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


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

Domingo L, Carey JC, Eckhauser A, Wilkes J, Menon SC.

Pediatr Cardiol. 2019 Feb;40(2):349-356. doi: 10.1007/s00246-018-2001-x. Epub 2018 Oct 5.

PMID: 30291384

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Take Home Points:

  • As expected, overall resource use is higher for patients with T13 and T18 who undergo surgery for cardiac lesions.
  • T13 had a higher mortality, tracheostomies, and gastrostomies than T18.
  • While there is increased risk for morbidity and mortality in this patient population, outcomes may be reasonable for a select cohort to undergo cardiac intervention.

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Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Intervention, especially cardiac surgery, for patients with Trisomy 13 (T13) and Trisomy 18 (T18), has historically rarely been considered or offered due to a high 1 year mortality rate. There seems to be some change in practice over the past few years, possibly influenced by parental desire to “do everything” when these patients are carried to term. As a majority of patients will have CHD, cardiologists are often intimately involved beginning in fetal life. Recent surveys have shown practice variabilities amongst different groups of physicians (e.g. neonatologists vs. cardiologists), but also depending on the type of cardiac lesion. This study was done to describe the clinical outcomes and resource use in those patients who underwent cardiac intervention using a large multicenter database of 45 pediatric hospitals.


Data included demographics, ICD-9 coding, and resource utilization information. Children were excluded if they had additional genetic defects, admitted for non-cardiac indication, or older than age 5 at the time of first cardiac intervention. CHD was categorized as a shunt lesion, left-sided obstruction, conotruncal defect, or single ventricle physiology. Interventions including diagnostic and catheter based interventions, palliative procedures, or complete surgical repair. RACHS and other demographic and historical information were reviewed. Mortality and resource use (total hospital charges adjusted to 2014 US Dollars) were the primary outcomes.


189 patients with T13 and T18 underwent cardiac intervention between 1999-2015. 26% had T13 and 74% T18. There were 238 hospitalizations for cardiac interventions, with 18% of T13 and 14% of T18 undergoing more than one intervention. A higher number of T13 had a tracheostomy or G-tube than T18 (12% vs. 4% and 18% vs. 6%, respectively). Shunt and conotruncal lesions were most common in both groups, but more T13 patients had single ventricle physiology. The majority of interventions were complete surgical repairs. 40% of the interventions occurred in the south US region, with more than half on government insurance. (See Table 1).


T18 patients were significantly older than T13 patients at time of initial intervention (5 vs. 2.5 months). T13 had longer median hospital LOS, but this was not statistically significant. Total median hospital charge was $305,990 for T13 and $252,780 for T18 (also not statistically significant). Mortality rate following surgery was high for both groups, but significantly higher in T13 (29% vs. 12%). Overall in-hospital mortality between groups was not statistically significant (T13 33% and T18 22%). (See Table 2). Regression modeling showed improved survival for white race in T13 patients and older age at initial intervention for T18. Tracheostomy or other comorbidities, cardiopulmonary bypass, type of cardiac lesion, and type of intervention did not affect postprocedural survival.


The authors note that ~70% of patients overall were discharged home with improved symptoms, suggesting a clear clinical benefit for a majority of patients. Compared to previous studies, the age at initial intervention was younger, suggesting a shift towards more aggressive medical intervention. The data also show a clear trend towards complete repair than palliative repair in the US. However, median costs were 1.5-2 times more and hospital LOS 2-3 times longer than those without these trisomies. While type of lesion was not associated with survival, the numbers are probably too low for some lesions to affect current decision making processes in most institutions (e.g. not intervening or palliating for single ventricle lesions). This data though could possibly suggest a benefit to intervening for some lesions if the costs and morbidity are acceptable. Additionally, long-term survival and quality of life data in those who underwent intervention have not yet been elucidated, so individual treatment plans and ethics teams will likely be necessary as we move forward. However, this study should hopefully lead to more discussion at pediatric hospitals as to how best to manage this challenging patient population.


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