Automated implantable cardioverter defibrillator device implantation in young children is a rare procedure that typically necessitates intrapericardial lead placement. The extravascular implantable cardioverter defibrillator device can be implanted with the patient in a substernal position and with a straightforward and minimally invasive technique. The largest incision is made to accommodate the generator device. The alignment of the coils and device provides a good vector for defibrillation while permitting somatic growth. Here we describe implantation of an extravascular implantable cardioverter defibrillator in a 2-year-old child, among the youngest patients to receive this device to date.

The total cavopulmonary connection, more commonly known as the Fontan-Kreutzer procedure, is the final surgical step in the palliation of single ventricle congenital heart defects. The overwhelming success of the Fontan procedure has resulted in a worldwide population of 50,000 to 70,000 patients with a Fontan procedure. However, the resultant Fontan physiology, characterized by parallel systemic and pulmonary circuits, inevitably results in fatal long-term complications as a result of passive, nonpulsatile pulmonary blood flow, inefficient hemodynamics, and static conduit size which are inherent to the Fontan. Partial heart transplantation has dramatically altered the landscape of congenital heart surgery by providing a source of semilunar valve implants with the potential for growth and self-repair. Here we hypothesize that partial heart transplantation can also provide an ideal Fontan conduit characterized by pulsatility, growth potential, low thrombogenicity, and optimal flow dynamics.

Intrathoracic left subclavian to common carotid artery translocation is advocated for patients undergoing vascular ring division in the setting of an enlarged Kommerell’s diverticulum and aberrant left subclavian artery. This approach poses technical challenges in larger patients and patients with unfavorable body habitus. Supraclavicular left subclavian artery to common carotid translocation greatly simplifies this procedure in select patients.

Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein’s anomaly following Starnes procedure. The patient’s postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.