Abstract
We report a case of a 1-year-old patient with aortopulmonary window, anomalous right coronary artery arising from the pulmonary artery, and a large perimembranous ventricular septal defect. Computed tomography was successful in diagnosing the coronary anomaly and the case was managed surgically with an intrapulmonary baffle, rerouting the right coronary ostium to the aortic root and a fenestrated patch closure of the ventricular septal defect. Pre-operative diagnosis and thorough planning were essential for the successful outcome of this case.
Keywords: Anomalous right coronary artery arising from the pulmonary artery, Aortopulmonary window, Ventricular septal defect
Introduction
Aortopulmonary window (APW) is a rare congenital anomaly with an incidence of 0.2–0.6% in patients with congenital heart disease [1]. It results from incomplete development of the conotruncal septum and is characterized by a communication between the main pulmonary artery (PA) and the ascending aorta, in the presence of 2 separate semilunar valves [2]. The clinical picture of this anomaly is characterized by early manifestation of congestive heart failure due to the left-to-right shunt and the severity of the pulmonary hypertension. APW can rarely be associated with other cardiac lesions. Even though conotruncal anomalies have been known to be associated with coronary artery anomalies, APW with coronary anomalies has been reported in very few cases. We report a case of a 1-year-old patient with APW, ventricular septal defect (VSD), and anomalous right coronary artery arising from the pulmonary artery (ARCAPA).
Case report
A 1-year-old boy (weight 5 kg; saturation 99%) presented to us with poor weight gain, feeding difficulty, and history of recurrent lower respiratory tract infections. Chest X-ray showed pulmonary plethora with cardiomegaly (Fig. 1A). Transthoracic echocardiography showed the findings of a large type I APW (based on Mori’s classification [3]) with large perimembranous VSD with bidirectional shunt. Additional findings were dilated left atrium and left ventricle with flow reversal in the descending aorta. Cardiac computed tomography (CT) scan confirmed the above findings, along with an additional finding of ARCAPA (Fig. 1B, C).
Fig. 1.
A Pre-operative chest X-ray. B Axial view of computed tomography showing anomalous origin of the right coronary artery from the pulmonary artery (white arrow). C 3D reconstructed view showing the rare association
He underwent surgical repair that included intrapulmonary baffle closure of the APW and rerouting of the right coronary artery (RCA) ostia to the aortic root along with fenestrated patch closure of VSD. The anomalous origin of the coronary artery was on the APW defect rim, interfering with proper suture placement for closure of APW (Fig. 2A, B); hence, incision was made on the PA and a patch larger than the defect was used, creating a pericardial baffle to close the APW, rerouting the RCA ostia to the aorta (Fig. 2C). Fenestrated polyester patch closure of VSD was done due to severely elevated PA pressures and late presentation of the patient. Cardiopulmonary bypass time and cross clamp time were 83 min and 58 min respectively. Post-operative course of the patient was uneventful. He was discharged a week after the surgery, with echocardiography showing unobstructed coronary flow through the baffle and through branch pulmonary arteries, with fenestration shunting left to right and PA pressures being 2/3rd systemic.
Fig. 2.
A Intra-operative image with arrow showing the origin and course of the right coronary artery. B After pulmonary arteriotomy, coronary probe in the ostium of the right coronary artery with the sucker tip in the right pulmonary artery. C Post-repair image showing the intrapulmonary baffle closing the aortopulmonary window and rerouting the right coronary artery to the aortic root; sucker tip in the right pulmonary artery
Comment
Incidence of coronary artery anomalies is 1.3% in the general population [4], while the incidence of ARCAPA is 0.002% in the general population [5]. Anomalous origin of either the left or the RCA may occur in isolation or in association with other forms of congenital heart disease, in particular conotruncal abnormalities [6]. ARCAPA has been rarely reported with APW and these patients may not have any ischemic symptoms as the left-to-right shunt provides oxygenated blood at high pressure to the RCA. Echocardiography and angiography sometimes may not be able to delineate coronary anomalies in APW. The origins of coronary arteries need to be outlined in all patients with congenital heart defects before beginning surgical repair.
We decided for surgical management of this case based on findings like clinical presentation of left-to-right shunt (symptoms of failure to thrive, forehead sweating), pulmonary plethora and cardiomegaly on the chest X-ray, echocardiography findings of dilated left atrium and left ventricle, and flow reversal in the descending aorta. VSD closure was done with a small fenestration due to the presence of 2 large left to right shunts pre-operatively and late presentation of the child; as in our experience, fenestration can be beneficial in avoiding pulmonary hypertensive crisis. In this case, the primary concern with regard to the origin of the RCA was its proximity to the APW defect rim, thus causing hindrance in suturing of the patch. The use of intrapulmonary baffle for rerouting of the RCA to the aorta was done due to its proximity to the APW. This technique is simple and preserves the native implantation of the coronary artery, avoiding the risk of kinking or stretching after transfer. Other options for repair include coronary ostia transfer to the aortic root [7] (beneficial in cases where rerouting by a patch may create a narrowed tunnel to the coronary artery) and tunneling [8]. There have been reports of incidental on-table discoveries of this anomaly; hence, a thorough assessment of the coronary origins and course is highly essential in every case. Early diagnosis and surgical correction are essential to prevent the onset of intractable congestive heart failure and irreversible pulmonary vascular disease. The anomalous RCA must be identified at operation and dealt with appropriately as closure of the APW can cause acute ischemic changes or myocardial infarction from a sudden fall in the RCA pressure in the absence of well-developed anastomotic channels from the left coronary artery. Additionally, coronaries should be visualized in all cases of APW with higher placement of the aortic cross clamp and prevention of inadvertent injury to the coronary, while taking incision on the APW. Late complications from myocardial ischemia may also occur.
Funding
None.
Declarations
Ethical approval
Not applicable.
Informed consent
Taken from the patient guardian to publish this case report.
Conflict of interest
None.
Footnotes
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