Cross-Sectional Study of the Association Between Plasma Brain Natriuretic Peptide Levels and Left Ventricular Shortening Fraction in Fukuyama Congenital Muscular Dystrophy

This study aimed to investigate the correlation between plasma brain natriuretic peptide levels and left ventricular shortening fraction in Fukuyama congenital muscular dystrophy. The correlation between brain natriuretic peptide levels and left ventricular shortening fraction in patients with genetically confirmed Fukuyama congenital muscular dystrophy was retrospectively investigated. The patients at Tokyo Women’s Medical University were followed-up from October 2015 to October 2020. The brain natriuretic peptide level was measured using a specific immunoradiometric assay kit. Left ventricular shortening fraction was defined as a degree of left ventricular systolic function within 3 months before and after brain natriuretic peptide measurements. In addition to left ventricular shortening fraction, the ratio of early filling to early diastolic and the maximum velocity of tricuspid regurgitation, which are measures of diastolic function, were investigated. In total, 27 sets of brain natriuretic peptide levels and echocardiography results were obtained from 16 patients with Fukuyama congenital muscular dystrophy. Excellent correlations were found between the brain natriuretic peptide levels and left ventricular shortening fraction (R2 = 0.8755). Patients with a left ventricular shortening fraction of > 20% had low brain natriuretic peptide levels. However, the brain natriuretic peptide values of patients with a left ventricular shortening fraction of < 20% were > 40 pg/mL. In Fukuyama congenital muscular dystrophy, the brain natriuretic peptide level was exponentially correlated with left ventricular shortening fraction. However, it did not elevate until the advanced stage of left ventricular systolic dysfunction.