Anomalous Right Coronary Artery Origin From Pulmonary Artery: ARCAPA Surgery

The authors present an extremely rare congenital heart disease that was operated on for a 2.5-year-old boy from the northeast of Brazil, who was born and still lives in an indigenous tribe. The clinical examination was normal; his family and tutors took him to the medical facility because he had an isolated syncope episode. There was no fatigue or chest pain. He was apparently healthy.

The electrocardiogram was normal, as was the chest roentgenogram. The echocardiogram showed an increased diameter of left coronary artery origin, besides the right one from the pulmonary trunk. The biventricular function was normal with moderately overloaded right chambers. The computed tomography angiogram on the axial view demonstrated mildly oversized right chambers, an enlarged ostium of the left coronary artery origin from the aorta, and the right coronary artery (RCA) arising from the anterior face of the pulmonary trunk (ARCAPA). The coronary angiogram showed the contrast medium injected into the left coronary artery crossing over into the right one through abundant collaterals draining straight into the pulmonary artery (PA).

The surgery was performed by a standard sternotomy; the pericardium was cut and pulled aside to the drapes. The procedure was done under mild hypothermic cardiopulmonary bypass (CPB, rectal temperature from 34°C to 36°C) using standard aortic and bicaval venous cannulations. Left heart decompression was achieved by a vent inserted into the left ventricle through the interatrial septum. The right atrium was widely opened to vent the right ventricle and PA. The RCA and the pulmonary-aorta window were freely dissected, a pretty small right ventricle outflow tract (RVOT) coronary artery was detached and ligated. An aortic cross-clamp was applied, and del Nido cardioplegia was infused. The initial dose was given into the aortic root and also into the right coronary ostium by opening the PA. A core temperature of 34°C was reached.

The right coronary ostium was harvested with a large cuff. The pulmonary trunk was reconstructed with an autologous pericardial patch with a standard running suture of 6-0. The remnant patent ductus arteriosus was ligated and divided to accomplish a better mobilization of the PA. An average 5 mm orifice was made in ascending aorta. A tension-free suture between RCA and aorta was done using 7-0 polypropylene, taking usual care to avoid malrotation. The procedure was completed, and the patient weaned from CPB. The initial inotropic support protocol included milrinone (0.4) and epinephrine (0.1). The cross-clamp time was 55 minutes. The patient had an uneventful postoperative course and was discharged from the hospital on the fifth postoperative day.

Several surgical techniques have been proposed for dealing with anomalous left coronary artery arising from the pulmonary artery and, in the current surgical era, the establishment of a dual coronary arterial system is considered the gold standard. Given an anterior position of the ARCAPA, it facilitates to carry out direct reimplantation avoiding further extensive mobilization and even allowing a tension-free anastomosis.

Even though the patient is living in a remote indigenous area, his follow-up has been done on a regular basis. He is asymptomatic after six months, and the echocardiogram shows that the RCA flow and biventricular function are both normal.