Noonan’s Syndrome Associated with Hypoplastic Left Heart

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Abstract

A case of a week old female baby, admitted because of apathy, hypothermia, dyspnea, jaundice and cyanosis is described. She had the characteristic phenotype of Turner’s syndrome with normal karyotype. Signs of severe heart failure were present. Therapy with diuretics, digoxin, dopamine and mechanical ventilation were unsuccessful, and the patient died several hours after her admission. The anatomopathological examination revealed the presence of hypoplastic left heart syndrome with mitral atresia and aortic atresia, atrial septal defect, double outlet right ventricle, and a patent ductus arteriosus.

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Journal

Journal ID (publisher-id): CRD

Journal ID (nlm-ta): Cardiology

Journal ID (doi): 10.1159/issn.0008-6312

Title: Cardiology

Publisher: S. Karger AG

ISSN (Print): 0008-6312

ISSN (Electronic): 1421-9751

Publication date Subscription-year: 1990

Publication date (Print): 1990

Publication date (Electronic): 12 November 2008

Volume: 77

Issue: 1

Pages: 62-65

Affiliations

^aDepartment of Cardiology and ^bPediatric Emergency Room, Central Emek Hospital, Afula, Israel

Article

Publisher ID: 174582 Other ID: Cardiology 1990;77:62–65

DOI: 10.1159/000174582

PubMed ID: 2354479

SO-VID: 8d20b4c8-f369-4599-9660-bae16c28df2d

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Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

History

Date received : 13 June 1989

Date accepted : 10 October 1989

Page count

Pages: 4

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