What is the management approach for an adult patient with a history of congenital heart disease or cardiac surgery and pulmonary regurgitation?

Management of Pulmonary Regurgitation in Adults with Congenital Heart Disease

Pulmonary valve replacement is indicated for severe pulmonary regurgitation when patients develop symptoms, decreased exercise tolerance, moderate-to-severe RV dysfunction or enlargement, or sustained arrhythmias. 1

Initial Assessment and Monitoring

Diagnostic Evaluation

• Echocardiography should assess PR severity, RV size and function, tricuspid regurgitation severity, and exclude residual hemodynamic lesions 1
• Cardiac MRI is the investigation of choice for quantifying RV size, function, and pulmonary regurgitation severity when echocardiographic data are inadequate 1
• ECG should evaluate QRS duration, as QRS >180 ms correlates with increased ventricular tachycardia risk and severe RV dysfunction 1
• Exercise testing objectively assesses functional capacity and screens for exertional arrhythmias 1
• Holter monitoring is indicated when hemodynamics are poor or symptoms suggest arrhythmias 1

Surveillance Intervals

• Patients with mild PR and normal RV size require follow-up every 3-5 years 1
• Those with moderate-to-severe PR require annual evaluation with ECG, echocardiography, and consideration of Holter monitoring or exercise testing 1
• Repeat imaging every 1-2 years is necessary when PR burden is high to monitor for progressive RV dysfunction 1

Indications for Pulmonary Valve Replacement

Class I Indications (Definitive)

Symptomatic patients with severe pulmonary regurgitation and any of the following warrant immediate intervention 1:

• Symptoms of heart failure or decreased exercise tolerance
• Progressive decline in functional capacity

Class IIa Indications (Reasonable)

Asymptomatic patients with severe pulmonary regurgitation should undergo valve replacement when any of these criteria are met 1:

• Moderate-to-severe RV dysfunction
• Moderate-to-severe RV enlargement
• Development of symptomatic or sustained atrial/ventricular arrhythmias
• Moderate-to-severe tricuspid regurgitation

Additional Surgical Considerations

Intervention is reasonable for 1:

• RVOT obstruction with peak gradient >50 mmHg
• RV/LV pressure ratio >0.7
• Residual VSD with left-to-right shunt >1.5:1
• Severe aortic regurgitation with symptoms or LV dysfunction
• Combination of multiple residual lesions causing RV enlargement or dysfunction

Treatment Options

Surgical Pulmonary Valve Replacement

• Surgeons with training and expertise in congenital heart disease must perform all operations 1
• Coronary artery anatomy must be determined preoperatively, specifically excluding anomalous anterior descending coronary artery across the RVOT 1
• Bioprosthetic valves demonstrate 90% freedom from reoperation at 10 years 1
• Surgical approach allows concurrent procedures including arrhythmia surgery, residual defect closure, or pulmonary artery reconstruction 1, 2

Transcatheter Pulmonary Valve Replacement

• Emerging as valuable non-surgical alternative for appropriate anatomy 3
• Long-term results comparable to surgical outcomes 3
• Collaboration between ACHD surgeons and interventional cardiologists determines optimal treatment approach 1
• Particularly suitable for conduit dysfunction or selected native RVOT cases 3

Medical Management

Pharmacotherapy

• Most patients require no regular medication in the absence of significant residual hemodynamic abnormality 1
• Heart failure medications (ACE inhibitors, beta-blockers, diuretics) are indicated when RV or LV dysfunction develops 1
• Diuretics treat elevated right-sided pressures, edema, and ascites when present 1

Arrhythmia Management

• Development of atrial or ventricular arrhythmias mandates search for underlying hemodynamic abnormality (commonly pulmonary regurgitation) 1
• Implantable cardioverter-defibrillators are indicated for documented sustained VT or cardiac arrest 1
• Consider electrophysiology study for symptomatic patients with palpitations, dizziness, or syncope 1

Critical Pitfalls to Avoid

Diagnostic Errors

• The murmur of pulmonary regurgitation is easily missed because it is soft and short due to rapid pressure equilibration between pulmonary artery and RV 1
• Echocardiography may underestimate PR severity due to minimal turbulence and small pressure gradients 1
• Progressively increasing heart size on chest x-ray should always prompt search for pulmonary regurgitation 1
• Cardiomegaly, RV enlargement, or new arrhythmias mandate evaluation for residual hemodynamic lesions 1

Management Errors

• Delaying intervention until severe RV dysfunction develops may result in incomplete reverse remodeling even after valve replacement 1, 2
• RV function may not normalize after pulmonary valve replacement if intervention is delayed too long 1
• Arrhythmias may persist despite successful valve replacement, particularly when intervention is delayed 1

Special Populations

Post-Tetralogy of Fallot Repair

• 35-year survival approximately 85% after repair 1
• Need for reintervention increases after second decade of life 1
• QRS duration >180 ms indicates high risk for ventricular arrhythmias and warrants closer monitoring 1

Conduit Patients

• All conduits in children deteriorate and require replacement, usually within 10 years 1
• Stenosis may occur at valve, subvalve, or anastomosis to pulmonary artery 1
• Longevity of replacement conduits remains unclear 1

Endocarditis Prophylaxis

• Prophylaxis is indicated for all patients with unrepaired or palliated cyanotic CHD, prosthetic valves, or previous endocarditis 1
• Prophylaxis is reasonable for completely repaired CHD with prosthetic materials during first 6 months after procedure 1
• Prophylaxis is reasonable for repaired CHD with residual defects at or adjacent to prosthetic patch/device sites 1