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[Hypoplastic left heart syndrome (general review)]
T Tláskal1, V Povýsilová, D Vondrys
1Dĕtské kardiocentrum, Fakultní nemocnice v Motole, Praha. tomas.tlaskal@lfmotol.cuni.cz
Insights
Hypoplastic left heart syndrome (HLHS), a severe congenital heart defect, has a near 100% neonatal mortality rate. Reassessing HLHS as operable, considering surgical and broader societal factors, is crucial for improving infant outcomes.
Area of Science:
- Cardiology
- Pediatric Surgery
Context:
- Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect characterized by underdevelopment of the left heart structures.
- Historically, HLHS has been considered inoperable due to unfavorable morphology, leading to high neonatal mortality rates.
Purpose:
- To challenge the notion of HLHS as an inoperable condition.
- To advocate for a reassessment of treatment strategies for HLHS, considering advancements in surgical interventions and transplantation.
Summary:
- HLHS involves a small left ventricle and abnormalities of the aortic and mitral valves, typically resulting in neonatal death.
- The Norwood procedure and neonatal heart transplantation have improved prognoses for HLHS patients.
- Current practice in some regions involves pregnancy termination or only symptomatic treatment for neonates with HLHS.
Impact:
- Revisiting the operability of HLHS could significantly improve survival rates for affected neonates.
- A comprehensive analysis encompassing morphological, clinical, surgical, philosophical, psychological, socio-economic, and healthcare organization aspects is necessary.
- This reassessment aims to offer better treatment options and improve the quality of life for children born with HLHS.
Abstract:
The hypoplastic left heart syndrome (HLHS) is a critical congenital heart lesion with a small left ventricle in combination with stenosis or atresia of the aortic and the mitral valve. This heart disease is associated with a nearly 100% mortality at neonatal age. Until recently, this heart lesion was considered inoperable because of extremely unfavourable morphology. Introduction of the Norwood three-step reconstructive operation on one hand, and heart transplantation in neonates, on the other hand, improved the prognosis of patients with this complex heart lesion. In our country, however, this heart disease, if found during the first months of pregnancy, represents an indication for its termination, and neonates with HLHS are offered only symptomatic medical treatment. On basis of good experience from several most experienced cardiac centres we believe that it is necessary to reassess the statement considering HLHS as an inoperable disease. The main change from this aspect necessitates, however, a detailed analysis of the whole problem not only from the morphological, clinical and surgical point of view but also from philosophical, psychological, socio-economic and health care organisation aspects.