Enliven: Surgery and Transplantation

Barth Syndrome and Left-Ventricular Non-Compaction: Case Report and Surveillance Plan Prior to Cardiac Transplantation
Author(s): Daniela Karall, Sabine Scholl-Burgi, Consolato Sergi, Ralf Geiger, Georg Engl, Thomas Karall, Joerg-Ingolf Stein, Frederic M. Vaz and Ulrich Schweigmann

Barth syndrome, an X-linked disorder of cardiolipin metabolism, shows cardioskeletal cardiomyopathy, neutropenia, psychomotor and growth retardation. A limiting factor is heart function. We present a ten year old boy who underwent heart transplantation after bridging with an assist device at 8 months, and propose a surveillance plan prior to transplantation.