Classic form of hypoplastic left heart syndrome diagnosed post-natally: an autopsy report

  • Cristiane Rúbia Ferreira Anatomic Pathology Service – Hospital Universitário – Universidade de São Paulo, São Paulo/SP
  • Vera Demarchi Aiello Laboratory of Pathology – Instituto do Coração – Hospital das Clínicas – Faculdade de Medicina – Universidade de São Paulo, São Paulo/SP
  • Ana Maria Andrello Gonçalves Pereira de Melo Department of Pediatrics – Hospital Universitário – Universidade de São Paulo, São Paulo/SP
  • Lucas Braga Mota Department of Internal Medicine – Hospital das Clínicas – Faculdade de Medicina – Universidade de São Paulo, São Paulo/SP
  • Ana Carolina Neves de Carvalho Department of Internal Medicine – Hospital das Clínicas – Faculdade de Medicina – Universidade de São Paulo, São Paulo/SP
  • Heli Samuel Pinto Souza Department of Internal Medicine – Hospital das Clínicas – Faculdade de Medicina – Universidade de São Paulo, São Paulo/SP
  • Diana Arrais de Souza Rangel Department of Internal Medicine – Hospital das Clínicas – Faculdade de Medicina – Universidade de São Paulo, São Paulo/SP
Keywords: Hypoplastic Left Heart Syndrome, Respiratory Insufficiency, Shock, Cardiogenic, Autopsy

Abstract

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.

Downloads

Download data is not yet available.
Published
2014-09-30
How to Cite
Ferreira, C., Aiello, V., Melo, A., Mota, L., Carvalho, A., Souza, H., & Rangel, D. (2014). Classic form of hypoplastic left heart syndrome diagnosed post-natally: an autopsy report. Autopsy and Case Reports, 4(3), 21-30. https://doi.org/10.4322/acr.%y.98474
Section
Article / Autopsy Case Report