title: Left ventricular apical torsion and architecture are not inverted in situs inversus totalis.
abstract: Occasionally, individuals have a complete, mirror-image reversal of their internal organ position, called situs inversus totalis (SIT). Whereas gross anatomy is mirror-imaged in SIT, this might not be the case for the internal architecture of organs, e.g. the myofiber pattern in the left cardiac ventricle. We performed a Magnetic Resonance Tagging study in nine controls and in eight subjects with SIT to assess the deformation pattern in the apical half of the LV wall. It appeared that both groups had the same LV apical deformation pattern. This implies that not only the superficial LV apical layers in SIT follow a normal, not inverted pattern, but the deeper layers as well. Apparently, the embryonic L/R controlling genetic pathway does determine situs-specific gross anatomy morphogenesis but it is not the only factor regulating fiber architecture within the apical part of the LV wall. We propose that mechanical forces generated in the not-inverted molecular structure of the basic right-handed helical contractile components of the sarcomere play a role in shaping the LV apex.
authors.affiliation: Department of Pediatrics, Cardiovascular Research Institute Maastricht, University Hospital Maastricht, PO Box 5800, AZ Maastricht, The Netherlands. t.delhaas@fys.unimaas.nl
authors.forename: Tammo
authors.initials: T
authors.lastname: Delhaas
authors.forename: Wilco
authors.initials: W
authors.lastname: Kroon
authors.forename: Peter
authors.initials: P
authors.lastname: Bovendeerd
authors.forename: Theo
authors.initials: T
authors.lastname: Arts
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006352:Heart Ventricles / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012857:Situs Inversus / Q000503:physiopathology*; D016277:Ventricular Function, Left