Hostname: page-component-54dcc4c588-nx7b4 Total loading time: 0 Render date: 2025-10-13T02:01:05.381Z Has data issue: false hasContentIssue false
  • English
  • Français

Risk stratification of patients with hypoplastic left heart syndrome and intact atrial septum using fetal MRI and echocardiography

Published online by Cambridge University Press:  14 May 2020

Ryan M. Serrano Open the ORCID record for Ryan M. Serrano [Opens in a new window] ,
Sabena Hussain ,
Brandon Brown ,
Eric S. Ebenroth  and
Anne Farrell
Ryan M. Serrano*
Affiliation:
Pediatric Cardiology, Riley Hospital for Children, Indianapolis, IN, USA
Sabena Hussain
Affiliation:
Pediatric Cardiology, Riley Hospital for Children, Indianapolis, IN, USA
Brandon Brown
Affiliation:
Department of Radiology and Imaging Sciences, Indiana University School of Medicine, Indianapolis, IN, USA
Eric S. Ebenroth
Affiliation:
Pediatric Cardiology, Riley Hospital for Children, Indianapolis, IN, USA
Anne Farrell
Affiliation:
Pediatric Cardiology, Riley Hospital for Children, Indianapolis, IN, USA
*
Address for Correspondence: Ryan M. Serrano MD, Pediatric Cardiology, 705 Riley Hospital Drive, RR 127, Indianapolis, IN46202, USA. Tel: +1 317 944-8906; Fax: +1 317 944-9330. E-mail: ryserran@iupui.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Despite prenatal diagnosis, prenatal intervention, and immediate postnatal intervention, patients with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have the highest risk for mortality. Charts for all infants diagnosed with hypoplastic left heart syndrome from 2009 to 2017 were retrospectively reviewed and compared, including pulmonary vein Doppler patterns on fetal echocardiogram and evidence of pulmonary lymphangiectasia on fetal MRI. Of the 81 newborns with hypoplastic left heart syndrome, we defined two groups. Group 1 patients had an adequate atrial septal communication (n = 69), while Group 2 met criteria for intact/restrictive septum (n = 12). No patient in Group 1 had a type C pulmonary vein Doppler pattern, while no patient in Group 2 had a type A pulmonary vein Doppler pattern. The two patients with pulmonary lymphangiectasia had type C pulmonary vein Doppler pattern and an intact atrial septum and did not survive. Survival to discharge for Group 1 was 83% compared to 58% for Group 2 (p = 0.116). Survival to stage 2 palliation was 71% for Group 1 compared to 50% for Group 2 (p = 0.186). Only 4 of the initial 12 patients from Group 2 are alive, which is an overall survival of 33%. Our experience supports previous evidence that fetal echocardiography can identify those patients with the greatest likelihood for postnatal intervention as well as those at highest risk for mortality. Fetal MRI is a novel imaging modality that may help providers separate patients at highest risk for mortality, regardless of pulmonary vein Doppler pattern.

Keywords

hypoplastic left heart syndromeintact atrial septumfetal MRIfetal echocardiographypulmonary lymphangiectasia

Information

Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 6 , June 2020 , pp. 790 - 798
Copyright
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

References

Morris, CD, Outcalt, J, Menashe, VD. Hypoplastic left heart syndrome: natural history in a geographically defined population. Pediatrics 1990; 85: 977–1000.Google Scholar
Sathanandam, SK, Philip, R, Gamboa, D, et al.Management of hypoplastic left heart syndrome with intact atrial septum: a two-center experience. Cardiol Young 2016; 26: 1072–1081.10.1017/S1047951115001791CrossRefGoogle Scholar
Hoque, T, Richmond, M, Vincent, JA, et al.Current outcomes of hypoplastic left heart syndrome with restrictive septum: a single center. Pediatr Cardiol 2013; 34: 1181–1189.CrossRefGoogle ScholarPubMed
Graziano, JN, Heidelberge, KP, Ensing, GJ, et al.The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome. Pediatr Cardiol 2002; 23: 146–151.CrossRefGoogle ScholarPubMed
Rychik, J, Rome, J, Collins, M, et al.The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 1999; 34: 554–560.CrossRefGoogle ScholarPubMed
Seed, M, Bradley, T, Bourgeois, J, et al.Antenatal MR imaging of pulmonary secondary to hypoplastic left heart. Pediatr Radiol 2009; 39: 747–749.CrossRefGoogle ScholarPubMed
Saul, D, Degenhardt, K, Iyoob, SD, et al.Left heart syndrome and the nutmeg lung in utero: a cause and effect relationship or prognostic indicator? Pediatr Radiol 2016; 46: 483–489.CrossRefGoogle ScholarPubMed
Better, DJ, Apfel, HD, Zidre, V, et al.Of blood flow in the left heart syndrome in the fetus. Heart 1999; 81: 646–649.CrossRefGoogle ScholarPubMed
Taketazu, M, Barrea, C, Smallhorn, J, et al.Intrauterine pulmonary venous flow and in fetal left heart syndrome. J Am Coll Cardiol 2004; 43: 1902–1907.CrossRefGoogle ScholarPubMed
Hill, K, Fudge, JK, Barker, P, et al.Novel transatrial septoplasty technique for neonates with left heart syndrome and an intact or highly restrictive atrial septum. Pediatr Cardiol 2010; 31: 545–549.CrossRefGoogle ScholarPubMed
Divanovic, A, Hor, K, Cnota, J, et al.Prediction and perinatal of severely restrictive atrial septum in fetuses with left heart obstruction: clinical experience using pulmonary venous Doppler analysis. J Thorac Cardiovasc Surg 2011; 141: 988–994.CrossRefGoogle ScholarPubMed
Javois, AJ, Van Bergen, AH, Cuneo, BF, et al.Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum. Catheter Cardiovasc Interv 2005; 66: 268–272.10.1002/ccd.20446CrossRefGoogle ScholarPubMed
Marshall, AC, Levine, J, Morash, D, et al.Results of in utero atrial in fetuses with hypoplastic left heart syndrome. Prenat Diagn 2008; 28: 1023–1028.CrossRefGoogle ScholarPubMed
Tanem, J, Hill, G, Rudd, R, et al. Survival after Norwood procedure in high risk patients. Abstract presented at the National AAP Conference 2018, Orlando, Florida.Google Scholar
Donofrio, MT, Moon-Grady, AJ, Hornberger, LK, et al.Diagnosis and treatment of fetal cardiac disease: a scientific statement from American Heart Association. Circulation 2014; 129: 2183–2242.CrossRefGoogle ScholarPubMed
Szwast, A, Tian, Z, McCann, M, et al.Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome. Circ Cardiovasc Imag 2010; 3: 172–178.CrossRefGoogle ScholarPubMed
Tworetzky, W, Wilkins-Haug, L, Jennings, RW, et al.Balloon dilation of severe aortic stenosis in the fetus potential for prevention of hypoplastic left heart syndrome candidate selection, technique, and results of successful intervention. Circulation 2004; 110: 2125–2131.CrossRefGoogle ScholarPubMed
Chaturvedi, RR, Ryan, G, Seed, M, et al.Fetal stenting of the atrial septum: technique and initial results in cardiac lesions with left atrial hypertension. Int J Cardiol 2013; 168: 2029–2036.10.1016/j.ijcard.2013.01.173CrossRefGoogle ScholarPubMed
Tweddell, JS, Hoffman, GM, Mussatto, KA, et al.Improved survival of patients undergoing palliation of Hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 2002; 106(Suppl 1): 82–89.Google ScholarPubMed
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: 2326–2330.CrossRefGoogle ScholarPubMed
Katz, JA, Tabbutt, S, Gaynor, W, et al.Hypoplastic left heart syndrome with atrial level restriction in the era of prenatal diagnosis. Ann Thorac Surg 2007; 84: 1633–1639.Google Scholar
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: 581–586.10.1016/j.athoracsur.2007.04.017CrossRefGoogle ScholarPubMed
Tanem, J, Hill, G, Rudd, R, et al. Survival after Norwood procedure in high risk patients. Abstract presented at the National AAP Conference 2018, Orlando, FloridaGoogle Scholar
Donofrio, MT, Moon-Grady, AJ, Hornberger, LKet al.Diagnosis and treatment of fetal cardiac disease: a scientific statement from American Heart Association. Circulation 2014; 129: 2183–2242.10.1161/01.cir.0000437597.44550.5dCrossRefGoogle ScholarPubMed
Szwast, A, Tian, Z, McCann, Met al.Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome. Circ Cardiovasc Imaging 2010; 3: 172–178.CrossRefGoogle ScholarPubMed
Venugopal, PS, Luna, KP, Anderson, DR, et al.Hybrid procedure as an alternative to surgical palliation of high-risk infants with hypoplastic left heart syndrome and its variants. J Thorac Cardiovasc Surg 2010; 139: 1211–1215CrossRefGoogle ScholarPubMed
Innovative management strategy for a fetus with hypoplastic left heart syndrome and intact atrial septum (2018). https://www.mayoclinic.org/medical-professionals/cardiovascular-diseases/news/innovative-management-strategy-for-a-fetus-with-hypoplastic-left-heart-syndrome-and-intact-atrial-septum/mac-20430219#Google Scholar