What is the significance of a split S1 heart sound in Tetralogy of Fallot (TOF)?

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Last updated: March 13, 2025View editorial policy

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From the Guidelines

A split S1 heart sound is not a characteristic finding in Tetralogy of Fallot (TOF) and is not related to the pathophysiology of the condition. The first heart sound (S1) represents closure of the mitral and tricuspid valves, and while splitting of S1 can occur normally, it is not a defining feature of TOF. Instead, TOF is characterized by four key abnormalities: pulmonary stenosis, ventricular septal defect, overriding aorta, and right ventricular hypertrophy, as noted in the management guidelines for adults with congenital heart disease 1. The classic auscultatory findings include a harsh systolic ejection murmur at the left upper sternal border due to pulmonary stenosis and often a single S2 heart sound (rather than the normal splitting) because pulmonary valve closure is delayed or inaudible.

Some key points to consider in the management of TOF include:

  • Regular follow-up with a cardiologist who has expertise in adult congenital heart disease (ACHD) is essential, with the frequency of follow-up determined by the extent and degree of residual abnormalities 1
  • Key postoperative issues in TOF include residual pulmonary regurgitation, RV dilation and dysfunction, residual RVOT obstruction, and branch pulmonary artery stenosis or hypoplasia 1
  • The most common problem encountered in the adult patient after repair is that of pulmonary regurgitation, which can lead to significant morbidity and mortality if not properly managed 1

If a split S1 is heard in a patient with TOF, it would be an incidental finding rather than related to the pathophysiology of the condition. The hemodynamic abnormalities in TOF primarily affect the second heart sound and create characteristic murmurs, while the first heart sound typically remains normal or may be accentuated due to the right ventricular hypertrophy. Therefore, the presence of a split S1 heart sound should not be used as a diagnostic criterion for TOF, and other clinical and echocardiographic findings should be relied upon for diagnosis and management.

From the Research

Significance of Split S1 Heart Sound in Tetralogy of Fallot (TOF)

  • The split S1 heart sound is not directly mentioned in the provided studies as a significant factor in Tetralogy of Fallot (TOF) 2, 3, 4, 5, 6.
  • However, the studies discuss the anatomical features and diagnostic criteria of TOF, which include a large ventricular septal defect, overriding aorta, right ventricular outflow obstruction, and right ventricular hypertrophy 4.
  • The presence of a split S1 heart sound is typically associated with other cardiac conditions, such as atrioventricular septal defects or mitral valve problems, but its significance in TOF is not explicitly stated in the provided evidence.
  • The studies focus on the surgical repair, follow-up, and long-term management of TOF, highlighting the importance of early total correction and careful monitoring for potential complications and residual lesions 2, 3, 4, 5, 6.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Echocardiographic evaluation of tetralogy of Fallot.

Echocardiography (Mount Kisco, N.Y.), 2015

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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