Serum Level of Brain Natriuretic Peptide (BNP) in Pediatric Patients with Muscular Dystrophy

Abstract

Background: Duchenne muscular dystrophy (DMD) patients used to die mainly from pulmonary problems. Echocardiography remains the standard diagnostic modality for cardiomyopathy in DMD patients, but is hampered by scoliosis in adult DMD patients. We investigated the usefulness of the plasma concentration of brain natriuretic peptide (BNP) for evaluating cardiac affection in patients with DMD. Methods: The plasma BNP concentration was measured by immunoassay in 15patients with DMD and in 15 healthy children. Cardiac function was evaluated by echocardiography which classified left ventricular function as preserved or depressed, and BNP. The function of skeletal muscle was evaluated by the disability of lower limb function, CPK. Results: The plasma concentration of BNP was increased in patients with DMD (95.700±58.901pg/ml, mean +/- SD) compared with that in normal children (16.007±7.135pg/ml). Four of the DMD patients had symptoms of heart failure, with markedly increased plasma BNP concentrations. The other DMD patients with increased plasma BNP concentrations showed abnormal cardiac function but no symptoms of heart failure. In addition, in patients with DMD, the plasma BNP concentration showed significant positive correlations with duration of cardiac affection, and negative correlations with CPK and LVEF. There was no significant correlation with duration of muscular dystrophy disease. Conclusion: The combination of echocardiography and BNP achieves good results in the evaluation of left ventricular function. We advise to add BNP to echocardiography in the routine cardiac assessment of DMD patients.