Utilizing Fetal Echocardiography to Risk Stratify and Predict Neonatal Outcomes in Fetuses Diagnosed with Congenital Heart Disease

Abstract

Objective Risk stratification of fetuses diagnosed with congenital heart disease (CHD) helps provide a delivery plan and prepare families and medical teams on expected course in the delivery room. Our aim was to assess the accuracy of echocardiographically determined risk-stratification assignments in predicting postnatal cardiac outcomes beyond the delivery room.

Study Design This was a retrospective study at a single center evaluating all fetuses with CHD who were risk-stratified by echocardiographically determined level of care (LOC) assignment, ranging from 1a (lowest risk) to 4 (highest risk). All data were collected from January 1, 2017, to November 1, 2021. Outcomes included any unexpected cardiac interventions and neonatal clinical outcomes including in-hospital mortality, the need for prostaglandins or inotropes, and defined critical illness. These outcomes were assessed for each LOC assignment by Fisher's exact test.

Results Out of 817 patients assigned a LOC, a total of 747 fetuses were included in our final cohort with a separate subanalysis of 70 fetuses diagnosed with coarctation of the aorta. The sensitivity and specificity were high for all LOC levels in predicting delivery room needs (93–100%). Higher LOC levels (3–4) had a lower positive predictive value (66–67%) indicating a high false-positive rate. Subjects with higher LOC assignments had a greater frequency of critical illness, hospital mortality, need for inotropes, need for neonatal surgical or catheterization interventions, and need for prostaglandins (p < 0.001 for all outcomes). A post-hoc analysis reviewing LOC assignments revealed a greater tendency to over-assign LOC at higher assignments (19% for LOC 3 and 4) compared to lower assignments (4% for LOC 1 and 2).

Conclusion Risk stratification based on fetal echocardiography can predict neonatal clinical outcomes and acuity of postnatal management needs. However, there is greater variability in expected clinical events and an expected degree of false positives for those with higher LOC assignments.

Key Points

• Risk stratification utilizing fetal echocardiography can be used to predict neonatal needs.

• Complex heart disease has lower positive predictive value in predicting postnatal clinical needs.

• There is a tendency to over-assign risk of acute hemodynamic instability for complex heart disease.

• False positives are expected when planning high-risk deliveries to avoid compromising situations.

Keywords

congenital heart disease - fetal - prenatal - echocardiography

Publication History

Received: 29 February 2024

Accepted: 09 July 2024

Article published online:
29 July 2024

• References

• 1 Donofrio MT, Skurow-Todd K, Berger JT. et al. Risk-stratified postnatal care of newborns with congenital heart disease determined by fetal echocardiography. J Am Soc Echocardiogr 2015; 28 (11) 1339-1349
• 2 Berkley EM, Goens MB, Karr S, Rappaport V. Utility of fetal echocardiography in postnatal management of infants with prenatally diagnosed congenital heart disease. Prenat Diagn 2009; 29 (07) 654-658
• 3 Donofrio MT, Levy RJ, Schuette JJ. et al. Specialized delivery room planning for fetuses with critical congenital heart disease. Am J Cardiol 2013; 111 (05) 737-747
• 4 Donofrio MT, Moon-Grady AJ, Hornberger LK. et al; American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation 2014; 129 (21) 2183-2242
• 5 Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation 2001; 103 (09) 1269-1273
• 6 Bonnet D, Coltri A, Butera G. et al. Detection of transposition of the great arteries in fetuses reduces neonatal morbidity and mortality. Circulation 1999; 99 (07) 916-918
• 7 Jaeggi ET, Sholler GF, Jones OD, Cooper SG. Comparative analysis of pattern, management and outcome of pre- versus postnatally diagnosed major congenital heart disease: a population-based study. Ultrasound Obstet Gynecol 2001; 17 (05) 380-385
• 8 Rychik J, Ayres N, Cuneo B. et al. American Society of Echocardiography guidelines and standards for performance of the fetal echocardiogram. J Am Soc Echocardiogr 2004; 17 (07) 803-810
• 9 American Institute of Ultrasound in Medicine. AIUM practice guideline for the performance of fetal echocardiography. J Ultrasound Med 2013; 32 (06) 1067-1082
• 10 Michelfelder E, Gomez C, Border W, Gottliebson W, Franklin C. Predictive value of fetal pulmonary venous flow patterns in identifying the need for atrial septoplasty in the newborn with hypoplastic left ventricle. Circulation 2005; 112 (19) 2974-2979
• 11 Divanović A, Hor K, Cnota J, Hirsch R, Kinsel-Ziter M, Michelfelder E. Prediction and perinatal management of severely restrictive atrial septum in fetuses with critical left heart obstruction: clinical experience using pulmonary venous Doppler analysis. J Thorac Cardiovasc Surg 2011; 141 (04) 988-994
• 12 Maskatia SA, Kwiatkowski D, Bhombal S. et al. A fetal risk stratification pathway for neonatal aortic coarctation reduced medical exposure. J Pediatr 2021; 237: 102-108.e3
• 13 Gellis L, Drogosz M, Lu M. et al. Echocardiographic predictors of neonatal illness severity in fetuses with critical left heart obstruction with intact or restrictive atrial septum. Prenat Diagn 2018; 38 (10) 788-794
• 14 Donofrio MT. Predicting the future: delivery room planning of congenital heart disease diagnosed by fetal echocardiography. Am J Perinatol 2018; 35 (06) 549-552
• 15 Caughey AB, Cahill AG, Guise JM, Rouse DJ. American College of Obstetricians and Gynecologists (College), Society for Maternal-Fetal Medicine. Safe prevention of the primary cesarean delivery. Am J Obstet Gynecol 2014; 210 (03) 179-193
• 16 Buca D, Winberg P, Rizzo G. et al. Prenatal risk factors for urgent atrial septostomy at birth in fetuses with transposition of the great arteries: a systematic review and meta-analysis. J Matern Fetal Neonatal Med 2022; 35 (03) 598-606
• 17 Masci M, Pasquini L, Alsaied T. et al. Reliability of fetal echocardiography in predicting postnatal critical hypoxia in patients with transposition of great arteries and intact ventricular septum. Pediatr Cardiol 2021; 42 (07) 1575-1584
• 18 Punn R, Silverman NH. Fetal predictors of urgent balloon atrial septostomy in neonates with complete transposition. J Am Soc Echocardiogr 2011; 24 (04) 425-430
• 19 Peyvandi S, Nguyen TATT, Almeida-Jones M. et al; University of California Fetal Consortium (UCfC). Timing and mode of delivery in prenatally diagnosed congenital heart disease- an analysis of practices within the University of California Fetal Consortium (UCFC). Pediatr Cardiol 2017; 38 (03) 588-595
• 20 Afshar Y, Hogan WJ, Conturie C. et al. Multi-institutional practice-patterns in fetal congenital heart disease following implementation of a standardized clinical assessment and management plan. J Am Heart Assoc 2021; 10 (15) e021598
• 21 Tegnander E, Williams W, Johansen OJ, Blaas HG, Eik-Nes SH. Prenatal detection of heart defects in a non-selected population of 30,149 fetuses–detection rates and outcome. Ultrasound Obstet Gynecol 2006; 27 (03) 252-265
• 22 Hornberger LK, Sahn DJ, Kleinman CS, Copel J, Silverman NH. Antenatal diagnosis of coarctation of the aorta: a multicenter experience. J Am Coll Cardiol 1994; 23 (02) 417-423