Expert consensus statement: Anatomy, imaging, and nomenclature of congenital aortic root malformations

Authors

Justin T. Tretter, Department of Pediatric Cardiology, Cleveland Clinic Children's and The Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
Diane E. Spicer, Heart Institute, Johns Hopkins All Children's Hospital, St Petersburg, Florida.
Rodney C. Franklin, Paediatric Cardiology Department, Royal Brompton & Harefield National Health Service Trust, London, United Kingdom.
Marie J. Béland, Division of Pediatric Cardiology, The Montreal Children's Hospital of the McGill University Health Centre, Montréal, Québec, Canada.Follow
Vera D. Aiello, Heart Institute (InCor), University of São Paulo School of Medicine, São Paulo, Brazil.Follow
Andrew C. Cook, Institute of Cardiovascular Science, University College London, London, United Kingdom.Follow
Adrian Crucean, Department of Paediatric Cardiac Surgery, Birmingham Women's and Children's Hospital, Birmingham, United Kingdom.Follow
Rohit S. Loomba, Advocate Aurora HealthFollow
Shi-Joon Yoo, Division of Cardiology, Department of Pediatrics, Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
James A. Quintessenza, Heart Institute, Johns Hopkins All Children's Hospital, St Petersburg, Florida.
Christo I. Tchervenkov, Division of Cardiovascular Surgery, The Montreal Children's Hospital of the McGill University Health Centre, Montréal, Québec, Canada.
Jeffrey P. Jacobs, Congenital Heart Center, UF Health Shands Hospital, Division of Cardiovascular Surgery, Departments of Surgery and Pediatrics, University of Florida, Gainesville, Florida.
Hani K. Najm, Division of Pediatric Cardiac Surgery, Cleveland Clinic Children's and the Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
Robert H. Anderson, Cardiovascular Research Centre, Biosciences Institute, Newcastle University, Newcastle upon Tyne, United Kingdom.Follow

Recommended Citation

Tretter JT, Spicer DE, Franklin RCG, et al. Expert Consensus Statement: Anatomy, Imaging, and Nomenclature of Congenital Aortic Root Malformations. Cardiol Young. 2023;33(7):1060-1068. doi:10.1017/S1047951123001233

Affiliations

Advocate Children's Hospital

Abstract

Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.

Type

Article

PubMed ID

37288941