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Congenital Heart Surgery Nomenclature and Database Project
Overview and Minimum Dataset

Table of Contents

Abstract
I. Introduction
II. Manuscripts Discussing Lesion Specific Nomenclature
III. Comprehensive Database Example
IV. Minimum Database Set
V. References
Appendix I. Minimum Database Data Set (PDF Format*)
Appendix II. Minimum Data Entry Form (PDF Format*)
Appendix III. Non-cardiac Abnormalities/General Preoperative Risk Factors Short List (PDF Format*)
Appendix IV. Diagnoses Short List (PDF Format*)
Appendix V. Procedures Short List (PDF Format*)
Appendix VI. Complications Short List (PDF Format*)
Appendix VII. European Congenital Heart Surgeons Foundation (PDF Format*)

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Constantine Mavroudis, M.D.
Professor of Surgery
Northwestern University Medical School
Division Head and A.C. Buehler Professor,
Division of Cardiovascular-Thoracic Surgery
Children's Memorial Hospital
Chicago, IL

Jeffrey P. Jacobs, M.D.
Division of Thoracic and Cardiovascular Surgery
All Children's Hospital
University of South Florida College of Medicine
St. Petersburg, FL

Address reprint requests and correspondence to:

Constantine Mavroudis, M.D.
Division of Cardiovascular-Thoracic Surgery
Children's Memorial Hospital
2300 Children's Plaza, m/c 22
Chicago, IL 60614
Telephone: 773.880.4378
FAX: 773.880.3054
E-mail: c-mavroudis@nwu.edu

Abstract

The International Congenital Heart Surgery Nomenclature and Database Project was organized for the purpose of standardizing nomenclature and reporting strategies which would establish the foundations for an international database. Worldwide representatives met for a series of conferences at which time issues of nomenclature were discussed and debated. Authors were chosen to review the various congenital heart diagnoses and reflect the mediated debate that followed. Manuscripts were prepared which reviewed the appropriate extant nomenclature, made recommendations for an inclusive rather than an exclusive method of reporting, and determined a hierarchial database scheme which would allow several levels of reporting based on the data input. This manuscript outlines two data sets for an international congenital heart surgery database, a minimum data set and a comprehensive data set. The comprehensive data set includes all the imagined variables, in a hierarchial scheme, which are detailed enough to generate risk stratification analyses. The minimum data set will include data points that would create an essential data set, which would be mandatory for data sharing and would lend itself to basic interpretation of trends. The minimum data set has four drop-down menus for short lists of 1) non-cardiac abnormalities / general preoperative risk factors, 2) diagnoses, 3) procedures, and 4) complications from which clinicians can chose for entry into the minimum data set. There was universal agreement for these data sets and short lists by the assembled members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. The data sets and short lists were also unanimously approved by the Congenital Heart Surgery Committee of The European Association for Cardiothoracic Surgery and adopted by the European Congenital Heart Surgeons Foundation.

I. Introduction

The idea of an International Congenital Heart Surgery Nomenclature and Database Project was conceived for a number of reasons. The development of the Society of Thoracic Surgeons-Summit Medical Systems National Congenital Heart Surgery Database and the obvious need for an international structure which would standardize nomenclature and reporting strategies were the important elements that propelled this initiative. The first reports of the STS-National Congenital Heart Surgery Database Committee were recently published [1,2] and included data from twenty-four centers which joined the program at various dates of entry between 1994 and 1997. There were 18,894 enrolled patient records from which 8,149 patient records were used to compile the relevant clinical features of eighteen congenital heart categories over the four-year period. Outcome data included operative death, complications, and length of stay, among others. Outcome analyses were segregated for age or weight at operation where appropriate, which varied from diagnosis to diagnosis. The data analysis was largely descriptive in character. Similar to the STS adult cardiac surgery database, this first report generated a massive amount of data. These data depicted many trends and were largely predictive of the established previous clinical reports from different centers. The analysis also demonstrated the strengths and weaknesses of a database, which, by necessity, limited the data input. On one hand, the four-page data form was readily available and concise; on the other hand, the information was limited and did not allow discriminating features that are necessary to establish risk stratification analysis. Unlike the STS adult cardiac surgery database, the STS Congenital Heart Surgery Database has numerous disease entities to analyze and by the nature of the subspecialty requires increased complexity in data analysis in order to produce meaningful risk stratification.

During the same time period, the European Congenital Heart Defects Database was founded through the European Congenital Heart Surgeons Foundation. By 1995, this database had collected data from 31 centers representing 18 countries. This data included the entire data set from four countries and gathered data on over 10,000 patients in the first 2 years. Uniform software was utilized for data collection, but the state of the art of data base software at that time was problematic. Like the first report of the STS-National Congenital Heart Surgery Database Committee (1), data analysis was largely descriptive in character. Furthermore, the data set was difficult to manage. This experience of the European Congenital Heart Defects Database of the early and mid 1990's also demonstrated the differences between data collection for adult heart surgery and congenital heart surgery. The European experience, like the STS experience, clearly demonstrated that the numerous disease entities cared for by congenital heart surgeons require increased complexity in data analysis in order to produce meaningful risk stratification.

As this initiative moves to an international focus, some agreement as to what to call things becomes imperative. The location of a lesion is not so controversial as to what it is called. What some call a conal VSD, others call a subarterial or type I VSD; what some call a type I truncus arteriosus, others call a type A1. Worse, in some cases, the classification schemes are not interchangeable and the scientific basis for the difference continues to be debated by well-established, well-intentioned, and well-known pediatric cardiac pathologists. Our solution to this problem is based on inclusion rather than exclusion. The contributing authors have reviewed the nomenclature literature on their assigned topic and made best efforts to include all the nomenclature schemes and integrate them by assigning the various appellations to the specific lesion, i.e. VSD, Type 1 (Subarterial VSD) (Supracristal VSD) (Conal septal defect)(Infundibular VSD). In this manner, different centers can use their own preference by simply placing the preferred version first. In time, a standardized version will evolve based on scientific principals and popular usage.

The inaugural efforts of the STS National Congenital Heart Surgery Database Committee and the European Congenital Heart Defects Database established the necessary foundation for future efforts to create an International Congenital Heart Surgery Database. The groundwork was created in areas of data organization for analysis. Important lessons learned from these initial databases will shape the development of the next generation database, which will be computer based in cyberspace rather than limited by the size of four sheets of paper. This will allow for the collection of increased data input, the elimination or at least reduction of missing data points, and the generation of data specific enough to possess discriminating features necessary to establish risk stratification analysis. This next generation database will need to meet the dual goals of facilitating multiinstitutional data analysis as well as providing data to support clinical programs, research, and teaching at individual institutions. The potential exists to create the first comprehensive international database for a medical subspecialty.

In order to meaningfully share data on a multi-institutional international level, two requirements must be met: minimum data sets for sharing data must be determined and a consensus on nomenclature terminology must be reached. This manuscript will outline two data sets for an international congenital heart surgery database, a comprehensive data set and a minimum data set. The comprehensive data set will include all imagined variables and be inclusive enough to generate detailed information. The minimum data set will include data points that would create an essential data set, which would be mandatory for data sharing and would lend itself to basic interpretation of trends. This minimum data set could still be useful for risk stratification, but in this case the comparative variables will be between different diagnoses - not within a given diagnoses. (For instance, the comprehensive database will be able to make risk stratification distinctions within the diagnosis of Truncus Arteriosus referable to truncal insufficiency, interrupted aortic arch, age at operation, coronary artery anomalies, etc. The minimum database, which will code all ASDs, VSDs, and Truncus Arteriosus as such without subcategories, can very effectively provide enough variables to make risk stratification distinctions between diagnoses such as comparing mortality and morbidity between ASD and VSD.)

The distinctions between the two approaches are obvious. On the one hand, the comprehensive database is time consuming and sophisticated which by virtue of its complexity increases the chance of error and heterogeneous reporting. If done correctly, however, the product is powerful and extremely informative. On the other hand, the minimum database is powerful in its simplicity and convenience. The chance of error is reduced, the chance of institutional compliance is increased and the cost of implementation, maintenance and manpower is reduced. (Of course, the minimum data set will not allow complex comparisons such as: postoperative arrhythmia's between sinus venosus ASDs and secundum ASDs, effect of coronary artery anatomy on the arterial switch operation, and the difference in complete heart block between inlet VSDs and outlet VSDs, etc.). Our purpose is to provide the necessary risk factors and data points for both types of databases. The key, of course, is to standardize what we call things so we can compare apples to apples and oranges to oranges regardless of the complexity of the respective database configuration.

The following manuscripts have been organized in a specific diagnosis-driven manner. The authors have: (1) reviewed and synthesized the nomenclature literature, (2) have determined a hierarchical scheme for data collection, and (3) offered potential database studies and outcome analyses specific for each diagnosis in tabular form. Each manuscript was presented at one or more of the following meetings:

Subcommittees for difficult subjects such as hypoplastic left heart syndrome, double outlet right ventricle, and single ventricle were organized to facilitate discussion.

In the end, agreement was reached by all participants on all topics, making the manuscripts all the more important. Those participating (in alphabetical order) are:

Carl L. Backer, M.D.

Chicago, IL

Redmond P. Burke, M.D.

Miami, FL

Duke Cameron, M.D.

Baltimore, MD

Andrew D. Cochrane F.R.A.C.S.

Melbourne, Australia

Victor F. Chu, M.D.

Monreal, Canada

Gordon Danielson, M.D.

Rochester, MN

Barbara J. Deal, M.D.

Chicago, IL

Joseph A. Dearani, M.D.

Rochester, MN

Ralph Delius, M.D.

Davis, CA

Martin J. Elliott, M.D., F.R.C.S.

London, England

J. William Gaynor, M.D.

Philadelphia, PA

René Herlong, M.D.

Durham, NC

Andreas Hoschtitzky, M.D.

London, England

Jeffrey Jacobs, M.D.

St. Petersburg, FL

Marshall L. Jacobs, M.D.

Philadelphia, PA

James Jaggers, M.D.

Durham, NC

Tom R. Karl, M.D.

Melbourne, Australia

François Lacour-Gayet, M.D.

Paris, France

John J. Lamberti, M.D.

San Diego, CA

Bohdan Maruszewski, M.D.

Warsaw, Poland

Constantine Mavroudis, M.D.

Chicago, IL

John E. Mayer Jr., M.D.

Boston, MA

John L. Myers, M.D.

Hershey, PA

Khanh H. Nguyen, M.D.

New York, NY

James A. Quintessenza, M.D.

St. Petersburg, FL

W. Steves Ring, M.D.

Dallas, TX

Albert P. Rocchini, M.D.

Ann Arbor, MI

Natalie Roy, M.D.

Montreal, Canada

Robert M. Sade, M.D.

Charleston, SC

Thomas L. Spray, M.D.

Philadelphia, PA

Jaroslav Stark, M.D.

London, England

Stephen A. Tahta

Montreal, Canada

Christo I. Tchervenkov, M.D.

Montreal, Canada

Ross M. Ungerleider, M.D.

Durham, NC

Henry L. Walters, M.D.

Detroit, MI

Paul Weinberg, M.D.

Philadelphia, PA

James L. Wilkinson, M.D.

Melbourne, Australia

The debt of gratitude that these authors and participants are owed is too much for any one person or guest editor to acknowledge. Their gratification is knowing that they performed an important task, which will significantly impact the way global information is shared well into the twenty-first century.

Special heartfelt thanks and noted appreciation are extended to Ms. Karen Graham, Project Coordinator, for her dedication and energy which drove this project to completion.

II. Manuscripts Discussing Lesion Specific Nomenclature

Subsequent manuscripts will present unified nomenclature proposals for the major disease entities of congenital heart surgery. A unified nomenclature proposal does not need to choose one classification proposal over another. Instead, a system can be developed allowing for congenital heart surgery centers utilizing any of the common nomenclature systems to meaningfully share data. This system would need to be inclusive of all nomenclature systems rather than exclusive. Furthermore, it should be hierarchial so that it permits each center to choose the level of detail to which it codes lesions and allows for the utilization of historical cases which might not be described and coded to the last level of the hierarchy. The myriad of more detailed coding choices is extremely manageable with the use of computer generated coding where only the subdivision of each hierarchy compatible with the previous hierarchial selection (one level above) is visualized at any time during data entry. Thus, long lists of diagnoses are available in drop-down menus in cyberspace while only short lists of coding possibilities are seen at any given time.

At any given level of coding, many surgeons might consider further description and classification into additional more detailed hierarchial choices as irrelevant. The advantage of this hierarchial nomenclature system is that these surgeons can stop at any level and still share data with other surgeons who choose to code in more detail. This more detailed coding might not play a role in multi-institutional data analysis, but it will clearly increase the clinical utility of the database at any given institution. Each lesion will be discussed utilizing the following format:

A. Background

A brief presentation of some of the more commonly utilized nomenclature systems for the lesion will be given.

B. Analysis: A Unified Nomenclature System

This section will present the hierarchial nomenclature for the diagnosis of given lesions. A proposal for standardized hierarchial nomenclature for the lesion will be given. Each new level of the hierarchy will be presented in a large box. Within this large box, smaller shaded boxes will indicate new coding choices given in this level of the hierarchy. Definitions of terms utilized in this hierarchial nomenclature system will be presented after each new level of the hierarchy. Only controversial or non-self-explanatory terms will be defined.

(In addition to the basic lesion based nomenclature given in this section, several other nomenclature issues must be addressed and coded in other areas of the database. Of primary importance, the segmental anatomy of the heart will need to be documented elsewhere in the database so that it is known when analyzing the lesion nomenclature. The database will offer the option of utilizing the segmental approach of Van Praagh and Vlad [3,4], the sequential segmental approach advocated by Anderson and Macartney [5,6,7], or both. The database will have a default to normal segmental anatomy as demonstrated below so that this information will not need to be re-entered for all cases with normal segmental anatomy.

The Van Praagh segmental approach documents the three major cardiac segments {atria, ventricles, great arteries} in a venoarterial sequence. Letters are coded in brackets {} to describe the visceroatrial situs [S = situs solitus, I = situs inversus, A = ambiguous], the ventricular loop [D = D-loop, L = L-loop]), and the great artery position [S = normally related great arteries, I = inverted normally related great arteries, D = D-transposition or D-malposition, and L = L-transposition or L-malposition] [8]. Thus, a normal heart would default to {S,D,S}.

Many surgeons and centers code segmental anatomy and connections by the sequential segmental approach advocated by Anderson. This approach has been applied utilizing the following terminology: visceroatrial situs [situs solitus, situs inversus, left isomerism, right isomerism], atrio-ventricular connection [Types: concordant, discordant, ambiguous, double inlet, absent right connection, or absent left connection; Modes: two perforate valves, single perforate valve, one perforate and one imperforate, and common valve], ventriculo-arterial connection [concordant, discordant [transposition], double outlet, single outlet - common arterial trunk, single outlet - solitary aortic trunk with pulmonary atresia, single outlet - solitary pulmonary trunk with aortic atresia, single outlet - solitary arterial trunk]. Although Anderson never described the side of the aortic arch in the sequential segmental analysis, many clinicians have added this descriptor to the sequential segmental approach: aortic arch side [L = left aortic arch, R = right aortic arch]. Thus, while the "Andersonian" approach never advocated an alphabetical shorthand, many clinicians would describe a normal heart as [SCCL or situs solitus, concordant atrio-ventricular connection, concordant ventriculo-arterial connection, left aortic arch].

C. Nomenclature for Treatment Options

A proposal for standardized hierarchial nomenclature for the lesion's treatment options will be given utilizing the format described above.

(In addition to the basic treatment options given in this section, several other therapeutic issues must be addressed and coded in other areas of the database. First, a separate module of the database must allow for coding of chest wall incisions [median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions {partial sternotomy, parasternal incision, "mini - thoracotomy"}, etc.]. Second, a separate part of the database must allow for coding of cardiac incisions [aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left atriotomy, left ventriculotomy, etc.]. Third, a separate module of the database must permit coding of patch materials [Dacron, Gore-Tex, Bovine pericardium. autologous pericardium, gluteraldehyde fixated autologous pericardium, etc.], conduit materials [Gore-Tex, Impra, Hemashield, etc,], homografts [fresh, chemically preserved, cryopreserved, etc,], valves [valve type and brand], pacemaker data, and AICD data.)

D. Diagnosis and Procedure Short Lists

Many centers may choose to utilize a list of diagnoses and procedures less cumbersome than the hierarchial nomenclature lists. A short list of 1) non-cardiac abnormalities / general preoperative risk factors, 2) diagnoses, 3) procedures, and 4) complications will be provided which will be compatible with the hierarchial nomenclature lists. For some lesions, this short list may be only the first level (hierarchy) of the hierarchial nomenclature list (rarer lesions such as AP window); while for other lesions, this list will be equivalent to the second or even third levels of the hierarchy (more common lesions such as ASD, VSD, or Single Ventricle).

This short list will allow data sharing between institutions coding lesions in great detail with those institutions coding to the short list only.

Editors' Note:

Personal communication with Professor Robert Anderson at the 13th Annual Meeting of The European Association for Cardiothoracic Surgery, Glasgow, Scotland, September 5-8, 1999 reveals that Professor Anderson prefers the term, "functionally single ventricle" rather than the term, "single ventricle" because these hearts generally have a functional single ventricle in addition to a diminutive or hypoplastic ventricle. We agree that the hearts which we classify as "single ventricle" in reality have a single well-developed ventricle and may also have an additional incomplete, rudimentary, or hypoplastic ventricle. Thus, our concept of "single ventricle" is consistent with Professor Anderson's concept of "functionally single ventricle". Our reluctance to using the term "functionally single ventricle" in this database scheme stems from the popular use of the term "single ventricle" in the surgical literature. As these initiatives progress, more debate by surgeons, anatomists, and pediatric cardiologists may result in nomenclature changes which will keep these initiatives as "works in progress" with the eventual goal of establishing a uniform nomenclature system across geographic boundaries and specialty preferences.

E. Potential Diagnostic Related (Lesion Specific) Risk Factors

Specific variables to be tracked related to a


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