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Prevalence of Barth syndrome in adult left ventricular hypertrabeculation / noncompaction

Objectives. Leukopenia, left ventricular hypertrabeculation/noncompaction (LVHT), cardiomyopathy, and myopathy are the clinical characteristics of Barth syndrome (BS). Aim of the present investigation was to determine the prevalence of these characteristics in a large cohort of adult LVHT patients. Material and methods. A retrospective review of the records of all patients in whom LVHT was diagnosed between 1995 and 2006 was carried out for the leukocyte and neutrophil granulocyte count, and the presence of dilated cardiomyopathy (dCMP) and myopathy. Results. Among 86 (50) LVHT patients in whom leukocyte (neutrophil granulocyte) counts were available, the leukocyte count was reduced (n, 4.0–9.0/nl) in three patients and the neutrophil granulocyte count reduced <50% in two patients. dCMP was diagnosed in 58 of the included patients. None of the three patients with a reduced leukocyte count and none of the two patients with reduced neutrophil granulocyte count had dCMP. One of the patients with reduced leukocyte count had Leber's hereditary optic neuropathy and the two others suspected metabolic myopathy. None of the 86 patients presented with all four classical features of BS. Conclusions. The presented data indicate that the prevalence of BS is either low among adult patients with LVHT or that BS cannot be diagnosed upon clinical findings alone.