Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Objective Conduction damage following transcatheter aortic valve implantation (TAVI) remains common. Anatomical risk factors remain elusive. We assessed the impact of variability in the dimensions of the membranous septum and position of the aortic root on the occurrence of conduction damage following TAVI. Methods The dimensions of the membranous septum, the rotational position of the aortic root correlating to variability in the central fibrous body width, and wedging of the aortic root were assessed on pre-TAVI CT datasets. The depth of implantation was measured from the final aortic angiogram. The variables were compared with the occurrence of both permanent pacemaker insertion (PPI) and left bundle branch block (LBBB) following TAVI. Results Of 200 patients who met inclusion criteria (mean age = 81 years ± 7.7, 49% men), 20.5 % underwent PPI after TAVI. New LBBB occurred in 23.5%, 21.3 % of whom required PPI. Preprocedural right bundle branch block (OR = 7.00; CI 3.13 to 15.64), valve type (OR=2.35; CI 1.13 to 4.87), depth of implantation (OR=1.62; CI 1.01 to 2.61) and the difference between depth of implantation and the distance from the virtual basal ring to the inferior margin of the membranous septum (OR=0.61; CI 0.38 to 0.99) were all associated with PPI, with similar associations with LBBB. No gross anatomical variable alone was associated with conduction damage. Conclusions Gross anatomical variation of the aortic root and its underlying support, including the membranous septum, were not associated with the occurrence of either PPI or new LBBB. Procedural characteristics associated with these adverse outcomes suggest that the depth of implantation and radial force of the bioprosthesis, regardless of gross anatomical variability, increase the risk for conduction damage.

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC. The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature. The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

There is continued interest in surgical repair of both the congenitally malformed aortic valve, and the valve with acquired dysfunction. Aortic valvar repair based on a geometric approach has demonstrated improved durability and outcomes. Such an approach requires a thorough comprehension of the complex 3-dimensional anatomy of both the normal and congenitally malformed aortic root. In this review, we provide an understanding of this anatomy based on the features that can accurately be revealed by contrast-enhanced computed tomographic imaging. We highlight the complimentary role that such imaging, with multiplanar reformatting and 3-dimensional reconstructions, can play in selection of patients, and subsequent presurgical planning for valvar repair. The technique compliments other established techniques for perioperative imaging, with echocardiography maintaining its central role in assessment, and enhances direct surgical evaluation. This additive morphological and functional information holds the potential for improving selection of patients, surgical planning, subsequent surgical repair, and hopefully the subsequent outcomes.