Case Report An Unusual Type of Totally Anomalous Pulmonary Venous Connection to the Superior Cavoatrial Junction Ahmad Mahir Shamsuddin, MD 1 , Abdul Rahim Wong, BMBS, MRCP, FNHAM 2 , Robert H. Anderson, BSc, MD, FRCPath 3 , and Antonio F. Corno, FRCS, FECTS, FACC 4 Abstract A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pul- monary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months. Keywords cardiopulmonary bypass, congenital heart defects, congenital heart surgery, totally anomalous pulmonary venous connection Submitted March 17, 2015; Accepted August 03, 2015. Introduction Totally anomalous pulmonary venous connection is said to account for 1.4% of all congenital cardiac defects. 1 It is usually classified as being supracardiac, intracardiac, infracardiac, or mixed, depending on the site of venous return. When the return is to the right atrium, then most usually all pulmonary veins drain into the coronary sinus. 2 Very few cases have been described with direct connection to the right atrium other than in the setting of right isomerism. 2,3 We report our recent expe- rience with an infant having an unusual connection of all pul- monary veins to the right superior cavoatrial junction. Case Report A boy born at term weighing 2.5 kg was admitted from home on day four of life four with tachypnea and reduced oxygen saturations. The peripheral oxygen saturations were 70% when breathing room air and increased only to 80% when oxygen was delivered via an oxygen hood (headbox). Doppler echocar- diography revealed totally anomalous pulmonary venous con- nection to the right atrium at the superior cavoatrial junction, with a restrictive interatrial communication and suprasystemic right ventricular pressures. Doppler echocardiography showed a velocity of 1.5 m/s across the foramen ovale. The pulse wave of the pulmonary veins showed a velocity of 50 cm/s. There was mild tricuspid valve regurgitation, with a velocity of 5 m/s. At four days of age, since neonatal cardiac surgery was not yet available in our hospital, and referral to another hospital was not practical, we made the decision to perform a balloon atrioseptostomy to improve the oxygenation, setting the scene for later surgical repair. The neonate was discharged home 4 days after the balloon septostomy, with markedly improved oxygenation, with saturations on room air having increased to 92%. Echocardiography at that time showed an unrestrictive interatrial communication, with systolic right ventricular pres- sure estimated to be half systemic blood pressure based on 1 Pediatric and Congenital Cardiac Surgery Unit, Department of Surgery, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia 2 Pediatric Cardiology Unit, Department of Pediatrics, Hospital Raja Perempuan Zainab II, Kota Bharu, Kelantan, Malaysia 3 Institute of Genetic Medicine, Newcastle University, International Centre for Life, Newcastle upon Tyne, United Kingdom 4 Pediatric Cardiac Surgery Unit, East Midlands Congenital Heart Center, Glenfield Hospital, Leicester, United Kingdom Corresponding Author: Ahmad Mahir Shamsuddin, Pediatric and Congenital Cardiac Surgery Unit, Department of Surgery, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia. Email: amahir@gmail.com World Journal for Pediatric and Congenital Heart Surgery 1-4 ª The Author(s) 2016 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135115603330 pch.sagepub.com by guest on January 23, 2016 pch.sagepub.com Downloaded from