The atrial septum in the hypoplastic left heart syndrome: evaluation of macro and microscopic characteristics for therapeutic and interventional procedures

• Grant number: 18/14607-8
• Support type: Scholarships in Brazil - Scientific Initiation
• Effective date (Start): November 01, 2018
• Effective date (End): April 30, 2020
• Field of knowledge: Health Sciences - Medicine - Pathological Anatomy and Clinical Pathology
• Principal researcher: Vera Demarchi Aiello
• Grantee: Bárbara Justo Carvalho
• Home Institution: Instituto do Coração Professor Euryclides de Jesus Zerbini (INCOR). Hospital das Clínicas da Faculdade de Medicina da USP (HCFMUSP). Secretaria da Saúde (São Paulo - Estado). São Paulo , SP, Brazil

Abstract

Title: The atrial septum in the hypoplastic left heart syndrome: evaluation of macro and microscopic characteristics for therapeutic and interventional procedures. Introduction: Hypoplastic Left Heart Syndrome (HLHS) encompasses a spectrum of cardiac malformations related to elements of the left heart and aorta that, despite the low incidence among congenital heart diseases, have high mortality rates that are related to the inability to generate adequate blood flow to the systemic circulation. In the usual procedures performed on syndromic newborns, phenotypic variations of the disease influence the success of the treatment, especially in the characteristics related to the atrial septum and atrial septal defect. Objectives: To study hearts with HLHS in order to analyze macro and microscopic features of the atrial septum to guide therapeutic procedures on this structure, correlating them with other aspects normally altered in this syndrome, such as cardiac valve morphology and ventricular size. Methods: This study will use a collection of 25 hearts diagnosed with SHCE and, as a control, 10 normal hearts of paired age, all belonging to the Laboratory of Pathological Anatomy at Instituto do Coração/HCFMUSP collection. The atrial septum will be evaluated for the patency of the foramen ovale and its size, bulging of its lamina to one or other atrial cavity, presence of septal defects and leftward displacement of its insertion. Other morphological features of the anatomical specimen, such as atresia or stenosis of the mitral and aortic valves, patency of the ductus arteriosus and caliber of the ascending aorta will be noted. The length of the entryway of both ventricles will also be measured. The atrial septum will be resected for measurements of maximum and minimum thickness according to topography and histological analysis to identify the characteristics of the extracellular matrix and muscle cells (smooth or cardiac).