scholarly journals Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases—Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease

2018 ◽  
Vol 106 (5) ◽  
pp. 1578-1589 ◽  
Author(s):  
Leo Lopez ◽  
Lucile Houyel ◽  
Steven D. Colan ◽  
Robert H. Anderson ◽  
Marie J. Béland ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
International Society ◽  
Congenital Heart ◽  
International Classification Of Diseases ◽  
International Classification ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Classification Of Diseases

scholarly journals Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature – The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11)

2021 ◽  
pp. 215013512110329
Author(s):  
Jeffrey P. Jacobs ◽  
Rodney C. G. Franklin ◽  
Marie J. Béland ◽  
Diane E. Spicer ◽  
Steven D. Colan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
International Classification Of Diseases ◽  
International Classification ◽  
Cardiac Care ◽  
Classification Of Diseases ◽  
Icd 10 ◽  
Cardiac Component

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.

scholarly journals Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature – The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11)

2021 ◽  
Vol 31 (7) ◽  
pp. 1057-1188
Author(s):  
Jeffrey P. Jacobs ◽  
Rodney C. G. Franklin ◽  
Marie J. Béland ◽  
Diane E. Spicer ◽  
Steven D. Colan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
International Classification Of Diseases ◽  
International Classification ◽  
Cardiac Care ◽  
Classification Of Diseases ◽  
Icd 10 ◽  
Cardiac Component

AbstractSubstantial 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.

Congenital heart disease in 37,294 births in Tunisia: birth prevalence and mortality rate

2013 ◽  
Vol 24 (5) ◽  
pp. 866-871 ◽  
Author(s):  
Dorra Abid ◽  
Anis Elloumi ◽  
Leila Abid ◽  
Souad Mallek ◽  
Hajer Aloulou ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Coarctation Of The Aorta ◽  
Ventricular Septal Defects ◽  
Birth Prevalence ◽  
Septal Defects

AbstractAim: To investigate the previously unknown birth incidence, treatment, and mortality of children with congenital heart disease in Tunisia. Methods: We undertook a retrospective review of medical records of all patients who were born in 2010 and 2011, and were diagnosed in Sfax (Tunisia) with congenital heart defect. Results: Among 37,294 births, 255 children were detected to have congenital heart disease, yielding a birth incidence of 6.8 per 1000. The most frequently occurring conditions were ventricular septal defects (31%), ostium secundum atrial septal defects (12.9%), and pulmonary valve abnormalities (12%). Coarctation of the aorta, tetralogy of Fallot, univentricular physiology, pulmonary atresia with ventricular septal defect, and transposition of the great arteries were found in 4.3%, 6.2%, 3.4%, 2.7%, and 2.7%, respectively. During the follow-up of 1 year, 23% of the children died. About three-quarters of those deaths happened before surgery. Conclusion: The present study is in line with the general estimates in the world. It has revealed a high case of mortality among the patients awaiting corrective surgery. These children need more facilities.

scholarly journals Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11)

2017 ◽  
Vol 27 (10) ◽  
pp. 1872-1938 ◽  
Author(s):  
Rodney C. G. Franklin ◽  
Marie J. Béland ◽  
Steven D. Colan ◽  
Henry L. Walters ◽  
Vera D. Aiello ◽  
...  
Keyword(s):  
World Health Organization ◽  
Congenital Heart ◽  
International Classification Of Diseases ◽  
International Classification ◽  
World Health ◽  
Short List ◽  
The World ◽  
Classification Of Diseases ◽  
Health Organization

AbstractAn internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many “short list” versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various “short lists”. In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the “short list” for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.

scholarly journals Congenital Heart Disease in Down Syndrome

1993 ◽  
Vol 14 (12) ◽  
pp. 488-494
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Chromosomal Abnormalities ◽  
Heart Defects ◽  
Endocardial Cushion ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Endocardial Cushion Defect

Trisomy 21, Down syndrome, is one of a number of chromosomal abnormalities associated with congenital heart disease. Recent studies indicate that approximately 5% of all congenital heart defects are associated with some form of chromosomal abnormality, the majority of which are Down syndrome. Reports of the incidence of congenital heart disease in patients who have Down syndrome have varied, but it is commonly accepted to be 50%. Endocardial cushion defect and ventricular septal defects both have been reported as the "most common," but the majority of investigators accept the endocardial cushion defect as being the more frequent. The association between endocardial cushion defects and Down syndrome is so striking that when an endocardial cushion defect is diagnosed in an infant, the possibility of Down syndrome always should be considered.

Letters to the Editor

PEDIATRICS ◽  
1967 ◽  
Vol 39 (5) ◽  
pp. 794-795
Author(s):  
NORMAN J. SISSMAN
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Alternative Methods ◽  
General Factor ◽  
Ventricular Septal Defects ◽  
Letters To The Editor ◽  
Septal Defects ◽  
Surgical Teams ◽  
Methods Of Treatment

The "controversy" over treatment of infants with ventricular septal defects published in the January 1967 issue of Pediatrics prompts me to comment on one general factor, vital in decisions between alternative methods of treatment of congenital heart disease, which has received little attention in the literature—namely, the experiences and capabilities of individual surgical teams. That Dr. Nadas takes this into account is reflected in his statements, ". . . our surgeons have been able to accomplish [banding of the pulmonary artery] at a mortality rate of 10% or less . . ." and ". . . the pediatrician unless he has surgeons with exceptional ability at his disposal, will do better to . . . proceed conservatively."

Smith-Magenis syndrome (interstitial deletion of chromosome 17p) and congenital heart disease

1993 ◽  
Vol 3 (4) ◽  
pp. 441-444 ◽  
Author(s):  
Dianne N. Abuelo ◽  
Robert D. Corwin
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Interstitial Deletion ◽  
Chromosome 17 ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
The Common ◽  
Pulmonary Valvar Stenosis ◽  
Specific Deletion

SummaryA syndrome with characteristic facial and skeletal features resulting from a specific deletion of material on the short arm of chromosome 17 was first described by Smith et al in 1982.1 The common findings are brachycephaly, hypoplasia of the middle face, and short broad hands associated with mental retardation. More than 50 cases have been reported, and about one third have congenital heart disease. We report here a patient with atrial and ventricular septal defects together with pulmonary valvar stenosis.

Reconstruction of the aortic arch in an adolescent female with complex congenital heart disease

1994 ◽  
Vol 4 (2) ◽  
pp. 164-167
Author(s):  
Rajiv Verma ◽  
Delores Danilowicz ◽  
Stephen Colvin
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Aortic Arch ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Adolescent Female ◽  
Ventricular Septal Defects ◽  
Postoperative Course ◽  
Septal Defects ◽  
Complete Repair

AbstractCoarctations of the aortic arch are rare, and when found, they are often associated with other complex congenital heart disease. An adolescent female with multiple coarctations of the aortic arch and ventricular septal defects underwent complete repair, entailing replacement of the aortic arch with a homograft and closure of the ventricular septal defects. The postoperative course was complicated by a tear in the homograft, possibly related to sepsis. After its resolution, her hemodynamic results remain excellent.

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