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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.


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:
  • Published online: 31 May 2011

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.

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:
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Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
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