A diagnosis of dilated cardiomyopathy was made. The child was intubated and ventilated, and managed with inodilation, adrenaline and isoprenaline for bradycardia. A cardiomyopathy screen returned normal. The child’s condition did not improve and the ECG developed progressive ST changes consistent with ischaemia as well as frequent ventricular ectopics and runs of tachycardia. The echocardiogram was repeated specifically to re-assess the LCA. The original appearance of a normal connection and direction of flow could be reproduced but was in fact misleading. The vessel originally thought to be the LCA was instead a small branch of the right coronary artery (RCA) coursing in the direction the LCA would ordinarily take, and therefore showed antegrade flow. A much larger vessel, the LCA, could be seen arising from the pulmonary artery. The direction of flow was retrograde (see Fig. 7.2c, d). A diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) was made. Reduction of the adrenaline and isoprenaline infusions resulted in less ventricular ectopy, and surgery was undertaken the same day.

At operation there was extensive collateralisation around the aorta and pulmonary artery, possibly accounting for the initial echocardiographic impression of a normal LCA. The LCA was transferred to the aorta, but despite good flow being seen on epicardial echocardiogram myocardial performance remained poor. The child was weaned off cardiopulmonary bypass, but due to the advanced state of left ventricular damage suffered persistent ventricular fibrillation and was placed on extra-corporeal membrane oxygenation (ECMO) prior to transfer to the intensive care unit. A week later the patient was separated from ECMO; however, she remained fully dependent on inotropic support and positive pressure ventilation, and experienced frequent ventricular tachyarrhythmias requiring resuscitation. Five weeks post-operatively one such arrest necessitated re-institution of EMCO support. Ventricular dysrhythmias continued despite a continuous amiodarone infusion and fastidious management of electrolyte balance.

There were grave concerns over the extent of irreversible myocardial damage by the time of presentation, and that transplantation represented the only possibility of survival. The patient was transferred to a transplant centre and support converted to a biventricular Berlin Heart. She was listed for urgent cardiac transplantation; however, she developed complications of mechanical circulatory support, including bacterial infection and evidence of neurologic injury manifested as choreoathetoid movements upon weaning of sedation. She also had persistent atelectasis of the left lower lobe due to gross cardiomegaly-induced compression of the left main bronchus. An initial CT demonstrated only a small sub-acute subdural haemorrhage; however, she subsequently became less responsive and developed fixed dilation of the left pupil. A repeat CT demonstrated a devastating intracranial haemorrhage. Following discussion with the patient’s parents, active support was withdrawn and care re-orientated.