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Hypoplastic left heart syndrome: a review
Roberts Gobergs1, Elza Salputra1, Ingūna Lubaua1,2
1Riga Stradiņš University, Riga, Latvia.
Acta Medica Lituanica
|March 31, 2017
Summary
Hypoplastic left heart syndrome (HLHS) treatment has advanced significantly, with staged surgical palliation now offering a five-year survival rate for over half of affected newborns. The optimal management strategy for HLHS remains individualized.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Research
- Neonatal Surgery
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect affecting 1 in 5,000 newborns.
- Historically, HLHS was uniformly fatal in early infancy.
- Recent advancements in surgical palliation have dramatically improved survival rates.
Purpose of the Study:
- To review the current understanding of HLHS, including its anatomy, embryology, and pathophysiology.
- To explore contemporary treatment options for HLHS, encompassing surgical and fetal interventions.
- To present a case study illustrating treatment approaches in Latvia.
Main Methods:
- Comprehensive literature review of HLHS using Pubmed and ClinicalKey databases.
- Inclusion of keywords: hypoplastic left heart syndrome, fetal aortic valvuloplasty, Norwood procedure, Glenn procedure, Fontan procedure, and hybrid procedure.
- Case report from Children's Clinical University Hospital, Riga, to exemplify clinical practice.
Main Results:
- Current therapeutic options for HLHS include heart transplantation, staged surgical palliation, and comfort care.
- Fetal therapy represents an emerging treatment modality for HLHS.
- Staged palliation, culminating in Fontan circulation, provides a pathway to survival pending heart transplantation, with 50-70% of patients reaching age five.
Conclusions:
- The optimal treatment for HLHS is not definitively established and requires a personalized approach.
- Management decisions should consider individual patient factors, institutional expertise, financial constraints, and parental preferences.
- Ongoing research is crucial for refining HLHS treatment strategies.