Mechanical Circulatory Support for Single Ventricle Failure

Abstract

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).

Hybrid approach for management of end-stage heart failure in complex congenital heart disease

“…In fact, paracorporeal pulsatile (PP) and continuous devices are usually applied in smaller and sicker children (weight <20 kg, more often with CHD, and INTERMACS 1-2), while intracorporeal devices are more frequently used in children >20 kg, with better INTERMACS level. 2 Also, when considering VAD support in children with CHD, it is fundamental to understand the mechanism of ventricular dysfunction, 3 since ESHF may be caused by LV dysfunction (amenable of LVAD support only), or by adverse ventricular-ventricular interdependence, in which LV dysfunction may be secondary to RV failure (e.g. dilated RV after TOF).…”

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

“…In the USA and Europe, patients with single-ventricular physiology have a higher incidence of adverse events during LVAD support. 554 The mortality rate of patients with CHD on the waiting list has been higher than that of those without. In 2018, UNOS (United Network for Organ Sharing) changed the donor allocation system (Table 29) 555 to give priority to CHD patients (New status 4) and to the patients with single-ventricular physiology on LVAD support (New status 2).…”

Review on Mechanical Support and Cell-Based Therapies for the Prevention and Recovery of the Failed Fontan-Kreutzer Circulation

“…This section will briefly identify the most common long-term MSDs that have been implanted in patients with a failing Fontan circulation. Reviews devoted to MSDs for patients with single ventricle physiology have been published and should be referenced for a detailed landscape of all mechanical assist options ( 36 – 39 ).…”