Heart abnormalities in children with neuromuscular diseases

Abstract

Neuromuscular diseases are a large group of diseases that are characterized by defective functions of peripheral nervous system, neuromuscular junction and/or muscle. Due to the similar structure of skeletal and cardiac muscles it is possible to associate neuromuscular diseases with cardiac disorders. The aim of the work was to identify the incidence rate of cardiovascular abnormalities in children with neuromuscular diseases. During the period from January to December 2008 at the Clinic of Neurology and Psychiatry for children and Youth the Faculty of Medicine in Belgrade, 44 patients with neuromuscular diseases had cardiovascular examination (physical, electrocardiographic and echocardiographic). All of the examined patients fell ill before turning 18 years of age. The patients’ ages (M: 25, F: 19) ranged from 3 to 38 years (X = 16 ± 8.35) at the time of cardiovascular examination. High incidence rate of mitral valve dysplasia, without haemodynamic changes, has been diagnosed (38.6%). Patients with distrophinopathy are often referred to cardiovascular examination. Five (26.3%) of the patients with distrophinopathy have dilated cardiomyopathy, and two patients with distrophinopathy have congenital heart disease and diseases of the valve. One of the examined patients had congenital heart disease as well as nondistrophinopathic dystrophy (LGM.D.), and spinal muscular atrophy (SMA) while patients with peripheral neuropathy hadn’t been diagnosed with pathological cardiovascular findings. Two out of five examined patients with disease of neuromuscular junction had results of the cardiovascular examination that matches the ones found in heart valve diseases. Dilatated cardiomyopathy, isolated or associated with other cardiology abnormalities can only be diagnosed in distrophinopathy. In other forms of dystrophy, as well as other neuromuscle diseases occurrence of described diseases of valve and congenital heart diseases has been diagnosed.