This is a case presentation of an 18-month-old female who presented to the emergency department with altered mental status. In the emergency room, the patient’s electrocardiogram (EKG) raised concerns for biventricular hypertrophy.
A transthoracic echocardiogram indicated that her right coronary artery originated from the main pulmonary artery (ARCAPA) and measured approximately 1.8 to 2.2 mm, widening distally to 2.2 to 2.4 mm (z-score approximately 2). The origin appeared to be at the medial wall of the pulmonary sinotubular junction. Additional findings showed a moderately dilated left main coronary (z-score approximately 3.6) and a severely dilated left anterior descending artery (z-score approximately 8).
The cardiothoracic surgical team elected to proceed with surgical repair of the ARCAPA using a coronary button reanastomosis.
Surgery
The patient was brought to the operating room, prepped accordingly, and the chest was opened in the standard fashion. Standard cardiopulmonary bypass was initiated following cannulation of the distal ascending aorta and bicaval venous cannulation. After application of the aortic cross-clamp, antegrade del Nido cardioplegia was administered at a dose of 15 cc/kg to achieve diastolic arrest.
Attention was directed back to the main pulmonary artery, which was then divided at its bifurcation. Using Pott’s scissors, a coronary button was excised and further mobilized away from the pulmonary trunk using electrocautery. An incision was made to the anterior aorta with an 11-blade scalpel and was further widened using an aortic punch. The coronary button was then anastomosed to the anterior side of the ascending aorta and secured using 6-0 Prolene in a running fashion. A piece of Cormatrix was then brought into the surgical field, trimmed to the appropriate size and shape, and used to repair the area of the coronary button graft. It was secured using 6-0 Prolene in a running fashion. Attention was then drawn back to the pulmonary trunk. The branch pulmonary arteries were then reanastomosed back to the main pulmonary trunk with 6-0 Prolene in a running fashion.
After adequate rewarming and hemostasis, the patient was separated from cardiopulmonary bypass and decannulated. The patient was extubated in the operating room and transferred to intensive care.
Postoperation
Cardiopulmonary bypass time was 45 minutes, and aortic cross-clamp time was 33 minutes. A postoperative transesophageal echocardiogram confirmed successful reimplantation of the right coronary artery to the ascending aorta above the sinotubular junction with predominantly prograde flow. There was no evidence of supravalvular pulmonary artery stenosis. The patient was discharged from the cardiovascular intensive care unit on day four.
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