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Predicting High-Risk Fetal Cardiac Disease Anticipated to Need Immediate Postnatal Stabilization and Intervention
Amol Moray1, Proscovia M Mugaba1, Chloe Joynt2
1Fetal & Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics University of Alberta, Stollery Children's Hospital Edmonton Alberta Canada.
Insights
Fetal echocardiography accurately identifies fetuses needing immediate cardiac intervention, improving care for critical congenital heart disease. This approach enhances surgical planning and outcomes for newborns with conditions like d-transposition of the great arteries/intact ventricular septum and hypoplastic left heart syndrome.
Area of Science:
- Pediatric Cardiology
- Fetal Medicine
- Neonatal Surgery
Background:
- Care for fetuses with cardiac disease at risk of cardiorespiratory instability is challenging due to delivery-to-cardiac-service distances.
- A fetal echocardiography-based algorithm was implemented in 2013 for high-risk neonates requiring delivery in a pediatric cardiac operating room.
- This study examines the experience with this algorithm for managing high-risk congenital heart disease.
Approach:
- Maternal and postnatal records of fetuses with cardiac disease from January 2013 to March 2022 were reviewed.
- Focus included fetuses with d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS) and hypoplastic left heart syndrome (HLHS).
- Analysis assessed the predictive value of fetal echocardiography for urgent intervention or mortality.
Key Points:
- Forty high-risk fetuses were identified, including those with d-TGA/IVS and HLHS with restrictive atrial septum.
- Fetal echocardiography predicted intervention/extracorporeal membrane oxygenation/death within 2 hours with 50% positive predictive value for all high-risk cases.
- For d-TGA/IVS, fetal echocardiography showed 67% sensitivity and 80% positive predictive value for intervention within 2 hours; for HLHS, it was 100% sensitive with 71% positive predictive value.
Conclusions:
- Fetal echocardiography effectively predicts the need for urgent intervention in most d-TGA/IVS and HLHS cases.
- The algorithm successfully identified fetuses requiring immediate postnatal cardiac intervention.
- This approach aids in planning delivery and surgical management for high-risk congenital heart disease.
Background:
Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience.
Methods And Results:
We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS) and hypoplastic left heart syndrome (HLHS) encountered over the study period. Forty fetuses were considered high risk for acute cardiorespiratory instability: 15 with d-TGA/IVS and 7 with HLHS with restrictive atrial septum, 4 with absent pulmonary valve syndrome, 3 with obstructed anomalous pulmonary veins, 2 with severe Ebstein anomaly, 2 with thoracic/intracardiac tumors, and 7 others. Pediatric cardiac operating room delivery occurred for 33 but not for 7 (5 with d-TGA/IVS, 2 with HLHS with restrictive atrial septum). For high-risk cases, fetal echocardiography had a positive predictive value of 50% for intervention/extracorporeal membrane oxygenation/death at ≤2 hours and 70% at ≤24 hours. Of "low-risk" cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. Fetal echocardiography for predicting intervention/extracorporeal membrane oxygenation/death at ≤2 hours had a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 80% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively.
Conclusions:
Fetal echocardiography can predict the need for urgent intervention in a majority with d-TGA/IVS and HLHS and in half of the entire spectrum of high-risk cardiac disease.
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