Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 160; no. 4; pp. 1021 - 1030
• Main Authors: Ahmed, Humera, Anderson, Jeffrey B., Bates, Katherine E., Fleishman, Craig E., Natarajan, Shobha, Ghanayem, Nancy S., Sleeper, Lynn A., Lannon, Carole M., Brown, David W.
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01.10.2020
• Subjects: cardiovascular surgery; congenital heart disease; mortality/survival; transplant; ECMO; IM; SVCHD; mBTS; HLHS; SVR; cardiovascular surgery; NPC-QIC; S2P; S1P; RV-PAC; congenital heart disease; transplant; mortality/survival; TR; CART
• ISSN: 0022-5223; 1097-685X
• DOI: 10.1016/j.jtcvs.2019.11.001

To develop a risk score to predict mortality or transplant in the interstage period. The “interstage” period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients (“learning cohort”) to build a risk score for the composite endpoint, that was validated in the remaining patients (“validation cohort”). In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score (“NEONATE” score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant. We created a validated risk score for patients with single-ventricle congenital heart defects ready for discharge to home in the interstage period after stage I/Norwood palliation, designed to guide objective decision-making regarding the safety of patient discharge. Multivariate modeling identified the 7 variables comprising the NEONATE risk score that had good predictive ability across the diverse patient group represented in both the learning and validation cohorts of the National Pediatric Cardiology Quality Improvement Registry. Patients with a NEONATE score of ≤17 were at low risk of interstage death and transplant compared with those with NEONATE score >17. CART, Classification and regression tree; ECMO, extracorporeal membrane oxygenator; O2, oxygen; TR, tricuspid regurgitation. [Display omitted]