What is the prognosis for a patient with pulmonary stenosis?

Prognosis of Pulmonary Stenosis

The prognosis of pulmonary stenosis is excellent, particularly for mild disease which rarely progresses and for moderate-to-severe disease after successful intervention, with patients achieving normal life expectancy and low rates of cardiovascular complications when appropriately managed. 1

Prognosis by Disease Severity

Mild Pulmonary Stenosis (Peak Gradient <36 mmHg)

• Mild pulmonary stenosis has a benign natural history with minimal progression over decades. 1
• 96% of patients with initial transpulmonary gradient <25 mmHg remained free of cardiac operation over a 25-year follow-up period. 1
• Patients with gradients below 40 mmHg at diagnosis remain stable and require no treatment, with follow-up intervals extending to 2 years or more. 2
• Intervention is not usually necessary for mild disease, and patients maintain reassuring long-term outcomes. 1

Moderate Pulmonary Stenosis (Peak Gradient 36-64 mmHg)

• Patients with moderate stenosis have variable but generally good long-term outcomes, whether managed conservatively or with intervention. 1
• Some patients progress to require intervention in adulthood due to worsening stenosis or development of significant pulmonary regurgitation after earlier procedures. 1
• Most patients followed medically or surgically had mild obstruction by Doppler echocardiography at long-term follow-up. 1

Severe Pulmonary Stenosis (Peak Gradient ≥64 mmHg)

• Severe pulmonary stenosis treated with balloon valvuloplasty or surgery has excellent prognosis with very low recurrence rates over 22-30 years. 1
• Patients usually require intervention in childhood but have good prognosis into adulthood after successful treatment. 1
• Long-term follow-up (20-30 years) after surgical repair demonstrates excellent outcomes with low mortality. 1

Post-Intervention Outcomes

After Balloon Valvuloplasty

• Early and midterm results (up to 10 years) suggest long-term outcomes similar to surgical valvotomy, with little or no recurrence. 1
• Balloon valvuloplasty is safe and effective in reducing pulmonary valve gradient and improving symptoms in most patients. 1
• Some pulmonary regurgitation almost invariably occurs after valvuloplasty but is rarely clinically important. 1

After Surgical Repair

• At 27 years median follow-up after surgical repair, 84.8% of patients remained in NYHA functional class I. 3
• Long-term mortality after surgical treatment is low, with 95% survival in one surgical series over extended follow-up. 4
• Freedom from cardiac operation was excellent in the Natural History Study cohort over 25 years. 1

Complications and Reintervention Rates

Need for Reintervention

• 38.6% of patients required reintervention during long-term follow-up, primarily pulmonary valve replacement (17.7%) for pulmonary regurgitation. 3
• Freedom from reintervention decreases significantly after 20 years from initial repair. 4
• Pulmonary valve replacement due to pulmonary regurgitation is the most common reintervention (67% of cases). 4

Cardiovascular Complications

• 12% of patients experienced at least one cardiovascular complication during long-term follow-up. 3
• Supraventricular arrhythmias occurred in 8.2% of patients, heart failure in 3.8%, and stroke in 3.2%. 3
• Infective endocarditis is uncommon, with an incidence of only 0.94 per 10,000 patient-years. 1

Residual Hemodynamic Abnormalities

• 45% of surgically treated patients develop moderate-to-severe pulmonary regurgitation, 38% have right ventricular dilatation, and 40% have tricuspid regurgitation. 4
• Among patients with pulmonary regurgitation after surgical valvotomy, 87% had evidence by Doppler echocardiography, though RV systolic dysfunction was uncommon (9%). 1
• Secondary infundibular obstruction may occur after successful valve dilation but frequently regresses over time without treatment. 1

Risk Factors for Poor Outcomes

Predictors of Cardiovascular Complications

• Older age at time of pulmonary stenosis repair (HR 1.08 per year) and presence of cyanosis before repair (HR 5.23) are independent predictors of cardiovascular complications. 3
• These patients merit stricter long-term surveillance and follow-up. 3

Special Considerations

• Sudden death is very unusual in pulmonary stenosis, even with severe obstruction, particularly in asymptomatic patients with good exercise tolerance. 1
• Prognosis deteriorates if severe obstruction remains untreated long-term, potentially leading to tricuspid regurgitation and right ventricular failure. 1
• Patients with patent foramen ovale and severe stenosis face increased risk of paradoxical emboli due to right-to-left shunting. 1, 5

Long-Term Surveillance Requirements

Lifelong follow-up is essential even after successful intervention, as patients are not "cured" despite excellent functional outcomes. 4

• Serial echocardiography every 5-10 years is recommended for monitoring progressive valve stenosis or regurgitation, RV hypertrophy, heart failure, and arrhythmias. 1
• Patients require ongoing cardiac follow-up to detect late complications including progressive pulmonary regurgitation, RV dysfunction, and arrhythmias. 1