Circulation: Cardiovascular Quality and Outcomes

CARDIOVASCULAR PERSPECTIVE

Towards a Unified Coding System for Congenital

Heart Diseases
Jason Chami , BSc; Geoff Strange, MD, PhD; Calum Nicholson, BSc (Hons); David S. Celermajer , MBBS (Hons), PhD, DSc

A
s congenital heart diseases (CHDs) are heterog-
enous, often complex and sometimes rare, many
constantly evolving coding systems have been
developed and used by CHD specialists, health care sys-
tems and databases. Unfortunately, these disparate cod-
ing systems mean that patient data are often siloed in
small institutional databases that are unable to be easily
combined or compared. To ensure continuity of care for
patients and improve our understanding of the burden
and outcomes of CHD, it is important to unify patient
data from different sources under a single classification
system. In the process of building a bi-national CHD reg-
istry in Australia and New Zealand, we aimed to iden-
tify the best coding system for our purposes and create
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Classification of Diseases (ICD), for example, which con-
tained no codes for CHD in its first version in 1900, con-
tained 73 codes in its tenth revision, and will contain 318
in its upcoming eleventh revision (Figure).1
Unfortunately, despite the efforts of the World Health
Organization to promote the use of ICD, the rapid pace
of research and the increasing digitization of medical
records quickly render each new revision insufficient
for the demands of modern electronic medical record
systems. Instead of being limited to the internation-
ally accepted standards, many national health services
have developed extensive addenda to the established
lists.2–6 Therefore, with increasing technological prog-
ress, the various coding systems for CHD lesions have

translation tables from all other coding systems in regu-
lar use worldwide.
Throughout the 1950s and 1960s, as pediatric car-
diology emerged as a sub-speciality, many countries
established their own pediatric cardiology and cardiac
surgery services. Soon after, to record and refer to stan-
dardized diagnoses, a variety of logical coding systems
for classification of CHDs were developed.1 The rarity
of many CHD lesions makes informative epidemiologi-
cal study difficult in single institutions, so regional and
national databases were set up, using the database tech-
nology available in the era.
Since then, technological advances in imaging (eg,
echocardiography) have led to greater understanding of
the anatomy of complex CHDs, necessitating more spe-
cific diagnostic coding systems to capture newly under-
stood variations. Thus, as small databases have swelled
in size and complexity, so too have the coding systems
they use. The World Health Organization’s International
grown further apart.
When patients, doctors, nurses, and other health work-
ers across the health care system use different codes to
describe the same disease, this poses a risk to appropri-
ate continuity of care. Indeed, without uniform diagnostic
codes, data cannot be easily shared or understood across
institutions or borders. For example, in Australia and New
Zealand, there are currently tens of thousands of CHD
cases siloed separately in institutional and regional data-
bases, classified using different systems of CHD coding.
This prevents combination or comparison and impedes
research and health services planning. Finally, databases
with patient information stored in free text without any
stated coding system are neither searchable by diagno-
sis, nor able to automatically manage follow-up sched-
ules based on disease severity, nor able to automatically
generate comprehensive patient summaries.
Unfortunately, CHD diagnostic coding is among the
most challenging in medicine because of the very large

Key Words: cardiology ◼ congenital heart disease ◼ registries ◼ technology

The opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.
Correspondence to: David Celermajer, MBBS (Hons), PhD, DSc, Royal Prince Alfred Hospital, Missenden Rd, Camperdown 2050, Sydney, Australia. Email david.
celermajer@health.nsw.gov.au
The Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCOUTCOMES.121.008216.
For Sources of Funding and Disclosures, see page 759.
© 2021 American Heart Association, Inc.
Circulation: Cardiovascular Quality and Outcomes is available at http://www.ahajournals.org/journal/circoutcomes

Circ Cardiovasc Qual Outcomes. 2021;14:e008216. DOI: 10.1161/CIRCOUTCOMES.121.008216 July 2021 757
 Chami et al
number of conditions, many of which overlap, combine or
are otherwise heterogeneous in nature. As an example,
a relatively straightforward CHD lesion is the ventricu-
lar septal defect: the commonest inborn structural heart
abnormality. Anatomically, there are at least 5 different
subtypes of ventricular septal defect, according to dif-
ferent anatomic locations within the ventricular septum.
Physiologically, the consequences of ventricular septal
defect vary according to size and the coexistence of up
to 10 different other CHDs, such as atrial septal defect,
transposition of the great arteries and coarctation of the
aorta. Many such combinations are common, but some
are exceedingly rare. Some combinations occur in rec-
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ognized patterns, such as Tetralogy of Fallot, while others
do not. This example serves to underscore the complexi-
ties of CHD classification and coding, even before one
considers the coding of operative interventions.
The ideal CHD coding system would be appropriately
detailed, universally adopted, and structured to allow
grouping of related defects. There should be enough
codes to describe defects with great specificity, but not
so much detail that excessive expertise or time commit-
ment is required on the part of coders themselves—a
system that is too cumbersome can lead various institu-
tions to create local Short Lists for their own purposes,
making comparison difficult. For research purposes, mis-
cellaneous codes should be avoided, as they encourage
nonspecific coding where more accurate codes are avail-
able. Conversion tables from existing coding systems
should be available and easy to automate.
Based on these criteria, we identified and evalu-
ated 5 coding systems for CHDs in widespread use
internationally: The International Statistical Classifica-
tion of Diseases and Related Health Problems, Ninth
Revision (ICD-9); ICD-10 and its national modifica-
tions; the European Paediatric Cardiac Code (EPCC),7
the Nomenclature System of the International Con-
genital Heart Surgery Nomenclature and Database
Project of the Society of Thoracic Surgeons and the
European Association for Cardio-Thoracic Surgery
Circ Cardiovasc Qual Outcomes. 2021;14:e008216. DOI: 10.1161/CIRCOUTCOMES.121.008216
Unified Coding for Congenital Heart Disease
Figure. Number of base congenital
heart disease (CHD) codes in a
selection of popular international
CHD coding systems.
EPCC indicates European Paediatric
Cardiac Code; ICD, International
Classification of Diseases; and STS-
ECATS, Society of Thoracic Surgeons
and the European Association for Cardio-
Thoracic Surgery.
(STS–EACTS),8,9 and the International Paediatric and
Congenital Cardiac Code (IPCCC).1
ICD-9 was ratified by the World Health Organiza-
tion in 1978 and grew in popularity along with some of
the very first large-scale electronic health databases. It
had only 29 CHD codes. ICD-9 was replaced by ICD-
10 in 1994 in response to a growing need for more
detailed clinical data as electronic medical records
grew in size and importance. With 73 codes for CHD,
it is more detailed than ICD-9 but not detailed enough
for modern research and administrative use. The Clini-
cal Modification (ICD-10-CM), adopted in the United
States in 2015, has >70 000 codes, several times more
than standard ICD-10.4 The Australian Modification
(ICD-10-AM) used since 1998 in Australia and New
Zealand also has added detail for clinical databases.3
Due to these regional modifications, international com-
parison and collaboration remains difficult.
EPCC and STS–EACTS were published near-simul-
taneously in 2000, both of which included several hun-
dred base codes and the ability to code much more
detailed diagnoses than ICD-10.7–9 The codes were
organized in a branching tree structure, meaning that
highly detailed diagnoses could be grouped together in
cases of low patient numbers—a common scenario for
CHD research. For convenience, they both came with
premade Short Lists to ensure uniformity across use
cases, but as direct competitors the codes needed to
be harmonized to achieve the aim of a universal CHD
coding system. By 2005, IPCCC had been created as
a one-to-one map between EPCC and STS–EACTS.1
Over time, as IPCCC has been updated with newly
described defects, these updates were pushed through
to the EPCC and STS–EACTS Short Lists, which are
now described as short lists of IPCCC itself, with the
EPCC Short List (EPCC-SL) having a more clinical
focus, while the STS–EACTS Short List (STS–EACTS-
SL) is surgically oriented.
Currently in development, ICD-11 will contain a
CHD section based directly upon the IPCCC, with 318
July 2021 758
 Chami et al
codes for CHD alongside extension codes for clinical
and administrative use that allow institutions to tailor
the system to their needs without affecting the base
codes.
When considering all aspects, including clarity,
detail, structure, and universality, we formed a view that
EPCC-SL has the optimal combination of features.
With a tree-structured code system, EPCC-SL avoids
the pitfalls of miscellaneous codes that are found in
droves in ICD-10-CM and ICD-10-AM, obscuring true
diagnoses in research databases. Unlike EACTS–STS-
SL, which is more surgically oriented, EACTS–SL has
a clinical focus that is ideal for use in our large-scale
clinical database. Furthermore, EPCC-SL is mapped
to the IPCCC Long List, which will form the basis of
the upcoming ICD-11. This allows not only for easy
upgrade when the time comes, but also the potential
for true universality. Finally, with around 600 codes
relevant to a CHD database, EPCC-SL contains suf-
ficient but not excessive detail for use in both hos-
pital administration and research, while allowing for
fast data entry and recall in a clinical setting. IPCCC
Long List, in contrast, is extremely detailed, containing
>11 000 unique codes—this is clearly too cumbersome
for routine use and requires considerable expertise by
the coders.
For these reasons, EPCC-SL will serve as the base
code for the upcoming bi-national ANZ CHD Registry,
which is being implemented as a relational database in
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FileMaker Pro (Claris International Inc, Cupertino USA).
This centralized database will be populated periodically
with data from each participating institution, each of which
will use the locally favored CHD coding system. To har-
monize the incoming data, we have developed a lookup
table which can be used to instantly translate codes from
ICD-9, ICD-10, ICD-10-AM, and STS–EACTS-SL into
EPCC-SL that is used in the Registry itself. This lookup
table is easily accessible and modifiable, so any mistakes
in translation can be easily corrected throughout the
database. Furthermore, extra translation protocols (eg, to
ICD-11 when it is published) can easily be added and
pushed through the whole database. The entire transla-
tion table is provided in Table I in the Data Supplement.
In general, we faced 2 types of problems when creat-
ing the translation table. One to many translations were
very common when translating from coding systems
lacking detail into EPCC-SL. For example, the ICD-
10-AM diagnosis of double outlet right ventricle (Q20.1)
could match up to 5 more specific codes in EPCC-SL.
Nonetheless, the tree structure of EPCC-SL allows us
to simply select a coarser description that matches the
level of detail of the imported code (Table II in the Data
Supplement). Many to one problems were less com-
mon, given the relatively high level of detail in EPCC-
SL. These generally occurred when the source code had
redundant miscellaneous codes that had no equivalent
Circ Cardiovasc Qual Outcomes. 2021;14:e008216. DOI: 10.1161/CIRCOUTCOMES.121.008216
Unified Coding for Congenital Heart Disease
in EPCC-SL (Table III in the Data Supplement). In some
cases, the dividing line between certain very closely
related conditions is difficult to draw with certainty, such
as with the variants of hypoplastic left heart syndromes.
Despite these difficulties, it was possible to create a
translation table that accurately converts ICD-9, ICD-10,
ICD-10-AM, and STS–EACTS-SL codes into EPCC-SL.
In conclusion, we propose that EPCC-SL is the most
suitable coding system for national or international CHD
registries. Furthermore, it is possible to create transla-
tion tables that match up any common congenital cardiac
codes to EPCC-SL for storage in a central database with
both clinical and research capabilities. Future directions
include the possibility of coding for common operations
(individual or serial) and developing more sophisticated
coding approaches in patients with multiple coexisting
CHD lesions. We acknowledge that almost all research
and health institutions are currently committed to 1 or 2
locally favored coding systems and that change would be
difficult. We hope that the translation protocols included
in the Data Supplement could ease this transition, and
that the clear benefits of coding harmonization for inter-
national cooperation make this endeavour worthwhile for
health care systems.
ARTICLE INFORMATION
Affiliations
Sydney Medical School, University of Sydney, Camperdown, NSW, Australia (J.C.).
School of Medicine, University of Notre Dame Australia, Freemantle, WA, Austra-
lia (G.S.). Heart Research Institute, Newtown, NSW, Australia (C.N.). Royal Prince
Alfred Hospital, Camperdown NSW, Australia (D.S.C.).
Acknowledgments
We would like to acknowledge Andrew McCallum for his work on the FileMaker
Pro database housing the new bi-national ANZ CHD Registry. We also thank
Drs Clare O’Donnell, Robert Weintraub, Mark Dennis, David Baker, and Michael
Cheung for their work producing the coding system translation table available in
the Data Supplement.
Sources of Funding
The development of a comprehensive ANZ CHD Registry, and the diagnosis
coding solutions described, was initially funded by philanthropic donations from
HeartKids Australia and the Kinghorn Foundation. Additional funding has been
provided by an Australian Department of Health grant through the Medical Re-
search Future Fund, grant code is ARGCHDG0000028.
Disclosures
None.
Supplemental Materials
Supplemental Tables I–III
REFERENCES
1. Franklin RCG, Béland MJ, Colan SD, Walters HL, Aiello VD, Anderson RH,
Bailliard F, Boris JR, Cohen MS, Gaynor JW, et al. Nomenclature for con-
genital and paediatric cardiac disease: the International Paediatric and Con-
genital Cardiac Code (IPCCC) and the Eleventh Iteration of the International
Classification of Diseases (ICD-11). Cardiol Young. 2017;27:1872–1938.
doi: 10.1017/S1047951117002244
2. Graubner B. ICD-10-GM 2014 Systematisches Verzeichnis: Inter-
nationale statistische Klassifikation der Krankheiten und verwandter
July 2021 759
 Chami et al
Gesundheitsprobleme 11. Revision - German Modification Version 2014.
Deutscher Ärzteverlag; 2013.
3. Roberts RF, Innes KC, Walker SM. Introducing ICD-10-AM in Australian
hospitals. Med J Aust. 1998;169(S1):S32–S35. doi: 10.5694/j.1326-
5377.1998.tb123473.x
4. National Center for Health Statistics. ICD-10-CM Official Guidelines for Coding and
Reporting. Center for Disease Control and Prevention. Hyattsville, MD; 2021.
5. Canadian Institute for Health Information. The Canadian Enhancement of
ICD-10. 2001.
6. Nitsuwat S, Paoin W. Development of ICD-10-TM ontology for a semi-auto-
mated morbidity coding system in Thailand. Methods Inf Med. 2012;51:519–
528. doi: 10.3414/ME11-02-0024
Downloaded from http://ahajournals.org by on June 6, 2023
Circ Cardiovasc Qual Outcomes. 2021;14:e008216. DOI: 10.1161/CIRCOUTCOMES.121.008216
Unified Coding for Congenital Heart Disease
7. Franklin R. The European paediatric cardiac code long list: structure and
function — the first revision. Cardiol Young. 2002;12(S2):9–17.
8. Mavroudis C, Jacobs JP. Congenital heart surgery nomenclature
and database project: overview and minimum dataset. Ann Tho-
rac Surg. 2000;69(4 suppl):S2–17. doi: 10.1016/s0003-4975(99)
01321-1
9. Béland MJ, Jacobs JP, Tchervenkov CI, Franklin RC; International Work-
ing Group for Mapping and Coding of Nomenclatures for Paediatric and
Congenital Heart Disease. Report from the executive of The International
Working Group for Mapping and Coding of Nomenclatures for Paediatric
and Congenital Heart Disease. Cardiol Young. 2002;12:425–430. doi:
10.1017/s1047951102000732
July 2021 760