Tricuspid Stenosis

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Background

Tricuspid valve dysfunction can result from morphological alterations in the valve or from functional aberrations of the myocardium. Tricuspid stenosis is almost always rheumatic in origin and is generally accompanied by mitral and aortic valve involvement.[1]

Most stenotic tricuspid valves are associated with clinical evidence of regurgitation that can be documented by performing a physical examination (murmur), echocardiography, or angiography. Stenotic tricuspid valves are always anatomically abnormal, and the cause is limited to a few conditions. With the exceptions of congenital causes or active infective endocarditis, tricuspid stenosis takes years to develop.[2, 3]



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A representation of a stenotic tricuspid valve. This image demonstrates fusion of the commissures (shown as dotted lines).

Pathophysiology

Tricuspid stenosis results from alterations in the structure of the tricuspid valve that precipitate inadequate excursion of the valve leaflets. The most common etiology is rheumatic fever, and tricuspid valve involvement occurs universally with mitral and aortic valve involvement. With rheumatic tricuspid stenosis, the valve leaflets become thickened and sclerotic as the chordae tendineae become shortened. The restricted valve opening hampers blood flow into the right ventricle and, subsequently, to the pulmonary vasculature. Right atrial enlargement is observed as a consequence. The obstructed venous return results in hepatic enlargement, decreased pulmonary blood flow, and peripheral edema. Other rare causes of tricuspid stenosis include carcinoid syndrome, endocarditis, endomyocardial fibrosis, systemic lupus erythematosus, and congenital tricuspid atresia.[2, 3, 4]

In the rare instances of congenital tricuspid stenosis, the valve leaflets may manifest various forms of deformity, which can include deformed leaflets, deformed chordae, and displacement of the entire valve apparatus. Other cardiac anomalies are usually present.[1]

Epidemiology

United States data

Tricuspid stenosis is rare, occurring in less than 1% of the population. While found in approximately 15% of patients with rheumatic heart disease at autopsy, it is estimated to be clinically significant in only 5% of these patients. The incidence of the congenital form of the disease is less than 1%.

International data

Tricuspid stenosis is found in approximately 3% of the international population. It is more prevalent in areas with a high incidence of rheumatic fever. The congenital form of the disease is rare and true incidence is not available.

Race-, sex-, and age-related demographics

No racial predisposition is apparent.

Tricuspid stenosis is observed more commonly in women than in men, similar to mitral stenosis of rheumatic origin. The congenital form of the disease has a slightly higher male predominance.

Tricuspid stenosis can present as a congenital lesion or later in life when it is due to some other condition. The congenital form accounts for approximately 0.3% of all congenital heart disease cases. The frequency of tricuspid stenosis in the older population, due to secondary causes, ranges from 0.3-3.2%.

Prognosis

Mortality/Morbidity

The mortality associated with tricuspid stenosis depends on the precipitating cause. The general mortality rate is approximately 5%.

History

Fatigue, due to limited cardiac output, may be present.

Systemic venous congestion leads to abdominal discomfort and swelling. The onset is usually gradual, but it may be rapid if atrial fibrillation or flutter develops. (For related information, see Medscape's Atrial Fibrillation Resource Center).

Dyspnea may be present but is not severe unless concomitant mitral valve disease is present.

Patients may complain about prominent pulsations in the neck.

When tricuspid stenosis occurs concomitantly with mitral stenosis, the decrement of cardiac output to the pulmonary bed may paradoxically diminish the dyspnea, hemoptysis, and orthopnea typically seen with mitral stenosis.

Obtain information regarding preceding rheumatic fever, symptoms of the carcinoid syndrome, and possible congenital abnormalities.

Physical

With sinus rhythm (more common with tricuspid stenosis than with mitral stenosis), the jugular venous pulse increases and the A wave is prominent (may be confused with an arterial pulse).

If atrial fibrillation occurs, the A wave is lost.

Peripheral edema and ascites are frequent.

Without significant mitral pathology, the patient should not be dyspneic and can probably lie flat without symptoms.

A prominent right atrium may be palpable to the right of the sternum. If not obscured by mitral stenosis sounds, a tricuspid opening snap may be heard. A diastolic murmur is audible along the left sternal border or at the xiphoid, which increases with inspiration. Often, tricuspid regurgitation is also present, represented by a holosystolic murmur in a similar location.

The first heart sound may be split widely. The second heart sound may be single. This single sound is due to the inaudible closure of the pulmonary valve from the decrease in blood flow through the stenotic tricuspid valve.

Causes

At least 4 conditions can cause obstruction of the native tricuspid valve. These include (1) rheumatic heart disease, (2) congenital abnormalities, (3) metabolic or enzymatic abnormalities, and (4) active infective endocarditis. Note the following:

Approach Considerations

A complete evaluation of the heart often necessitates a multimodality approach, including radiography, echocardiography, computed tomography (CT), magnetic resonance (MR) imaging, and invasive angiography.[5]

Laboratory Studies

The following laboratory studies may be useful:

Imaging Studies

The following imaging studies may be useful:

Other Tests

Electrocardiogram

Arrhythmias are frequent in this patient population. Because of the enlargement of the right atrium, the presence of atrial flutter and/or fibrillation should not be surprising. In sinus rhythm, right atrial enlargement or abnormality (tall P waves on inferior leads) may be noted.

Procedures

Cardiac catheterization

This may be required prior to surgery in older patients to assess for concomitant coronary artery disease. Right heart catheterization can be used to determine the gradient across the valve and valve area (ie, severity of stenosis) and assess the presence of associated congenital defects (eg, septal defects, intracardiac shunts, anomalous veins) if present. Assessment of aortic and mitral valves via left heart catheterization is useful in patients with rheumatic disease.

Histologic Findings

Most commonly, stenotic tricuspid valves are secondary to rheumatic fever. These generally demonstrate fibrous tissue proliferation without calcium deposits. The leaflet tissue is composed of dense collagen and elastic fibers, producing major distortions of the normal leaflet layers. Congenitally abnormal valves can show a wide spectrum of incompletely developed leaflets, abnormal chordae tendineae, or dysplastic papillary muscles.

Medical Care

In the treatment of tricuspid stenosis, medical care consists of assessment and treatment of the underlying cause of the valvular pathology, as follows:

 

Surgical Care

Tricuspid stenosis remains a surgical disease and requires either commissurotomy or replacement of the valve if right heart failure or low cardiac output has resulted. Surgery is rarely performed solely on the tricuspid valve; it is usually performed in combination with mitral and/or aortic valve disease repair. Note the following:

Consultations

Consultation with infectious disease specialists may be appropriate if the stenosis is secondary to an infectious process.

An endocrinologist may be of assistance if carcinoid syndrome or an inborn error of metabolism is the cause of the pathology.

Diet

No specific dietary restrictions are necessary before therapy.

Fluid and sodium restriction is prudent if signs of venous congestion are present.

If a valve replacement is undertaken and the patient must be anticoagulated, dietary instructions must be provided regarding those foods that interfere with anticoagulation and are rich in vitamin K.

Activity

Activity is usually self-limited by the patient because of easy fatigability secondary to oxygen deprivation.

Once the pathology has been corrected, no activity restrictions are necessary.

Guidelines Summary

In 2014, the AHA/ACC released a revision to its 2008 guidelines for management of patients with valvular heart disease (VHD)18; and ESC/EACTS issued a revision of its 2007 guidelines in 2012.[11, 12]

The AHA/ACC guidelines include the following recommendations for diagnostic testing and initial diagnosis of tricuspid stenosis (TS)[11] :

Both guidelines concur that Class I indications for tricuspid valve surgery are as follows[11, 12] :

The AHA/ACC guidelines also recommend consideration of percutaneous balloon tricuspid commissurotomy in patients with isolated, symptomatic severe TS without accompanying TR. (Class IIb; Level of Evidence: C)

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Digoxin (Lanoxin)

Clinical Context:  Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. Acts directly on cardiac muscle and increases myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

Class Summary

Alter the electrophysiologic mechanisms responsible for arrhythmia.

Warfarin (Coumadin)

Clinical Context:  Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Tailor dose to maintain an INR in the range of 2-3.

Class Summary

Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.

Further Outpatient Care

Outpatient care consists of routine follow-up care with echocardiography studies to assess valvular function. Check the INR monthly to regulate anticoagulation. In those instances in which the tricuspid stenosis is secondary to some other process (eg, carcinoid, tumor), consider continual surveillance of the underlying disease state.

Further Inpatient Care

Inpatient care consists of treating the underlying precipitating condition. For acute bacterial endocarditis or rheumatic causes, antibiotic therapy is indicated until the acute phase has resolved. Then, valve surgery can be considered, if indicated.

After valve replacement, inpatient care consists of regulating the anticoagulation and treating postoperative arrhythmias until stability has been achieved. After valve replacement, adjust anticoagulation to an international normalized ratio (INR) of 3-4 because of the low-pressure and low-flow state of the right side. Because of the high risk of thrombosis in this low-pressure system, some authors recommend warfarin therapy for bioprosthetic or mechanical valves.

Inpatient & Outpatient Medications

Generally, outpatient medications consist of the anticoagulant warfarin and any antiarrhythmic used to treat atrial fibrillation or flutter, if present. Diuretics may be needed depending on the volume status of the patient.

Deterrence/Prevention

For those cases in which intravenous drug use or bacterial endocarditis was the precipitating event, emphasize careful dental hygiene. Maximize drug detoxification efforts. Of course, do not forget that routine antibiotic coverage should be administered for prevention of endocarditis.

Complications

Complications that can be encountered after tricuspid valve replacement include infection of the prosthetic valve, particularly in those instances when endocarditis was present preoperatively. Tricuspid insufficiency and thromboembolization can also occur.

Prognosis

The prognosis is generally good if therapy is provided for tricuspid stenosis. For those cases in which tumors are the cause of the stenosis, the prognosis is directly related to the prognosis of the underlying disease. In those cases of precipitating infection, if the behavior that caused the initial infection (eg, intravenous drug use) can be modified, prognosis for the patient is good.

Patient Education

Educate patients regarding the adverse effects of anticoagulation. Emphasize instructions regarding dental hygiene and subacute bacterial endocarditis prophylaxis for invasive procedures.

Author

Mary C Mancini, MD, PhD, MMM, Surgeon-in-Chief and Director of Cardiothoracic Surgery, Christus Highland

Disclosure: Nothing to disclose.

Specialty Editors

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald J Oudiz, MD, FACP, FACC, FCCP, Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Actelion, Bayer, Gilead, Lung Biotechnology, United Therapeutics<br/>Received research grant from: Actelion, Bayer, Gilead, Ikaria, Lung Biotechnology, Pfizer, Reata, United Therapeutics<br/>Received income in an amount equal to or greater than $250 from: Actelion, Bayer, Gilead, Lung Biotechnology, Medtronic, Reata, United Therapeutics.

Chief Editor

Richard A Lange, MD, MBA, President, Texas Tech University Health Sciences Center, Dean, Paul L Foster School of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Frank M Sheridan, MD to the development and writing of this article.

References

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  2. Waller BF. Morphological aspects of valvular heart disease: Part I. Curr Probl Cardiol. 1984 Oct. 9(7):1-66. [View Abstract]
  3. Waller BF. Morphological aspects of valvular heart disease: Part II. Curr Probl Cardiol. 1984 Nov. 9(8):1-74. [View Abstract]
  4. Acikel M, Erol MK, Yekeler I, Ozyazicioglu A. A case of free-floating ball thrombus in right atrium with tricuspid stenosis. Int J Cardiol. 2004 Apr. 94(2-3):329-30. [View Abstract]
  5. Malik SB, Kwan D, Shah AB, Hsu JY. The right atrium: gateway to the heart--anatomic and pathologic imaging findings. Radiographics. 2015 Jan-Feb. 35(1):14-31. [View Abstract]
  6. Faletra F, La Marchesina U, Bragato R, De Chiara F. Three dimensional transthoracic echocardiography images of tricuspid stenosis. Heart. 2005 Apr. 91(4):499. [View Abstract]
  7. Badheka AO, Shah N, Ghatak A, et al. Balloon mitral valvuloplasty in the United States: a 13-year perspective. Am J Med. 2014 Nov. 127(11):1126.e1-12. [View Abstract]
  8. Reddy G, Ahmed M, Alli O. Percutaneous valvuloplasty for severe bioprosthetic tricuspid valve stenosis in the setting of infective endocarditis. Catheter Cardiovasc Interv. 2015 Apr. 85(5):925-9. [View Abstract]
  9. Roberts PA, Boudjemline Y, Cheatham JP, et al. Percutaneous tricuspid valve replacement in congenital and acquired heart disease. J Am Coll Cardiol. 2011 July 5. 58(2):117-22.
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  11. [Guideline] Nishimura RA, Otto CM, Bonow RO, et al. 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. 2014 Jun 10. 63(22):e57-185. [View Abstract]
  12. [Guideline] Vahanian A, Alfieri O, Andreotti F, et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J. 2012 Oct. 33(19):2451-96. [View Abstract]

A representation of a stenotic tricuspid valve. This image demonstrates fusion of the commissures (shown as dotted lines).

A representation of a stenotic tricuspid valve. This image demonstrates fusion of the commissures (shown as dotted lines).