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Hybrid stage 1 palliation for HLHS: the experience of a tertiary center in a developing country
Fouad Bitar1, Issam M El-Rassi2, Rana Zareef1
1Department of Pediatrics and Adolescent Medicine, The American University of Beirut Medical Center (AUBMC), Beirut, Lebanon.
Insights
The hybrid approach for hypoplastic left heart syndrome (HLHS) shows promise in developing countries. This surgical technique yielded excellent outcomes, comparable to developed nations, offering hope for improved pediatric congenital heart disease management globally.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Global Health
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect affecting 2.6% of cases.
- HLHS is often fatal without treatment, particularly in developing countries with limited resources.
- Annual incidence of HLHS is approximately 33,750 newborns in developing nations.
Purpose of the Study:
- To analyze the outcomes of the hybrid approach for HLHS management in a developing country.
- To assess the feasibility and effectiveness of this strategy in a resource-limited tertiary care setting.
Main Methods:
- Retrospective single-center study of neonates with HLHS over five years.
- Review of medical records for patients undergoing hybrid stage 1 palliation.
- Data collection included baseline characteristics, procedural details, and patient outcomes.
Main Results:
- Eight of 18 HLHS patients underwent hybrid stage 1 palliation.
- Mean weight at procedure: 3.3 kg; mean age: 6.4 days.
- Zero surgical mortality; one interstage mortality due to sepsis. All survivors had preserved cardiac function.
Conclusions:
- The hybrid procedure for HLHS can achieve outcomes comparable to developed countries in developing nations.
- Successful implementation requires a strong foundational healthcare system.
- This approach contributes to a more equitable global landscape for congenital heart disease care.
Background:
Hypoplastic left heart syndrome (HLHS) accounts for 2.6% of congenital heart disease and is an invariably fatal cardiac anomaly if left untreated. Approximately 33,750 babies are born annually with HLHS in developing countries. Unfortunately, the majority will not survive due to the scarcity of resources and the limited availability of surgical management.
Aim:
To describe and analyze our experience with the hybrid approach in the management of HLHS in a developing country.
Methods:
We performed a retrospective single-center study involving all neonates born with HLHS over five years at the Children's Heart Center at the American University of Beirut. The medical records of patients who underwent the hybrid stage 1 palliation were reviewed, and data related to baseline characteristics, procedure details and outcomes were collected to describe the experience at a tertiary care center in a developing country.
Results:
A total of 18 patients were diagnosed with HLHS over a five-year period at our institution, with male to female ratio of 1:1. Of those, eight patients underwent the hybrid stage I procedure. The mean weight at the time of the procedure was 3.3 ± 0.3 kg with an average age of 6.4 ± 4 days. The mean hospital length of stay was 27.25 days, with an interquartile range of 33 days. The cohort's follow-up duration averaged 5.9 ± 3.5 years. The surgical mortality was zero. Only one mortality was recorded during the interstage period between stage I and II and was attributed to sepsis. Notably, all surviving patients maintained preserved and satisfactory cardiac function with good clinical status.
Conclusion:
Our limited experience underscores the potential of developing countries with proper foundations to adopt the hybrid procedure for HLHS, yielding outcomes on par with those observed in developed countries. This demonstrates the viability of establishing a more balanced global landscape for children with congenital heart disease.
