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Published on: June 16, 2023
Hypoplastic left heart syndrome: from the prenatal to the postnatal period
Anna Wojtowicz1, Marek Raczka2, Zbigniew Kordon2
1Collegium Medicum, Jagiellonian University, Cracow, Poland. anna.3.wojtowicz@uj.edu.pl.
Insights
Hypoplastic left heart syndrome (HLHS) affects 9.7% of fetuses with cardiovascular abnormalities. Prognosis for HLHS depends on associated cardiac, extracardiac, and genetic defects, necessitating comprehensive evaluation.
Area of Science:
- Cardiology
- Prenatal Diagnosis
- Medical Genetics
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect.
- Accurate prenatal diagnosis and characterization are crucial for management.
Purpose of the Study:
- To analyze the population of fetuses diagnosed with hypoplastic left heart syndrome (HLHS).
- To understand the spectrum of associated anomalies and outcomes in HLHS cases.
Main Methods:
- Retrospective study of fetuses diagnosed with HLHS between 2013 and 2017.
- Analysis of cardiovascular abnormalities (CVA), extracardiac malformations, and genetic disorders.
Main Results:
- HLHS was identified in 9.7% of fetuses with CVA.
- Associated anomalies (cardiac, extracardiac, genetic) were common, impacting outcomes.
- Postnatal diagnosis rates for anomalies were significant (42% cardiac, 25% extracardiac).
- Surgical outcomes varied, with higher mortality in cases with additional anomalies.
Conclusions:
- HLHS diagnosis warrants thorough cardiac and noncardiac structural examination.
- Genetic testing and microarray assessment are recommended.
- Prognosis is significantly influenced by coexisting anomalies and genetic defects.
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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