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

Abstract

Anomalous origin of the right coronary artery (RCA) from the pulmonary artery, ARCAPA, is an extremely rare congenital heart disease. Only 200 cases were reported from 1885 to the present. Patients diagnosed with ARCAPA can be either asymptomatic or can experience symptoms, such as heart murmur, dyspnea, or angina, shortly after birth or around 40–60 years of life. Usually, those with isolated ARCAPA are diagnosed later in life compared to those who associate other structural cardiac defects. We report two cases of anomalous origin of the right coronary artery at the level of the pulmonary artery trunk (ARCAPA) that were diagnosed by invasive coronary angiography. Although asymptomatic, general recommendations suggest an early corrective intervention to prevent complications such as myocardial ischemia and cardiac dysfunction, which can lead to sudden cardiac death.

Anomalous right coronary artery from the pulmonary artery in an infant with tetralogy of Fallot

“…In such instances, the major structural heart defect gets highlighted in the preoperative work and the coronary anatomy may be relegated to a secondary “checklist” status with potentially disastrous consequences if the ARCAPA is undetected. [ 3 ] Although both coronary ostia can be seen clearly on the majority of echocardiograms done on infants, occasionally, artifacts may be mistaken for a coronary ostium. [ 2 ] The artifacts may be pericardial spaces or drop-out artifacts when the wall is parallel to the sonography beam [ Figure 3a ].…”

“…The investigative modality of choice beyond echocardiography in such a situation is angiography or a CT scan. [ 3 ] We chose to do a CT scan as it is less invasive. The surgical options for ARCAPA are translocation and reimplantation or ligating the RCA with or without a bypass graft.…”

“…[ 1 ] It is often discovered incidentally through imaging or may manifest as heart failure, sudden cardiac death, or syncope. [ 2 3 4 ] In addition, ARCAPA can be associated with other congenital heart defects (CHD) such as tetralogy of Fallot (TOF), aortopulmonary window, and simple shunts in a third of cases, emphasizing the importance of diligent preoperative assessment.…”

Discovery and functional investigation of BMP4 as a new causative gene for human congenital heart disease

“…Congenital heart disease (CHD) is the most frequent type of human birth malformation globally, occurring in approximately 0.8%-1.2% of all live births and roughly 10% of miscarriages globally, accounting for nearly 33% of all congenital deformations [1][2][3]. Notably, if minor cardiac developmental aberrations are encompassed, such as patent foramen ovale and aortic bicuspid valve, the prevalence of CHD rises to around 5% of all live births [4][5][6]. As an array of cardiovascular developmental deformations, CHD is clinically categorized into > 30 distinct isoforms, encompassing double-outlet right ventricle (DORV) and ventricular septal defect (VSD) [2,[7][8][9][10][11][12][13][14].…”

Anomalous Arising of Right Coronary Artery from the Pulmonary Artery

“…Coronary artery origin anomalies are rare congenital heart diseases (CHD) that are divided into four groups: anomalous origin of the coronary artery from the pulmonary artery (PA); the ectopic origin of the coronary artery from the aortic sinus; the absence of a coronary artery; and a coronary artery fistula [1]. Coronary artery anomalies are seen in less than 1% of the general population and in 1.6% of cardiac catheterization cases [1,2]. Coronary artery origin from the PA is a part of this group, with an incidence of 0.01% in the general population [3,4].…”