A case of a single double inlet cardiac ventricle with transposition of great vessels

Presented was an observation of a 19-year old patient with a congenital heart defect (a single double inlet right cardiac ventricle in combination with a corrected transposition of the great vessels), which was complicated by acute heart rhythm disturbance (atrial flutter), and heart failure. With the help of echocardiography (2D-, M-mode, color Doppler scan) diagnosed was the single ventricle with transposition of the great vessels (a rare variant of B III). The peculiarity of this clinical case is the preservation of the sinus rhythm during 19 years, despite the marked remodeling of the myocardium. The sharp deterioration in clinical status of the patient was due to a disturbance of the cardiac rhythm: atrial flutter with a 2:1 conduction on to the ventricle. Clinical improvement was achieved by pharmacological blockade of atrioventricular conduction in combination with an increase of the inotropic myocardial function.