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Hypoplastic left heart syndrome with intact atrial septum

Attempt of an interventional palliation by ductal and interatrial stent implantation

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Summary

In patients with hypoplastic left heart syndrome (HLHS) and intact atrial septum, the blood entering the left atrium cannot egress. Emergency treatment interventionally or surgically is mandatory immediately after birth. We describe a patient with HLHS and intact atrial septum who underwent successful transvenous atrial septostomy immediately after birth. When the interatrial communication became restrictive, stent implantation into the arterial duct and into the atrial septum was performed on the 7th day of life. Despite good hemodymanic response, the lung damage was severe and persistent, rendering staged surgical correction impossible. The child died on the 23rd day of life. Autopsy showed patent and correct placed stents in the duct and the atrial septum. There was severe dilatation of pulmonary lymphatic and venous vessels, suggestive of long–standing pulmonary venous hypertension. In conclusion, this form of HLHS has a poor prognosis despite early and aggressive interventional treatment.

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Authors and Affiliations

  1. Klinik für Kinderkardiologie, Herzzentrum Leipzig GmbH, Universität Leipzig, Strümpellstr. 39, 04289, Leipzig, Germany

    Michael Weidenbach MD & Ingo Daehnert MD

  2. Department of Pathology, University of Leipzig, Liebigstr. 26, 04103, Leipzig, Germany

    Paul Caffier MD

  3. Department of Neuropathology, University of Leipzig, Liebigstr. 26, 04103, Leipzig, Germany

    Taja Harnisch MD

Authors
  1. Michael Weidenbach MD
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  2. Paul Caffier MD
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  3. Taja Harnisch MD
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  4. Ingo Daehnert MD
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Correspondence to Michael Weidenbach MD.

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Weidenbach, M., Caffier, P., Harnisch, T. et al. Hypoplastic left heart syndrome with intact atrial septum. Clin Res Cardiol 95, 110–114 (2006). https://doi.org/10.1007/s00392-006-0327-5

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  • DOI: https://doi.org/10.1007/s00392-006-0327-5

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