A Case of Double-Inlet Left Ventricle Reaching Adulthood Without Surgery

Double-inlet left ventricle (DILV) are rarely encountered congenital heart defects which are also known as single-ventricle defects and have a complex structure in which the blood exiting both atriums flows into a single ventricle, and they constitute approximately 1.5% of all congenital heart diseases. The 37-year-old female patient who did not have a history of cardiac disease visited our outpatient clinic for routine cardiological assessments. Transthoracic echocardiography (TTE) was performed upon hearing a 3/6 pansystolic murmur at the mesocardiac focus and a 3/6 systolic ejection murmur at the pulmonary focus on cardiac auscultation. The TTE of the patient revealed that the atrioventricular valves opened into one ventricular chamber in four-chamber apical imaging. There was no noticeable interventricular septum, while a rudimentary right ventricle and a ventricular septal defect (VSD) were observed. There was a gradient of 38 mmHg in the pulmonary valve. Mild-to-moderate pulmonary stenosis was present. In the transesophageal echocardiography (TEE), a rudimentary interventricular septum and the right ventricle were observed. Because the patient did not have any symptoms or cyanosis, the patient was given information about a potential need for occasional infective endocarditis prophylaxis and phlebotomy, and close medical follow-up was decided. A double-inlet left ventricle is known as a severe congenital heart anomaly that is typically symptomatically diagnosed in childhood and requires surgical intervention. However, in the literature, cases with an asymptomatic course reaching adulthood without surgical intervention, albeit very rare, have been reported.