Trends in Long-Term Mortality After Congenital Heart Surgery

Spector, Logan G.; Menk, Jeremiah; Knight, Jessica; McCracken, Courtney; Thomas, Amanda S.; Vinocur, Jeffrey M.; Oster, Matthew E.; Louis, James D. St.; Moller, James H.; Kochilas, Lazaros

doi:10.1016/j.jacc.2018.03.491

Cited by 168 publications

(113 citation statements)

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“…Using the PCCC cohort and mortality data from the NDI, we recently reported that PCCC patients with SV anatomy had survival estimates of 84.1%, 82.7%, 80%, and 78.4% at 5, 10, 15, and 20 years, respectively. 11 As part of the same cohort, we now report the in-hospital and long-term survival for one of the largest series of patients with T21 following SV palliation.…”

Section: Discussionmentioning

confidence: 98%

“…Additionally, we compare transplant‐free survival following the Fontan procedure between SV patients with T21 and without a known genetic anomaly. We used the linked dataset of the Pediatric Cardiac Care Consortium (PCCC), a large multi‐center registry for interventions for CHD, with the National Death Index (NDI) for information on outcomes and causes of death, and with the Organ Procurement and Transplantation Network (OPTN) for organ transplant information …”

Section: Introductionmentioning

confidence: 99%

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Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Peterson

Setty

Knight

et al. 2019

Congenital Heart Disease

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Objective Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation. Design This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anomaly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers. Results We identified 118 children with T21 who underwent initial surgical SV palliation. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in‐hospital survival of 80.3% and 76.0%, respectively. Fifty‐three patients had sufficient identifiers for PCCC‐NDI‐OPTN linkage. Ten‐year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34‐7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD). Conclusions Children with T21 and SV are at high risk for procedural and long‐term mortality related to their genetic condition and underlying CHD. Nevertheless, a select group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long‐term survival.

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Peterson

Setty

Knight

et al. 2019

Congenital Heart Disease

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“…On the one hand, medicolegal issues require the physicians to provide extensive and fair information, which may contribute to increase the anxiety level of the parents in a disproportionate way. Indeed, currently 99% of children with a mild CHD and 97% of those with a moderate CHD are discharged alive after first cardiac surgery . On the other hand, a reassuring medical speech in response to worried parents may seem benevolent but sometimes results in negative effects.…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, currently 99% of children with a mild CHD and 97% of those with a moderate CHD are discharged alive after first cardiac surgery. 39 On the other hand, a reassuring medical speech in response to worried parents may seem benevolent but sometimes results in negative effects. For example, Rempel et al have shown that medical advice to parents of children with a hypoplastic left heart syndrome to "treat their child normally" was a bad advice, especially by delaying management of neurodevelopmental issues.…”

Section: Discussionmentioning

confidence: 99%

Parental anxiety before invasive cardiac procedure in children with congenital heart disease: Contributing factors and consequences

Werner

Louali

Fouilloux

et al. 2019

Congenital Heart Disease

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Objective Medical information provided to parents of a child with a congenital heart disease can induce major stress. Visual analog scales have been validated to assess anxiety in the adult population. The aim of this study was to analyze parental anxiety using a visual analog scale and to explore the influencing factors. Design This prospective cross‐sectional study. Setting Tertiary care regional referral center for congenital heart disease of Marseille—La Timone university hospital. Patients Parents of children with a congenital heart disease, as defined by the ACC‐CHD classification, referred for cardiac surgery or interventional cardiac catheterization, were offered to participate. Intervention and outcome measure The parental level of anxiety was assessed using a visual analog scale (0‐10) before intervention and after complete information given by the cardiologist, the surgeon or the anesthetists. Results Seventy‐three children [7 days‐13 years], represented by 49 fathers and 71 mothers, were included in the study. A total of 42 children required cardiac surgery and 31 children underwent interventional cardiac catheterization. The mean score of maternal anxiety was significantly higher than the paternal anxiety (8.2 vs 6.3, P < .01). A high level of maternal anxiety (visual analog scale > 8) was associated with paternal anxiety (P = .02), the child's comorbidity (P = .03), the distance between home and referral center (P = .04), and the level of risk adjustment for congenital heart surgery (P = .01). In multivariate analysis, maternal anxiety was associated with paternal anxiety (OR = 4.9; 95% confidence interval [1.1‐19.2]), and the level of risk adjustment for congenital heart surgery (OR = 11.4; 95% confidence interval [1.2‐116.2]). No significant association was found between parental anxiety and prenatal diagnosis. Conclusion This study highlighted several factors associated with the parental anxiety. Identifying the parents at risk of high stress can be useful to set up psychological support during hospitalization.

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“…1,54) In addition, the morbidity and mortality in adult patients affected with CHDs are much higher than those in the general population. [55][56][57][58] In spite of important clinical significance, the causes of CHDs remain largely obscure.…”

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confidence: 99%

A New <i>ISL1</i> Loss-of-Function Mutation Predisposes to Congenital Double Outlet Right Ventricle

et al. 2019

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Occurring in about 1% of all live births, congenital heart defects (CHDs) represent the most frequent type of developmental abnormality and account for remarkably increased infant morbidity and mortality. Aggregating studies demonstrate that genetic components have a key role in the occurrence of CHDs. Nevertheless, due to pronounced genetic heterogeneity, the genetic causes of CHDs remain unclear in most patients. In this research, 114 unrelated patients affected with CHDs and 218 unrelated individuals without CHDs served as controls were recruited. The coding regions and splicing donors/acceptors of the ISL1 gene, which codes for a transcription factor required for proper cardiovascular development, were screened for mutations by sequencing in all study participants. The functional characteristics of an identified ISL1 mutation were delineated with a dual-luciferase reporter assay system. As a result, a new heterozygous ISL1 mutation, NM_002202.2: c.225C>G; p.(Tyr75*), was discovered in an index patient with double outlet right ventricle and ventricular septal defect. Analysis of the proband's family unveiled that the mutation co-segregated with the CHD phenotype. The nonsense mutation was absent in the 436 control chromosomes. Biological analysis showed that the mutant ISL1 protein had no transcriptional activity. Furthermore, the mutation nullified the synergistic activation between ISL1 and TBX20, another CHD-associated transcription factor. This research for the first time links an ISL1 loss-of-function mutation to double outlet right ventricle in humans, which adds insight to the molecular pathogenesis underpinning CHDs, suggesting potential implications for timely personalized management of CHD patients. (Int Heart J 2019; 60: 1113-1122

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Trends in Long-Term Mortality After Congenital Heart Surgery

Abstract: In this large U.S. cohort, long-term mortality after congenital heart surgery was elevated across all forms of CHD. Survival has improved over time, particularly for severe defects with significant changes in their management strategy, but still lags behind the general population.

Cited by 168 publications

References 50 publications

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Parental anxiety before invasive cardiac procedure in children with congenital heart disease: Contributing factors and consequences

A New <i>ISL1</i> Loss-of-Function Mutation Predisposes to Congenital Double Outlet Right Ventricle

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