Babliak O. Surgical treatment of conotruncal diseases with major aortopulmonary collateral arteries

The thesis presents the analysis of consecutive 83 patients with conotruncal heart defects and major aortopulmonary collateral arteries (MAPCAs), in whom 143 surgical operations, 247 angioprocedures and 65 angiooperations in GI "The scientific-practical medical center of pediatric cardiology and cardiac surgery of Ministry of Health of Ukraine" from 2007y. to 2014y. were performed. Of the 83 patients who underwent surgical intervention for conotruncal heart defects with MAPCAs, 44 (53 %) were male. The median age at the time of the first operation was 9.7 months (range, 1 month - 336 months), and 54 (65 %) were less than 1 year of age at the time of the first operation. The most common cardiac malformation was pulmonary atresia and ventricular septal defect (VSD) (n = 69 [83 %]). The remaining 14 patients had tetralogy of Fallot (6 patients) and double outlet right ventricle (tetralogy of Fallot type) (8 patients). For quantification of pulmonary arborization anomalies we divided patients in three groups: 29 (35 %) patients had pulmonary arborization anomalies in less than 10 pulmonary segments, 23 (28 %) patients had pulmonary arborization anomalies in 10 to 14 pulmonary segments, and 31 (37 %) - had pulmonary arborization anomalies in 15-20 pulmonary segments. For assessment of impact of hypertensive MAPCAs we divided patients in two groups: 26 (31 %) patients had pulmonary in 10 or more pulmonary segments and 47 (69 %) - had pulmonary hypertension in less than 10 pulmonary segments. Surgical methods. We decided to use several surgical strategies in treatment of these 83 patients. In 26 patients (I group) we used one-stage UF and simultaneous total repair. In 28 patients (III group) we used multistage UF and delayed total repair. Multistage UF included rehabilitation of native pulmonary arteries and UF of MAPCAs. In 29 patients (II group) we developed alternative approach (since 2008), according to which we perform one-stage UF and central shunt. Total repair was delayed for a mean of 12 months. Follow-up was done in 97 % of patients. Two patients were lost from follow-up. Overall survival of patients was 94 % with median follow-up of 4 years (from 1 to 9 years). There were 3 in-hospital deaths (1st, 10th, 12th days after surgery) and 2 sudden out-hospital deaths (4 months post UF and 6 months post total repair). All patients who died had DiGeorge syndrome (positive for 22q11 deletion). There was no mortality in group of patients without 22q11 deletion. The difference in survival was statistically significant. The overall surgical mortality in 143 operations was 2.1 %. The overall mortality in 65 angiooperations was 0 %. Revision of surgical strategies has made the positive impact on the surgical results. Introduction of surgical algorithm in 2010 year, that helps to choose between the multistage UF, one-stage UF with shunt and one-stage UF with total repair, reduced early surgical mortality from 8,8 % (3 mortalities in 34 operations in 2007-2009 period) to 0 % (no mortality in 109 operations in 2010-2014 period).