No Relationships to Disclose Department of Pediatric Cardiology and - - PowerPoint PPT Presentation

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No Relationships to Disclose Department of Pediatric Cardiology and - - PowerPoint PPT Presentation

Sep 18, 2022 240 likes •527 views

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

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SLIDE 1

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

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SLIDE 2
  • Analysis of results of a fifteen-year experience with

surgical treatment of PA-VSD-MAPCAs

  • Identification of variables influencing both early and late
  • utcome with a particular focus on the impact of

unifocalization procedures

PA-VSD-MAPCAs

Aim of the Study

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SLIDE 3

PA-VSD-MAPCAs

Management protocol

PA-VSD-MAPCAs Hypoplastic dominant pulmonary arteries (PAI<100mm2/m2)

  • Abnormal P.Art. arborization
  • Dominant MAPCAs
  • Exclusive MAPCAs

RVOTR

  • r

Central shunt Midline complete unifocalization and flow-study Unifocalization and staged intracardiac repair Low PA pressure (≤30 mmHg) High PA pressure (>30 mmHg) Staged intracardiac repair Angiography Simultaneous intracardiac repair

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SLIDE 4

January 1994 – September 2009

PA-VSD-MAPCAs

Demographics

  • Number of pts

 1994-2000  2001-2009 90 27 (30%) 63 (70%)

  • Chromosome 22q11 deletion (pts)

33 (37%)

  • Median age  at 1st treatment:

 at unifocalization: 12 mos (20 dd - 35 yrs) 15 mos (20 dd - 35 yrs)

  • Median weight  at 1st treatment:

 at unifocalization: 8.2 kg (2.5-54 kg) 8.7 kg (2.5-68 kg)

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SLIDE 5

PA-VSD-MAPCAs

Characteristics of pulmonary blood supply

  • N. pts

% TNPAI = <150 mm2/m2 ≥150 mm2/m2 26 64 29 71 Dominant collateral or pulmonary arteries Spa:Sca <1 Spa:Sca >1 40 50 44 56 Central pulmonary artery/ies No Yes, single Yes, bilaterally 12 20 58 13 22 65 Confluent intrapericardial pulmonary arteries No Yes 28 62 31 69 Confluent intraparenchimal pulmonary arteries No Yes 28 62 31 69

Cross sectional area of MAPCAs + PAs (mm2) Body Surface Area (m2)

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SLIDE 6

PA-VSD-MAPCAs

Surgical treatment

Pts with PA-VSD-MAPCAs n = 90

  • Abnormal P.Art. arborization n = 17
  • Dominant MAPCAs

n = 40

  • Exclusive MAPCAs

n = 12 Complete unifocalization n = 69 Flow-study n = 56 Simultaneous VSD closure n = 44 Hypoplastic dominant pulmonary arteries n = 21 RVOTR n = 19 Central shunt n = 2 Complete unifocalization n = 17 Flow-study n = 15 Simultaneous VSD closure n = 15 VSD left open n = 25 Delayed VSD closure n = 11 In-staging n = 4 In-staging n = 2 Late death n = 2 Early death n = 1 Late death n = 4 In-staging n = 7 Early death n = 6 Late death n = 1 Early death n = 1

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SLIDE 7

PA-VSD-MAPCAs

Outcome

N.pts Staging to promote native pulmonary arterial growth 21 23% Total unifocalization

  • 1st stage
  • 2nd stage

86 69 17 95% 80% 20% VSD management at unifocalization

  • Intraoperative pulmonary flow study
  • VSD closed
  • VSD left open

71 59 27 82% 69% 31% VSD delayed closure 11 41% Complete repair 70 78% Postoperative early pRV/pLV ratio after repair

(all patients)

70 0.48±0.14 Postoperative early pRV/pLV ratio after repair

(patients who entered paired t test analysis)

35 0.51±0.14

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SLIDE 8
  • N.Observations

78

  • Median interval (mos)

46 (1.5-165)

  • Conduit reinterventions: N.Pts

25

  • Percutaneous intervention on PAs: N.Pts
  • Once (N. Pts)
  • Twice or more times (N. Pts)

30

20 10

  • Bronchial stenting: N.Pts

3

PA-VSD-MAPCAs

Follow-up

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SLIDE 9

95% CI Freedom from percutaneous intervention on PAs

months

95% CI Survival function

months

95% CI Freedom from conduit reintervention

months

PA-VSD-MAPCAs

Kaplan-Meier Estimates

Survival ≤30 days: 92%

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SLIDE 10

Variable Obs Mean Std.Err. Std.Dev. p Value pRV/pLV p.o. pRV/pLV f.u. Diff 35 35 35 .51 .53

  • 1,71

.02 .03 .03 .14 .18 .18 0.579

PA-VSD-MAPCAs

pRV/pLV Comparison (paired t test)

0,1 0,2 0,3 0,4 0,5 0,6 0,7 0,8 0,9 1

early p.o. median 95 mos f.u. (1.5-164 mos)

Early p.o. Follow-up

0.5 1

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SLIDE 11

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
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SLIDE 12
  • Age

Categorical ≤30 vs >30 days Continuous

  • Weight

Categorical ≤3 vs >3 kgs Continuous

  • TNPAI

<150 vs ≥150 mm2/m2

  • 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

Variables examined

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SLIDE 13

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
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SLIDE 14

Group Obs (n=90) No (n=4) Yes (n=86) p Value

Age ≤30 days >30 days 3 87 4 3 83 1.000 Weight ≤3 kgs >3 kgs 3 87 4 3 83 1.000 TNPAI <150 mm2/m2 ≥150 mm2/m2 26 64 4 22 64 0.006 Confluent intrapericardial PAs No Yes 28 62 4 28 58 0.306 Confluent intraparenchimal PAs No Yes 28 62 4 28 58 0.306 Central pulmonary artery/ies No Yes, single Yes, bilaterally 12 20 58 4 12 20 54 0.758 Chromosome 22q11.2 microdeletion No Yes 57 33 4 53 33 0.292 Dominant collateral or PAs MAPCAs PAs 40 50 4 40 46 0.126 Era at treatment 1994-2000 2001-2009 27 63 4 27 59 0.312

PA-VSD-MAPCAs

Univariate model fit for suitability to one-stage unifocalization (χ2- Fisher)

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SLIDE 15

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 Yes 4 86 9 44.1

  • 6.9 24.9

29.7 58.3 0.30 Weight No Yes 4 86 5.9 13.7 2.1 9.8 10.9 16.5 0.24

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SLIDE 16

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
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SLIDE 17

PA-VSD-MAPCAs

Univariate model fit for suitability to simultaneous VSD closure (χ2- Fisher)

Group Obs (n=86) No (n=27) Yes (n=59) p Value

Age ≤30 days >30 days 3 83 2 25 1 58 0.231 Weight ≤3 kgs >3 kgs 3 83 2 25 1 58 0.231 TNPAI <150 mm2/m2 ≥150 mm2/m2 22 64 7 20 15 44 1.000 Confluent intrapericardial PAs No Yes 28 58 10 18 17 41 0.623 Confluent intraparenchimal PAs No Yes 28 58 10 18 17 41 0.623 Central pulmonary artery/ies No Yes, single Yes, bilaterally 12 20 54 4 9 14 8 11 40 0.271 Chromosome 22q11.2 microdeletion No Yes 53 33 13 14 40 19 0.098 Dominant collateral or PAs MAPCAs PAs 40 46 16 11 24 35 0.162 Era at treatment 1994-2000 2001-2009 27 59 4 23 23 36 0.027

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SLIDE 18

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 Yes 27 59 35.7 47.8 13.8 57.7 29.2 66.5 0.40 Weight No Yes 27 59 12.1 14.4 7.5 16.7 10.8 17.9 0.40

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SLIDE 19

PA-VSD-MAPCAs

Logistic regression model fit for suitability to simultaneous VSD closure Group Odds Ratio p Value 95% CI Chromosome 22q11 deletion Era at treatment .399 .249 0.064 0.025 0.150 1.056 0.074 0.839

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SLIDE 20

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
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SLIDE 21

PA-VSD-MAPCAs

Univariate model fit for postoperative pRV/pLV (ANOVA)

Group (Obs = 70) Coef. p Value 95% CI

Age Categorical ≤30 vs >30 days Continuous

  • .08
  • .00

0.55 0.20

  • .37 .20
  • .00 .00

Weight Categorical ≤3 vs >3 kgs Continuous

  • .08
  • .00

0.55 0.39

  • .37 .20
  • .00 .00

TNPAI <150 vs ≥150 mm2/m2

  • .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
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SLIDE 22

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
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SLIDE 23

Age at unifocalization: ≤30 days >30 days Weight at unifocalization: ≤3 kg >3 kg del 22q: No Yes Simultaneous VSD closure: No Yes

months months months months

PA-VSD-MAPCAs

Kaplan-Meier survival estimates comparison (log-rank test)

p = 0.0004 p = 0.0004 p = 0.001 p = 0.01

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SLIDE 24

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 Dominant collateral or PAs 4.174 1.666 0.006 0.351 1.616 16.566 .569 4.873 Chromosome 22q11 deletion Era at treatment 5.556 .925 0.003 0.889 1.758 17.558 .309 2.768

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SLIDE 25
  • Single-center retrospective study in which a treatment

protocol was analyzed in the absence of a control group

  • Follow-up was incomplete for 8 out of 78 patients (10%),

due to insufficient data retrieval from foreign countries

  • Some results of the analysis are probably biased by the

use of specific parameters (e.g. TNPAI) as selection criteria

PA-VSD-MAPCAs

Limitations of the Study

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SLIDE 26

PA-VSD-MAPCAs

Conclusions 1

  • Results of one-stage complete unifocalization for PA-

VSD-MAPCAs are satisfactory and durable despite the need

  • f

repeated percutaneous

  • r

surgical reinterventions

  • The

use

  • f

MAPCAs for neo-pulmonary arterial reconstruction assures functional pulmonary blood flow, compensates for defects in true pulmonary arterial distribution, and is the only option in cases with absent pulmonary arteries

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SLIDE 27

PA-VSD-MAPCAs

Conclusions 2

  • Avoiding

neonatal age and low body weight at unifocalization may increase the chance of positive

  • utcome, as it does the simultaneous closure of

ventricular septal defect, when feasible

  • Presence
  • f

confluent intrapericardial pulmonary arteries does not improve the hemodynamic result after VSD closure

  • Chromosome 22q11 deletion remains an independent

variable affecting survival