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. 2023 Sep:260:113530.
doi: 10.1016/j.jpeds.2023.113530. Epub 2023 May 31.

Congenital Heart Disease with Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes

Latoya A Stewart  1 , Rebecca R Hernan  2 , Christopher Mardy  3 , Eunice Hahn  3 , Wendy K Chung  2 , Emile A Bacha  4 , Ganga Krishnamurthy  2 , Vincent P Duron  5 , Usha S Krishnan  6
Affiliations

Congenital Heart Disease with Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes

Latoya A Stewart et al. J Pediatr. 2023 Sep.

Abstract

Objective: To describe the types of congenital heart disease (CHD) in a congenital diaphragmatic hernia (CDH) cohort in a large volume center and evaluate surgical decision making and outcomes based on complexity of CHD and associated conditions.

Study design: A retrospective review of patients with CHD and CDH diagnosed by echocardiogram between 01/01/2005 and 07/31/2021. The cohort was divided into 2 groups based on survival at discharge.

Results: Clinically important CHD was diagnosed in 19% (62/326) of CDH patients. There was 90% (18/20) survival in children undergoing surgery for both CHD and CDH as neonates, and 87.5 (22/24) in those undergoing repair initially for CDH alone. A genetic anomaly identified on clinical testing was noted in 16% with no significant association with survival. A higher frequency of other organ system anomalies was noted in nonsurvivors compared with survivors. Nonsurvivors were more likely to have unrepaired CDH (69% vs 0%, P < .001) and unrepaired CHD (88% vs 54%, P < .05), reflecting a decision not to offer surgery.

Conclusions: Survival was excellent in patients who underwent repair of both CHD and CDH. Patients with univentricular physiology have poor survival and this finding should be incorporated into pre and postnatal counseling about eligibility for surgery. In contrast, patients with other complex lesions including transposition of the great arteries have excellent outcomes and survival at 5 years follow-up at a large pediatric and cardiothoracic surgical center.

Keywords: cardiac and thoracic surgery; genetics; outcomes; prenatal counseling.

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Conflict of interest statement

Declaration of Competing Interest Supported by the National Institute of Health grant NICHDR-01 HD057036, P01HD068250, UL1 RR024156. The authors declare no conflicts of interest.

Figures

Figure 1:
Figure 1:
Flow diagram showing CHD-CDH cohort composition IUFD: intrauterine fetal demise Simple: (STAT Score 1,2) Complex (STAT Score 3,4,5)
Figure 2:
Figure 2:
CHD-CDH survival status based on repair status Bolded percentages: refer to survival rate at discharge for patients with CDH and CHD within each repair category; N (%): refers to proportion of cohort in each of the 4 categories
Figure 3:
Figure 3:
Percentage survival by CHD severity and presence of additional anomalies in patients undergoing cardiac repair Simple CHD - STAT Score 1,2 Complex CHD -STAT Score 3,4,5 + Additional anomalies present • No additional anomalies.
See this image and copyright information in PMC

References

    1. CDC. Data and statistics on birth defects. Division of Birth Defects and Developmental Disabilities, NCBDDD, Centers for Disease Control and Prevention; https://www.cdc.gov/ncbddd/birthdefects/data.html [Internet]. 2021.
    1. Akinkuotu AC, Cruz SM, Cass DL, Lee TC, Cassady CI, Mehollin-Ray AR, et al. An evaluation of the role of concomitant anomalies on the outcomes of fetuses with congenital diaphragmatic hernia. J Pediatr Surg. 2016;51(5):714–7. - PubMed
    1. Graziano JN; Congenital Diaphragmatic Hernia Study Group. Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg. 2005;40(6):1045–50. - PubMed
    1. Menon SC, Tani LY, Weng HY, Lally PA, Lally KP, Yoder BA; Congenital Diaphragmatic Hernia Study Group. Clinical characteristics and outcomes of patients with cardiac defects and congenital diaphragmatic hernia. J Pediatr. 2013;162(1):114–119.e2. - PubMed
    1. Lin AE, Pober BR, Adatia I. Congenital diaphragmatic hernia and associated cardiovascular malformations: type, frequency, and impact on management. Am J Med Genet C Semin Med Genet. 2007;15;145C(2):201–16. - PMC - PubMed

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