Treatment Approach for Tricuspid Valve Atresia in Pediatric Patients
Tricuspid atresia requires staged surgical palliation culminating in a Fontan procedure, as medical management alone is universally fatal. 1, 2, 3
Initial Stabilization and Early Palliation
Neonatal Period Management
The immediate postnatal approach depends on the degree of pulmonary blood flow obstruction:
For severe cyanosis (pulmonary blood flow obstruction): Perform a modified Blalock-Taussig shunt (systemic-to-pulmonary artery shunt) in early infancy to augment pulmonary blood flow and prevent life-threatening hypoxemia 1, 4
For congestive heart failure (unrestricted pulmonary flow): Perform pulmonary artery banding to decrease pulmonary flow and pressure, preventing pulmonary vascular disease and heart failure symptoms 1
For coarctation with systemic outflow obstruction: Repair coarctation simultaneously with pulmonary artery banding to relieve systemic outflow obstruction 1
For total anomalous pulmonary venous connection (particularly in right-isomerism heterotaxia): Repair the anomalous connection at the time of systemic arterial shunt placement 1
Associated Lesions Requiring Attention
Look specifically for ventricular septal defects, atrial septal defects, patent ductus arteriosus, atrioventricular septal defects, pulmonary stenosis or atresia, coarctation, interrupted aortic arch, and anomalous pulmonary venous connections 1
Staged Surgical Palliation
Stage 1: Bidirectional Glenn Procedure (4-6 months of age)
- Perform superior vena cava to right pulmonary artery anastomosis (bidirectional cavopulmonary connection) to reduce volume load on the single functional ventricle 1, 5
- This intermediate stage prepares the pulmonary vasculature for the definitive Fontan procedure 4
Stage 2: Fontan Procedure (3-4 years of age)
The Fontan operation is the definitive surgical procedure of choice for tricuspid atresia, creating a connection between systemic venous and pulmonary circulation. 6, 4
- Anastomose the pulmonary artery to the right atrium (or use total cavopulmonary connection modifications) to direct systemic venous return directly to the pulmonary arterial tree 1, 6, 4
- This eliminates cyanosis by separating systemic and pulmonary circulations 4
- Early operative mortality is 5-7%, but more than half of patients survive into their second decade 6
Critical Timing Considerations
Without surgical treatment, these children die. 6 The natural history shows:
- Patients with balanced pulmonary and systemic circulations may survive to ages 21-41 years without surgery, but this is rare 7
- At approximately 15 years of age, mortality increases due to congestive heart failure, pulmonary vascular obstructive disease, and dysrhythmias 6
- Early recognition and timely surgical intervention are pivotal, as the condition is invariably fatal if left untreated 3
Anatomic Classification for Surgical Planning
Use left cineventriculography (particularly biplane with 60-degree left anterior oblique with cranial angulation) to define ventricular and great vessel relations 7:
- Type I (most common): Normal great arterial relations 7
- Type II: Transposed great arteries 7
- Type III: "Corrected transposition of the great arteries" 7
This anatomic classification determines the specific surgical approach and associated lesions to address 7
Long-Term Outcomes and Monitoring
- Survival free of late reoperation after Fontan is 86% at 5 years, 74% at 10 years, 62% at 15 years, and 46% at 20 years 1
- Monitor for late complications including arrhythmias, ventricular dysfunction, protein-losing enteropathy, and thromboembolic events 1, 2
- Lifelong specialized cardiac follow-up is mandatory in tertiary congenital heart disease centers 1, 2
Common Pitfalls to Avoid
- Delaying initial palliation: Severe cyanosis or heart failure in the neonatal period requires urgent intervention to prevent death 1, 3
- Inadequate assessment of pulmonary vascular resistance: Elevated pulmonary vascular resistance is a contraindication to Fontan completion and must be assessed before definitive surgery 1
- Missing associated lesions: Failure to identify and address coarctation, anomalous venous connections, or ventricular outflow obstruction compromises surgical outcomes 1
- Suboptimal timing of Fontan: Performing Fontan too early (before adequate pulmonary artery development) or too late (after irreversible ventricular dysfunction develops) worsens outcomes 6, 4