Table 3.

Select Features of Barth Syndrome

Feature% of Persons w/Feature 1Comment
Skeletal myopathy97%6MWT is abnormal in almost all individuals.
Cardiomyopathy 273%Dilated cardiomyopathy is most common. 2
Neutropenia70%-85%
Prepubertal growth delay58% 3
Prolonged QTc25%-43%
Arrhythmia10%-20% 4

6MWT = distance walked on six-minute walk test

1.

The vast majority of affected individuals are male.

2.

At presentation

3.
4.

Based on the study by Kang et al [2016] and Spencer et al [2006]

From: Barth Syndrome

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