Can the Pulmonary Valve Be Replaced?
Yes, the pulmonary valve can be replaced, and pulmonary valve replacement is a well-established intervention performed both surgically and via transcatheter approaches for specific indications in patients with congenital heart disease. 1
Primary Indications for Pulmonary Valve Replacement
Symptomatic Patients with Pulmonary Regurgitation
Pulmonary valve replacement is recommended (Class I) in symptomatic patients with moderate or greater pulmonary regurgitation (PR) following treatment of isolated pulmonary stenosis who have right ventricular (RV) dilation or RV dysfunction. 1 Symptoms include:
- Dyspnea
- Chest pain
- Exercise intolerance attributable to PR 1
This recommendation reflects that valve replacement can improve functional class, reduce RV size, and improve RV function when performed in symptomatic patients. 1
Asymptomatic Patients with Progressive Disease
In asymptomatic patients with moderate or greater PR and progressive RV dilation and/or RV dysfunction, pulmonary valve replacement may be reasonable (Class IIb). 1 The rationale is that RV dilation or dysfunction should improve, or at least not progress further, if the volume overload from PR is alleviated. 1
Patients with Repaired Tetralogy of Fallot
Pulmonary valve replacement is most compelling when 2 or more of the following criteria are met: 1
- Mild or moderate RV or left ventricular (LV) systolic dysfunction
- Severe RV dilation (RV end-diastolic volume index ≥160 mL/m², or RV end-systolic volume index ≥80 mL/m², or RV end-diastolic volume ≥2x LV end-diastolic volume)
- RV systolic pressure due to right ventricular outflow tract (RVOT) obstruction ≥2/3 systemic pressure
- Progressive reduction in objective exercise tolerance
Available Valve Options
Surgical Valve Replacement
Both biological and mechanical valves can be used for pulmonary valve replacement. 2, 3, 4
Biological valves are most commonly used, especially in children, due to good durability and no need for anticoagulation, though structural valve deterioration is inevitable long-term. 2, 5, 4
Mechanical valves require lifelong anticoagulation but offer superior durability with freedom from reoperation of 97% at 5 years and 91% at 10 years. 4 Freedom from valvular thrombosis is 91% at 5 years and 86% at 10 years when proper anticoagulation is maintained. 4 Thrombolysis is successful in up to 88% of thrombotic events. 4
Transcatheter Pulmonary Valve Replacement
Transcatheter approaches are FDA-approved for specific anatomic situations and offer results at least equivalent to surgery in the short and mid-term. 5, 6
- Melody valve®: FDA-approved for failing pulmonary surgical conduits (2010) and failing bioprosthetic surgical pulmonic valves (2017). 5
- Sapien XT valve®: FDA-approved for failing pulmonary conduits (2016), offering larger diameters. 5
Critical Monitoring Requirements
Asymptomatic Patients
For asymptomatic patients with residual PR and a dilated right ventricle, serial follow-up is recommended (Class I). 1 This includes:
- Clinical evaluation
- Cardiopulmonary exercise testing (CPET)
- Echocardiography
- Advanced imaging (cardiac MRI or CT) to evaluate for symptoms, exercise intolerance, and/or RV dilation or dysfunction 1
Post-Replacement Surveillance
Patients with right ventricle-to-pulmonary artery conduits require evaluation for conduit complications, including fluoroscopy for stent fracture and blood cultures for infective endocarditis. 1
Important Caveats
If a patient has moderate or greater PR but normal RV size, the estimation of PR severity is likely inaccurate, or there may be restrictive RV physiology requiring further investigation. 1 Significant PR causes RV dilation by definition.
For patients with significant LV or RV dysfunction, pulmonary valve replacement may not be tolerated or sufficient; evaluation by adult congenital heart disease (ACHD) cardiologists and heart failure specialists is appropriate to determine if mechanical circulatory support or heart transplant is needed. 1
Pulmonary valve replacement alone has not consistently been demonstrated to reduce risk of ventricular tachycardia or sudden cardiac death, so additional interventions such as VT surgery and/or ICD implantation may be considered. 1