title: Heart transplantation for progressive cardiomyopathy as a manifestation of MELAS syndrome.
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.
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of North Carolina, Chapel Hill, North Carolina 27599, USA.
authors.forename: Rajendra S
authors.initials: RS
authors.lastname: Bhati
authors.forename: Brett C
authors.initials: BC
authors.lastname: Sheridan
authors.forename: Michael R
authors.initials: MR
authors.lastname: Mill
authors.forename: Craig H
authors.initials: CH
authors.lastname: Selzman
mesh_terms: D000293:Adolescent; D009202:Cardiomyopathies / Q000209:etiology* / Q000601:surgery*; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications*; D008297:Male; D018579:Patient Selection; D011788:Quality of Life; D016896:Treatment Outcome