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J Heart Lung Transplant. 2005 Dec;24(12):2286-9. Epub 2005 Sep 15.

Heart transplantation for progressive cardiomyopathy as a manifestation of MELAS syndrome.

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Division of Cardiothoracic Surgery, Department of Surgery, University of North Carolina, Chapel Hill, North Carolina 27599, USA.


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.

[Indexed for MEDLINE]

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