From the Guidelines
The pulmonic valve is frequently removed during surgical repair of Tetralogy of Fallot, as it is often stenotic or malformed and needs to be replaced to ensure proper blood flow to the lungs. This procedure, called a pulmonary valvectomy, is often performed as part of the complete repair when there is significant pulmonary stenosis obstructing blood flow to the lungs 1. The surgeon typically removes the stenotic valve and places a transannular patch across the right ventricular outflow tract and pulmonary artery to widen the narrowed area. While removing the valve improves immediate blood flow to the lungs, it results in pulmonary regurgitation (backward flow of blood) 1.
Key Considerations
- The decision to remove the valve depends on the specific anatomy of each patient, with surgeons attempting to preserve valve function when possible 1.
- However, in many cases of Tetralogy of Fallot, the pulmonic valve is too stenotic or malformed to preserve, making valvectomy a necessary component of the repair 1.
- Late survival after tetralogy repair is excellent, with 35-year survival approximately 85%, but the need for reintervention, usually for pulmonary valve insertion, increases after the second decade of life 1.
- Surgical intervention is indicated for symptomatic patients with severe pulmonary regurgitation or asymptomatic patients with severe PS or pulmonary regurgitation in association with signs of progressive or severe RV enlargement or dysfunction 1.
Management and Outcomes
- Pulmonary valve replacement is reasonable in adults with previous tetralogy of Fallot, severe pulmonary regurgitation, and any of the following: moderate to severe RV dysfunction, moderate to severe RV enlargement, development of symptomatic or sustained atrial and/or ventricular arrhythmias, or moderate to severe TR 1.
- Collaboration between ACHD surgeons and ACHD interventional cardiologists is reasonable to determine the most feasible treatment for pulmonary artery stenosis 1.
From the Research
Pulmonic Valve Removal in Tetralogy of Fallot
- The pulmonic valve is not frequently removed for patients with Tetralogy of Fallot, as pulmonary valve-sparing repair is preferable 2.
- However, in some cases, the pulmonary valve may need to be replaced due to severe pulmonary regurgitation, which can occur after complete repair of Tetralogy of Fallot 3, 4, 5, 6.
- The decision to replace the pulmonary valve is typically made on a case-by-case basis, taking into account factors such as the severity of pulmonary regurgitation, symptoms, and right ventricular function 4, 6.
- Recent studies suggest that pulmonary valve replacement can be beneficial in asymptomatic patients with significant pulmonary regurgitation, and that transcatheter pulmonary valve implantation may become the future gold standard intervention 4, 6.
Indications for Pulmonary Valve Replacement
- Severe pulmonary regurgitation is a common indication for pulmonary valve replacement in patients with Tetralogy of Fallot 3, 4, 5, 6.
- Symptoms such as heart failure, exercise intolerance, and arrhythmias may also indicate the need for pulmonary valve replacement 4, 5.
- Right ventricular dysfunction and dilatation are also important considerations in the decision to replace the pulmonary valve 3, 5, 6.
Timing of Pulmonary Valve Replacement
- The timing of pulmonary valve replacement is not well established, but it is generally recommended to replace the valve before the development of severe right ventricular dysfunction 4, 5, 6.
- Recent studies suggest that pulmonary valve replacement can be beneficial in asymptomatic patients with significant pulmonary regurgitation, and that the decision to replace the valve should be made on a case-by-case basis 4, 6.