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    This article has been cited by the following publications. This list is generated based on data provided by CrossRef.

    Krupickova, Sylvia Muthurangu, Vivek Hughes, Marina Tann, Oliver Carr, Michelle Christov, Georgi Awat, Ram Taylor, Andrew and Marek, Jan 2016. Echocardiographic arterial measurements in complex congenital diseases before bidirectional Glenn: comparison with cardiovascular magnetic resonance imaging. European Heart Journal – Cardiovascular Imaging, p. jew069.


    Krupickova, Sylvia Quail, Michael A. Yates, Robert Gebauer, Roman Hughes, Marina and Marek, Jan 2016. The comparative role of echocardiography and MRI for identifying critical lesions in patients with single-ventricle physiology, before bidirectional cavopulmonary connection. Cardiology in the Young, p. 1.


    Ruotsalainen, Hanna Bellsham-Revell, Hannah Bell, Aaron Pihkala, Jaana Ojala, Tiina and Simpson, John 2016. Right ventricular systolic function in hypoplastic left heart syndrome: a comparison of velocity vector imaging and magnetic resonance imaging. European Heart Journal – Cardiovascular Imaging, Vol. 17, Issue. 6, p. 687.


    Bentham, James R. Baird, Christopher W. Porras, Deigo P. Rathod, Rahul H. and Marshall, Audrey C. 2015. A reinforced right-ventricle-to-pulmonary-artery conduit for the stage-1 Norwood procedure improves pulmonary artery growth. The Journal of Thoracic and Cardiovascular Surgery, Vol. 149, Issue. 6, p. 1502.


    Corsini, Chiara Biglino, Giovanni Schievano, Silvia Hsia, Tain-Yen Migliavacca, Francesco Pennati, Giancarlo and Taylor, Andrew M. 2014. The Effect of Modified Blalock-Taussig Shunt Size and Coarctation Severity on Coronary Perfusion After the Norwood Operation. The Annals of Thoracic Surgery, Vol. 98, Issue. 2, p. 648.


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Lessons from inter-stage cardiac magnetic resonance imaging in predicting survival for patients with hypoplastic left heart syndrome

  • Marina L. Hughes (a1), Victor T. Tsang (a2), Martin Kostolny (a2), Alessandro Giardini (a1), Vivek Muthurangu (a2), Andrew M. Taylor (a3) and Katherine Brown (a4)
  • DOI: http://dx.doi.org/10.1017/S104795111100059X
  • Published online: 31 May 2011
Abstract
Abstract

This study describes single-unit experience and explores risk stratification, with protocolised inter-stage cardiac magnetic resonance imaging for infants with hypoplastic left heart syndrome. Survival was retrospectively analysed among the cohort of locally followed survivors of Norwood Stage I procedure, who underwent magnetic resonance imaging under general anaesthesia from 2003 to 2008. This included 32 patients: 17 with Sano conduit and 15 with arterio-pulmonary shunt. The median (inter-quartile range) age and weight at scan were 3.1 (2.6–4.6) months and 5.0 (4.5–5.3) kilograms, respectively. Using morphologic definitions, the median coarctation index was 0.71 (0.57–0.83). The degree of proximal right and left pulmonary artery narrowing was 25% (14–44%) and 25% (11–50%), respectively. The median right ventricular ejection fraction was 54% (48–59%). The ejection fraction was not related to the coarctation index or to pulmonary artery narrowing. Patients were followed up for a median of 19.2 (10.8–46.0) months, during which 13 (41%) had an intervention in addition to routine Norwood Stage II surgery and seven died. Risk of death was related to reduced right ventricular ejection fraction, with a hazard ratio of 0.91 (95% confidence interval 0.85–0.98, p = 0.02), and the cumulative number of focal stenoses of neo-aortic arch and pulmonary arteries (hazard ratio 2.71, 95% confidence interval 1.14–6.44, p = 0.02). Conclusions: In addition to comprehensive three-dimensional morphologic imaging, inter-stage cardiac magnetic resonance imaging provides a ventricular functional index that may predict outcome in patients with hypoplastic left heart syndrome. Measures to preserve right ventricular systolic function and relieve stenoses are paramount within the complex management strategies for these patients.

Copyright
Corresponding author
Correspondence to: M. L. Hughes, DPhil, MRCP, FRACP, Centre for Cardiovascular Imaging, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, United Kingdom. Tel: +44 207 407 9200, ext. 7857; Fax: +44 2078138262; E-mail: hughem@gosh.nhs.uk
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1.CL Cua , RR Thiagarajan , R Taeed , Improved inter-stage mortality with modified Norwood procedure: a meta-analysis. Ann Thorac Surg 2005; 80: 4449.

2.RG Ohye , JW Gaynor , NS Ghanayem , Design and rationale of a randomized trial comparing the Blalock–Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure. J Thorac Cardiovasc Surg 2008; 136: 968975.

4.RG Ohye , LA Sleeper , L Mahony , Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 19801992.

5.V Muthurangu , AM Taylor , SR Hegde , Cardiac magnetic resonance imaging after Stage 1 Norwood operation for hypoplastic left heart syndrome. Circulation 2005; 112: 32563263.

6.DW Brown , K Gauvreau , AJ Powell , Cardiac magnetic resonance versus routine cardiac catheterization before bidirectional Glenn anastomosis in infants with a functional single ventricle: a prospective randomized trial. Circulation 2007; 116: 27182725.

7.JA Noonan , AS Nadas . The hypoplastic left heart syndrome: an analysis of 101 cases. Pediatr Clin North Am 1958; 5: 10261056.

9.MA Fogel , MT Donofrio , C Ramaciotti , Magnetic resonance and echocardiographic imaging of pulmonary artery size throughout stages of Fontan reconstruction. Circulation 1994; 90: 29272936.

10.MS Lemler , TM Zellers , KA Harris , Coarctation index: identification of recurrent coarctation in infants with hypoplastic left heart syndrome after the Norwood procedure. Am J Cardiol 2000; 86: 697699.

11.S Sano , K Ishino , M Kawada , Right ventricle-pulmonary artery shunt in first stage palliation of hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 7: 2231.

12.S Denslow , HB Wiles , LF McKellar , Right ventricular volume estimation using an ellipsoidal shell model and single-plane magnetic resonance imaging. Invest Radiol 1996; 31: 1725.

13.MM Winter , FJP Bernink , M Groenink , Evaluating the systemic right ventricle by CMR: the importance of consistent and reproducible delineation of the cavity. J Cardiovasc Magn Reson 2008; 10: 4048.

14.LA Larrazabal , ESS Tierney , DW Brown , Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome. Ann Thorac Surg 2008; 86: 869874.

15.M Chessa , A Dindar , JJ Vettukattil , Balloon angioplasty in infants with aortic obstruction after the modified stage I Norwood procedure. Am Heart J 2000; 140: 227231.

16.DA Hehir , TE Dominguez , JA Ballweg , Risk factors for inter-stage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants. J Thorac Cardiovasc Surg 2008; 136: 9499.

17.JC Nielson , AJ Powell , K Gauvreau , Magnetic resonance imaging predictors of coarctation severity. Circulation 2005; 111: 622628.

18.RH Mohiaddin , PJ Kilner , S Rees , Magnetic resonance volume flow and jet velocity mapping in aortic coarctation. J Am Coll Cardiol 1993; 22: 15151521.

19.IA Simpson , KJ Chung , RF Glass , Cine magnetic resonance imaging for evaluation of anatomy and flow relations in infants and children with coarctation of the aorta. Circulation 1988; 78: 142148.

20.MA Walsh , BW McCrindle , A Dipchand , Left ventricular morphology influences mortality following the Norwood operation. Heart 2009; 95: 12381244.

21.JW Gaynor , WT Mahle , MI Cohen , Risk factors for mortality after the Norwood procedure. Eur J Cardiothoracic Surg 2002; 22: 8289.

22.K Altmann , BF Printz , DE Solowiejczyk , Two-dimensional echocardiographic assessment of right ventricular function as a predictor of outcome in hypoplastic left heart syndrome. Am J Cardiol 2000; 86: 964968.

23.DS Lim , BB Peeler , GP Matherne , Cardiovascular magnetic resonance of pulmonary artery growth and ventricular function after Norwood procedure with Sano modification. J Cardiovasc Magn Reson 2008; 10: 3441.

24.NS Ghanayem , GM Hoffman , KA Mussatto , Home surveillance program prevents inter-stage mortality after the Norwood procedure. J Thorac Cardiovasc Surg 2003; 126: 13671377.

25.EA Bacha , LA Larrazabal , FA Pigula , Measurement of technical performance in surgery for congenital heart disease: the stage I Norwood procedure. J Thorac Cardiovasc Surg 2008; 136: 993997.

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Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
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