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Clarifying the anatomy of hearts with concordant ventriculo-arterial connections but abnormally related arterial trunks

Published online by Cambridge University Press:  27 August 2015

Saurabh K. Gupta ,
Sivasubramanian Ramakrishnan ,
Gurpreet S. Gulati ,
G. William Henry ,
Diane E. Spicer ,
Carl L. Backer  and
Robert H. Anderson
Saurabh K. Gupta*
Affiliation:
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
Sivasubramanian Ramakrishnan
Affiliation:
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
Gurpreet S. Gulati
Affiliation:
Department of Cardiac Radiology, All India Institute of Medical Sciences, New Delhi, India
G. William Henry
Affiliation:
Bailliard Henry Pediatric Cardiology, Raleigh, North Carolina, United States of America
Diane E. Spicer
Affiliation:
Division of Pediatric Cardiology, University of Florida College of Medicine, Gainesville, United States of America Congenital Heart Institute of Florida, St. Petersburg, Florida, United States of America
Carl L. Backer
Affiliation:
Division of Cardiovascular-Thoracic Surgery, Department of Pediatrics, Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois, United States of America
Robert H. Anderson
Affiliation:
Institute of Genetic Medicine, University of Newcastle, Newcastle-upon-Tyne, United Kingdom
*
Correspondence to: Dr Saurabh K. Gupta, MD, DM, Assistant Professor, Department of Cardiology, Room No. 23, 7th floor CT Centre, All India Institute of Medical Sciences, New Delhi 110029, India. Tel: +91-11-26594681; Fax: +91-11-26588663; E-mail: drsaurabhmd@gmail.com
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Abstract

Hearts in which the arterial trunks arise from the morphologically appropriate ventricles, but in a parallel manner, rather than the usual spiralling arrangement, have long fascinated anatomists. These rare entities, for quite some time, were considered embryological impossibilities, but ongoing experience has shown that they can be found in various segmental combinations. Problems still exist about how best to describe them, as the different variants are often described with esoteric terms, such as anatomically corrected malposition or isolated ventricular inversion. In this review, based on our combined clinical and morphological experience, we demonstrate that the essential feature of all hearts described in this manner is a parallel arrangement of the arterial trunks as they exit from the ventricular mass. We show that the relationship of the arterial roots needs to be described in terms of the underlying ventricular topology, rather than according to the arrangement of the atrial chambers. We then discuss the importance of determining atrial arrangement on the basis of the morphology of the appendages, following the precepts as set out in the so-called “morphological method” and distinguished according to the extent of the pectinate muscles relative to the atrioventricular junctions as opposed to basing diagnosis on the venoatrial connections. We show that, when approached in this manner, the various combinations can be readily diagnosed in the clinical setting and described in straightforward way.

Keywords

Anatomically corrected malpositionisolated ventricular inversionsequential segmental analysisventricular topologyterminology
Type
Review Articles
Information
Cardiology in the Young , Volume 26 , Issue 1 , January 2016 , pp. 1 - 18
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Copyright
© Cambridge University Press 2015 

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