Mechanical circulatory support (MCS) for failing single ventricle (SV) physiology is a complex and challenging problem, which has not yet been satisfactorily addressed. Advancements in surgical strategies and techniques along with intensive care management have substantially improved the outcomes of neonatal palliation for SV physiology, particularly for hypoplastic left heart syndrome (HLHS). This is associated with a steady increase in the number of SV patients who are susceptible to develop heart failure (HF) and would potentially require MCS at a certain stage in their palliation. We have reviewed the literature regarding the reported modalities of MCS use in the management of SV patients. This includes analysis of various devices and strategies used for failing circulation at distinct stages of the SV pathway: after neonatal palliation, after the superior cavo-pulmonary connection (SCPC), and after total cavo-pulmonary connection (TCPC).

HeartMate III is an emerging, small-sized centrifugal ventricular assist device. Its lower pump thrombosis and stroke rates make it favorable for use in pediatrics. We report the use of HeartMate III as a bridge to transplantation in an adolescent with failed Fontan circulation. (Level of Difficulty: Advanced.)

Ventricular assist device (VAD) management continues to be a challenge in the presence of restrictive physiology. Left atrial (LA) decompression is not satisfactory even with good function and position of the left ventricular cannula. We describe an alternate approach with LA cannulation via the left atrial appendage (LAA) as a rescue strategy in a patient who had restrictive physiology, in our case was secondary to viral myocarditis acute systolic heart failure with subsequent insidious diffuse endomyocardial fibrosis and superimposed massive calcification, causing inadequate emptying of the left ventricle despite optimal VAD apical cannula position.

1p36 deletion is the most common terminal deletion syndrome in humans. Herein, we report two cases, a 5-month-old female and a 14.5-year-old female, both with 1p36 deletion and left ventricular non-compaction cardiomyopathy. They presented with severely depressed left ventricle function and underwent heart transplantation with excellent outcomes. Given the incidence of heart defects and cardiomyopathy in 1p36 deletion syndrome, it should be recommended that children with this genetic condition have screening for cardiac disease. These cases add to the current literature by demonstrating the potential therapeutic options for non-compaction in 1p36 deletion syndrome and showed the favorable outcomes.

We report a 6-year-old with single ventricle physiology secondary to tricuspid atresia who had cardiorespiratory failure who was not a candidate for further single ventricle palliation. The patient underwent planned staged left pneumonectomy for recurrent pneumonias secondary to bronchomalacia followed by orthotopic heart transplantation. This aggressive approach improved the patient candidacy for heart transplantation by removing the source of recurrent infection and respiratory failure (left lung).