Quality Improvement for the Treatment of Patients with Pediatric and Congenital Cardiac Disease: The Role of the Clinical Database

Nomenclature

Substantial effort has been devoted to the standardization of nomenclature and definitions related to surgery for pediatric and congenital cardiac disease. During the 1990s, both the European Association for Cardio-Thoracic Surgery (EACTS) and the Society of Thoracic Surgeons (STS) created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature and a common core minimal dataset were adopted by EACTS and STS and published in the Annals of Thoracic Surgery . In 2000, the International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD). By 2005, members of the ISNPCHD cross-mapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of the EACTS and STS with the European Paediatric Cardiac Code (EPCC) of the Association for European Paediatric Cardiology (AEPC), and therefore created the International Paediatric and Congenital Cardiac Code (IPCCC), which is available for free download at http://www.ipccc.net .

Most international databases of patients with pediatric and congenital cardiac disease use the IPCCC as their foundation. Two versions of the IPCCC are used in the overwhelming majority of multi-institutional databases throughout the world:

• 1

  The version of the IPCCC derived from the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of the EACTS and the STS

• 2

  The version of the IPCCC derived from the nomenclature of the EPCC of the AEPC

These two versions of the IPCCC are also often cited with the following abbreviated short names:

• 1

  EACTS-STS–derived version of the IPCCC

• 2

  AEPC-derived version of the IPCCC

The STS Congenital Heart Surgery Database, the EACTS Congenital Heart Surgery Database, and the Japan Congenital Cardiovascular Surgery Database (JCCVSD) all use the EACTS-STS–derived version of the IPCCC.

The ISNPCHD has published review articles that provide a unified and comprehensive classification, with definitions, for several complex congenital cardiac malformations: the functionally univentricular heart, hypoplastic left heart syndrome, discordant atrioventricular connections, and cardiac structures in the setting of heterotaxy. These review articles include definitions and a complete listing of the relevant codes and terms in both versions of the IPCCC.

In collaboration with the World Health Organization (WHO), the ISNPCHD is developing the pediatric and congenital cardiac nomenclature that will be used in the eleventh version of the International Classification of Diseases (ICD-11). With a grant funded by the Children's Heart Foundation ( www.childrensheartfoundation.org ), the ISNPCHD has also linked images and videos to the IPCCC. These images and videos are acquired from cardiac morphologic specimens and imaging modalities such as echocardiography, angiography, computerized axial tomography, and magnetic resonance imaging, as well as intraoperative images and videos. ^†† These images and videos are available for free download from the internet at ( http://www.IPCCC-awg.NET ). The IPCCC itself is available for free download from the internet at ( http://www.IPCCC.NET ).

†† References .

The EACTS-STS–derived version of the IPCCC, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are currently used by the STS Congenital Heart Surgery Database, the EACTS Congenital Heart Surgery Database, and the JCCVSD. Between 1998 and January 1, 2014 inclusive, this nomenclature and database was used by STS, EACTS, and JCCVSD to analyze the outcomes of 479,000 operations.

Database

The STS Congenital Heart Surgery Database is the largest database in North America cataloging congenital cardiac malformations. It has grown annually since its inception, both in terms of the number of participating centers submitting data and the number of operations analyzed ( Figs. 133-1, 133-2, and 133-3 ). As of January 1, 2014, the STS Congenital Heart Surgery Database currently had 111 participating centers, representing 120 hospitals performing pediatric and congenital cardiac surgery in North America: 117 of an estimated 125 centers from the United States of America that perform pediatric and congenital heart surgery and 3 of 8 centers from Canada that perform pediatric and congenital heart surgery. (The Report of the 2005 STS Congenital Heart Surgery Practice and Manpower Survey, undertaken by the STS Workforce on Congenital Heart Surgery, estimated that 122 centers in the United States of America perform pediatric and congenital heart surgery and 8 centers in Canada perform pediatric and congenital heart surgery. The Report of the 2010 STS Congenital Heart Surgery Practice and Manpower Survey, undertaken by the STS Workforce on Congenital Heart Surgery, estimated that 125 centers in the United States of America perform pediatric and congenital heart surgery and 8 centers in Canada perform pediatric and congenital heart surgery. )

As of January 1, 2014, the STS Congenital Heart Surgery Database therefore contained data from an estimated 93.6% of hospitals (117 of 125) performing pediatric cardiac surgery in the United States. With penetrance of greater than 90%, the data in the STS Congenital Heart Surgery Database is representative of pediatric and congenital heart surgery in the United States. As of January 1, 2014, the number of cumulative total operations in the STS Congenital Heart Surgery Database was 292,828. The aggregate Participant Feedback Report from the Fall 2013 Harvest of the STS Congenital Heart Surgery Database includes 136,617 operations performed in the 4-year analytic window of July 1, 2009 to June 30, 2013, inclusive, submitted from 120 hospitals in North America—117 in the United States and 3 in Canada. In collaboration with EACTS, the STS has developed standardized methodology for tracking mortality and morbidity associated with the treatment of patients with congenital and pediatric cardiac disease.

The EACTS Congenital Heart Surgery Database is the largest database in Europe dealing with congenital cardiac malformations ( Fig. 133-4 ). As of May 2013, the EACTS Congenital Heart Surgery Database contained 157,772 operations performed in 130,534 patients. As of May 2013, the EACTS Congenital Heart Surgery Database had 348 centers from 76 countries registered, with 173 active centers from 46 countries submitting data.

The JCCVSD has recently been operationalized based on nomenclature and database standards identical to those used by EACTS and STS. The JCCVSD began enrolling patients in 2008. By December 2011, more than 100 hospitals were submitting data, and by April 2013, more than 29,000 operations were entered into the JCCVSD, in just under 5 years of data collection ( Fig. 133-5 ). In Japan, it is mandatory for specialists to enroll in this benchmarking project to examine their own performance objectively and to make efforts for continuous improvement. In the future, certification is to be performed solely on the basis of empirical data registered by the project. The developers of the JCCVSD hope to collaborate with their colleagues across Asia to create an Asian Congenital Heart Surgery Database.

In the United Kingdom, the United Kingdom Central Cardiac Audit Database (UKCCAD) uses the AEPC-derived version of the IPCCC as the basis for its national, comprehensive, validated, and benchmark-driven audit of all pediatric surgical and transcatheter procedures undertaken since 2000. All 13 tertiary centers in the United Kingdom performing cardiac surgery or therapeutic cardiac catheterization in children with congenital cardiac disease submit data to the UKCCAD. Data about mortality are obtained from both results volunteered from the hospital databases, and by independently validated records of deaths obtained by the Office for National Statistics, using the patient's unique National Health Service number, or the general register offices of Scotland and Northern Ireland. Efforts are underway to link the UKCCAD to the EACTS Congenital Heart Surgery Database. Linkage of the UKCCAD to the EACTS Congenital Heart Surgery Database will require use of the cross-map of the AEPC-derived version of the IPCCC (used by the UKCCAD) to the EACTS-STS–derived version of the IPCCC (used by the EACTS, STS, and JCCVSD).

As of January 1, 2014, the STS Congenital Heart Surgery Database contains data from 292,828 operations, the EACTS Congenital Heart Surgery Database contains data from more than 157,772 operations, and the JCCVSD contains data from more than 29,000 operations. Therefore, the combined dataset of the STS Congenital Heart Surgery Database, the EACTS Congenital Heart Surgery Database, and JCCVSD contains data from more than 479,000 operations, all coded with the EACTS-STS–derived version of the IPCCC, and all coded with identical data specifications.