A follow up study of myocardial involvement in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)

Title

A follow up study of myocardial involvement in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)

Creator

Okajima Y; Tanabe Y; Takayanagi M; Aotsuka H

Publisher

Heart (british Cardiac Society)

Date

1998

Subject

Child; Female; Humans; Male; Adult; Follow-Up Studies; adolescent; Q3 Literature Search; Hypertrophy; Echocardiography; Electrocardiography; Left Ventricular/physiopathology/ultrasonography; MELAS Syndrome/physiopathology/ultrasonography; Stroke Volume

Description

OBJECTIVE: To investigate cardiac function in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and clarify the clinical features of cardiomyopathy in MELAS. PATIENTS: 11 consecutive patients with MELAS (mean age at initial examination 11.3 years, range 4 to 16 years) were enrolled in the study. Six were followed for more than five years. RESULTS: On echocardiographic examination, three patients showed increased left ventricular end diastolic posterior wall thickness (LVPWTd), exceeding 140% of the normal value. Four patients, including these three, had an ejection fraction of less than 50%, and two also had increased left ventricular end diastolic volume (LVEDV) exceeding 140% of the normal value (%N). The LVPWTd%N was correlated positively with the LVEDV%N (R = 0.669, p < 0.05) and negatively with the ejection fraction (R = -0.6701, p < 0.05). One patient died of heart failure aged 22 years. CONCLUSIONS: The cardiomyopathy in MELAS is characterised by an abnormally thick left ventricular wall with progressive dilatation and poor left ventricular contraction developing over several years, indicating hypertrophic cardiomyopathy advancing to dilated cardiomyopathy.
1998

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Citation

Okajima Y; Tanabe Y; Takayanagi M; Aotsuka H, “A follow up study of myocardial involvement in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS),” Pediatric Palliative Care Library, accessed March 28, 2024, https://pedpalascnetlibrary.omeka.net/items/show/11890.