Interconnected puzzle pieces forming a heart, symbolizing CHD diagnosis and treatment in Canada.

Decoding Congenital Heart Disease: A Canadian Guide to Standardized Care

Reese Marlowe in Health & Wellness November 2025 • 4 min read.

"How Canada is leading the way in standardizing nomenclature for congenital heart disease research and treatment to improve patient outcomes."

Congenital heart disease (CHD), the most common type of birth defect, presents significant challenges in diagnosis, treatment, and research due to its wide range of phenotypes. The complexity of CHD demands a standardized approach to ensure accurate classification and effective care. Addressing this need, Canada is taking proactive steps to standardize the nomenclature used in CHD diagnosis and research, aiming to improve outcomes and advance the understanding of this condition nationwide.

With advances in medical care, survival rates for individuals with CHD have increased, leading to a growing adult population with congenital heart defects. This demographic shift underscores the importance of consistent and comprehensive data collection to inform healthcare policy and improve patient care across the lifespan. Canada, leveraging its universal health insurance system and unique patient identifiers, is uniquely positioned to conduct large-scale longitudinal studies to enhance outcomes for individuals with CHD.

However, the absence of a uniformly accepted nomenclature for CHD across Canadian healthcare centers and cardiac sub-specialties has hindered collaborative research and data sharing. Recognizing this obstacle, key organizations such as the Canadian Pediatric Cardiology Association (CPCA), the Canadian Adult Congenital Heart Network (CACH), and the Canadian Pediatric Cardiology Research Network are collaborating to establish a standardized nomenclature. This initiative aims to facilitate Canada-wide research cohorts, enhance the quality of care, and solidify Canada's leadership in CHD research.

Why Standardized Nomenclature Matters: Boosting CHD Research

Interconnected puzzle pieces forming a heart, symbolizing CHD diagnosis and treatment in Canada.

Historically, the nomenclature for CHD has evolved differently among various medical specialties, including fetal, pediatric, and adult cardiology, as well as cardiac morphology and cardiovascular surgery. Many hospitals still rely on center-specific nomenclature for clinical coding, further complicating data sharing and collaborative research efforts. While Canadian administrators use the International Classification of Diseases (ICD) of the World Health Organization (WHO) for coding purposes, clinicians often find these lists lack the necessary scope, precision, and clinical relevance.

In the 1990s, the Canadian Pediatric Cardiology Association (CPCA) attempted to standardize CHD nomenclature by developing a list of terms distinct from the ICD. However, this effort was not finalized or widely adopted. The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD) began creating a more definitive list in the early 2000s, eventually named the International Paediatric and Congenital Cardiac Code or IPCCC.

IPCCC: The IPCCC consists of thousands of terms for diagnoses and procedures related to CHD.
Unique Codes: Each term is linked to a unique six-digit numerical code to facilitate cross-mapping to other lists, including those in different languages.
Adoption: The CACH Network, the Western Canadian Children's Heart Network, and various surgical groups have adopted portions of the IPCCC for coding purposes.
Challenges: The IPCCC "long list" is unwieldy in its detail, making it less conducive to uniform coding.

Recognizing the clinical shortcomings of previous versions of the International Classification of Diseases (ICD), the WHO involved more clinicians in constructing its newest version, ICD-11. The ISNPCHD was invited to participate and devise a list of CHD terms for ICD-11 that would meet the needs of clinicians in the field.

Moving Forward: The Future of CHD Care in Canada

The adoption of a common nomenclature for CHD across Canada will facilitate inter-institutional studies and improve the quality of CHD research, particularly as big data sources become increasingly available. This unified approach will capitalize on Canada's healthcare system and well-characterized data sources to enhance Canadian leadership in CHD research, ultimately improving patient outcomes and advancing the understanding of congenital heart disease.

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Everything You Need To Know

What is the primary goal of standardizing nomenclature in the context of Congenital Heart Disease (CHD) in Canada?

The primary goal of standardizing nomenclature in CHD in Canada is to improve patient outcomes and advance the understanding of the condition nationwide. This is achieved through more accurate classification, effective treatment, enhanced collaborative research, and improved data sharing across healthcare centers and specialties. The standardization aims to create a unified approach that capitalizes on Canada's healthcare system and data sources to bolster Canadian leadership in CHD research.

How does the International Paediatric and Congenital Cardiac Code (IPCCC) contribute to CHD nomenclature standardization, and what are its limitations?

The IPCCC provides a comprehensive list of thousands of terms for diagnoses and procedures related to CHD, each associated with a unique six-digit numerical code. This system is designed to facilitate cross-mapping to other lists, including those in different languages. The CACH Network, the Western Canadian Children's Heart Network, and several surgical groups have integrated parts of the IPCCC into their coding practices. However, a significant limitation of the IPCCC is its extensive detail, which makes it challenging to apply uniformly across all clinical settings.

Why is it important to standardize CHD nomenclature across different medical specialties and institutions in Canada?

Standardizing CHD nomenclature is crucial for several reasons. Historically, nomenclature has varied across specialties like fetal, pediatric, and adult cardiology, as well as cardiac morphology and cardiovascular surgery. This variation has hindered data sharing and collaborative research. A unified approach facilitates inter-institutional studies, enhances the quality of research, and allows for the effective use of big data sources. By aligning on a common language, healthcare providers can share information more easily, leading to improved patient care and advancements in CHD research.

How are Canadian organizations like the CPCA, CACH, and others collaborating to address the challenges of CHD nomenclature, and what are they aiming to achieve?

Several key organizations in Canada are actively working to standardize CHD nomenclature. The Canadian Pediatric Cardiology Association (CPCA), the Canadian Adult Congenital Heart Network (CACH), and the Canadian Pediatric Cardiology Research Network are collaborating on this effort. Their primary goals include establishing a standardized nomenclature to facilitate Canada-wide research cohorts, improving the quality of care, and reinforcing Canada's leadership in CHD research. Their combined efforts will facilitate the adoption of unified diagnostic and procedural codes.

In what ways is Canada uniquely positioned to advance CHD research and improve patient outcomes through standardized nomenclature and data collection?

Canada's universal health insurance system and unique patient identifiers provide a strong foundation for large-scale longitudinal studies in CHD. The standardization of nomenclature will allow for the effective use of these advantages. By unifying the diagnostic and procedural language, Canada can leverage its well-characterized data sources to conduct comprehensive research, facilitate inter-institutional collaboration, and ultimately enhance outcomes for individuals with CHD. This strategic approach underscores Canada's opportunity to lead in CHD research and improve patient care across the lifespan.