IMR Press / RCM / Volume 19 / Issue 2 / DOI: 10.31083/j.rcm.2018.02.903
Open Access Review
Repeat Cardiac Transplant Indicated by Severe Cardiac Allograft Vasculopathy in a Patient With Danon Disease
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1 Division of Cardiology, Baylor University Medical Center, Dallas, TX, 75246
2 Texas A&M University College of Medicine, Dallas, TX, 75246
3 Center for Advanced Heart and Lung Disease, Baylor University Medical Center, Dallas, TX, 75246
4 Annette C. and Harold C. Simmons Transplant Institute, Baylor Scott and White Research Institute, Dallas, TX, 75246
5 Soltero Cardiovascular Research Center, Baylor Scott and White Research Institute, Dallas, TX, 75246

*Correspondence: Aasim.Afzal@BSWHealth.org (Aasim Afzal)

Rev. Cardiovasc. Med. 2018, 19(2), 69–71; https://doi.org/10.31083/j.rcm.2018.02.903
Published: 30 June 2018
Abstract
Danon disease is a rare, X-linked dominant, lysosomal storage disorder, presenting with cardiomyopathy mostly in adolescent men. Male patients face a high mortality rate and rarely live to the age of 25 years unless they receive a heart transplant. Because they generally undergo heart transplantation at a young age, many patients ultimately face both short- and long-term complications. We present a 32-year-old man diagnosed with Danon disease; a nonsense mutation in the LAMP-2 gene. Progressive heart failure symptoms resulted in initial heart transplant at age 27 years. He subsequently developed severe cardiac allograft vasculopathy that led to graft failure requiring a redo orthotopic heart transplant. This is one of only two reported Danon disease cases described to date surviving repeat orthotopic heart transplants. We present this case to highlight the importance of heart transplantation in the management of Danon disease, to emphasize the risk of cardiac allograft vasculopathy post-transplant, and to discuss management strategies.
Keywords
Danon disease
Cardiac allograft vasculopathy
Cardiac transplantation
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