Originally named The International Nomenclature Committee for Paediatric and Congenital Heart Disease, The ISNPCHD was formed in Frankfurt, Germany in October 2000 to explore the possibility of establishing a common international system of nomenclature for congenital heart disease. The Nomenclature Committee was established after the near-simultaneous publication of two complementary systems of nomenclature for congenital cardiac malformations and their procedure-based treatments in early 2000:

• The European Paediatric Cardiac Code (EPCC) of the Association for European Paediatric Cardiology (AEPC);
• The nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of the Society of Thoracic Surgeons (STS)

This led to the problematic situation of having two lexicons that were to be widely adopted, with the potential risks of invalidating multi-center projects due to confusion between the two systems and duplicate or inaccurate entries within institutions. Both systems of nomenclature followed the same basic structure:

• A comprehensive Long List (thousands of terms)
• A Short List (~650 terms), designed to be used as part of a minimum dataset
• Long Lists mapped fully to the Short Lists

The Frankfurt meeting of interested parties led to an agreement to establish the Nomenclature Committee with an aim to produce a bidirectional map between the two systems. This work was then presented and endorsed at the Third World Congress of Pediatric Cardiology and Cardiac Surgery in Toronto, Canada on May 27, 2001, during the First International Summit on Nomenclature for Congenital Heart Disease.