Outcomes of Berlin Heart EXCOR^® pediatric ventricular assist device support in patients with restrictive and hypertrophic cardiomyopathy

Abstract

The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR were identified. Of these, nine (45%) were successfully bridged to transplant, one (5%) weaned from support, and 10 (50%) died after support was withdrawn. Of patients under age 3 years (n = 13), 38.5% survived, whereas those aged 3 or older (n = 7) had 71.4% survival (P = .35). Biventricular assist device (BiVAD) patients experienced a 27.3% survival, vs 77.8% for patients with left ventricular assist device only (P = .07). Primary causes of death included stroke, infection, acidosis, multisystem organ failure, and bleeding. Pediatric patients with diastolic heart failure comprise a high-risk population for mechanical circulatory support. However, half of patients with this physiology have been successfully supported to explant with EXCOR . The trends toward higher mortality for younger patients and those receiving BiVAD support warrant consideration.

Impact of the 2016 revision of US Pediatric Heart Allocation Policy on waitlist characteristics and outcomes

“…Candidates with CHD, HCM, and RCM are less well supported by VAD than are children with DCM . In addition, infants and smaller children are also less well supported .…”

Mathematical modeling of cardiac function to evaluate clinical cases in adults and children

“…In the future, it is aimed to use this cardiovascular system model to simulate heart failure for pediatric patients in patient-specific studies to evaluate mechanical circulatory support. An important portion of the pediatric patients implanted with a heart pump exhibit complications associated with mechanical circulatory support [80]. Continuous and pulsatile flow LVADs are being tested to use in children.…”

Pediatric Restrictive Cardiomyopathies

“…Left atrial cannulation (such as in EXCOR ® Berlin Heart) is therefore an interesting option in patient with small ventricular cavity, preserved systolic function, and enlarged atria ( 80 ). A recent review of the American registry of EXCOR ® Berlin Heart implantation in pediatric patients affected by RCM showed a survival rate of 50%, which is significantly less than that of the overall EXCOR ® pediatric population (75%) ( 81 ). Primary causes of death included stroke, infection, acidosis, multisystem organ failure, and bleeding.…”