Cubillos Celis María Paz, Maccioni Romero Andrea, Galleguillos Galindo Constanza, Garrido Sepúlveda Claudia, Martinovic Carolina, Vargas Innocenti Paula
Complejo Asistencial Dr. Sótero del Río, Santiago, Chile.
Pontificia Universidad Católica de Chile, Santiago, Chile.
Andes Pediatr. 2021 Aug;92(4):576-583. doi: 10.32641/andespediatr.v92i4.2787.
Hypoplastic Left Heart Syndrome (HLHS) is the most common form of functional single-ventricle heart disease. Despite improvements in medical and surgical care, morbidity and mortality remain high, especially around the first surgical stage.
To describe the clinical characteristics of newborns with HLHS, seen at a perinatal referral center.
Retrospective des criptive study of all newborns with pre and/or postnatal diagnosis of HLHS between 2014 and 2019 in the Complejo Asistencial Dr. Sótero del Rio. Medical records were reviewed collecting demographic and perinatal variables, clinical evolution, mortality associated with the first surgery, and survival at one year.
Nineteen patients were included. Most were term newborns and 13/19 were fema le. All had a prenatal diagnosis. Five patients had associated extracardiac anomalies. 16/19 required vasoactive drugs and 17/19 used invasive mechanical ventilation. Seven patients received enteral fe eding, without episodes of enterocolitis. The median age at transfer for surgery was 8 days. Thirteen patients underwent the Norwood procedure, with 31% of mortality, and 47% of survival at one year.
Neonatal management of patients with HLHS is complex and the multidisciplinary approach is fundamental. Mortality around the first surgical stage of our cohort is greater than the international reports which could be influenced by the absence of fetal therapy and the higher inci dence of extracardiac malformations.
左心发育不全综合征（HLHS）是功能性单心室心脏病最常见的形式。尽管医疗和手术治疗有所改善，但发病率和死亡率仍然很高，尤其是在第一个手术阶段前后。
描述在围产期转诊中心就诊的HLHS新生儿的临床特征。
对2014年至2019年在索特罗·德尔里奥医生综合医疗中心产前和/或产后诊断为HLHS的所有新生儿进行回顾性描述性研究。查阅病历，收集人口统计学和围产期变量、临床病程、与首次手术相关的死亡率以及一岁时的生存率。
纳入19例患者。大多数为足月儿，19例中有13例为女性。均有产前诊断。5例患者伴有心外异常。19例中有16例需要使用血管活性药物，19例中有17例使用有创机械通气。7例患者接受肠内喂养，无坏死性小肠结肠炎发作。转至手术的中位年龄为8天。13例患者接受了诺伍德手术，死亡率为31%，一岁时生存率为47%。
HLHS患者的新生儿管理复杂，多学科方法至关重要。我们队列中第一个手术阶段前后的死亡率高于国际报告，这可能受到缺乏胎儿治疗以及心外畸形发生率较高的影响。
