Asymptomatic Tetralogy of Fallot with Severe Pulmonary Regurgitation and/or Right Ventricular Outflow Tract Obstruction
Clinical Scenario
This protocol addresses patients who are currently asymptomatic but present with severe pulmonary regurgitation (PR) and/or right ventricular outflow tract obstruction (RVOTO), together with objective evidence of at least one of the following progressive findings:
- Decrease in objective exercise capacity
- Progressive RV dilation — RVESVi ≥80 mL/m² and/or RVEDVi ≥160 mL/m² — and/or progression of tricuspid regurgitation to at least moderate
- Progressive RV systolic dysfunction
- RVOTO with RVSP >80 mmHg
Treatment Approach
In this setting, pulmonary valve replacement is the intervention warranting consideration. The full structured regimen — selection criteria, procedural pathway, and evidence grading — is detailed in the complete protocol.
Partial summary only. Specific clinical decision criteria are available in the full regimen below.
Clinical Goals
The primary aims are improvement of symptoms and reduction of right ventricular volume.
References
- PVRep should be considered in asymptomatic patients with severe PR and/or RVOTO when one of the following criteria is present.
- Decrease in objective exercise capacity.
- Progressive RV dilation to RVESVi ≥80 mL/m², and/or RVEDVi ≥160 mL/m², and/or progression of TR to at least moderate.
- Progressive RV systolic dysfunction.
- RVOTO with RVSP >80 mmHg.
- A recent meta-analysis demonstrated that PVRep can improve symptoms and reduce RV volume, but a survival benefit still needs to be shown.
DOI: 10.1093/eurheartj/ehaa554
View source ↗