Introduction
Coronary anomalies are a rare but potentially fatal congenital defect that may occur in isolation or be associated with other CHDs. Although most patterns of coronary anomalies are benign, a few may be associated with high morbidity and mortality, and some are reported only in autopsies. Higher risk patterns include interarterial course, extensive myocardial bridges, and anomalous coronary origin of the pulmonary trunk. The totally anomalous origin of the coronary arteries from the pulmonary trunk is a rare type among these anomalies, with only 57 cases reported in the literature. Reference Fuenzalida1 For such a high-risk pattern, surgical correction is the treatment of choice. We present the case of a 14-month-old female who was referred to our centre with cardiogenic shock secondary to a totally anomalous origin of the coronary arteries from the pulmonary trunk and had a successful surgical repair and good cardiac function recovery.
Case report
A 14-month-old girl was transferred to our hospital for surgical evaluation after presenting with cardiogenic shock at another service. Her past medical history was remarkable for low weight gain and a recent history of tachypnoea, syncope, and epilepsy. After having four cardiac arrest episodes, she was diagnosed with anomalous left coronary artery from the pulmonary trunk. Admission tests at our centre revealed an electrocardiogram with left ventricular conduction delay, left ventricular hypertrophy, and altered inferolateral wall repolarisation abnormalities, as well as severe left ventricular dysfunction and hyperrefringence of the papillary muscles of the mitral valve on the echocardiogram. The left coronary artery arose from the right aspect of the pulmonary trunk; however, it was not possible to define the origin of the right coronary artery. A subsequent angiotomography reported a single coronary ostium originating from the right aspect of the pulmonary trunk, the anterior descending artery, the right coronary artery, and the circumflex artery. This arrangement was confirmed intraoperatively (Figure 1). Surgical correction was performed via direct re-implantation of the common coronary trunk in the left lateral wall of the aorta (Figure 2). The post-operative course was remarkable for progressive cardiac function recovery, and an echocardiogram without vasoactive support on post-operative day 45 showed preserved biventricular function and adequate single trunk re-implantation.
Preoperative tests. ( a ) 12-lead electrocardiogram. ( b ) Common coronary artery origin from pulmonary trunk (yellow arrow) on echocardiogram. ( c ) Angiotomography depicting total anomalous coronary artery origin from pulmonary trunk (yellow arrow) and its branches. ( d ) Angiotomographic 3D reconstruction showing common coronary origin from pulmonary trunk (yellow arrow) and its branches. Ao = Aorta; PT = Pulmonary trunk; LAD = Left anterior descending artery; RCA = Right coronary artery; Cx = Circumflex artery.
Surgical view. ( a ) Common coronary artery origin from pulmonary trunk (yellow arrow). ( b ) Common coronary ostium (yellow arrow). ( c ) Surgical repair: coronary aortic re-implantation (red arrow), pulmonary trunk reconstruction with autologous pericardium (green arrow): closure of the excised part of the pulmonary artery containing the coronary ostium with a patch (yellow asterisk) and left atrial line for post-operative monitoring (blue arrow). Ao = aorta; PT = Pulmonary trunk; RA = Right atrium; RV = Right ventricle.
Discussion
Coronary anomalies are rare cardiac lesions with an incidence that varies around 1%. Reference Fuenzalida1 Most congenital coronary artery anomalies are benign, but a few of them are associated with ischaemia, heart failure, arrhythmias, and sudden cardiac death in the paediatric population. Among the congenital coronary anomalies, anomalous origin of the coronary artery from the pulmonary trunk is an uncommon subtype, and its most common presentation is the anomalous left coronary artery. In this entity, antegrade deoxygenated blood flow occurs early in life, when pulmonary vascular resistance is high. As it decreases, collateral vessels develop, and retrograde flow is established and depends on higher aortic pressures from the normally originated coronary artery. Reference Singh, Di Carli, Sullivan, Leonen and Morrow2 However, the totally anomalous origin of coronary arteries from the pulmonary trunk is a rare entity among these rare coronary anomalies, where the entire coronary circulation emerges from the pulmonary trunk, which does not allow for compensatory mechanisms such as collateral circulation development to provide oxygenated blood to the territory supplied by the anomalous circulation. Therefore, it usually presents early in life, most often in the neonatal period, with a median age of those who were alive at presentation of 10 days of life. Reference Guenther3 A recent systematic review reported 57 cases, with almost half of them being autopsy reports. Reference Guenther3 However, there have been reported cases of later presentation, including a 7-year-old girl. Reference Feldt, Ongley and Titus4 Different from our patient, most cases have an associated cardiac lesion, which most frequently is a ventricular septal defect or patent ductus arteriosus. This finding may have a pathophysiological explanation: since cardiac perfusion is directly related to pulmonary trunk pressure, any lesion that increases it can provide adequate coronary perfusion. Additionally, left-to-right shunting leading to higher oxygen content in the pulmonary trunk of these patients increases oxygen delivery for the anomalous coronary arteries. Presentation usually includes cyanosis, respiratory distress, and signs of heart failure and cardiogenic shock, with low ejection fraction. Surgical repair aims at reestablishing coronary flow from the aortic root and may be achieved via aortic re-implantation or a Takeuchi-type repair, among other techniques. Reference Alzghari5 Although this condition has a high post-operative mortality rate, those who survive have a high chance of cardiac function improvement.
Conclusion
Although rare, a totally anomalous origin of the coronary arteries from the pulmonary trunk is a mandatory differential diagnosis for coronary anomalies in the paediatric population. Diagnosis awareness is key for this high-risk population, since the mortality is high and cardiac function recovery is achieved by most of those who have the chance of surgical correction. The most frequent clinical presentation is heart failure, and the treatment of choice is surgery through aortic re-implantation of the common coronary trunk in the aorta.
Acknowledgements
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Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
None.
