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

Anna Wojtowicz, Marek Raczka, Zbigniew Kordon, Agnieszka Ochoda-Mazur, Krzysztof Janowiec, Hubert Huras, Piotr Werynski, Agata Włoch
DOI: 10.5603/GP.a2020.0160

open access

Ahead of Print
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.

Get Citation

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

Ahead of Print

Article type

Research paper

Published online

2021-01-26

DOI

10.5603/GP.a2020.0160

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 Włoch

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