IMAGES IN PEDIATRIC CARDIOLOGY Double-Outlet Left Ventricle With L-Malposition of the Great Arteries and Subpulmonary Ventricular Septal Defect Vladislav Vukomanovic • Sergej Prijic • Bojko Bjelakovic Received: 31 May 2012 / Accepted: 29 August 2012 / Published online: 19 September 2012 Ó Springer Science+Business Media, LLC 2012 Abstract Double-outlet left ventricle is a very rare con- genital cardiac anomaly with various anatomic types. This report describes an infant with the aorta anterior and to the left of the pulmonary trunk, a subpulmonary ventricular septal defect, and pulmonary artery stenosis. This variation has not been described to date in patients with a double- outlet left ventricle. Keywords Anatomy Á Double-outlet left ventricle Á L-TGA Á Subpulmonary VSD Case Report An infant boy was referred to our institution on his first day of life for suspected transposition of the great arteries. The course of the pregnancy had been complicated in the first month by surgical removal of a uterine polyp. The boy was born vaginally with an Apgar score of 9 at 1 min and a the birth weight of 3,000 g. Respiratory dis- tress was noted immediately after delivery and associated with moderate cyanosis (oxygen saturation, 85 %), tach- ypnea (respiratory rate, 55 per min), and a 4/6 systolic murmur. The boy had a blood pressure of 113/45 mmHg, with a heart rate of 110 beats/min. Echocardiographic examination showed a double-outlet left ventricle (DOLV) with the aorta anterior and to the left of the pulmonary trunk, a subpulmonary ventricular septal defect (VSD), and pulmonary artery stenosis (Fig. 1a). Both chambers were well developed, with preserved con- tractility. The huge inlet-outlet VSD was nonrestrictive as well as a secundum artial septal defect. Fibrous continuity between the mitral valve and the overriding pulmonary valve was present (Fig. 2a). The aorta emerged exclusively from the left ventricle (Fig. 2b). The pulmonary artery was stenotic (pressure gradient, 45 mmHg) and received blood mostly from the left ventricle. The aorta was more than twice the size of the pulmonary artery (14 vs 5.5 mm). In addition, patent ductus arteriosus with bidirectional shunting was present. According to symbolic terminology, this patient had an SDL segmental set (Fig. 3) involving situs solitus (S), ventricular D-loop (D), and L-malposition of the great arteries (L) [2]. Right heart catheterization confirmed the diagnosis (Fig. 1b). Discussion The prevalence of DOLV is estimated to be less than 1 per 200,000 births [4]. To the best of our knowledge, DOLV with L-malposition of the great arteries and subpulmonary VSD, as in the reported patient, has not been described in V. Vukomanovic (&) Á S. Prijic Cardiology Department, Mother and Child Health Institute of Serbia, R. Dakica Street 6–8, 11070 Belgrade, Serbia e-mail: vvladavuk@ptt.rs S. Prijic e-mail: sergej@scnet.rs V. Vukomanovic School of Medicine, University of Belgrade, Belgrade, Serbia B. Bjelakovic Pediatric Cardiology Department, Clinical Center Nis, Nis, Serbia e-mail: bojko968@gmail.com B. Bjelakovic School of Medicine, University of Nis, Nis, Serbia 123 Pediatr Cardiol (2013) 34:476–477 DOI 10.1007/s00246-012-0514-2