What are the symptoms and management of a bicuspid tricuspid valve?

Symptoms and Management of Bicuspid Tricuspid Valve

Bicuspid tricuspid valve is an extremely rare congenital anomaly that requires specialized management focused on monitoring for progression to tricuspid regurgitation (TR) or tricuspid stenosis (TS) and early surgical intervention when indicated to prevent right ventricular dysfunction.

Clinical Presentation

Symptoms

• Asymptomatic in early stages
• Progressive symptoms of right heart failure:
  • Fatigue and exercise intolerance
  • Lower extremity edema
  • Abdominal distention and hepatic congestion
  • Jugular venous distention
  • Right upper quadrant discomfort
  • Ascites in advanced cases

Physical Examination Findings

• Holosystolic murmur at left lower sternal border that increases with inspiration (Carvallo's sign) if TR develops 1
• Abnormal systolic c and v waves in jugular venous pulse 1
• Systolic hepatic pulsation in severe TR 1
• In tricuspid stenosis: giant a wave and diminished y descent in jugular venous pulse, tricuspid opening snap, and presystolic/middiastolic murmur that increases on inspiration 1

Diagnostic Evaluation

Echocardiography

• Transthoracic echocardiography (TTE) is the primary diagnostic tool 1
• Assessment should include:
  • Valve morphology (bicuspid structure)
  • Degree of regurgitation and/or stenosis
  • Tricuspid annular size (significant if ≥40 mm) 1
  • Right ventricular size and function
  • Estimation of pulmonary artery pressure 1

Additional Testing

• Cardiac MRI: Useful for precise quantification of RV volumes and function, tricuspid valve anatomy and regurgitant volume 2
• Cardiopulmonary exercise testing: To evaluate functional capacity and exercise-induced changes in pulmonary pressures 2
• Right heart catheterization: To confirm pulmonary artery pressures and evaluate right atrial pressure when clinical and non-invasive data are discordant 2

Management Approach

Medical Management

• Loop diuretics (furosemide) for symptomatic relief of congestion 2
• Aldosterone antagonists (spironolactone) particularly beneficial for hepatic congestion 2
• Regular monitoring of:
  • Symptoms
  • Electrolytes and renal function
  • TR severity via echocardiography
  • Right ventricular function 2

Surgical Indications

Surgical intervention is indicated in the following scenarios:

1. Severe TR with symptoms despite medical therapy (Class I recommendation) 1
2. Severe TR in patients undergoing left-sided valve surgery (Class I recommendation) 1
3. Asymptomatic or mildly symptomatic patients with severe TR and progressive RV dilatation or deterioration of RV function (Class IIa recommendation) 1
4. Severe isolated TR with mild or no symptoms but progressive RV dilation (Class IIb recommendation) 1

Surgical Options

• Valve repair is preferred over replacement when technically feasible 1
• Repair techniques include:
  • Ring annuloplasty for annular dilatation 2
  • Chordal reconstruction for damaged chordae 1
• Valve replacement options:
  • Bioprosthetic valves are generally preferred over mechanical valves 2
  • Mechanical valves may be considered in younger patients who can tolerate long-term anticoagulation

Percutaneous Interventions

• Transcatheter tricuspid valve interventions may be considered for high-risk surgical patients at specialized centers with expertise in tricuspid valve disease 2
• Balloon valvuloplasty has limited success for tricuspid stenosis due to high risk of causing severe TR 1

Prognosis and Follow-up

Prognosis

• Untreated severe TR has poor long-term outcomes due to progressive RV dysfunction 1
• Early intervention before the onset of significant RV dysfunction improves outcomes 2
• Operative mortality for isolated tricuspid valve surgery ranges from 4-13% depending on center experience 1

Follow-up

• Regular echocardiographic surveillance every 6-12 months for asymptomatic patients with significant valve disease 2
• More frequent monitoring if symptoms develop or RV function deteriorates
• Post-surgical monitoring for recurrent regurgitation, valve dysfunction, and RV function 2

Special Considerations

Pitfalls to Avoid

• Delaying surgery until severe RV dysfunction develops significantly worsens outcomes 2
• Underestimating TR severity due to low right-sided pressures
• Focusing only on left-sided valve disease while missing significant tricuspid pathology
• Failing to recognize that bicuspid tricuspid valve is distinct from the more common bicuspid aortic valve and requires specific management approaches

Contraindications to Surgery

• Severe irreversible RV dysfunction
• Advanced pulmonary hypertension
• Irreversible liver cirrhosis 2

By following these guidelines, patients with bicuspid tricuspid valve can be appropriately monitored and receive timely intervention to prevent progression to severe right heart failure and improve long-term outcomes.