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Mortality and Resource Use Following Cardiac Interventions in Children with Trisomy 13 and Trisomy 18 and Congenital Heart Disease

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Abstract

We sought to evaluate the mortality, risk factors for mortality, and resource utilization following cardiac interventions in trisomy 13 (T13) and 18 (T18) children. All T13 and T18 children who underwent a cardiac intervention from January 1999 to March 2015 were identified from the Pediatric Health Information System database. Data collected included demographics, type of congenital heart disease (CHD), cardiac interventions, comorbidities, length of stay (LOS), hospital charges, and deaths (within 30 days). Logistic regression analysis was used to determine factors associated with mortality. There were 49 (47% females) T13 and 140 (67% females) T18 subjects. The two cohorts were similar in distribution for race, geographic region, insurance type, and median household income. The most common CHD in both groups was a shunt lesion followed by conotruncal defects. Compared to T18, the T13 cohort had higher mortality (29% vs. 12%), tracheostomies (12% vs. 4%), gastrostomies (18% vs. 6%), and overall resource use (P < 0.05 for all). White race (OR 0.23, 95% CI 0.06–0.81) in T13 and older age (in weeks) at surgery in T18 (OR 0.75, 95% CI 0.64–0.86) were associated with lower mortality. A select group of T13 and T18 CHD patients can undergo successful cardiac interventions, albeit with a higher mortality and resource use. T13 patients have higher mortality and resource use compared to T18. In T13 and T18 patients, interventions for CHD may be an acceptable and ethical option following a careful individualized selection and counseling by a team of experts.

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Abbreviations

T13:

Trisomy 13

T18:

Trisomy 18

CHD:

Congenital heart disease

LOS:

Length of stay

PHIS:

Pediatric Health Information Systems

RACHS:

Risk Adjustment for Congenital Heart Surgery

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Funding

No funding was received for this study.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, University of Utah, Salt Lake City, UT, USA

    Liezl Domingo & Shaji C. Menon

  2. Division of Pediatric Cardiology, Augusta University, 1120 15th Street BA, 8300, Augusta, GA, 30909, USA

    Liezl Domingo

  3. Division of Medical Genetics, University of Utah, Salt Lake City, UT, USA

    John C. Carey

  4. Division of Cardiothoracic Surgery, University of Utah, Salt Lake City, UT, USA

    Aaron Eckhauser

  5. Pediatric Specialties Clinical Program, Intermountain Healthcare, Salt Lake City, UT, USA

    Jacob Wilkes

Authors
  1. Liezl Domingo
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  2. John C. Carey
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  3. Aaron Eckhauser
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  4. Jacob Wilkes
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  5. Shaji C. Menon
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Corresponding author

Correspondence to Liezl Domingo.

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Conflict of Interest

Dr. John C. Carey is a medical adviser for the Support Organization for Trisomy 18, 13, and Related Disorders (SOFT organization). All other authors declare that they have no conflicts of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

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Domingo, L., Carey, J.C., Eckhauser, A. et al. Mortality and Resource Use Following Cardiac Interventions in Children with Trisomy 13 and Trisomy 18 and Congenital Heart Disease. Pediatr Cardiol 40, 349–356 (2019). https://doi.org/10.1007/s00246-018-2001-x

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  • DOI: https://doi.org/10.1007/s00246-018-2001-x

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