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Total Anomalous Pulmonary Venous Connection: Results of Surgical Repair of 100 Patients at a Single Institution
Angela M Kelle1, Carl L Backer1, Jeffrey G Gossett1, Sunjay Kaushal1, Constantine Mavroudis2 1Children's Memorial Hospital, Chicago, IL;2Cleveland Clinic, Cleveland, OH
BACKGROUND: Surgical repair of total anomalous pulmonary venous connection (TAPVC) continues to be associated with significant mortality and morbidity rates, especially in single ventricle patients. This study will analyze the results of surgical repair of TAPVC at one institution to look for trends and indicators of positive outcome. METHODS: Our cardiac surgery database was used to identify all patients who underwent surgical repair of TAPVC from 1990-2008 (n=100). Specific TAPVC types were supracardiac (52), cardiac (15), infracardiac (23) and mixed (10). Median age at repair was 14.6 days (range, 0-4 years) and median weight was 4.0 kg (range, 1.3-15 kg). Patients were divided into two groups based on biventricular (BV, n=83) or univentricular (SV, n=17) anatomy for analysis. RESULTS: There were 12 operative deaths (4 BV [5%] vs. 8 SV [47%], p<0.01) and 9 late deaths (6 BV [7%] vs. 3 SV [18%], p<0.05). Death by TAPVC type was supracardiac 12/52 (23.1%), cardiac 1/15 (6.7%), infracardiac 3/23 (13.0%), and mixed 5/10 (50%). Pulmonary venous obstruction was present in 32 patients which included 24 BV (29%) vs 8 SV (47%), p = 0.16. The pulmonary veins were obstructed in 8/21 patients (38%) that died versus 13/21 (16%) in unobstructed patients (p = 0.06). Mean cardiopulmonary bypass time was 95.1±40.5 minutes (86.2±31.0 BV vs. 139.4±53.3 SV, p<0.01) and mean aortic cross-clamp time was 42.9±23.1 minutes (43.9±22.4 BV vs. 37.3±26.7 SV, p=NS). Deep hypothermic circulatory arrest (DHCA) was used in 38 patients (27 BV, 32.5%; 11 SV, 64.7%). Mean DHCA time was 31.4±10.7 minutes with no difference between groups. Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen surviving patients required reoperation for pulmonary venous stenosis (14 BV vs. 5 SV); median time to reoperation was 104 days (range, 4-753 days). CONCLUSIONS: TAPVC continues to be a surgical challenge. Outcomes are good for patients with BV anatomy. Patients with SV anatomy have very high mortality and are at increased risk for pulmonary vein stenosis leading to reoperation. Mortality is highest in mixed-type TAPVC. Pulmonary venous obstruction was not associated with increased mortality.
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