What is the most common congenital heart defect?

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Most Common Congenital Heart Defect

Ventricular septal defect (VSD) is the most common congenital heart defect at birth, occurring in approximately 3.0 to 3.5 per 1000 live births. 1

Epidemiology and Context

  • VSD accounts for approximately 40% of all congenital heart malformations, making it definitively the most prevalent structural cardiac anomaly present at birth 2

  • VSDs are more frequent than other major congenital heart defects including atrial septal defects, transposition of the great arteries, tetralogy of Fallot, and aortic coarctation 1

  • Multiple research studies from high-quality journals confirm VSD as the most common congenital cardiac malformation 3, 4, 5

Important Clinical Distinction: Birth vs. Adulthood

A critical caveat exists when considering adults rather than newborns:

  • In adults, bicuspid aortic valve becomes the most common congenital heart anomaly, followed by atrial septal defect 6

  • This shift occurs because many VSDs close spontaneously during childhood (particularly muscular VSDs which have high spontaneous closure rates), while bicuspid aortic valves persist throughout life 7, 1

  • Approximately 10% of patients with common congenital heart defects survive undetected until adulthood 6

Anatomic Classification of VSDs

Perimembranous VSDs (Type 2) represent approximately 80% of all ventricular septal defects, making them the most common subtype 1

Other VSD types include:

  • Type 1 (Subarterial/Outlet): 6% in non-Asian populations, up to 33% in Asian patients 1
  • Type 3 (Inlet): Commonly associated with Down syndrome 1
  • Type 4 (Muscular): Often multiple with high spontaneous closure rates 1

Clinical Significance

  • Among adults presenting with unrepaired VSDs, manifestations include asymptomatic murmur, infective endocarditis, aortic regurgitation from valve prolapse, or cyanosis with exercise intolerance from pulmonary vascular disease 1

  • Tetralogy of Fallot, which includes a VSD as one of its four components, is noted as the most common complex congenital defect with the longest surgical history 7

References

Guideline

Ventricular Septal Defect Epidemiology and Classification

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Research

The Natural and Unnatural History of Ventricular Septal Defects Presenting in Infancy: An Echocardiography-Based Review.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 2020

Research

Ventricular septal defect.

Orphanet journal of rare diseases, 2014

Research

Ventricular septal defects.

Circulation, 2006

Research

[Patch Closure of the Ventricular Septal Defect].

Kyobu geka. The Japanese journal of thoracic surgery, 2024

Guideline

Most Common Cardiac Anomalies in Adults

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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