A rare case of two-chambered heart in an adult patient

Abstract

Congenital heart diseases are third most prevalent congenital disorders right after congenital musculoskeletal and central nervous system disorders. Up to 30-35 thousand of children with congenital heart disease are born annually in USA, 20-22 thousand - in Russia, 320-350 children - in the Republic of Tatarstan. Univentricular heart is among rare and unique congenital heart disorders. The natural course of the disease is poor, with two thirds of patients passing away within the first year due to severe pulmonary hypertension, progressing heart failure, and severe hypoxia and heart arrhythmias. Prenatal influence of the disorder on hemodynamics is not significant, and babies are born with normal body weight and length. In most of the children, the disease is diagnosed within the first months after birth. If combined with pulmonary artery stenosis and transposition of the great vessels, clinical manifestations are associated with serous hypoxia and cyanosis. Intensive cyanosis is observed since birth, peripheral signs of hypoxia - phalangeal and nail deformities are seen early. Such children do not frequently have respiratory diseases, despite shortness of breath; there is no crackles in lungs. In the absence of pulmonary artery stenosis, the clinical picture reminds congenital heart disorder with big ventricular septal defect and left to right shunt with the expressed hypervolemia and moderate cyanosis, which becomes more severe while crying and at feeding. Children often have recurrent pneumonia and bronchitis, lag behind in physical development, they constantly have a shortness of breath and tachycardia, hepatomegaly occurs early. A clinical case of the patient with congenital heart disease (univentricular heart) and severe congestive heart failure is presented. The peculiarity of this case is the presence of the single atrium communicating through a common atrioventricular valve with a single ventricle, from which two main vessels begins with the aortic and pulmonary valves, as well as atypical long clinical course associated with heart arrhythmia and extensive cardiac remodeling.