Tricuspid stenosis overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rim Halaby, M.D. [2] Fatimo Biobaku M.B.B.S [3]

Overview

Tricuspid stenosis (TS) is a type of valvular heart disease where there is narrowing of the orifice of the tricuspid valve of the heart.Tricuspid stenosis is quite uncommon, it is usually caused by rheumatic heart disease and generally accompanies mitral and/or aortic valve involvement.[1] A majority of stenotic tricuspid valves are associated with evidence of regurgiation that has been clinically documented through a physicial examination (murmur), echocardiogram, or angiogram. Stenotic tricuspid valves are anatomically abnormal and usually take years to develop, with some exceptions such as congenital causes, active infective endocarditis.

Classification

Pathophysiology

TS is characterized by structural changes in the tricuspid valve. The pathophysiology of the tricuspid valve stenosis depends on the underlying etiology. In rheumatic heart disease which is the most common cause of TS, there is diffuse scarring and fibrosis of the valve leaflets, fusion of the commissures, and shortening of the chordae tendineae as a result of inflammation.[2] These abnormalities limit leaflet mobility and reduce the size of the tricuspid orifice, thereby obstructing right ventricular filling.

Causes

The most common cause of TS is rheumatic heart disease. Other causes of TS include carcinoid syndrome, congenital abnormalities, endocarditis, lupus, and mechanical obstruction by a tumor.[3][2]

Differential Diagnosis

The differential diagnosis of TS includes other valvular abnormalities such as aortic regurgitation due to the similarity in the type of murmur, and also diseases resulting in systemic venous congestion such as constrictive pericarditis, right ventricular dysfunction, restrictive cardiomyopathy, atrial myxoma, etc.

Epidemiology and Demographics

TS is the least common valvular disease. TS is rarely an isolated disease, it is mostly associated with mitral valve abnormalities and/or concomitant tricuspid regurgitation. Approximately 8% of patients with rheumatic heart disease develop isolated TS, while up to 50% develop tricuspid regurgitation and TS.[4] The prevalence of TS is lower in developed countries compared to developing countries due to the low prevalence of rheumatic heart disease, the most common cause of TS.

Risk Factors

One of the most recognized risk factors of TS is rheumatic fever.

Natural History, Complications, and Prognosis

TS rarely exists in isolation, it is usually associated with existing mitral valve abnormality and/or tricuspid regurgitation. Complications of TS include heart failure, liver failure, and stroke.[5]

Diagnosis

History and Symptoms

TS is mostly associated with mitral valve abnormalities. Common symptoms include dyspnea, peripheral edema, and fatigue.

Physical Examination

Tricuspid stenosis often co-exists with mitral stenosis, thus depending on the severity of mitral valve pathology, symptoms differ. The diagnosis of TS may also be missed when they coexist. Patients can lay flat without any symptoms in the absence of serious mitral valve pathology and thus, not present with any signs of dyspnea. Characteristic findings of TS include an opening snap and a diastolic rumbling murmur that is localized to the lower left sternal border at the fourth intercostal space and it increases with inspiration.

Electrocardiogram

The electrocardiogram of patients with TS can demonstrate a sinus rhythm with or without right atrial hypertrophy.[5] Patients with TS experience frequent arrhythmias, particularly atrial flutter and/or atrial fibrillation due to the enlargement of the right atrium.

Chest X-Ray

The chest X-ray in a patient with TS may be significant for a pronounced right atrial enlargement. The heart size can range from a normal-sized heart to cardiomegaly.

Echocardiography

Transthoracic echocardiography (TTE) should be performed among patients with suspected TS to confirm the diagnosis, determine the etiology, and establish the baseline severity. TTE commonly reveals findings associated with other valvular diseases, such as tricuspid regurgitation and/or mitral stenosis. TS is mainly characterized by an elevated transvalvular gradient.[3]

Cardiac MRI

While echocardiography remains the diagnostic imaging modality of choice, cardiac MRI is useful to evaluate TS when the results of the echocardiography are insufficient.

Cardiac Catheterization

While echocardiography remains the diagnostic imaging modality of choice, cardiac catheterization is useful to evaluate TS when the results of the non-invasive testing are insufficient, particularly among patients who are being evaluated for other conditions such as mitral stenosis and pulmonary hypertension.[6] In the older pre-surgery population, cardiac catheterization may be necessary in order to assess concomitant artery disease.

Treatment

Medical Therapy

Medical therapy with diuretics and sodium restriction is the mainstay of treatment among patients with TS complicated by systemic and pulmonary congestion. Patients with TS should receive medical therapy for left heart failure, and/or pulmonary hypertension if case they are present.[7]

Surgery

Surgical tricuspid valve replacement in TS is recommended among patients undergoing surgical intervention for left valvular disease as well as among patients with severe symptomatic isolated TS.[7]

References

  1. Roguin A, Rinkevich D, Milo S, Markiewicz W, Reisner SA (1998). "Long-term follow-up of patients with severe rheumatic tricuspid stenosis". Am Heart J. 136 (1): 103–8. PMID 9665226.
  2. 2.0 2.1 Waller BF, Howard J, Fess S (1995). "Pathology of tricuspid valve stenosis and pure tricuspid regurgitation--Part I." Clin Cardiol. 18 (2): 97–102. PMID 7720297.
  3. 3.0 3.1 Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP; et al. (2009). "Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice". Eur J Echocardiogr. 10 (1): 1–25. doi:10.1093/ejechocard/jen303. PMID 19065003.
  4. Goswami KC, Rao MB, Dev V, Shrivastava S (1999). "Juvenile TS and rheumatic tricuspid valve disease: an echocardiographic study". Int J Cardiol. 72 (1): 83–6. PMID 10636636.
  5. 5.0 5.1 Diaof M, Ba SA, Kane A, Sarr M, Diop IB, Diouf SM (2004). "[Tricuspid valve stenosis. A prospective study of 35 cases]". Dakar Med. 49 (2): 96–100. PMID 15786615.
  6. Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA; et al. (2014). "2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines". J Am Coll Cardiol. 63 (22): e57–185. doi:10.1016/j.jacc.2014.02.536. PMID 24603191.
  7. 7.0 7.1 Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA; et al. (2014). "2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines". Circulation. doi:10.1161/CIR.0000000000000029. PMID 24589852.

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