Determinants of Outcome after Surgical Treatment of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries Presenter Disclosure Adriano Carotti, MD The following relationships exist related to this presentation: No Relationships to Disclose Department of Pediatric Cardiology and Cardiac Surgery Bambino Gesu’ Children’s Hospital Roma, Italy
PA-VSD-MAPCAs Aim of the Study • Analysis of results of a fifteen-year experience with surgical treatment of PA-VSD-MAPCAs • Identification of variables influencing both early and late outcome with a particular focus on the impact of unifocalization procedures
PA-VSD-MAPCAs Management protocol PA-VSD-MAPCAs Angiography • Abnormal P.Art. arborization Hypoplastic dominant pulmonary arteries • Dominant MAPCAs (PAI<100mm 2 /m 2 ) • Exclusive MAPCAs Midline complete RVOTR unifocalization or and Central shunt flow-study Low PA pressure High PA pressure Unifocalization and ( ≤30 mmHg) (>30 mmHg) staged intracardiac repair Simultaneous Staged intracardiac repair intracardiac repair
PA-VSD-MAPCAs Demographics January 1994 – September 2009 Number of pts 90 1994-2000 27 (30%) 2001-2009 63 (70%) Chromosome 22q11 deletion (pts) 33 (37%) Median age at 1st treatment: 12 mos (20 dd - 35 yrs) at unifocalization: 15 mos (20 dd - 35 yrs) Median weight at 1st treatment: 8.2 kg (2.5-54 kg) at unifocalization: 8.7 kg (2.5-68 kg)
PA-VSD-MAPCAs Characteristics of pulmonary blood supply N. pts % Cross sectional area of MAPCAs + PAs (mm 2 ) TNPAI = Body Surface Area (m 2 ) <150 mm 2 /m 2 26 29 ≥ 150 mm 2 /m 2 64 71 Dominant collateral or pulmonary arteries Spa:Sca <1 40 44 Spa:Sca >1 50 56 Central pulmonary artery/ies No 12 13 Yes, single 20 22 Yes, bilaterally 58 65 Confluent intrapericardial pulmonary arteries No 28 31 Yes 62 69 Confluent intraparenchimal pulmonary arteries No 28 31 Yes 62 69
PA-VSD-MAPCAs Surgical treatment Pts with PA-VSD-MAPCAs n = 90 Hypoplastic dominant pulmonary arteries • Abnormal P.Art. arborization n = 17 n = 21 • Dominant MAPCAs n = 40 • Exclusive MAPCAs n = 12 RVOTR n = 19 Central shunt n = 2 Complete unifocalization n = 17 Complete unifocalization n = 69 Flow-study n = 15 Flow-study n = 56 Simultaneous VSD closure n = 15 Early death n = 1 Simultaneous VSD closure n = 44 Late death n = 4 In-staging n = 2 Early death n = 6 VSD left open n = 25 Late death n = 1 In-staging n = 4 Early death n = 1 Delayed VSD closure n = 11 Late death n = 2 In-staging n = 7
PA-VSD-MAPCAs Outcome N.pts Staging to promote native pulmonary arterial growth 21 23% Total unifocalization 86 95% - 1 st stage 69 80% - 2 nd stage 17 20% VSD management at unifocalization - Intraoperative pulmonary flow study 71 82% - VSD closed 59 69% - VSD left open 27 31% VSD delayed closure 11 41% Complete repair 70 78% 0.48 ± 0.14 Postoperative early pRV/pLV ratio after repair 70 (all patients) 0.51 ± 0.14 Postoperative early pRV/pLV ratio after repair 35 (patients who entered paired t test analysis)
PA-VSD-MAPCAs Follow-up • N.Observations 78 • Median interval (mos) 46 (1.5-165) • Conduit reinterventions: N.Pts 25 • Percutaneous intervention on PAs: N.Pts 30 - Once (N. Pts) 20 - Twice or more times (N. Pts) 10 • Bronchial stenting: N.Pts 3
Survival ≤30 days: 92% PA-VSD-MAPCAs Kaplan-Meier Estimates months 95% CI Survival function months 95% CI Freedom from conduit reintervention months 95% CI Freedom from percutaneous intervention on PAs
PA-VSD-MAPCAs pRV/pLV Comparison (paired t test) 1 1 0,9 0,8 0,7 early p.o. 0,6 0,5 0.5 median 95 mos f.u. 0,4 (1.5-164 mos) 0,3 0,2 0,1 Early p.o. Follow-up 0 0 Variable Obs Mean Std.Err. Std.Dev. p Value pRV/pLV p.o. 35 .51 .02 .14 pRV/pLV f.u. 35 .53 .03 .18 Diff 35 -1,71 .03 .18 0.579
PA-VSD-MAPCAs End-points of univariate and multivariate analysis • Suitability to one-stage unifocalization • Suitability to simultaneous VSD closure • Postoperative pRV/pLV ratio after VSD closure • Survival
PA-VSD-MAPCAs Variables examined • Age Categorical ≤30 vs >30 days Continuous • Weight Categorical ≤3 vs >3 kgs Continuous • TNPAI <150 vs ≥ 150 mm 2 /m 2 • Confluent intrapericardial pulmonary arteries No vs Yes • Confluent intraparenchimal pulmonary arteries No vs Yes • Central pulmonary artery/ies No vs Yes • Chromosome 22q11.2 microdeletion No vs Yes • Dominant collateral or pulmonary arteries MAPCAs vs PAs • Era at treatment 1994-2000 vs 2001-2009
PA-VSD-MAPCAs End-points of univariate and multivariate analysis • Suitability to one-stage unifocalization • Suitability to simultaneous VSD closure • Postoperative pRV/pLV ratio after VSD closure • Survival
PA-VSD-MAPCAs Univariate model fit for suitability to one- stage unifocalization (χ 2 - Fisher) Group Obs No Yes p Value (n=90) (n=4) (n=86) ≤30 days Age 3 0 3 1.000 >30 days 87 4 83 ≤3 kgs Weight 3 0 3 1.000 >3 kgs 87 4 83 TNPAI <150 mm 2 /m 2 26 4 22 0.006 ≥ 150 mm 2 /m 2 64 0 64 Confluent intrapericardial PAs No 28 0 28 0.306 Yes 62 4 58 Confluent intraparenchimal PAs No 28 0 28 0.306 Yes 62 4 58 Central pulmonary artery/ies No 12 0 12 0.758 Yes, single 20 0 20 Yes, bilaterally 58 4 54 Chromosome 22q11.2 microdeletion No 57 4 53 0.292 Yes 33 0 33 Dominant collateral or PAs MAPCAs 40 0 40 0.126 PAs 50 4 46 Era at treatment 1994-2000 27 0 27 0.312 2001-2009 63 4 59
PA-VSD-MAPCAs Univariate model fit for suitability to one-stage unifocalization (two-sample t test) GROUP Obs Mean 95% CI p Value Age No 4 9 -6.9 24.9 Yes 86 44.1 29.7 58.3 0.30 Weight No 4 5.9 2.1 9.8 Yes 86 13.7 10.9 16.5 0.24
PA-VSD-MAPCAs End-points of univariate and multivariate analysis • Suitability to one-stage unifocalization • Suitability to simultaneous VSD closure • Postoperative pRV/pLV ratio after VSD closure • Survival
PA-VSD-MAPCAs Univariate model fit for suitability to simultaneous VSD closure (χ 2 - Fisher) Group Obs No Yes p Value (n=86) (n=27) (n=59) ≤30 days Age 3 2 1 0.231 >30 days 83 25 58 ≤3 kgs Weight 3 2 1 0.231 >3 kgs 83 25 58 <150 mm 2 /m 2 TNPAI 22 7 15 1.000 ≥ 150 mm 2 /m 2 64 20 44 Confluent intrapericardial PAs No 28 10 17 0.623 Yes 58 18 41 Confluent intraparenchimal PAs No 28 10 17 0.623 Yes 58 18 41 Central pulmonary artery/ies No 12 4 8 0.271 Yes, single 20 9 11 Yes, bilaterally 54 14 40 Chromosome 22q11.2 microdeletion No 53 13 40 0.098 Yes 33 14 19 Dominant collateral or PAs MAPCAs 40 16 24 0.162 PAs 46 11 35 Era at treatment 1994-2000 27 4 23 0.027 2001-2009 59 23 36
PA-VSD-MAPCAs Univariate model fit for suitability to simultaneous VSD closure (two-sample t test) GROUP Obs Mean 95% CI p Value Age No 27 35.7 13.8 57.7 Yes 59 47.8 29.2 66.5 0.40 Weight No 27 12.1 7.5 16.7 Yes 59 14.4 10.8 17.9 0.40
PA-VSD-MAPCAs Logistic regression model fit for suitability to simultaneous VSD closure Group Odds p Value 95% CI Ratio Chromosome 22q11 deletion .399 0.064 0.150 1.056 Era at treatment .249 0.025 0.074 0.839
PA-VSD-MAPCAs End-points of univariate and multivariate analysis • Suitability to one-stage unifocalization • Suitability to simultaneous VSD closure • Postoperative pRV/pLV ratio after VSD closure • Survival
PA-VSD-MAPCAs Univariate model fit for postoperative pRV/pLV (ANOVA) Group Coef. p Value 95% CI (Obs = 70) Age Categorical ≤30 vs >30 days -.08 0.55 -.37 .20 Continuous -.00 0.20 -.00 .00 Weight Categorical ≤3 vs >3 kgs -.08 0.55 -.37 .20 Continuous -.00 0.39 -.00 .00 TNPAI <150 vs ≥ 150 mm 2 /m 2 -.02 0.66 -.09 .06 Confluent intrapericardial PAs No(0) vs Yes(1) .07 0.04 -.00 .15 Confluent intraparenchimal PAs No vs Yes -.03 0.46 -.10 .04 Central pulmonary artery/ies No vs Yes .03 0.18 -.01 .08 Chromosome 22q11 deletion No vs Yes .05 0.14 -.01 .12 Dominant collateral or pulmonary arteries MAPCAs vs PAs -.00 0.97 -.07 .07 Era at treatment 1994-2000 vs 2001-2009 -.04 0.22 -.11 .02
PA-VSD-MAPCAs End-points of univariate and multivariate analysis • Suitability to one-stage unifocalization • Suitability to simultaneous VSD closure • Postoperative pRV/pLV ratio after VSD closure • Survival
PA-VSD-MAPCAs Kaplan-Meier survival estimates comparison (log-rank test) p = 0.0004 p = 0.0004 months months ≤30 days ≤3 kg Age at unifocalization: >30 days Weight at unifocalization: >3 kg p = 0.001 p = 0.01 months months del 22q: No Yes Simultaneous VSD closure: No Yes
PA-VSD-MAPCAs Cox regression model fit for mortality Group Haz.ratio p Value 95% CI Age (continous) 1.001 0.710 .994 1.008 Weight (continuous) 1.004 0.804 .967 1.044 Chromosome 22q11 deletion 4.174 0.006 1.616 16.566 Dominant collateral or PAs 1.666 0.351 .569 4.873 Chromosome 22q11 deletion 5.556 0.003 1.758 17.558 Era at treatment .925 0.889 .309 2.768
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