Congenital heart disease
Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial

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Objective

For infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock–Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors.

Methods

Participants in the Single Ventricle Reconstruction trial who survived to discharge after the Norwood procedure were included (n = 426). Interstage mortality was defined as death postdischarge after the Norwood procedure and before the stage II procedure. Univariate analysis and multivariable logistic regression were performed adjusting for site.

Results

Overall interstage mortality was 50 of 426 (12%)—13 of 225 (6%) for RVPAS and 37 of 201 (18%) for MBTS (odds ratio [OR] for MBTS, 3.4; P < .001). When moderate to severe postoperative atrioventricular valve regurgitation (AVVR) was present, interstage mortality was similar between shunt types. Interstage mortality was independently associated with gestational age less than 37 weeks (OR, 3.9; P = .008), Hispanic ethnicity (OR, 2.6; P = .04), aortic atresia/mitral atresia (OR, 2.3; P = .03), greater number of post-Norwood complications (OR, 1.2; P = .006), census block poverty level (P = .003), and MBTS in subjects with no or mild postoperative AVVR (OR, 9.7; P < .001).

Conclusions

Interstage mortality remains high at 12% and is increased with the MBTS compared with the RVPAS if postoperative AVVR is absent or mild. Preterm delivery, anatomic, and socioeconomic factors are also important. Avoiding preterm delivery when possible and close surveillance after Norwood hospitalization for infants with identified risk factors may reduce interstage mortality.

CTSNet classification

20

Abbreviations and Acronyms

AVVR
atrioventricular valve regurgitation
BSA
body surface area
CPR
cardiopulmonary resuscitation
DHCA
deep hypothermic circulatory arrest
ECMO
extracorporeal membrane oxygenation
HLHS
hypoplastic left heart syndrome
MBTS
modified Blalock–Taussig shunt
OR
odds ratio
RVEDV
right ventricular end-diastolic volume
RVEF
right ventricular ejection fraction
RVESV
right ventricular end-systolic volume
RVPAS
right ventricle-to-pulmonary artery shunt
SVR
Single Ventricle Reconstruction

Cited by (0)

Supported by Grants HL068269, HL068270, HL068279, HL068281, HL068285, HL068288, HL068290, HL068292, and HL085057 from the National Heart, Lung, and Blood Institute. This work is solely the responsibility of the authors and does not necessarily represent the official views of the National Heart, Lung, and Blood Institute.

Disclosures: Authors have nothing to disclose with regard to commercial support.

Kerstin R. Allen's current affiliation is Infinity Pharmaceuticals, Boston, Mass; Sarah Tabbutt's current affiliation is University of California San Francisco, San Francisco, Calif; Peter J. Gruber's current affiliation is Primary Children's Medical Center and University of Utah, Salt Lake City, Utah; David S. Cooper's and Pirooz Egthesady's current affiliation is Cincinnati Children's Medical Center, Cincinnati, Ohio.

Pediatric Heart Network Investigators are listed in Appendix 1.