in patients with pulmonary atresia with intact ventricular septum (PA/IVS) were described only in a few cases.

A 15 year old adolescent with PA/IVS and severely hypoplastic tricuspid valve was referred for CT and invasive hemodynamic evaluation of his coronary arteries. He underwent Rashkind maneuver at the age of 3 days due to restrictive atrial septal defect. BT-anastomosis was performed at the age of 15 days. He received a bidirectional Glenn anastomosis with 9 months and a total intracardial cavopulmonary connection at the age of 3 years. Due to stenosis of the RA-SVC anastomosis a CP-Stent (8 × 45 mm) was implanted in the superior vena cava at the age of 13. Myocardial infarction or ischemia has never occurred, Oxygen saturation was 96%. The patient is taking phenprocoumon for years.

Cardiac CT showed left and right coronary artery (RCA) arising from the same left facing coronary sinus. While the LAD interrupts after short course, the ramus circumflexus (LCX) mainly supplies the left ventricle. The right coronary artery originates more anterior, it was dilated and shows two aneurysms. It empties with two sinusoids into the RV. Cardiac catheterization on the following day confirmed two aneurysmata. One nonpulsatile in the RCA (12 × 11 mm), and the other one pulsatile in an RCA branch (9 × 7.5 mm). Cine mode reveals systolic filling and diastolic downsizing of the smaller aneurysm. There was no diastolic retrograde coronary perfusion from the right ventricle (RV) to the RCA and no coronary artery stenosis. A closer look at the angiograms of the patient before Glenn procedure revealed one aneurysm at the same location as we now see the bigger aneurysm with a diameter of 8 × 8 mm.

Coronary aneurysms of the RCA in a patient with PA/IVS following total cavopulmonary anastomosis have not yet been described. The case shows relatively stable size of one of the aneurysms over years without ischemic complications. Since one of the aneurysms shows pulsatility, an RV dependent circulation must exist.

It is not known whether any kind of intervention will prevent complications (e.g., rupture or embolism) of such aneurysms. Any intervention should be taken very cautiously, since decompression of the right ventricle could lead to coronary steal phenomenon with ischemia. However, close follow up with adequate imaging and anticoagulation therapy is mandatory.