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Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
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The systemic and pulmonary circuits are crucial components of the circulatory system, working together to transport blood between the heart, lungs, and the rest of the body. The process begins with pulmonary circulation, where deoxygenated blood is pumped from the right ventricle to the lungs via the pulmonary trunk and arteries. Upon reaching the lungs, the blood becomes oxygenated and returns to the heart, specifically to the left atrium, via the pulmonary veins.
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Pulmonary Atresia and Ventricular Septal Defect: Definitions, Nomenclature, and Classification.

Christo I Tchervenkov1, Richard Tang1, Giles J Peek2

  • 1Division of Cardiovascular Surgery, The Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada.

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|February 6, 2025
PubMed
Summary

Pulmonary atresia with ventricular septal defect is classified based on pulmonary circulation anatomy. This classification, using 2021 IPCCC and ICD-11 standards, guides surgical approach and patient outcomes.

Keywords:
pulmonary atresiapulmonary atresia with ventricular septal defect (VSD)tetralogy of Fallottetralogy of Fallot with pulmonary atresia, pulmonary atresia with ventricular septal defect (VSD) and MAPCA(s), tetralogy of Fallot with pulmonary atresia and MAPCA(s)

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Area of Science:

  • Congenital cardiovascular malformations
  • Pediatric cardiology
  • Medical coding and classification

Background:

  • Pulmonary atresia with ventricular septal defect (PA+VSD) is a complex congenital heart defect.
  • Accurate definitions and classifications are crucial for patient management and research.
  • Existing classifications may not fully capture the nuances of pulmonary circulation in PA+VSD.

Purpose of the Study:

  • To provide updated definitions, nomenclature, and classification for PA+VSD.
  • To align with the 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and ICD-11.
  • To establish a classification system based on pulmonary circulation anatomy.

Main Methods:

  • Review and synthesis of definitions from 2021 IPCCC and ICD-11.
  • Analysis of the role of intracardiac anatomy versus pulmonary circulation in PA+VSD.
  • Development of a three-group classification system (Type A, B, C) based on pulmonary blood flow sources.

Main Results:

  • Tetralogy of Fallot with pulmonary atresia is a specific type of PA+VSD.
  • The presence of major aortopulmonary collateral arteries (MAPCA[s]) does not alter the primary definitions.
  • A classification system based on pulmonary circulation (native pulmonary arteries, MAPCA[s], or both) was established.

Conclusions:

  • The classification of PA+VSD should prioritize pulmonary circulation anatomy over intracardiac details.
  • The proposed classification (Type A, B, C) aids in determining surgical strategies and predicting outcomes.
  • Standardized nomenclature and classification are essential for advancing the understanding and treatment of PA+VSD.