Ferreira Cristiane Rúbia, Aiello Vera Demarchi, de Melo Ana Maria Andrello Gonçalves Pereira, Mota Lucas Braga, de Carvalho Ana Carolina Neves, Souza Heli Samuel Pinto, Rangel Diana Arrais de Souza
Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Laboratory of Pathology - Instituto do Coração - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.
Autops Case Rep. 2014 Sep 30;4(3):21-30. doi: 10.4322/acr.2014.025. eCollection 2014 Jul-Sep.
Hypoplastic left heart syndrome (HLHS) is a congenital heart disease, which, despite the current improved knowledge about its management and surgical treatment, is still associated with high mortality, especially in the early neonatal period and before the second stage of reconstruction surgery. The low rate of prenatal diagnosis and delayed diagnostic suspicion results in unsuccessful therapeutic intervention, even though the real impact of early diagnosis and intervention on mortality and quality of life of patients is still uncertain. Fortunately, this syndrome of challenging treatment is not that frequent. It involves a spectrum of obstructions to the blood flow within the left heart and is characterized by an inappropriate size of the left ventricle associated with a wide variety of valvular dysfunctions. Treatment ranges from heart transplantation to palliative surgical procedures. The authors describe a case of a newborn with HLHS, whose diagnosis was made after birth because of early respiratory failure. Despite the use of prostaglandin the newborn died. An autopsy was performed and the anatomical findings were described.
左心发育不全综合征（HLHS）是一种先天性心脏病，尽管目前对其管理和外科治疗的认识有所提高，但它仍然与高死亡率相关，尤其是在新生儿早期和二期重建手术之前。产前诊断率低和诊断怀疑延迟导致治疗干预失败，尽管早期诊断和干预对患者死亡率和生活质量的实际影响仍不确定。幸运的是，这种治疗具有挑战性的综合征并不常见。它涉及左心内一系列血流梗阻，其特征是左心室大小不合适，并伴有多种瓣膜功能障碍。治疗范围从心脏移植到姑息性外科手术。作者描述了一例HLHS新生儿病例，该患儿因早期呼吸衰竭在出生后确诊。尽管使用了前列腺素，该新生儿仍死亡。进行了尸检并描述了解剖学发现。
