August 16, 2023/Pediatrics/Cardiology

New Consensus Statement Seeks To Clarify Aortic Root Terminology

Malformation classification provides uniform nomenclature for use in patient care and procedure coding

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For decades, experts in cardiology worldwide have worked to come to agreement on a lexicon for aortic root abnormalities that can be used in clinical practice and to support procedure coding. Several categorization schemes have been published in recent years, all of which have utility but also a few drawbacks.

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Building on those previous efforts, a new consensus statement is designed to eliminate some of the ambiguity around the nomenclature. Developed by a panel of international experts, including a Cleveland Clinic cardiac anatomist-imager and cardiac surgeon, the recommendations focus on clinically and surgically relevant terminology and take into consideration not only the anatomy of the aortic root, but how it functions.

“Our aim was to create a comprehensive, evidence-based scientific terminology scheme for congenital cardiac disease that would contribute to refinement of language in the World Health Organization’s [WHO] International Classification of Diseases [ICD]” says Justin T. Tretter, MD, Co-Director of the Congenital Valve Procedural Planning Center. “The standardized terms and definitions we’ve proposed are suitable for acquired or congenital disease in children and adults and can be used by those working in all cardiac specialties.”

Co-published in The Annuals of Thoracic Surgery and Cardiology in the Young, the classification is an outgrowth of categorizations for aortic root abnormalities created over the past 30 years. Included are terms and definitions suggested for use by pediatric and adult cardiologists to describe trisinuate, bisinuate and quadrisinuate variants.

The key to describing the congenitally malformed aortic root, the authors believe, lies in recognizing that the normal root is made up of three leaflets, supported by their own sinuses, which are separated by the interleaflet triangles.

“When discussing a patient with congenital heart disease, clinicians often talk about aortic valve cusps. But they define a ‘cusp’ in different ways,” says Dr. Tretter. “One person may mean the leaflets, another the sinuses in the aortic root, and a third may believe that a ‘cusp’ is both things. That leads to confusion, which can negatively impact patient care.”

Historical perspective on congenital heart disease nomenclature

The first classification of congenital heart disease was the 1936 Atlas of Congenital Cardiac Disease. It wasn’t until the 1990s that two separate pediatric and congenital cardiac systems of nomenclature were created. One was by the Association for European Paediatric and Congenital Cardiology and the other a collaboration of the Society of Thoracic Surgeons, the European Association for Cardio-Thoracic Surgery (EACTS), and the European Congenital Heart Surgeons Foundation.

“As a result, clinicians in North American have been using different ‘languages’ for congenital heart disease than clinicians in Europe and Asia,” says Dr. Tretter, a cardiac anatomist and first author of the new consensus statement. “They have some similarities but many differences, and each has its positives and negatives.”

Recognizing the potential for duplication, inaccurate coding and confusion between the systems, the two groups convened an expert panel in 2000 to create a common nomenclature. The result was subsequent formation of the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD) and creation of the International Paediatric and Congenital Cardiac Code (IPCCC), which is in use worldwide, has been unified with the WHO’s ICD-11 and includes more than 300 terms.

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Consensus panel composition and clinical impact

“While the value of the IPCCC can’t be overstated, the 2021 update was limited to the bicuspid aortic valve. It didn’t cover all the congenital problems associated with the aortic valve and aortic root,” says Dr. Tretter. “It also lacked a hierarchical breakdown of terminology, which is necessary for databases.”

To help fill those gaps, the panel convened for the new consensus statement included a broad group of experts – from cardiac anatomists, imagers and surgeons to experts in database development. Dr. Tretter and Hani K. Najm, MD, brought a unique perspective, as co-directors of Cleveland Clinic’s Congenital Valve Procedural Planning Center and the only anatomist-imager and cardiac surgeon team from the same institution.

“Through our clinical and research work, we’ve come to appreciate that the aortic valve is part of the complex 4-dimensional structure aortic root, which includes the valve and its leaflets, the sinuses and the interleaflet triangles,” says Dr. Tretter. “Understanding the anatomy and how it moves has led us to a more tailored approach to imaging underlying structures not visible to the surgeon and better personalizing surgical procedures.”

In the year and a half since the valve center has been in existence, the team has collaborated on care of more than 50 patients with various aortic valve issues. “With a more precise approach, utilizing the very clear terminology in the consensus statement, coupled with an understanding of an individual’s anatomy and a personalized surgical blueprint, Dr. Najm’s results, which were great before, have become even better.”

Dr. Najm remarks, “The improved understanding of the aortic root with advanced imaging adds a next level planning of the repair technique prior to surgical intervention. The valve procedural planning center is founded to improve the quality and durability of valve interventions.”

Featured image: Image captured from a video displaying a 3-dimensional plane of the aortic valvar opening area. The featured image is reproduced under the Creative Commons CC-BY-NC-ND license. Originally appearing in Tretter JT, Spicer DE, Franklin RCG, et al. Expert Consensus Statement: Anatomy, Imaging, and Nomenclature of Congenital Aortic Root Malformations. Ann Thorac Surg. 2023 Jul;116(1):6-16. doi: 10.1016/j.athoracsur.2023.03.023. Epub 2023 Jun 8.

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