Journal Of Indian College Of Cardiology

: 2021  |  Volume : 11  |  Issue : 1  |  Page : 37--39

Anomalous right coronary artery from the pulmonary artery in a young infant with ventricular septal defect

Anil Kumar Singhi1, Richard Hawker2, Gary Sholler3,  
1 Department of Pediatric Cardiology, Medica Superspecialty Hospital, Kolkata, West Bengal, India
2 Heart Centre for Children, Children's Hospital at Westmead, Sydney, Australia
3 SCHN Cardiac Service, Heart Centre for Children, Children's Hospital at Westmead, University of Sydney, Sydney, Australia

Correspondence Address:
Dr. Anil Kumar Singhi
Senior Consultant, Department of Pediatric Cardiology, Medica Super Specialty Hospital, Mukundapur, Kolkata - 700 099, West Bengal


We report a case of anomalous right coronary artery from pulmonary artery (ARCAPA), which is a very rare congenital coronary defect. It can be an isolated lesion or associated with congenital cardiac defects such as tetralogy of Fallot or shunt lesions in up to 40' of cases. The clinical presentation varies from asymptomatic to signs of myocardial ischemia, cardiac failure, and/or ventricular arrhythmia. The electrocardiogram can be normal in a significant fraction of the cases. The diagnosis of ARCAPA may be confirmed by cardiac catheterization, computed tomographic angiogram, and cardiac magnetic resonance angiogram. Reimplantation of anomalous coronary artery is the treatment of choice. In the current era, systematic preoperative screening by echo is recommended.

How to cite this article:
Singhi AK, Hawker R, Sholler G. Anomalous right coronary artery from the pulmonary artery in a young infant with ventricular septal defect.J Indian coll cardiol 2021;11:37-39

How to cite this URL:
Singhi AK, Hawker R, Sholler G. Anomalous right coronary artery from the pulmonary artery in a young infant with ventricular septal defect. J Indian coll cardiol [serial online] 2021 [cited 2021 Apr 12 ];11:37-39
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Full Text


An anomalous right coronary artery from the pulmonary artery (ARCAPA) is a very rare congenital abnormality. In young patients, it is frequently asymptomatic and therefore undetected. From a review of past cases of anomalous coronary arteries, we report the case of a young infant with a large ventricular septal defect (VSD) and anomalous right coronary artery (RCA) from the pulmonary artery, whose abnormality was obscured by abnormal preoperative hemodynamics and was identified on the operating table.

 Case Report

A 3-month-old girl weighing 4 kg was symptomatic with an unrestrictive perimembranous VSD with significant left-to-right shunt. She was admitted for the repair of the defect. During the operation, there was an unexpected finding of an ARCAPA. The surgical team wished to obtain more information about the coronary anomaly and wished to rule out any additional lesion. The chest was closed under aseptic precaution, and the baby was moved directly to the cardiac catheterization Laboratory. The preoperative electrocardiogram (ECG) was reanalyzed in view of an anomalous coronary artery. The preoperative ECG showed sinus rhythm @148/min, with Q-waves in the leads II III aV, and no evidence of any significant ischemia [Figure 1]. The cardiac catheterization demonstrated the lesion with an aortic root angiogram in the anteroposterior and lateral view showing a normal origin of the left coronary artery (LCA) from the left aortic sinus [[Figure 2]a and [Figure 2]b, Movie 1]. The LCA divided normally to left anterior descending and left circumflex artery.{Figure 1}{Figure 2}

The RCA was not seen originating from the aorta. The pulmonary artery angiogram revealed a smaller caliber RCA originating from the main pulmonary artery [[Figure 3], Movie 2] A left ventricular angiogram showed a single large subaortic VSD, unobstructed left ventricular outflow tract [[Figure 4], Movie 3]. The patient was relocated to the operating theater. The baby underwent VSD surgical closure along and reimplantation of the RCA to aorta. The postoperative recovery was uncomplicated. At follow-up over 8 years, she exhibited normal growth and development, with a normal ECG was normal and normal function on echocardiographic study.{Figure 3}{Figure 4}


Congenital coronary artery anomalies are seen in 0.2'–1.4' of the general population. Anomalous origin of the RCA from pulmonary artery is an extremely rare among them with an incidence of 0.002'. The anomaly was first reported by Brooks in 1885. ARCAPA has a wide range of clinical presentations. It is associated with other congenital cardiac anomalies in 30'–40' of the cases. The common associations are tetralogy of Fallot, shunt lesions like aorto-pulmonary window, VSD, atrial septal defect, anomalous pulmonary venous drainage, and arch anomalies, etc.[1],[2],[3] The hemodynamic effects of ARCAPA depend on the magnitude of the left-to-right shunt, myocardial oxygen demand, and the extent of the collateralization. The less oxygen demand of the right ventricle makes this entity have less ischemia, then more frequently found anomalous LCA from pulmonary artery.[4],[5] In the patients of ARCAPA with posttricuspid shunt, the increased pulmonary blood flow and elevated pulmonary artery pressure prevent retrograde flow from LCA to pulmonary artery. This also restricts the coronary steal phenomenon and limits the ischemia. When the pulmonary vascular resistance reduces and the oxygen demand increases in different circumstances, then the ischemia sets in. Up to 40' of the ARCAPA patients remain asymptomatic and are incidentally detected. In symptomatic cases, the manifestation ranges from myocardial ischemia, heart failure ventricular arrhythmia, and episodes of sudden cardiac death. Interestingly, the ARCAPA does not show the features of classic ischemic changes in ECG of all the patients. The ECG may be completely normal in the significant number of patients, while in others, it can show the features of ventricular enlargement. Q-wave in inferior leads and ST- and T-wave changes.[2] Echocardiogram reveals anomalous connection of the RCA to pulmonary artery and the evidence of retrograde flow in the coronary artery to pulmonary artery when pulmonary vascular resistance reduces. In our patient, the large VSD allowed for persistent pulmonary hypertension to counterbalance the effects of abnormal connection. The ischemia resulting from an undiagnosed, significant ARCAPA may result in myocardial dysfunction, papillary muscle scarring more so in advanced age. Echocardiographic study may miss the diagnosis in suspected cases,[6] however, in the current era, regularized imaging of the coronary origins forms the part of routine preoperative imaging. Where there is continued uncertainty, the diagnosis of ARCAPA can be confirmed with the next level imaging modality such as coronary angiography, computed tomographic angiogram, or cardiac magnetic resonance angiogram. Some patients can develop myocardial ischemia which can lead to ventricular arrhythmia and sudden cardiac death. Hence, all cases of ARCAPA with or without associated congenital heart disease should be surgically repaired. Translocation of the anomalous coronary artery to the aorta is the treatment of choice. ARCAPA surgery has good short- and long-term outcomes as observed in our index case.[3],[7]


ARCAPA is a rare and unique congenital anomaly with varied clinical presentation. It can be associated with other congenital heart diseases. The anomaly may be missed in routine evaluation unless regularized focus on the coronary origins is part of preoperative and general pediatric cardiac echocardiography. Correction is achievable by reimplantation or baffle redirection with excellent outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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