Tricuspid Stenosis

Tricuspid stenosis (TS) is a valvular defect that obstructs blood flow from the right atrium to the right ventricle during diastole. This condition most commonly results from rheumatic heart disease or a congenital defect, and is usually found in conjunction with other valvular disease. A mid-diastolic murmur is best heard at the lower left sternal border. Mild TS may be asymptomatic or present with systemic venous congestion due to increased right atrial and venous pressures. Echocardiography can establish the diagnosis. Treatment focuses on heart failure management, and surgery is reserved for severe disease.

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Overview

Definition and epidemiology

Tricuspid stenosis (TS) is the narrowing of the tricuspid valve, which obstructs the flow of blood from the right atrium to the right ventricle, resulting in a transvalvular gradient.

  • Rare
    • < 1% of the population in the United States
    • Approximately 3% worldwide (more prevalent in areas with a high incidence of rheumatic fever)
  • More common in women
  • Usually not seen as an isolated condition; most commonly associated with mitral stenosis and tricuspid regurgitation

Etiology

  • Inflammatory or systemic disease
    • Rheumatic fever (most common cause) 
    • Infective endocarditis
    • Systemic lupus erythematosus (Libman-Sacks)
  • Congenital
    • Tricuspid valve atresia
    • Ebstein’s anomaly (atypical presentation)
  • Malignancy
    • Carcinoid syndrome
    • Tumors of the right heart may cause a functional TS (valve is not affected, but blood flow is obstructed by the tumor).
  • Iatrogenic
    • Injury from cardiac procedures leading to fibrosis of the valve:
      • Pacemaker lead placement
      • Endomyocardial biopsy
    • Radiation therapy

Pathophysiology

  • Obstruction of blood flow from the right atrium to the right ventricle → diastolic pressure gradient → increased blood volume in the right atrium → increased mean right atrial pressure → systemic venous congestion
  • Transvalvular gradient will increase with inspiration and decrease with expiration.
  • In severe TS, cardiac output will be reduced at rest and will not increase with exercise.
  • Low cardiac output prevents elevation of right ventricular, pulmonary artery, and left atrial pressures: may mask the signs and symptoms of concurrent mitral stenosis

Diseased valve is obstructing blood flow out of the right atrium.

Image by Lecturio.

Clinical Presentation

Symptoms

  • Mild or no dyspnea
  • Fatigue due to reduced cardiac output 
  • Abdominal discomfort or distension
  • Peripheral edema

Physical exam

  • Mid-diastolic murmur heard best at the left lower sternal border or over the xiphoid. 
    • Carvallo’s sign: 
      • Accentuation of the murmur during inspiration, leg raise, squatting, and exercise
      • Due to increased blood flow across the valve
  • May hear an opening snap and widely split S1
  • Evidence of systemic venous congestion:
    • Jugular venous distension
    • Hepatomegaly
    • Hepatojugular reflux
    • Abdominal distension from ascites
    • Peripheral edema

Diagnosis and Management

Diagnosis

  • Echocardiography (diagnostic modality of choice)
    • Thickening and/or distortion of the tricuspid valve
    • Limited leaflet mobility
    • Right atrium and inferior vena cava enlargement
    • Associated valvular and structural abnormalities
    • Doppler for estimation of the transvalvular gradient
  • Cardiac catheterization
    • Not required, but useful if there is diagnostic uncertainty from the clinical presentation and echo
    • Measures right atrial and ventricular pressures to calculate the pressure gradient
    • Evaluates cardiac output and valve area
  • Electrocardiogram
    • Not diagnostic, but may raise suspicion during workup
    • Evidence of right atrial enlargement 
      • Tall P waves in leads II, III, and aVF 
      • Upright P wave in V1
    • Known mitral stenosis + absent signs of right ventricular hypertrophy → should suspect TS

Management

  • Systemic venous congestion treatment:
    • Salt restriction
    • Fluid restriction
    • Diuretics
  • Treat underlying causes (endocarditis, cardiac tumors).
  • Invasive interventions (reserved for severe, symptomatic stenosis):
    • Balloon valvotomy 
      • Percutaneous procedure to separate and stretch the valve leaflets to increase the valve opening
      • Preferred for high-risk patients
      • May worsen concurrent tricuspid regurgitation
      • Vegetations, thrombi, and tumors are contraindications due to embolism risk.
    • Surgery
      • Valve repair
      • Valve replacement

Transthoracic echocardiogram of apical 4-chamber view showing thickened and doming mitral valve and tricuspid valve in rheumatic heart disease

Image: “Transthoracic echocardiogram” by US National Library of Medicine. License: CC BY 2.0

Differential Diagnosis

  • Congestive heart failure: results when the heart cannot maintain a normal cardiac output. Etiologies can include ischemic, structural, inflammatory, and valvular disease. Symptoms depend on the side of involvement but include dyspnea, orthopnea, and edema. Diagnosis is made by echocardiogram, and treatment involves diuretics and salt/fluid restriction. This condition can occur in conjunction with tricuspid stenosis and will be established by echocardiogram.
  • Cirrhosis: chronic disease of the liver marked by fibrosis of the parenchyma and impaired function. Symptoms include jaundice, ascites, hepatosplenomegaly, and edema. Diagnosis is made based on liver function test abnormalities and an ultrasound showing distorted hepatic architecture with portal hypertension. Treatment includes management of the underlying cause, diuretics, and salt  restriction. Echo findings will help differentiate this condition from tricuspid stenosis.
  • Mitral regurgitation: valve disorder where blood refluxes from the left ventricle to the left atrium during systole. Signs and symptoms are based on severity and can include exertional dyspnea, fatigue, or edema. Exam will be notable for a systolic murmur at the cardiac apex, and echocardiogram can establish the diagnosis and differentiate it from tricuspid stenosis. Treatment includes sodium restriction, diuretics, and surgery for severe cases.
  • Mitral stenosis: narrowing of the mitral valve, which results in obstruction of blood flow from the left atrium to the left ventricle. Rheumatic heart disease is the most common cause. Patients may be asymptomatic or present with dyspnea. Exam may reveal a low-pitched, rumbling, diastolic murmur at the cardiac apex. Diagnosis is made by echocardiography and will differentiate this condition from tricuspid stenosis.
  • Tricuspid regurgitation: valve disorder allowing blood to reflux into the right atrium from the right ventricle during systole. Patients may be asymptomatic or present with signs and symptoms of systemic venous congestion. A holosystolic murmur at the left lower sternal border distinguishes this condition from tricuspid stenosis. Echocardiogram will establish the diagnosis. Management involves treating the underlying cause, sodium restriction, diuretics, and surgery for severe cases.

References

  1. Mancini, M.C. (2016). Tricuspid stenosis. In Lange, R.A. (Ed.), Medscape. Retrieved October 21, 2020, from https://emedicine.medscape.com/article/158604-overview
  2. Kasper, D.L., Fauci, A. S., Longo, D.L., Bruanwald, E., Hauser, S. L., Jameson, J.L., (2007). Harrison’s principles of internal medicine (16th edition.). New York: McGraw Hill Education.
  3. Peters, F. (2020). Tricuspid Stenosis. In Yeon, S. B. (Ed.), UpToDate. Retrieved October 23, 2020, from https://www.uptodate.com/contents/tricuspid-stenosis

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