Case Study Left isomerism syndrome with total anomalous systemic connection Anh Tuan Vo, Khang Dang Cao, Khoi Minh Le and Dinh Hoang Nguyen Abstract We present a case of left isomerism with total anomalous systemic venous connection where the inferior vena cava was absent and all other systemic veins connected abnormally to the left atrium. The right atrium was hypoplastic with an intact atrial septum. Blood flow to the lungs was through a large ventricular septal defect. The diagnosis was made with echocardiography, angiography, and computed tomography. Complete repair was performed successfully, and the 7-year- old patient had an uneventful recovery. Keywords Heart atria, Heart defects, congenital, Heart septal defects, atrial, Vascular malformations, Vena cava, inferior, Vena cava, superior Introduction Anomalous systemic venous connections are congenital malformations of the course or destination of systemic venous return. They can be either partial, affecting part of the systemic venous return, or total, affecting all sys- temic return. The most common type is persistent left superior vena cava. Total anomalous systemic venous connection is an unusual form of this defect; all sys- temic venous flow, including the right superior vena cava, left superior vena cava, coronary sinus, and infer- ior vena cava if persistent, drains directly into the left atrium. In the case of interruption of the inferior vena cava, there should be an azygos or hemiazygos continu- ation of the inferior vena cava to the superior vena cava on the corresponding side. Surgical correction is often technically challenging. We report a case of left isom- erism with total anomalous systemic venous return and interrupted inferior vena cava. Case report A 7-year-old girl was admitted to our department with dyspnea and cyanosis on exertion. Her oxygen satur- ation on room air was 89%. Vital signs were normal, and her body mass index was in the 12th percentile for girls of her age. Physical examination revealed a 3/6 harsh holosystolic murmur at the 3rd–4th intercostal space, radiating along the left sternal border. Transthoracic echocardiography showed situs solitus, levocardia, abnormal drainage of the right superior vena cava, persistent left superior vena cava, and the coronary sinus and hepatic veins draining into the left atrium (Figure 1). The inferior vena cava was absent and lower body venous blood returned to the heart through the hemiazygos continuation to the left super- ior vena cava. Both atrial appendages had left atrial morphology. The atrial septum was intact and the blood flowed to the lungs through a large perimembra- nous ventricular septal defect (20.4 mm). Because the blood for pulmonary circulation was from the left ven- tricle through the ventricular septal defect, there was no flow through the tricuspid valve, however, both ventricles were balanced and the tricuspid annulus diameter was 24 mm, with a z-score of 0.18. Ventriculoarterial connection was concordant, and the Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315618507 aan.sagepub.com Department of Cardiovascular Surgery, University Medical Center, University of Medicine and Pharmacy in Ho Chi Minh City, Ho Chi Minh City, Vietnam Corresponding author: Anh T Vo, Department of Cardiovascular Surgery, University Medical Center, University of Medicine and Pharmacy in Ho Chi Minh City, Ho Chi Minh City, Vietnam. Email: dranhtuanvo@gmail.com by guest on November 29, 2015 aan.sagepub.com Downloaded from