Heart transplantation in pediatric cardiomyopathies

Abstract

Cardiomyopathies represent an uncommon but serious cause of heart disease in the pediatric population and can be categorized as dilated, hypertrophic, restrictive and left ventricular non-compaction. Each of these subtypes has multiple potential genetic etiologies in addition to possible non-genetic causes. Many patients with cardiomyopathies can benefit from transplantation, although there is not insignificant morbidity and mortality for those patients. Outcomes both prior to and following transplantation depend on the underlying etiology, the amount of support needed prior to transplantation and the illness severity of the patient prior to transplantation. Mechanical circulatory support is frequently used to bridge patients to transplantation, and newer technologies are currently in development.

Keywords::

• dilated cardiomyopathy
• heart failure
• heart transplantation
• hypertrophic cardiomyopathy
• mechanical circulatory support
• pediatrics

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• • A thorough evaluation prior to transplantation to ensure that the patient does not have a severe, irreversible etiology as the underlying cause of their cardiomyopathy is necessary in pediatric patients.

• • In comparison to extracorporeal membrane oxygenation, ventricular assist device (VAD) therapy allows an increasing number of patients to undergo transplantation, although complications are not uncommon in patients with VADs.

• • Common complications in VADs include serious bleeding, infection and stroke.

• • Children diagnosed with hypertrophic cardiomyopathy under 1 year of age have relatively poor outcomes, regardless of underlying etiology.

• • Although restrictive cardiomyopathy progresses more rapidly than other cardiomyopathies, patients fare well long term following transplantation in this group.

• • Though not specifically defined, a level of illness exists for which transplantation is clearly not beneficial. Need for ventilatory support is consistently a risk factor for poor outcomes both prior to and following transplantation.

• • Clinical trials involving new malformation syndrome technologies will occur over the next several years in an effort to provide more options in this challenging group of patients.

Notes