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Foetal therapy: what works? Closed interatrial septum

Published online by Cambridge University Press:  27 August 2014

Ulrike Herberg ,
Christoph Berg ,
Annegret Geipel ,
Ulrich Gembruch  and
Johannes Breuer
Ulrike Herberg*
Affiliation:
Department of Pediatric Cardiology, University of Bonn, Bonn, Germany
Christoph Berg
Affiliation:
Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn, Germany Department of Obstetrics and Prenatal Medicine, University of Cologne, Bonn, Germany
Annegret Geipel
Affiliation:
Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn, Germany
Ulrich Gembruch
Affiliation:
Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn, Germany
Johannes Breuer
Affiliation:
Department of Pediatric Cardiology, University of Bonn, Bonn, Germany
*
Correspondence to: Dr U. Herberg, Department of Pediatric Cardiology, University of Bonn, Adenauerallee 119, 53113 Bonn, Germany. Tel: +49 228 287 33350; Fax: +49 228 287 33360; E-mail: Ulrike.Herberg@ukb.uni-bonn.de
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Abstract

Hypoplastic left-heart syndrome and critical aortic stenosis with severely restricted or intact foramen ovale are associated with high neonatal mortality and poor long-term outcome. Despite accurate foetal diagnosis and successful postnatal catheter-based and surgical intervention, the 1-month survival rate is about 33%. Changes in pulmonary vascular architecture resulting in pulmonary hypertension result in important long-term morbidity. Prenatal relief of left atrial and pulmonary hypertension may promote normal pulmonary vascular and parenchymal development and improve short- and long-term outcomes. Foetal atrial balloon septostomy, laser perforation, and stenting of the foetal interatrial septum are the current options for foetal therapy. This paper provides an overview of foetal diagnosis, selection of patients for foetal intervention, and interventional techniques, and also reviews the current status of foetal and postnatal outcomes after intrauterine intervention.

Keywords

Hypoplastic left-heart syndromecritical aortic stenosisrestrictive foramen ovalefoetal atrial septostomyfoetal atrial stent placement
Type
Original Article
Information
Cardiology in the Young , Volume 24 , Issue S2: Fetal cardiovascular medicine: current perspectives and future hopes , October 2014 , pp. 47 - 54
Copyright
© Cambridge University Press 2014 

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References

1.Rychik, J, Rome, JJ, Collins, MH, DeCampli, WM, Spray, TL. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 1999; 342: 554–560.Google Scholar
2.Vlahos, AP, Lock, JE, McElhinney, DB, van der Velde, ME. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation 2004; 109: 2326–2330.Google Scholar
3.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: 1633–1638.Google Scholar
4.Atz, AM, Feinstein, JA, Jonas, RA, Perry, SB, Wessel, DL. Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect. Am J Cardiol 1999; 83: 1224–1228.Google Scholar
5.Lowenthal, A, Kipps, AK, Brook, MM, Meadows, J, Azakie, A, Moon-Grady, AJ. Prenatal diagnosis of atrial restriction in hypoplastic left heart syndrome is associated with decreased 2-year survival. Prenat Diagn 2012; 32: 485–490.CrossRefGoogle ScholarPubMed
6.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: 581–585.Google Scholar
7.Jaeggi, E, Ryan, G, Seed, M, Chaturvedi, R. Hypoplastic left heart syndrome with restrictive atrial septum: echocardiographic criteria for the need of in-utero catheter intervention and first results of human fetal atrial stenting. Cardiol Young 2012; 22: S23.Google Scholar
8.Taketazu, M, Barrea, C, Smallhorn, JF, Wilson, GJ, Hornberger, LK. Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome. J Am Coll Cardiol 2004; 43: 1902–1907.Google Scholar
9.Graziano, JN, Heidelberger, KP, Ensing, GJ, Gomez, CA, Ludomirsky, A. 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.Google Scholar
10.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: 1023–1028.Google Scholar
11.Chintala, K, Tian, Z, Du, W, Donaghue, D, Rychik, J. Fetal pulmonary venous Doppler patterns in hypoplastic left heart syndrome: relationship to atrial septal restriction. Heart 2008; 94: 1446–1449.Google Scholar
12.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: 988–994.Google Scholar
13.Quintero, RA, Huhta, J, Suh, E, Chmait, R, Romero, R, Angel, J. In utero cardiac fetal surgery: laser atrial septotomy in the treatment of hypoplastic left heart syndrome with intact atrial septum. Am J Obstet Gynecol 2005; 193: 1424–1428.Google Scholar
14.Kohl, T, Müller, A, Tchatcheva, K, Achenbach, S, Gembruch, U. Fetal transesophageal echocardiography: clinical introduction as a monitoring tool during cardiac intervention in a human fetus. Ultrasound Obstet Gynecol 2005; 26: 780–785.CrossRefGoogle Scholar
15.Schmidt, M, Jaeggi, E, Ryan, G, et al. Percutaneous ultrasound-guided stenting of the atrial septum in fetal sheep. Ultrasound Obstet Gynecol 2008; 32: 923–928.Google Scholar
16.Herberg, U. Fetal therapy. What works? Closed interatrial septum. Oral presentation. Update fetal cardiology course. 46. Meeting of the Association for European Pediatric and Congenital Cardiology, Istanbul, 23–26 May 2012.Google Scholar
17.Vogel, M, McElhinney, DB, Wilkins-Haug, LE, et al. Aortic stenosis and severe mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary experience with pre-natal cardiac intervention. J Am Coll Cardiol 2011; 57: 348–355.Google Scholar
18.Tulzer, G. Fetal therapy. What works? Aortic stenosis. Oral presentation. Update fetal cardiology course. 46. Meeting of the Association for European Pediatric and Congenital Cardiology, Istanbul, 23–26 May 2012.Google Scholar
19.Arzt, W, Wertaschnigg, D, Veit, I, Klement, F, Gitter, R, Tulzer, G. Intrauterine aortic valvuloplasty in fetuses with critical aortic stenosis: experience and results of 24 procedures. Ultrasound Obstet Gynecol 2011; 37: 689–695.CrossRefGoogle ScholarPubMed
20.Gembruch, U, Geipel, A, Herberg, U, Berg, C. Fetal cardiac interventions. Z Geburtshilfe Neonatol 2012; 216: 162–172.Google ScholarPubMed
21.Rogers, LS, Peterson, AL, Gaynor, JW, Rome, JJ, Weinberg, PM, Rychik, J. Mitral valve dysplasia syndrome: a unique form of left-sided heart disease. J Thorac Cardiovasc Surg 2011; 142: 1381–1387.Google Scholar
22.McElhinney, DB, Tworetzky, W, Lock, JE. Current status of fetal cardiac intervention. Circulation 2010; 121: 1256–1263.Google Scholar
23.Tchatcheva, K, Herberg, U, Gembruch, U, Geipel, A, Lachmann, R, Berg, C. Premature obstruction of the foramen ovale (POFO) – a single centre experience. OC02.04. Presentation on the 22nd World Congress on Ultrasound in Obstetrics and Gynecology. Copenhagen, September 9–12, 2012; Ultraqsound Obst Gyn 40: 3.Google Scholar