Resource Use and Morbidities in Pediatric Cardiac Surgery Patients with Genetic Conditions - 13/12/17

Doi : 10.1016/j.jpeds.2017.09.085

Jamie Furlong-Dillard, DO ¹, David Bailly, DO ¹, Venugopal Amula, MD, MBBS ¹, Jacob Wilkes, BS ², Susan Bratton, MD, MPH ¹
¹ Department of Pediatrics, Division Critical Care, University of Utah School of Medicine, Salt Lake City, UT
² Quality and Informatics, Intermountain Healthcare, Salt Lake City, UT

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Abstract

Objective

To evaluate and describe resource use and perioperative morbidities among those patients with genetic conditions undergoing cardiac surgery.

Study design

Using the Pediatric Health Information System database, we identified patients ≤18 years old with cardiac surgery classified by Risk Adjustment for Congenital Heart Surgery (RACHS) during 2003-2014. A total of 95 253 patients met study criteria and included no genetic conditions (84.6%), trisomy 21 (9.9%), trisomy 13 or 18 (0.2%), 22q11 deletion (0.8%), Turner syndrome (0.4%), and “other” genetic conditions (4.2%). We compared perioperative complications and procedures in each genetic condition with patients without genetic conditions using regression analysis.

Results

All groups with genetic conditions, excluding trisomy 21 RACHS 3-5, experienced increased length of stay and cost among survivors. Complications varied by genetic condition, with patients with trisomy 21 having increased odds of pulmonary hypertension and nosocomial infections. Patients with 22q11 only had increased odds of infection. Patients with Turner syndrome had increased odds of acute renal failure (OR 2.35). Patients with trisomy 13 or 18 had increased odds of pulmonary hypertension (OR 3.13), acute renal failure (OR 2.93), cardiac arrest (OR 2.84), and nosocomial infections (OR 3.53), and those with “other” genetic conditions had increased odds of all complications.

Conclusions

Children with congenital heart disease and genetic conditions, except trisomy 21 RACHS 3-5, had increased costs and length of stay. Perioperative morbidities were more common and differed across genetic condition subgroups. Patient-specific risk factors are important for risk stratification, benchmarking, and counseling with families.

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Keywords : Congenital heart disease, Trisomy 21, Trisomy 13, Trisomy 18, 22q11, Turner syndrome, cost, length of stay

Abbreviations : ARF, CHD, CLABSI, ECMO, ICD-9, IVH, LBW, LOS, PH, PHIS, RACHS, VAP, XO

Plan

S.B. served on the American Board of Pediatric Critical Care Medicine until December 2016 and receives an honorarium. She also serves as an associate editor for Pediatric Critical Care Medicine. D.B. received research support from the National Institutes of Health (NIH) and received funding from the Primary Children's Hospital Early Career Development Award, the NIH loan repayment grant, and Orca Health. The other authors declare no conflicts of interest.