Anomalous Pulmonary Venous Return (APVR) Author : Andrei Feier Co-authors : Denisa Pop, Marta Ostopovici, Pavel Bujorean, Miriam Huma Scientific coordinators : prof. Horatiu Suciu, MD Valentin Stroe MD,PhD Student ; MARISIENSIS 2014 International Congress for Students Young Physicians and Pharmacists 1

a rare heart defect in which a child’s pulmonary veins don’t drain normally into the left atrium Introduction Video they are re-directed to the right atrium by an abnormal connection 1-2 % of all congenital cardiac malformations 2

Video source: ©Cincinnati Children's Hospital Medical Center 3

History Wilson: described a "monstrous formation of the heart“(1798) [1] Muller: first closed partial approach(1951) Lewis & Varco: successful open repair (1956) 4

Supracardiac (45%) Classification Infracardiac (25%) Cardiac (25%) Mixed (5%) 5

Classification Supracardiac TAPVR (45%): the pulmonary veins drain superiorly into the innominate vein or superior vena cava via an ascending vertical vein [2][3] 6

Classification Infracardiac TAPVR (25%): The pulmonary veins drain through the diaphragm into the portal vein or ductus venosus [2][3] 7

Classification Cardiac TAPVR (25%): the pulmonary veins drain into the coronary sinus or, on rare occasions, individual pulmonary veins will connect directly into the right atrium [2][3] Mixed TAPVR(5%) 8

Etiology idiopathic morphological malformation in early embriological development disconnection of the 4 pulmonary veins from the left atrium 9

Diagnosis Echocardiography pulmonary veins not connected to the left atrium dilated right atrium presence of atrial septal defect Clinical presentation cyanosis, tachypnea respiratory distress prominent S2/fixed split S2 Hepatomegaly Chest X-ray it can show the classic “snowman pattern” [6] 10

Management and treatment The surgical approach was made by median sternotomy Aorto-bicaval cannulation and full-flow cardiopulmonary bypass at moderate hypothermia was used The aorta was clamped, and cold potassium crystalloid cardioplegia is administered. 11

Management and treatment [7] A.vertical vein is ligated and a longitudinal incision is made in the pulmonary confluence B.ASD is closed and pulmonary veins are anastomosed to the left atrium C.Right atrioraphy SUPRACARDIAC 12

Management and treatment [7] A. - vertical vein ligated at the diaphragm level -incision of the posterior wall of the left atrium and pulmonary veins confluence B. pulmonary venous confluence and LA are anastomosed INFRACARDIAC 13

Management and treatment [7] A.pulmonary veins connected into the dilated coronary sinus B.the roof of the coronary sinus is incised into the left atrium and the atrial septal defect is enlarged to communicate with the unroofed coronary sinus C.Closure of the ASD with the channeling of the pulmonary veins and coronary sinus into the left atrium CARDIAC 14

Objective to evaluate the surgical management of total and partial anomalous pulmonary venous return at Institutul de Urgenţă pentru Boli Cardio-Vasculare şi Transplant Tîrgu-Mure 15

Material and method retrospective study 24 patients,16 male and 8 female underwent surgical repair between January 2011 and March

Material and method  Mean age : 1.3 years (2 days - 18 years)  Mean weight : 7.6 kg ( kg) 17

Material and method Type APVR Patients NumberPercentage Supracardiac 937.5% Infracardiac 28.3% Cardiac 520.8% PAPVR 833.3% The atrial septal defect was present in all TAPVR cases and in 7 cases (87,5%) of PAPVR 18

Results In TAPVR cases: ligation of the vertical(innominate) vein connection of the common pulmonary veins collector to the posterior aspect of the left atrium In PAPVR cases: the anomalous pulmonary vein was redirected and connected to the left atrium The ASD was corrected using a pericardial patch 19

Results CPB : min. AoX : min. Supracardiac CPB : min. AoX : min. Cardiac CPB : min. AoX : min. Infracardiac CPB : min. AoX : min. Partial Cardiopulmonary bypass times (CPB) & Aortic cross clamp times (AoX) 20

Conclusion there has been a significantly increase in the surgical treatment of APVR showing great results with low mortality rate early diagnosis leads to good surgical and therapeutic outcome further clinical studies are needed to evaluate the postoperative status 21

Bibliography [1] Cirstoveanu C. et al. Prostaglandin E1 on Infradiaphragmatic Type of Total Anomalous Pulmonary Venous Connection, 2012; [2] Bove EL, Hirsch JC. Total anomalous pulmonary venous drainage and cor triatriatum. In: Gardner TJ, Spray TL, editors. Operative Cardiac Surgery, London: Arnold Publishers; 2004:581–592; [3] Hirsch JC, Bove EL Prostaglandin E1 on Infradiaphragmatic Type of Total Anomalous Pulmonary Venous Connection, MMCTS 2007; [4] Hedwig HG,Seghaye MC. Pulmonary venous return anomaly; 2003; [5] Total Anomalous Pulmonary Venous Connection; Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery; [6] Shen Q., et. al. (2012). Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection. Pediatric Radiology, Epub ahead of print. [7] Hirsch JC, Bove EL, Total anomalous pulmonary venous connection;

Thank you! 23