Surgical treatment for anomalous origin of the right coronary artery from the pulmonary artery: a case report with five-year follow-up

Abstract

Background Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital heart disease affecting about 0.002% of the population. Knowledge of ARCAPA is almost collected from case reports. The aim of this study was to provide a rare case to better understand this rare congenital coronary anomaly. Case presentation We report a rare case of an 18-year-old male who was initially referred because of heart murmur. Dilated and tortuous coronary arteries were detected by echocardiography and congenital coronary anomaly was suggested. Further coronary CT angiography confirmed the diagnosis of ARCAPA. Although dual coronary system provides favorable long-term outcome, bypass surgery was considered technically difficult due to the huge mismatch of caliber between the right coronary artery and graft vessels. Eventually, simple right coronary artery ligation was performed. The patient was followed up for about 5 years without evidence of atherosclerosis or myocardial ischemia. Conclusions ARCAPA presents as a rare congenital heart disease with variable clinical manifestations. Surgical treatment is highly recommended to re-establish dual coronary system and prevent further complications. To our best knowledge, only about 200 cases of ARCAPA has been reported.

Cardiac imaging findings in anomalous origin of the coronary arteries from the pulmonary artery; narrative review of the literature

“…If this phenomenon happens, symptoms of ischemia appear in the early years. As for later onset symptoms, because the single functioning coronary system is more prone to atherosclerosis, ARCAPA patients develop ischemia around 40–60 [ 6 ].…”

“…The left coronary artery is usually enlarged and tortuous and collateral arteries are present in the ventricular septum as seen in ALCAPA. Wall motion abnormalities in ventricles can also be seen in TTE [ 4 , 6 , 10 – 14 , 28 , 32 ]. When there is a dilated LCA and continuous or diastolic retrograde flow from an anomalous vessel to the pulmonary artery, ARCAPA can be mistaken for a coronary fistula.…”

Challenging Diagnosis of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery

“…According to the pathophysiology, the age of the presentation has a bimodal distribution with one peak near after birth (insufficient collateral circulation) and another around 40 to 60 years of age (good collaterals) [ 7 , 14 ]. A review conducted in 2019 has identified a total of 223 cases in 193 case reports and the median age at presentation was 14.0 years old [ 14 ].…”

“…Although asymptomatic, general recommendations suggest an early corrective intervention to prevent complications such as myocardial ischemia and cardiac dysfunction, which can lead to sudden death [ 26 , 27 , 28 , 29 ]. Among the corrective interventions applied in this type of coronary anomaly, three surgical strategies are described: reimplantation of the RCA into the aorta, RCA ligation, and RCA ligation with coronary artery bypass grafting and the treatment of the associated cardiac pathology, sometimes being necessary to combine surgery with interventional cardiology for associated lesions [ 7 , 30 , 31 ].…”

“…When not combined with other congenital heart defects, this condition is often asymptomatic and is found incidentally. There is a significant difference compared to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), considered the most common type of coronary artery origination anomaly from the pulmonary artery, especially regarding the high rate of mortality in infancy and early childhood [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ]. However, some patients with ARCAPA also may suffer from exertion angina, and cases of sudden cardiac death were reported as well [ 8 ].…”