Bridge to heart transplantation with nearly 800-day intracorporeal biventricular assistance in a pediatric patient with Danon disease: a case report.

Danon disease is a rare inherited disorder, and its prognosis is significantly impacted by cardiac complications. Because of the severe shortage of donors, a ventricular assist device (VAD) is necessary as a bridge to heart transplantation. However, reports on VAD implantation for cardiomyopathy in Danon disease patients are limited. Herein, we report a successful pediatric case with nearly 800-day of biventricular assistance as a bridge to heart transplantation. A 12-year-old girl with severe heart failure due to hypertrophic cardiomyopathy associated with Danon disease underwent left implantation of a Heartware VAD (Heartware Inc., Miami Lakes, FL, USA). Owing to severe right ventricular failure, she was unable to be weaned off the cardiopulmonary bypass, and a right VAD implantation was undertaken using a Gyro pump (Kyosera, Kyoto, Japan). However, the right ventricular function did not recover postoperatively, and she subsequently underwent right implantation of a Heartware VAD on postoperative day 34. Despite several VAD-related complications, including aortic and pulmonary valve insufficiency and a driveline infection requiring hospitalization, the patient was able to attend junior high school during the waiting period for a heart transplantation. After a total waiting period of 882 days, including 791 days of biventricular Heartware VAD assistance, the patient underwent a heart transplantation.