Decision-making in congenital cardiac care, although sometimes appearing simple, may prove challenging due to lack of data, uncertainty about outcomes, underlying heuristics, and potential biases in how we reach decisions. We report on the decision-making complexities and uncertainty in management of five commonly encountered congenital cardiac problems: indications for and timing of treatment of subaortic stenosis, closure or observation of small ventricular septal defects, management of new-onset aortic regurgitation in ventricular septal defect, management of anomalous aortic origin of a coronary artery in an asymptomatic patient, and indications for operating on a single anomalously draining pulmonary vein. The strategy underpinning each lesion and the indications for and against intervention are outlined. Areas of uncertainty are clearly delineated. Even in the presence of “simple” congenital cardiac lesions, uncertainty exists in decision-making. Awareness and acceptance of uncertainty is first required to facilitate efforts at mitigation. Strategies to circumvent uncertainty in these scenarios include greater availability of evidence-based medicine, larger datasets, standardised clinical assessment and management protocols, and potentially the incorporation of artificial intelligence into the decision-making process.

We report a case of a patient with an anomalous left coronary artery originating from the opposite coronary sinus with evidence of intramural course at the level of the septal commissure and no slit-like deformation of the coronary ostium, acute take-off, or proximal vessel narrowing. According to previous publications, patients with anomalous coronary artery and intramural segments identified at surgery had coronary CT findings of acute take-off angle or proximal vessel narrowing; slit-like orifice; and elliptical cross-sectional shape. Although further investigation is required, we suggest that the intramural course may not be ruled out based on the absence of slit-like ostium, acute take-off, or proximal vessel narrowing.

We report on a patient with bicuspid aortic valve and anomalous right coronary artery from the opposite sinus without evidence of intramural course. Different authors support the universal presence of intramural course in patients with origin of the right coronary artery from the opposite sinus of Valsalva in normal heart. The occurrence of both bicuspid aortic valve and the absence of intramural course may not be accidental. This might suggest a developmental interaction between bicuspid aortic valve and anomalous coronary artery. Large observational study including characterisation by intravascular ultrasonography in patients with bicuspid aortic valve and anomalous coronary is needed.

We report on the diagnosis of anomalous coronary artery in two brothers. Following the diagnosis of anomalous coronary artery in one sibling, we screened immediate family relatives and found the same anomaly in the older brother. Familiarity in this pathology is extremely rare. We analysed and compared clinical, echocardiographic and radiological findings in the two brothers.

Myocarditis and coronary artery anomalies are both potentially life-threatening aetiologies of cardiac chest pain in children. We present a case of a young man presenting with non-exertional chest pain and subsequently found to have an anomalous origin of the right coronary artery from the left coronary sinus with an interarterial course in addition to a diagnosis of myocarditis. The patient subsequently was able to undergo surgical correction of his anomalous coronary to mitigate the risk of sudden cardiac death.

CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.

Percutaneous pulmonary valve placement in patients with an anomalous coronary artery is rare and can be complicated by coronary artery compression. We report successful implantation of a percutaneous pulmonary valve in a patient with an anomalous left anterior descending artery.

Stress echocardiography in paediatrics is used to evaluate pre- and post-operative coronary artery conditions, as well as to gain haemodynamic information for a variety of diagnoses, although evidence regarding sensitivity, specificity, and predictive value is lacking. This review will consider the available literature with a focus on anomalous aortic origin of the coronary arteries and discuss a practical approach to test selection and use.

In the United States, hypertrophic cardiomyopathy and coronary artery anomalies account for the leading two causes of sudden death in athletes. We present a case of a patient with an anomalous origin of the left main from the right coronary sinus with associated gene-confirmed hypertrophic cardiomyopathy. The patient underwent surgical repair with unroofing of the intramural portion of the left main coronary artery with a good result. We also review the reported cases in the medical literature describing this uncommon association between anomalous coronary artery origin and hypertrophic cardiomyopathy.

We report the case of a 1-month-old boy with an unusual association of supraventricular tachycardia and anomalous origin of the left coronary artery from the pulmonary artery. Although signs of infarction were visible on the first electrocardiogram, the presence of an arrhythmia did not immediately suggest a coronary anomaly. Echocardiography allowed the diagnosis, thus leading to appropriate care.

We describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.

Anomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly. Diagnosis is usually made at autopsy or incidentally in asymptomatic adults, but cardiopul-monary arrest and sudden death have been reported. The angiographic findings have been well described but the echocardiographic findings are less well documented.

This report describes the echo and colour Doppler findings in a 3-year-old child with an anomalous right coronary artery arising from the pulmonary trunk, as well as observations on myocardial perfusion demonstrated by nuclear angiography.

A common brachiocephalic trunk is an anatomic variant in which both common carotid arteries and the right subclavian artery arise from the aortic arch via a single trunk. The impact of this condition on associated congenital cardiac malformations is presently unknown. Out of a total of 1480 cardiac catheterizations performed in children over a period of 10 years, we discovered 48 patients (3.2%) to have a common brachiocephalic trunk, of whom 98% had associated congenital cardiac malformations. A spectrum of associated lesions was identified, including left-to-right shunts in 19 patients, right-sided anomalies in 18 patients, left-sided obstructive lesions in 12 patients, and coronary arterial abnormalities in 10 patients, eight of whom had other cardiac defects. Genetic syndromes were present in one-fifth of the cases. When found with left-sided malformations, the common trunk was associated with persistent hypoplasia of the aortic arch, likely related to diminished flow through the arch during development. In each of four patients in whom the brachiocephalic trunk had been used during construction of a palliative shunt, we observed inadequate growth and deformation of the pulmonary arteries. Thus, angiographic identification of a common brachiocephalic trunk may be a marker for the presence of accompanying congenital cardiac defects and coronary arterial abnormalities. Understanding the pathophysiologic effects of the common trunk is important when planning the palliative or corrective procedures, and when assessing the potential benefit of the surgical repair over the long term.

We report a rare variant of anomalous origin of the left coronary artery from the non-adjacent sinus of the pulmonary trunk. The patient also had the right coronary artery arising from the non-facing sinus of the aorta.