open access

Vol 92, No 4 (2021)
Research paper
Published online: 2021-01-26
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Hypoplastic left heart syndrome: from the prenatal to the postnatal period

Anna Wojtowicz1, Marek Raczka1, Zbigniew Kordon1, Agnieszka Ochoda-Mazur1, Krzysztof Janowiec1, Hubert Huras1, Piotr Werynski1, Agata Wloch2
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Pubmed: 33751510
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Ginekol Pol 2021;92(4):289-299.
Affiliations
  1. Collegium Medicum, Jagiellonian University, Cracow, Poland
  2. Department of Obstetrics and Gynecology in Ruda Slaska, Medical University of Silesia, Ruda Slaska, Poland, Poland

open access

Vol 92, No 4 (2021)
ORIGINAL PAPERS Obstetrics
Published online: 2021-01-26

Abstract

Objectives: To analyse a population of foetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS). Material and methods: Retrospective study of foetuses diagnosed with HLHS between 2013 and 2017 in a referral centre. Results: HLHS was found in 9.7% (65/665) of foetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, HLHS was present in 40% of cases; in 24.5% other CVA were detected; in 14%, CVA and extracardiac anomalies; and in 21.5% only extracardiac malformations. Genetic disorders were present in 18.4% (12/65) of foetuses. 42% of cardiovascular and 25% of extracardiac anomalies were diagnosed postnatally. There were 10 (15.4%) elective terminations, 1 (1.5%) spontaneous foetal demise. Two newborns died after birth before surgery. Of the 52 children who underwent Norwood surgery, 13 (25%) died (9 with additional anomalies, and 4 with isolated HLHS). Of the 38 children who underwent stage II surgery, 2 (5.2%) with isolated HLHS died, and 1 (2.6%) with CVA. Conclusions: A diagnosis of HLHS is an indication for a detailed examination of cardiac and noncardiac structures. It is advisable to consider genetic testing, together with the microarray assessment. The prognosis depends on underlying cardiac and extracardiac anomalies and coexisting genetic defects.

Abstract

Objectives: To analyse a population of foetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS). Material and methods: Retrospective study of foetuses diagnosed with HLHS between 2013 and 2017 in a referral centre. Results: HLHS was found in 9.7% (65/665) of foetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, HLHS was present in 40% of cases; in 24.5% other CVA were detected; in 14%, CVA and extracardiac anomalies; and in 21.5% only extracardiac malformations. Genetic disorders were present in 18.4% (12/65) of foetuses. 42% of cardiovascular and 25% of extracardiac anomalies were diagnosed postnatally. There were 10 (15.4%) elective terminations, 1 (1.5%) spontaneous foetal demise. Two newborns died after birth before surgery. Of the 52 children who underwent Norwood surgery, 13 (25%) died (9 with additional anomalies, and 4 with isolated HLHS). Of the 38 children who underwent stage II surgery, 2 (5.2%) with isolated HLHS died, and 1 (2.6%) with CVA. Conclusions: A diagnosis of HLHS is an indication for a detailed examination of cardiac and noncardiac structures. It is advisable to consider genetic testing, together with the microarray assessment. The prognosis depends on underlying cardiac and extracardiac anomalies and coexisting genetic defects.

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Keywords

foetus; hypoplastic left heart syndrome; outcome; prenatal; postnatal

About this article
Title

Hypoplastic left heart syndrome: from the prenatal to the postnatal period

Journal

Ginekologia Polska

Issue

Vol 92, No 4 (2021)

Article type

Research paper

Pages

289-299

Published online

2021-01-26

Page views

1476

Article views/downloads

1174

DOI

10.5603/GP.a2020.0160

Pubmed

33751510

Bibliographic record

Ginekol Pol 2021;92(4):289-299.

Keywords

foetus
hypoplastic left heart syndrome
outcome
prenatal
postnatal

Authors

Anna Wojtowicz
Marek Raczka
Zbigniew Kordon
Agnieszka Ochoda-Mazur
Krzysztof Janowiec
Hubert Huras
Piotr Werynski
Agata Wloch

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