What is the treatment for tricuspid valve atresia?

Treatment of Tricuspid Valve Atresia

Tricuspid atresia requires staged surgical palliation culminating in a Fontan procedure, with the specific surgical pathway determined by pulmonary blood flow status and pulmonary artery anatomy. 1

Initial Assessment and Surgical Planning

The treatment strategy depends critically on the anatomic subtype and pulmonary blood flow pattern:

• Type I (normally related great arteries) with decreased pulmonary flow (Group A) represents 54% of cases and has only 10% survival beyond the first year without intervention 2
• Type II (transposed great arteries) requires different surgical considerations 3
• Pulmonary artery size and pressure are the key determinants of surgical candidacy—arteries must be large enough and at sufficiently low pressure to allow cavopulmonary connections 4

Staged Surgical Approach

Stage 1: Initial Palliation (Infancy)

For symptomatic infants with diminished pulmonary flow, a systemic-to-pulmonary artery shunt is the procedure of choice: 2, 5

• Classic Blalock-Taussig or Gore-Tex shunts carry 7% early mortality and 78% 5-year survival 5
• Waterston shunt is specifically recommended for symptomatic small infants with decreased pulmonary vascularity 2
• For patients with increased pulmonary blood flow (Group B, Type II), pulmonary arterial banding is indicated 2

Stage 2: Bidirectional Glenn (Intermediate Stage)

The bidirectional cavopulmonary anastomosis (superior vena cava to right pulmonary artery) should be performed as an intermediate stage to reduce volume load on the single functional ventricle: 6, 1

• This provides stable pulmonary blood flow without volume loading the systemic ventricle 1
• Approximately 50% survival at 20 years, though progressive cyanosis may occur from pulmonary arteriovenous fistulas 1
• Generally should not be the sole source of pulmonary blood flow except in specific circumstances 1

Stage 3: Fontan Completion (Definitive Palliation)

The Fontan procedure (right atrium to pulmonary artery connection) is the definitive treatment, with optimal timing after adequate pulmonary artery development: 1, 5

• A nonvalved connection between the right atrium and right ventricular infundibulum or pulmonary artery is preferred (used in 92% of cases) 5
• Modern era mortality is 7% when performed without complex additional procedures 5
• Actuarial survival is approximately 90% at 10 years in the absence of risk factors, 80% for all patients 1
• 96% of survivors achieve NYHA Functional Class I or II 5

Risk Factors for Poor Surgical Outcome

Critical factors that increase operative mortality include: 5

• Young age at time of Fontan (p = 0.0003)
• Complex associated cardiac procedures (p = 0.02)
• Inadequate pulmonary artery size or elevated pulmonary vascular resistance 4

Late Complications Requiring Surveillance

All patients require lifelong specialized follow-up at GUCH (Grown-Up Congenital Heart) centers for: 1

• Atrial arrhythmias (common and increase with age) 1
• Thrombus formation requiring anticoagulation 1
• Protein-losing enteropathy (PLE) 1
• Progressive systemic ventricular failure 1
• Progressive AV valve regurgitation 1
• Sinus node dysfunction potentially requiring pacing 1

Special Considerations

Patients with Balanced Circulation

• Four patients with naturally balanced pulmonary and systemic circulations survived to ages 21-41 years without surgery 3
• However, this represents a minority and most require intervention 2

Surgical Expertise Requirement

Surgeons with specific training and expertise in congenital heart disease must perform all operations for single-ventricle anatomy (Class I recommendation) 1

Common Pitfalls to Avoid

• Delaying initial palliation in symptomatic infants with decreased pulmonary flow—these patients have dismal natural history without intervention 2
• Performing Fontan procedure in patients with elevated pulmonary vascular resistance or inadequate pulmonary artery size 4, 5
• Failing to address associated anomalies (ASD, VSD, pulmonary stenosis) during staged repairs 2
• Inadequate long-term arrhythmia surveillance—atrial arrhythmias are nearly universal and increase with time 1

Prognosis

Overall survival to 15 years is approximately 50% with appropriate surgical intervention, compared to 10% survival beyond 1 year for untreated Type I, Group A patients 2. The Fontan procedure offers good intermediate survival with few reoperations, though late complications remain significant 5, 1.