Congenital heart disease
Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: Risk factors and their interaction with shunt type

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Objective

The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock–Taussig shunt or a right ventricle-to-pulmonary artery shunt.

Methods

We used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors.

Results

Data from 549 subjects (follow-up, 2.7 ± 0.9 years) were analyzed. Mortality risk was characterized by early and constant phases; transplant was characterized by only a constant phase. Early phase factors associated with death included lower socioeconomic status (P = .01), obstructed pulmonary venous return (P < .001), smaller ascending aorta (P = .02), and anatomic subtype. Constant phase factors associated with death included genetic syndrome (P < .001) and lower gestational age (P < .001). The right ventricle-to-pulmonary artery shunt demonstrated better survival in the 51% of subjects who were full term with aortic atresia (P < .001). The modified Blalock–Taussig shunt was better among the 4% of subjects who were preterm with a patent aortic valve (P = .003). Lower pre-Norwood right ventricular fractional area change, pre-Norwood surgery, and anatomy other than hypoplastic left heart syndrome were independently associated with transplantation (all P < .03), but shunt type was not (P = .43).

Conclusions

Independent risk factors for intermediate-term mortality include lower socioeconomic status, anatomy, genetic syndrome, and lower gestational age. Term infants with aortic atresia benefited from a right ventricle-to-pulmonary artery shunt, and preterm infants with a patent aortic valve benefited from a modified Blalock–Taussig shunt. Right ventricular function and anatomy, but not shunt type, were associated with transplantation.

Abbreviations and Acronyms

AA
aortic atresia
AS
aortic stenosis
HLHS
hypoplastic left heart syndrome
HR
hazard ratio
MA
mitral atresia
MBTS
modified Blalock–Taussig shunt
MS
mitral stenosis
OPVR
obstructed pulmonary venous return
PHN SVR
Pediatric Heart Network Single Ventricle Reconstruction
RVPAS
right ventricle-to-pulmonary artery shunt
SES
socioeconomic status

CTSNet classification

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Supported by U01 grants from the National Heart, Lung, and Blood Institute (HL068269, HL068270, HL068279, HL068281, HL068285, HL068292, HL068290, HL068288, HL085057). Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the National Heart, Lung, and Blood Institute. Clinical Trial Registration NCT00115934.

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