Mechanical Circulatory Support for End-Stage Heart Failure in Repaired and Palliated Congenital Heart Disease

Joseph B Clark,Linda B Pauliks,Akif Undar,John L Myers

doi:10.2174/157340311797484222

Joseph B Clark, Linda B Pauliks + Show 2 more

Open Access

https://doi.org/10.2174/157340311797484222

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Abstract

Approximately one in one hundred children is born with congenital heart disease. Most can be managed with corrective or palliative surgery but a small group will develop severe heart failure, leaving cardiac transplantation as the ultimate treatment option. Unfortunately, due to the inadequate number of available donor organs, only a small number of patients can benefit from this therapy, and mortality remains high for pediatric patients awaiting heart transplantation, especially compared to adults. The purpose of this review is to describe the potential role of mechanical circulatory support in this context and to review current experience. For patients with congenital heart disease, ventricular assist devices are most commonly used as a bridge to cardiac transplantation, an application which has been shown to have several important advantages over medical therapy alone or support with extracorporeal membrane oxygenation, including improved survival to transplant, less exposure to blood products with less immune sensitization, and improved organ function. While these devices may improve wait list mortality, the chronic shortage of donor organs for children is likely to remain a problem into the foreseeable future. Therefore, there is great interest in the development of mechanical ventricular assist devices as potential destination therapy for congenital heart disease patients with end-stage heart failure. This review first discusses the experience with the currently available ventricular assist devices in children with congenital heart disease, and then follows to discuss what devices are under development and may reach the bedside soon.

Highlights

Survival rates for patients with repaired or palliated congenital heart disease are quite good, there remain a minority of patients who may experience either acute heart failure in the early postoperative period or end-stage heart failure much later [1]
Extracorporeal life support (ECLS) in the setting of congenital heart disease is most commonly applied in the early postoperative period for acute heart failure, and these patients are typically supported with extracorporeal life support systems, such as extracorporeal membrane oxygenation (ECMO)
For the repaired or palliated congenital heart disease patient with severe and intractable ventricular dysfunction, mechanical circulatory support can serve as a bridge to transplantation

Summary

Introduction

Survival rates for patients with repaired or palliated congenital heart disease are quite good, there remain a minority of patients who may experience either acute heart failure in the early postoperative period or end-stage heart failure much later [1]. Mechanical circulatory support can offer several important applications in the management of both acute and chronic heart failure following surgery for congenital heart disease [2,3,4]. Extracorporeal life support (ECLS) in the setting of congenital heart disease is most commonly applied in the early postoperative period for acute heart failure, and these patients are typically supported with extracorporeal life support systems, such as extracorporeal membrane oxygenation (ECMO). For the repaired or palliated congenital heart disease patient with severe and intractable ventricular dysfunction, mechanical circulatory support can serve as a bridge to transplantation. Lower survival rates have been observed in patients who were younger and had cardiac failure, with recent survival rates of 39% for neonatal cardiac patients [6]

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