What are the indications for Pulmonic Valve Replacement (PVR) in an adult with a history of Tetralogy of Fallot (ToF) repair, and what are the benefits of open versus transcatheter approaches?

From the Guidelines

Pulmonary valve replacement (PVR) is indicated in this 24-year-old female with repaired tetralogy of Fallot due to her severe pulmonic valve regurgitation with right ventricular dilation. The primary indications for PVR in adults with repaired tetralogy of Fallot include severe pulmonary regurgitation with progressive right ventricular dilation, decreased right ventricular function, exercise intolerance, arrhythmias, or heart failure symptoms. In this patient's case, the severe pulmonic regurgitation with mild RV dilation warrants intervention before irreversible RV dysfunction develops. Both surgical and transcatheter PVR options exist, with transcatheter PVR offering advantages of avoiding sternotomy, cardiopulmonary bypass, shorter hospital stays, and quicker recovery, as noted in the guidelines comparison by 1. However, it requires suitable anatomy and may have higher rates of reintervention. Surgical PVR remains the gold standard, especially for patients with complex anatomy or those requiring additional repairs. A "watch and wait" approach is not recommended in this case given the severe regurgitation and RV dilation already present, as delaying intervention could lead to irreversible RV dysfunction, arrhythmias, and heart failure. The patient should undergo comprehensive evaluation including cardiac MRI to quantify RV volumes and function, exercise testing to assess functional capacity, and cardiac catheterization to evaluate hemodynamics before finalizing the PVR approach, as recommended by 1 and further supported by 1. Key considerations include:

• Severe pulmonic regurgitation with right ventricular dilation
• Progressive right ventricular dilation or dysfunction
• Exercise intolerance or arrhythmias
• Heart failure symptoms
• Suitability for transcatheter versus surgical PVR based on anatomy and other factors. Given the most recent and highest quality evidence from 1, transcatheter PVR is preferred over surgical PVR in patients with suitable anatomy to reduce the number of surgeries over a patient’s lifetime. However, the decision between transcatheter and surgical PVR should be individualized based on patient-specific factors, including anatomy, symptoms, and overall clinical condition, as emphasized by 1 and 1.

From the Research

Indications for Pulmonary Valve Replacement (PVR) in Adults with Tetralogy of Fallot

• Severe chronic pulmonary regurgitation can lead to symptomatic, irreversible right ventricular dilatation and dysfunction, making PVR a necessary intervention 2
• Pulmonary valve replacement is associated with male gender, older age at surgical repair, and longer QRS duration in adulthood 2
• Patients with significant pulmonary regurgitation are at risk for exercise intolerance, heart failure, arrhythmias, and sudden death, making PVR a crucial treatment option 3

Transcatheter Pulmonary Valve Replacement (TPVR)

• TPVR is a viable option for patients with severe pulmonary regurgitation and large right ventricular outflow tracts 4
• The Venus P-valve has been shown to be effective in improving ventricular function and reducing pulmonary regurgitation in patients with tetralogy of Fallot 5
• TPVR has been associated with durable relief of stenosis and regurgitation, and is considered a future gold standard intervention for pulmonary valve replacement 2, 6

Open vs Transcatheter PVR

• Transcatheter PVR is a less invasive option compared to open surgery, with benefits including reduced recovery time and decreased risk of complications 6
• However, open surgery may still be necessary for certain patients, such as those with complex anatomy or conduit rupture 6
• The choice between open and transcatheter PVR depends on individual patient factors and should be determined on a case-by-case basis 6

Watch and Wait Approach

• Severe chronic pulmonary regurgitation can be tolerated for decades, but if left untreated, can progress to symptomatic, irreversible right ventricular dilatation and dysfunction 2
• Regular monitoring and follow-up are necessary to determine the optimal timing for PVR, as the risk of complications increases with delayed intervention 3