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

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 cardiovasc...

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Published in:Ginekologia polska 2021-01, Vol.92 (4), p.289-299
Main Authors: Wojtowicz, Anna, Raczka, Marek, Kordon, Zbigniew, Ochoda-Mazur, Agnieszka, Janowiec, Krzysztof, Huras, Hubert, Werynski, Piotr, Włoch, Agata
Format: Article
Language:English
Subjects:
Coronary vessels
Counseling
Genetic disorders
Gestational age
Heart
Medical prognosis
Obstetrics
Pediatrics
Pregnancy
Surgery
Ultrasonic imaging
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Summary: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.
ISSN:0017-0011
2543-6767
DOI:10.5603/GP.a2020.0160