Myocardial glucose and fatty acid metabolism is altered and associated with lower cardiac function in young adults with Barth syndrome.

ABSTRACT:

Background

Barth syndrome (BTHS) is a rare X-linked condition resulting in cardiomyopathy, however; the effects of BTHS on myocardial substrate metabolism and its relationships with cardiac high-energy phosphate metabolism and left ventricular (LV) function are unknown. We sought to characterize myocardial glucose, fatty acid (FA), and leucine metabolism in BTHS and unaffected controls and examine their relationships with cardiac high-energy phosphate metabolism and LV function.

Methods/results

Young adults with BTHS (n = 14) and unaffected controls (n = 11, Control, total n = 25) underwent bolus injections of ¹⁵O-water and 1-¹¹C-glucose, palmitate, and leucine and concurrent positron emission tomography imaging. LV function and cardiac high-energy phos

SUBMITTER: Cade WT 

PROVIDER: S-EPMC7205570 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature