Double-inlet left ventricle with transposition of great arteries in an asymptomatic adult

Double-inlet left ventricle is a congenital heart disease characterized with a single ventricle receiving the whole blood from both atria via AV valves. Prognosis is usually poor unless early surgical correction is done. Rarely, cases of patients reaching the sixth decade have been reported.
34-year-old women admitted with palpitation. The physical examination revealed IV/VI systolic murmur with thrill over the entire precordium. Increased cardiothoracic ratio was seen in the telecardiogram. ECG revealed sinusal rhythm. Transthoracic echocardiography showed that both atria were connected at the same level to a chamber forming the morphologic left ventricle (MLV), which was seperated from the hypoplastic right ventricle via a rudimentary septum with large VSD. This MLV was connected to the transposed anteriorly located aorta and to the posteriorly located pulmonary artery. The atrioventricular valves were connected to the MLV (Figure 1,2). There was a subvalvular hypertrophic band leading to a 68-mmHg maximum systolic gradient in pulmonary outflow tract. The diagnosis was double inlet left ventricle with rudimentary right ventricle, transposition of the great arteries and subvalvular pulmonary stenosis. Subvalvular pulmonary stenosis probably prevented pulmonary hypertension providing a favorable survival. Holter monitorization revealed monomorphic ventricular extrasystoles with 2 couplets and 26 triplets. Beta-blocker medication was the final decision for her treatment.
This case deserves submission as being a patient reaching adulthood free of symptoms with nonoperated double-inlet left ventricle.