Case report
A 6-year-old boy presented to our clinic due to a heart murmur detected during routine health screening. A systolic thrill was noted at the left sternal edge, accompanied by a grade 4/6 continuous ejection systolic murmur. The electrocardiogram revealed left ventricular dilatation. Chest radiography showed a dilated main pulmonary artery, resulting in a cardiothoracic ratio of 65%. Echocardiographic examination indicated enlargement of the left heart chambers. The color flow started just above the pulmonary valve and extended to the left pulmonary artery (Fig. 1). Although the outflow of the left coronary artery was observed, the outflow of the right coronary artery could not be clearly evaluated. As a result, it was planned to perform computerized tomography and angiography procedures for further differential diagnosis.
Figure 1. The color Doppler echocardiography image shows the flow of blood from the aortic root to the pulmonary artery and the right coronary artery flow jet directed into the pulmonary artery.
Computerized tomography imaging revealed a 7 mm diameter aortopulmonary window at the ascending aorta and proximal region of the pulmonary artery. Furthermore, it was determined that the right coronary artery originated from the proximal part of the pulmonary artery.
During cardiac catheterization, when injecting into the ascending aorta, it was observed that the pulmonary artery filled simultaneously, providing support for the diagnosis of an aortopulmonary window (Fig. 2a). Additionally, retrograde filling of the right coronary artery was observed during selective left coronary arteriography (Fig. 2b).
Figure 2. (a) The injection into the aortic root and the passage of contrast material into the pulmonary artery through the aortopulmonary defect. (b) The right coronary artery showed retrograde filling during selective injection of the left coronary artery.
Based on these findings, a surgical operation was planned for the patient. During the operation, a 7 mm diameter defect between the aorta and the main pulmonary artery was observed and primarily repaired. It was also noted that the right coronary artery was located adjacent to the pulmonary artery. The right coronary artery was prepared as a button and reimplanted into the ascending aorta. The patient was extubated on the first day of intensive care follow-up and discharged on the fifth day of hospitalization without any complications. Subsequent follow-up at our pediatric cardiology clinic revealed that the patient remained asymptomatic.
Discussion
The coexistence of aortopulmonary window and an abnormal origin of the right coronary artery from the pulmonary artery is rare. There are limited studies in the literature demonstrating the presence of both conditions together Reference Gabbieri, Guadalupi and Stefanelli1,Reference Barnes, Mitchell and Tweddell2 . Typically, this condition is detected in the neonatal period. However, in our case, it is noteworthy that the patient was 6 years old and remained asymptomatic.
Agarwala et al Reference Agarwala, Varga, Hijazi and Ziemer3 reported a case of a 5-month-old female infant with aortopulmonary window and an abnormal origin of the right coronary artery from the pulmonary artery. The patient was born prematurely at 34 weeks with a birth weight of 2.15 kg. Continuous heart murmur was detected, and after undergoing surgical operation, the patient was discharged without complications. Similarly, Alakhfash et al Reference Alakhfash, Tagelden, Almesned and Alqwaiee4 described a premature neonate who was diagnosed with aortopulmonary window and underwent a successful operation at the age of 4 months.
In both of these cases, the patients were in the neonatal age group, and they were treated with early diagnosis. In contrast, our case was 6 years old at the time of diagnosis and had not yet developed any clinical symptoms.
Atalay et al Reference Atalay, Soran Turkcan, Kulahcioglu, Eris, Ece and Aydın5 reported a case of a 45-day-old male infant who presented with congestive cardiac failure, murmur, and tachycardia. The patient underwent a successful one-step surgery. In this case, the detected diameter of the aortopulmonary window was around 7 mm, similar to our patient. However, it is important to note that the patient in this case had additional pathologies with left-right shunt, such as atrial septal defect and ventricular septal defect.
Wadhawa et al Reference Wadhawa, Mishra and Kothari6 described a case of a 19-year-old patient with tetralogy of Fallot and an anomalous origin of the right coronary artery from the aortopulmonary window. The patient presented with progressive dyspnea on exertion since childhood.
It is worth noting that in our case, the patient did not have any additional conditions other than the coexistence of the aortopulmonary window and the abnormal origin of the right coronary artery from the pulmonary artery. Furthermore, our patient was asymptomatic at the time of diagnosis.
In conclusion, when evaluating pediatric patients with only a murmur and no other clinical findings, the possibility of an aortopulmonary window should be considered. Although rare, the association between aortopulmonary window and coronary artery anomalies should be investigated in diagnosed cases. In situations where the coronary artery anatomy cannot be clearly evaluated or when there is suspicion, differential and diagnostic methods such as catheterization and computed tomography should be employed.
Acknowledgements
We would like to thank the paediatric cardiac surgeons (Dr Sertaç Haydin), who was involved in the care of this patient.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
None.
Ethical standard
The patient’s parents provided informed consent for the publication of this case report.
