Ventricular noncompaction and the association with congenital heart defects

• Grant number: 13/25059-8
• Support type: Scholarships in Brazil - Scientific Initiation
• Effective date (Start): April 01, 2014
• Effective date (End): March 31, 2015
• Field of knowledge: Health Sciences - Medicine - Pathological Anatomy and Clinical Pathology
• Principal researcher: Vera Demarchi Aiello
• Grantee: Laís Costa Marques
• 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

Ventricular non-compation is a rare anomaly, characterized by an aberration in the morphology of the trabeculation in both ventricles, especially in the left. This anomaly has received attention in the last decade and there are few studies approaching the subject. The most accepted hypothesis is that this malformation occurs prenatally. The gold standard method to identify this anomaly in life is the echocardiography. The diagnostic criteria are: presence of more than 3 prominent trabeculae apical to the papillary muscles, evidence of blood flow in intratrabecular recesses detected by Doppler and the existence of a double layer of the ventricular wall, with a thin compacted epicardial band and an uncompacted muscular layer (relationship between uncompressed layer / compressed >2). Non-systematic observation shows that anatomical specimens with congenital heart anomalies can show myocardium non-compaction, without a detected dysfunction during the clinical or hemodynamic evaluation. The functional and clinical significance of this finding is unclear, as is the actual prevalence of myocardial phenotype in these hearts.4. GoalsThe goal of this study is to evaluate the prevalence of non-compacted myocardium in hearts (anatomical specimens) from children with congenital heart disease, and its possible relation to different predominant abnormalities.5.MethodologyThe hearts belong to the anatomical collection of the Laboratory of Pathology, Heart Institute of HCFMUSP, and will be evaluated macroscopically. Hearts from children with a primary diagnosis of cardiomyopathy, dilated or hypertrophic, will not be included. The complete collection comprises more than 500 hearts, each of them will be individually evaluated, provided the method of opening of the specimen allows inclusion in the study. Once the hearts are selected, trabecular and the compacted myocardium will be measured with a ruler at the distal portion of the inlet - corresponding to the line of insertion of a papillary muscle and at the apical region, corresponding to the midpoint between the first measurement and the apex itself. In each case, the ration between compact and non-compacted myocardium will be calculated. The results of these measurements will be correlated with the type of heart defect present.

Scientific publications

(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)

MARQUES, LAIS COSTA; LIGUORI, GABRIEL ROMERO; AMARANTE SOUZA, ANA CAROLINA; AIELLO, VERA DEMARCHI. Left Ventricular Noncompaction Is More Prevalent in Ventricular Septal Defect Than Other Congenital Heart Defects: A Morphological Study. JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE, v. 7, n. 4 DEC 2020. Web of Science Citations: 0.