Objective. There are many reasons why a hypertrophy of cardiac muscle might occur during the neonatal period. The most common one is hypertrophy due to mother’s primary or gestational diabetes. On other occasions, cardiac hypertrophy develops due to metabolic, neuromuscular, infiltrative disorders or malformation syndromes. Neonatal hypertrophic cardiomyopathy is a primary heart muscle disease inherited in an autosomal dominant manner, characterized by thickening of the heart walls, usually in the interventricular septum. This disease is rarely diagnosed in newborns. It might be a life threatening disease causing sudden death of a newborn or an infant.
Aim. To analyze the clinical signs, the evaluation possibility and outcomes of hypertrophic cardiomyopathy diagnosed in neonatal period. The article reviews literature about hypertrophic cardiomyopathy epidemiology, symptoms, diagnosis, complications, treatment, diagnosis and situation in a single center of the neonatology.
Methods. We analyzed the medical data of newborns born from 2005 to 2017 who were diagnosed and treated with hypertrophic cardiomyopathy at Neonatology Center, Vilnius University Children’s Hospital. This article reviews literature and one Neonatology center experience about features of hypertrophic cardiomyopathy during neonatal period.
Results. In total there were 27 neonates diagnosed with hypertrophy of the cardiac muscle. Usually concentric hypertrophy occurs due to mother‘s primary or gestational diabetes and less often it is caused because of other neonatal period
conditions such as perinatal hypoxia or congenital infection. 5 newborns were confirmed to have rare and isolated right ventricular hypertrophy which is associated with antenatal closure of the ductus arteriosus. 2 newborns were diagnosed with Pompe disease. From 27 neonates only 6 were diagnosed with primary hypertrophic cardiomyopathy. The asymmetrical type of hypertrophy was identified in 5 cases out of 6.
Conclusions. Hypertrophic cardiomyopathy is rarely diagnosed in newborns. Usually it appears in a transient way. The majority of the cases showed the interventricular septum hypertrophy. Diagnosis in the early childhood of the disease can lead to more serious course and prognosis. However it is important for patients’ observation and treatment. Methods which improved understanding about disease and patient care include genetic analysis, 24 hour ECG monitoring, and magnetic resonance tomography.
