Bitar Fouad, El-Rassi Issam M, Zareef Rana, Jassar Yehya, Abboud Jennifer, Bulbul Ziad, Bitar Fadi, Arabi Mariam
Department of Pediatrics and Adolescent Medicine, The American University of Beirut Medical Center (AUBMC), Beirut, Lebanon.
Department of Pediatric Cardiac Surgery, Heart Center of Excellence, Al Jalila Children's Specialty Hospital, Dubai, United Arab Emirates.
Front Cardiovasc Med. 2024 Mar 7;11:1355989. doi: 10.3389/fcvm.2024.1355989. eCollection 2024.
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.
To describe and analyze our experience with the hybrid approach in the management of HLHS in a developing country.
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.
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.
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.
左心发育不全综合征（HLHS）占先天性心脏病的2.6%，若不治疗，这是一种必然致命的心脏畸形。在发展中国家，每年约有33750名患有HLHS的婴儿出生。不幸的是，由于资源稀缺和手术治疗的可及性有限，大多数患儿无法存活。
描述和分析我们在一个发展中国家采用杂交手术治疗HLHS的经验。
我们进行了一项回顾性单中心研究，纳入了在贝鲁特美国大学儿童心脏中心出生并患有HLHS的所有新生儿，研究时间跨度为五年。对接受杂交一期姑息手术的患者的病历进行了回顾，并收集了与基线特征、手术细节和结果相关的数据，以描述一个发展中国家三级医疗中心的经验。
在我们机构的五年时间里，共有18例患者被诊断为HLHS，男女比例为1:1。其中，8例患者接受了杂交一期手术。手术时的平均体重为3.3±0.3千克，平均年龄为6.4±4天。平均住院时间为27.25天，四分位间距为33天。该队列的随访时间平均为5.9±3.5年。手术死亡率为零。在一期和二期之间的过渡期仅记录到1例死亡，原因是败血症。值得注意的是，所有存活患者的心脏功能均保持良好且令人满意，临床状况良好。
我们有限的经验强调了具备适当基础的发展中国家采用杂交手术治疗HLHS的潜力，其结果与发达国家观察到的结果相当。这表明为先天性心脏病患儿建立更平衡的全球格局是可行的。
