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Published on: January 27, 2023
Early Left Ventricular Dysfunction and Severe Pulmonary Hypertension Predict Adverse Outcomes in "Low-Risk"
Duy T Dao1,2, Neil Patel3, Matthew T Harting4
1Department of Surgery, Boston Children's Hospital, Boston, MA.
Insights
Early cardiac dysfunction and pulmonary hypertension predict adverse outcomes in low-risk congenital diaphragmatic hernia (CDH) infants. Identifying these risk factors can guide interventions to improve survival in this population.
Area of Science:
- Pediatric Cardiology
- Neonatal Surgery
- Critical Care Medicine
Background:
- Congenital diaphragmatic hernia (CDH) management often prioritizes high-risk cases.
- Risk factors for adverse outcomes in "low-risk" CDH populations require further identification.
- Early cardiac dysfunction and pulmonary hypertension are potential predictors of poor outcomes.
Purpose of the Study:
- To identify risk factors for suboptimal outcomes in "low-risk" congenital diaphragmatic hernia (CDH) infants.
- To investigate the association between early cardiac dysfunction, pulmonary hypertension, and adverse events in this cohort.
Main Methods:
- Retrospective cohort study using the Congenital Diaphragmatic Hernia Study Group registry.
- Defined "low-risk" CDH as defect size A/B without cardiac or chromosomal anomalies.
- Analyzed left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension on postnatal echocardiograms as risk factors for composite adverse events.
Main Results:
- Seven hundred seventy-eight "low-risk" CDH infants were analyzed.
- Left ventricular dysfunction (10.8%), right ventricular dysfunction (20.5%), and severe pulmonary hypertension (57.5%) were present.
- Left ventricular dysfunction and severe pulmonary hypertension independently predicted adverse outcomes (composite events including death, ECMO, prolonged oxygen, or extended hospitalization).
Conclusions:
- Early left ventricular dysfunction and severe pulmonary hypertension are significant predictors of adverse outcomes in "low-risk" CDH infants.
- These findings highlight the need for early recognition and intervention in this specific patient group.
- Improved outcomes may be achievable through targeted interventions based on early echocardiographic findings.
Objectives:
Given significant focus on improving survival for "high-risk" congenital diaphragmatic hernia, there is the potential to overlook the need to identify risk factors for suboptimal outcomes in "low-risk" congenital diaphragmatic hernia cases. We hypothesized that early cardiac dysfunction or severe pulmonary hypertension were predictors of adverse outcomes in this "low-risk" congenital diaphragmatic hernia population.
Design:
This is a retrospective cohort study using data from the Congenital Diaphragmatic Hernia Study Group registry. "Low-risk" congenital diaphragmatic hernia was defined as Congenital Diaphragmatic Hernia Study Group defect size A/B without structural cardiac and chromosomal anomalies. Examined risk factors included left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension on the first postnatal echocardiogram. The primary outcome was composite adverse events, defined as either death, extracorporeal membrane oxygenation utilization, oxygen requirement on day 30 of life, or hospitalization greater than or equal to 8 weeks. Multivariable adjustment was performed with logistic regression and inverse probability weighting.
Setting:
Neonatal index hospitalization for congenital diaphragmatic hernia.
Patients:
"Low-risk" congenital diaphragmatic hernia infants born between January 2015 and December 2018.
Interventions:
First postnatal echocardiogram performed within 24 hours from birth.
Measurements And Main Results:
Seven-hundred seventy-eight patients were identified as "low-risk" congenital diaphragmatic hernia. Left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension were present in 10.8%, 20.5%, and 57.5%, respectively. The primary outcome occurred in 21.3%. Death occurred in 3.0% and 9.1% used extracorporeal membrane oxygenation. On unadjusted analysis, all three risk factors were associated with the primary outcome. On all multivariable adjustment methods, left ventricular dysfunction and severe pulmonary hypertension remained significant predictors of adverse outcomes while right ventricular dysfunction no longer demonstrated any effect.
Conclusions:
Early left ventricular dysfunction and severe pulmonary hypertension are independent predictors of adverse outcomes among "low-risk" congenital diaphragmatic hernia infants. Early recognition may lead to interventions that can improve outcome in this at-risk cohort.
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