Article

Heart transplantation for progressive cardiomyopathy as a manifestation of MELAS syndrome

Department of Surgery, University of North Carolina at Chapel Hill, North Carolina, United States
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation (Impact Factor: 5.61). 01/2006; 24(12):2286-9. DOI: 10.1016/j.healun.2005.05.012
Source: PubMed

ABSTRACT Mitochondrial diseases represent a heterogeneous group of disorders associated with a wide array of clinical manifestations. The presentation of patients with mitochondrial pathology largely depends upon the dysfunction of organ systems with large metabolic/energy requirements, including cardiac, neurologic, and musculoskeletal. In particular, mitochondrial myocardial disease can be progressive resulting in congestive heart failure and end-stage heart disease. This article reviews the role of heart transplantation for a particular variant of mitochondrial disorder, mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome, and discusses perioperative management issues related to transplantation for mitochondrial cardiomyopathies.

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    • "rate has been reported in six patients with mitochondrial cardiomyopathy (Bonnet et al. 2001). More recently, a successful recipient outcome for two patients with MELAS was reported (Bhati et al. 2005). Liver function is often impaired, especially in mtDNA depletion syndromes. "
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