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Hypoplastic Left Heart Syndrome: Introductory Concepts

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Modelling Congenital Heart Disease

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Abstract

Hypoplastic Left Heart Syndrome (HLHS) includes a spectrum of congenital underdevelopment of the left-sided heart structures such that the systemic cardiac output relies on the function of the right ventricle and the patency of the arterial duct. HLHS is incompatible with life if untreated timely after birth. Early attempts to surgically treat HLHS were fated by high mortality until the pioneering work of William Norwood in the 80s, vastly changed the prognosis of these patients. The surgical approach has appreciably evolved since the first description: today’s HLHS treatment, by “bending” the physiology of these babies, allows them to survive after birth in the majority of the cases and reach adulthood in a considerable number of patients. Although the management of HLHS remains challenging, the outlook is steadily improving and this testifies that with ongoing research, these patients may stand a good chance to a fairly normal life.

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Author information

Authors and Affiliations

  1. Bambino Gesù Hospital, Rome, Italy

    Lorenzo Galletti

  2. Congenital Cardiac Unit, Papa Giovanni XXIII Hospital, Bergamo, Italy

    Nicola Uricchio

Authors
  1. Lorenzo Galletti
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  2. Nicola Uricchio
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Correspondence to Lorenzo Galletti .

Editor information

Editors and Affiliations

  1. Department of Pediatric Cardiology, Cardiac Surgery and Heart Lung Transplantation - ERN GUARD HEART, Bambin Gesù Hospital and Research Institute, Rome, Italy

    Gianfranco Butera

  2. UCL Institute of Cardiovascular Science and Great Ormond St Hospital for Children, London, UK

    Silvia Schievano

  3. University of Bristol, Bristol Medical School, Bristol, UK

    Giovanni Biglino

  4. Stanford University, Lucile Packard Children’s Hospital, Palo Alto, CA, USA

    Doff B. McElhinney

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Galletti, L., Uricchio, N. (2022). Hypoplastic Left Heart Syndrome: Introductory Concepts. In: Butera, G., Schievano, S., Biglino, G., McElhinney, D.B. (eds) Modelling Congenital Heart Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-88892-3_9

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  • DOI: https://doi.org/10.1007/978-3-030-88892-3_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-88891-6

  • Online ISBN: 978-3-030-88892-3

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