Abstract
Background: The pathognomonic feature of tetralogy of Fallot (ToF) is the antero-cephalad deviation of the outlet septum in combination with an abnormal arrangement of the septoparietal trabeculations.Aims: The aim of this article was to study perinatal hearts using Polarized Light Imaging (PLI) in order to investigate the deep alignment of cardiomyocytes that bond the different components of the ventricular outflow tracts both together and to the rest of the ventricular mass, thus furthering the classic description of ToF.Methods and Materials: 10 perinatal hearts with ToF and 10 perinatal hearts with no detectable cardiac anomalies (control) were studied using PLI. The orientation of the myocardial cells was extracted and studied at high resolution. Virtual dissections in multiple section planes were used to explore each ventricular structure.Results and Conclusions: Contrary to the specimens of the control group, for all ToF specimens studied, the deep latitudinal alignment of the cardiomyocytes bonds together the left part of the Outlet septum (OS) S to the anterior wall of the left ventricle. In addition, the right end of the muscular OS bonds directly on the right ventricular wall (RVW) superior to the attachment of the ventriculo infundibular fold (VIF). Thus, the OS is a bridge between the lateral RVW and the anterior left ventricular wall. The VIF, RVW, and OS define an “inverted U” that roofs the cone between the interventricular communication and the overriding aorta. The opening angle and the length of the branches of this “inverted U” depend however on three components: the size of the OS, the size of the VIF, and the distance between the points of insertion of the OS and VIF into the RVW. The variation of these three components accounts for a significant part of the diversity observed in the anatomical presentations of ToF in the perinatal period.
Highlights
Tetralogy of Fallot (ToF) is a common cyanotic congenital heart disease
The main differences were found in the basal half of the ventricular mass, from the equatorial level to the atrioventricular orifices and the right ventricular outflow tract (RVOT)
The myoarchitecture of the ventricular mass is a new field of investigation allowed by the technological development of Computer Tomography (CT), Micro CT, Polarized Light Imaging (PLI), and diffusion tensor imaging (DTI)-magnetic resonance imaging (MRI) that should be extended to the description of all cardiac malformations
Summary
The malformation was described in 1888 by Etienne-Louis Arthur Fallot as the association of inter-ventricular communication, sub-pulmonary stenosis, overriding of the aorta, and hypertrophy of the right ventricle. These anatomical features had been described before him, but his article and clearly stated the clinico-pathological correlations of what he called the “maladie bleue.”. The pathognomonic feature of tetralogy of Fallot (ToF) is the antero-cephalad deviation of the outlet septum in combination with an abnormal arrangement of the septoparietal trabeculations
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
