Prenatally diagnosed foramen ovale restriction in fetuses with hypoplastic left heart syndrome may be a predictor of longer hospitalization, but not of a need for an urgent rashkind procedure
Abstract
Objectives: This prospective study aimed to assess the effectiveness of foramen ovale examination in classifying prenatal hypoplastic left heart syndrome (HLHS) in accordance with the new classification groupings for congenital heart defects.
Material and methods: The analysis included 145 fetuses with HLHS, diagnosed and monitored between 2008 and 2015 in Prenatal Cardiology Department at Polish Mother’s Memorial Hospital Research Institute in Lodz. The main criteria for classifying our study population into three sub-groups was was the presence of a foramen ovale restriction, which we diagnosed by evaluating the diameter and blood flow through the foramen ovale. Of the total group, 73.8% (n = 107) were classified as severe planned, 24.1% (n = 35) as severe urgent, and 2.1% (n = 3) as the severest group.
Results: Comparing the severe planned and the severe urgent HLHS groups showed: gestational age of delivery 38 vs 38 weeks respectively (p = 0.45); cesarean delivery 62% vs 79.2% (p = 0.15); neonatal birth weight 3110 g vs 2985 g (p = 0.2); Apgar score 9 vs 9 points; survival rate 65.8% vs 61.9% (p = 0.8); and hospitalization 38 vs 46.5 days (p = 0.059). Prenatal qualification for the group of severe urgent HLHS was characterized by 100% sensitivity, 80.6% specificity and a low posi- tive predictive value of 9.5%.
Conclusions: 1. Prenatal qualification into the group of severe urgent CHD based on the features of foramen ovale was characterized by high sensitivity, a satisfying specificity and a low positive predictive value. 2. Prenatally diagnosed foramen ovale restriction may be a predictor of longer hospitalization, but not of a need for an urgent Rashkind procedure. 3. New classifications of CHDs allowed clinicians to determine prognoses and to plan optimal multi-specialized care which resulted in similar outcomes between the severe planned and severe urgent HLHS groups.
Keywords: heart defectscongenitalhypoplastic left heart syndromeechocardiographyfetusprenatal diagnosis
References
- Kordjalik P, Respondek-Liberska MH. on the basis of a Nationwide Registry of Fetal Cardiac Pathology www.orpkp.pl. Prenat Cardio. 2013 Sep. ; 3(3): 18–21.
- Słodki M, Respondek-Liberska M. Proposal of screening fetal heart examination form granted by Polish Ministry of Health Program Kardio-Prenatal 2008. Ginekol Pol. 2009; 80: 466–470.
- Sanapo L, Pruetz JD, Słodki M, et al. Fetal echocardiography for planning perinatal and delivery room care of neonates with congenital heart disease. Echocardiography. 2017; 34(12): 1804–1821.
- Słodki M, Respondek-Liberska M, Pruetz JD, et al. Fetal cardiology: changing the definition of critical heart disease in the newborn. J Perinatol. 2016; 36(8): 575–580.
- Respondek-Liberska M. ATLAS of prenatal heart defects- Selected examples and their clinical significance. ADI Publishers. (PL): 2011.
- Słodki M, Respondek-Liberska M. New classifications of prenatally diagnosed congenital heart defects and their influence of neonatal survivability. . Prenat Cardio. 2015 Sep.; 5(3): 6–8.
- Hypoplastic left heart syndrome. Comprehensive Surgical Management of Congenital Heart Disease. 2012.
- Słodki M. Developing a model of care for pregnant women with congenital heart disease in the fetus on the basis of a new division for prenatal heart defects. Habilitation thesis. Medical University Lodz, PWSZ Plock. 2012.
- Pruetz JD, Carroll C, Trento LU, et al. Outcomes of critical congenital heart disease requiring emergent neonatal cardiac intervention. Prenat Diagn. 2014; 34(12): 1127–1132.
- Vida VL, Bacha EA, Larrazabal A, et al. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center. Ann Thorac Surg. 2007; 84(2): 581–5; discussion 586.
- Donofrio MT, Levy RJ, Schuette JJ, et al. Specialized delivery room planning for fetuses with critical congenital heart disease. Am J Cardiol. 2013; 111(5): 737–747.
- Glatz JA, Tabbutt S, Gaynor JW, et al. Hypoplastic left heart syndrome with atrial level restriction in the era of prenatal diagnosis. Ann Thorac Surg. 2007; 84(5): 1633–1638.
- Rychik J, Rome J, Collins M, et al. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. Journal of the American College of Cardiology. 1999; 34(2): 554–560.
- Vlahos AP, Lock JE, McElhinney DB, et al. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation. 2004; 109(19): 2326–2330.
- Jacobs M, Blackstone E, Bailey L. Intermediate survival in neonates with aortic atresia: A multi-institutional study. The Journal of Thoracic and Cardiovascular Surgery. 1998; 116(3): 417–431.
- Moon-Grady AJ, Morris SA, Belfort M, et al. International Fetal Cardiac Intervention Registry. International Fetal Cardiac Intervention Registry: A Worldwide Collaborative Description and Preliminary Outcomes. J Am Coll Cardiol. 2015; 66(4): 388–399.
- Jantzen DW, Moon-Grady AJ, Morris SA, et al. Hypoplastic Left Heart Syndrome With Intact or Restrictive Atrial Septum: A Report From the International Fetal Cardiac Intervention Registry. Circulation. 2017; 136(14): 1346–1349.
- Marshall AC, Levine J, Morash D, et al. Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome. Prenat Diagn. 2008; 28(11): 1023–1028.
- Słodki M, Janiak K, Szaflik K, et al. Fetal echocardiography before and after prenatal aspiration of a fetal ovarian cyst. Ginekol Pol. 2009; 80: 629–631.
- Słodki M, Janiak K, Szaflik K, et al. Fetal echocardiography in fetal ovarian cysts. Ginekol Pol. 2008; 79: 347–351.
- Michelfelder E, Gomez C, Border W, et al. 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.
- Divanović A, Hor K, Cnota J, et al. 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(4): 988–994.
- Taketazu M, Barrea C, Smallhorn JF, et al. Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome. J Am Coll Cardiol. 2004; 43(10): 1902–1907.
- 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.
- Enzensberger C, Axt-Fliedner R, Degenhardt J, et al. Pulmonary Vasoreactivity to Materno-Fetal Hyperoxygenation Testing in Fetuses with Hypoplastic Left Heart. Ultraschall Med. 2016; 37(2): 195–200.
- Kohl T. Chronic intermittent materno-fetal hyperoxygenation in late gestation may improve on hypoplastic cardiovascular structures associated with cardiac malformations in human fetuses. Pediatr Cardiol. 2010; 31(2): 250–263.
- Schidlow DN, Donofrio MT. Prenatal Maternal Hyperoxygenation Testing and Implications for Critical Care Delivery Planning among Fetuses with Congenital Heart Disease: Early Experience. Am J Perinatol. 2018; 35(1): 16–23.
