eMedicine Specialties > Radiology > Cardiac

Tricuspid Valve Disease

Author: Vibhuti N Singh, MD, MPH, FACC, FSCAI, Director, Suncoast Cardiovascular Center; Chair, Cardiology Division and Cath Labs, Department of Medicine, Bayfront Medical Center; Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine
Coauthor(s): Hanumanth K Reddy, MD, FACC, Clinical Professor of Medicine, St Louis University Medical School; Associate Chief, Department of Cardiovascular Services, Three Rivers Healthcare; Kul Aggarwal, MD, FACC, Professor of Clinical Medicine, Department of Internal Medicine, Division of Cardiology, University of Missouri School of Medicine; Chief, Cardiology Section, Harry S Truman Veterans Hospital
Contributor Information and Disclosures

Updated: Sep 24, 2008

Introduction

Background

Tricuspid valve disease involves the atrioventricular valve apparatus that separates the right atrium (RA) from the right ventricle (RV). Tricuspid regurgitation (TR) and tricuspid stenosis (TS) are commonly encountered. Isolated tricuspid valve disease is rare. It most often occurs as a secondary condition.

TR is more common than TS and usually develops in association with pulmonary hypertension in patients with mitral stenosis or regurgitation. Most patients with rheumatic tricuspid valve disease present with TR or a combination of stenosis and regurgitation. Rheumatic TS does not occur as an isolated lesion, but it often accompanies mitral and aortic valve disease. Some patients may develop TR in association with trauma or infective endocarditis. TS is nearly always rheumatic in origin. However, congenital tricuspid atresia, RA tumors, tricuspid valve vegetations, the presence of a pacemaker lead, or compression caused by extracardiac tumors may produce a clinical picture similar to that of TS. In addition, in patients with carcinoid syndrome, endomyocardial fibrosis may lead to obstruction of RV inflow, resulting in a clinical presentation similar to that of TS.^1,2,3,4,5,6

Echocardiography provides excellent images and may be used to detect and quantitate tricuspid regurgitation. It also permits assessment of the dimensions of cardiac chambers, determination of RV and pulmonary pressures, and delineation of associated valvular diseases.

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Pathophysiology

In most patients with tricuspid regurgitation (TR), right atrium (RA) and right ventricle (RV) pressures are increased. The RA pressure tracing usually reveals an augmented a wave, an absence of x descent, and a prominent v or c-v wave. These findings may not be discernible in mild cases of TR, but they are always present when TR is moderate or severe. As the severity of TR increases, the shape of the RA pressure pulse begins to resemble that of the RV pressure pulse. The usual decrease in RA pressure on deep inspiration is not seen; rather, a paradoxical increase may be noted.

Measurement and analysis of pulmonary arterial pressure or RV systolic pressure may indicate whether the TR is primarily caused by disease of the valve or is secondary to RV dilatation. A pulmonary arterial or RV systolic pressure of less than 40 mm Hg suggests a primary cause, whereas a pressure greater than 55 mm Hg suggests that TR is secondary.

Frequency

United States

Organic tricuspid valve disease is rare in the United States. Secondary tricuspid regurgitation is more common.

International

Organic tricuspid valve disease is more common in India, Pakistan, and other developing nations near the equator than in North America or Western Europe. Reports of findings at autopsy indicate that on the Indian subcontinent, organic tricuspid valve disease occurs in more than one third of patients with rheumatic heart disease.

Mortality/Morbidity

Most patients with tricuspid valve disease remain asymptomatic for many years.

• Most patients are treated medically.
• In rare cases, surgical intervention is needed. The tricuspid valve is usually repairable. Replacement may be required in certain patients with endocarditis whose valve leaflets have been destroyed.

Race

No particular racial predilection is observed, though endomyocardial fibrosis with shortening of the tricuspid leaflets and chordae tendineae is an important cause of tricuspid regurgitation in tropical Africa.

Sex

Tricuspid stenosis is more common in women than in men.

Age

• Tricuspid valve disease of rheumatic etiology usually occurs in teenage patients.
• In the Western world, secondary tricuspid regurgitation (TR) usually develops in older patients, because most of the diseases that cause TR develop in older individuals.

Anatomy

Tricuspid stenosis (TS)

TS involves commissural fusion of the valve cusps, as well as fusion and shrinkage of the chordae tendineae, similar to the changes seen in rheumatic mitral stenosis. When the valve opens, it resembles a tentlike structure with a fixed central opening. Valvular calcification is rare. The right atrium (RA) develops pressure overload; the walls of the RA thicken, and the RA becomes dilated. The pressure overload leads to further passive congestion, causing enlargement of the liver and spleen. In many cases, a small increase of 5 mm Hg in the transvalvular pressure gradient may augment the mean RA pressure enough to cause systemic venous congestion. Jugular venous distention, ascites, and edema develop as a result.

In patients with a sinus rhythm, the a wave of the RA may be tall, and it may increase to the level of the right ventricle (RV) systolic pressure. Resting cardiac output is decreased, and there is an absence of augmentation on exercise. The mean diastolic pressure gradient across the tricuspid valve may be as low as 2 mm Hg; this finding may suffice to suggest the diagnosis of TS. An increase in the gradient is seen with exercise, deep inspiration, rapid fluid infusion, or the use of intravenous atropine.

In most cases, TS is rheumatic in origin. Unusual causes include congenital tricuspid atresia; right atrial tumors, which may produce a clinical picture suggesting rapidly progressive TS; carcinoid syndrome, which more frequently produces tricuspid regurgitation; and obstruction of right ventricular inflow as a result of endomyocardial fibrosis, tricuspid valve vegetations, a pacemaker lead, or compression by extracardiac tumors. Marfan syndrome usually causes aortic regurgitation. It may cause tricuspid regurgitation, but it does not cause TS.

Tricuspid regurgitation (TR)

TR is usually secondary to increases in pulmonary pressures and to mitral valve disease. A systolic RV pressure of 55 mm Hg may cause functional TR. Other causes include RV infarction, pulmonic stenosis, primary pulmonary hypertension, and cor pulmonale. TR may also occur as a consequence of dilatation of the annulus in Marfan syndrome. Functional TR may decrease or disappear as the RV decreases in size as a result of the treatment of heart failure with diuretics.

Primary TR may occur as part of an Ebstein anomaly or in association with an atrioventricular canal defect or with correction of the transposition of the great arteries. Occasionally, it occurs in association with an aneurysm of the ventricular septum. Rheumatic fever may directly involve the tricuspid valve by causing scarring of the valve leaflets and chordae tendineae; such scarring restricts leaflet mobility and leads either to isolated TR or to a combination of TR and TS. Tricuspid involvement is almost always associated with rheumatic involvement of the mitral values and, often, the aortic valves.

In carcinoid syndrome, focal or diffuse deposits of fibrous tissue on the endocardium of the valve leaflets may cause TR or the combination of TR and TS. The white, fibrous carcinoid plaques are most extensive on the right side of the heart, where they are usually deposited on the ventricular surfaces of the tricuspid valve.

In tropical Africa, endomyocardial fibrosis is sometimes a cause of TR. It results in shortening of the tricuspid leaflets and the chordae tendineae.

TR may also be a result of tricuspid valve prolapse caused by myxomatous changes in the valve and chordae tendineae. It is usually coexistent with the prolapse of the mitral valve. Tricuspid valve prolapse is seen in about 20% of all patients with mitral valve prolapse. It is sometimes associated with an atrial septal defect.

Other causes of TR include penetrating and nonpenetrating trauma, dilated cardiomyopathy, and infective endocarditis (particularly staphylococcal endocarditis, as seen in narcotics addicts). TR also occurs in patients who have infective endocarditis that is unresponsive to medical management and who undergo surgical excision of the tricuspid valve. Less common causes of TR include cardiac tumors (particularly RA myxoma), the use of transvenous pacemaker leads, repeated endomyocardial biopsy procedures in a transplanted heart, endomyocardial fibrosis, methysergide-induced valvular disease, the administration of fenfluramine-phentermine, and systemic lupus erythematosus involving the tricuspid valve.

Presentation

Tricuspid stenosis (TS)

History

The clinical history of patients with tricuspid valve disease includes progressive fatigue, edema, and anorexia; in addition, two thirds of patients have a history of rheumatic fever. TS has a female preponderance. Orthopnea, paroxysmal nocturnal dyspnea, pulmonary edema, and hemoptysis are rare.

Physical examination

Physical findings may include wasting, peripheral cyanosis, and distention of the neck veins with prominent v waves. Other findings include hepatic pulsation, ascites, and peripheral edema.

On palpation, right ventricle (RV) lift may be appreciated over the precordium.

On auscultation, TR manifests as a holosystolic murmur that is maximal at the lower left sternal border and that is accentuated with inspiration. A diastolic rumble may be audible in some patients at the lower left sternal border, which increases in intensity with inspiration.

Diagnostic studies

ECG shows tall right atrium (RA) P waves and no RV hypertrophy.

Chest radiography shows a dilated RA without an enlarged pulmonary-artery segment.

Tricuspid regurgitation (TR)

History

Patients generally remain asymptomatic in the absence of pulmonary hypertension. After pulmonary hypertension develops, cardiac output declines, and the manifestations of right-sided heart failure become apparent.

Symptoms include dyspnea on exertion, fatigue, and, in late stages, edema and abdominal swelling and pain. Patients occasionally have throbbing pulsations in the neck, which increase on effort and are caused by jugular venous distention. Systolic pulsations of the eye orbits may be seen.

Physical examination

On inspection, signs include evidence of weight loss and cachexia. Cyanosis and jaundice are often observed in patients with severe TR. When patients present with palpitations, evidence of atrial fibrillation is seen on the ECG. A venous systolic thrill and murmur in the neck may be present in patients with severe TR.

The jugular venous pressure is elevated. Normal x and x' descents disappear, and a prominent systolic v wave (also called a c-v wave or an s wave) may be appreciated. The y descent is characteristically sharp.

On palpation, the RV impulse is hyperdynamic and may be thrusting in quality. Systolic pulsations of an enlarged, tender liver may be present initially. Ascites and edema are frequently associated findings. As liver cirrhosis develops, hepatic enlargement recedes.

On auscultation, the systolic murmur of TR is high pitched and occupies the entire systole. It is most prominent in the fourth intercostal space in the left parasternal region. In patients with mild TR, the murmur may be short. When TR occurs in the absence of pulmonary hypertension, such as in infective endocarditis or trauma, the murmur is usually of low intensity and occurs in early systole. A right-sided S3 may be present; such a finding represents RV enlargement and dysfunction. In patients with pulmonary hypertension, P2 becomes louder.

The TR murmur is characteristically augmented in the following settings: (1) in patients who exhibit the Carvallo sign (in such patients, the TR murmur is enhanced during inspiration; as RV becomes dysfunctional, the inspiratory augmentation may still be elicited by having the patient stand up from a recumbent position); (2) in association with the Mueller maneuver (the murmur also increases during the forced inspiration against a closed glottis); (3) with exercise; (4) with leg raising; and (5) with direct hepatic compression.

Tricuspid valve prolapse, like mitral valve prolapse, may cause a midsystolic click and a late systolic murmur. The murmur is most prominent at the lower left sternal border. With inspiration, the click is delayed, whereas the murmur becomes louder and shortens.

Diagnostic studies

ECG findings are frequently nonspecific. Incomplete right bundle-branch block, Q waves in lead V1, and atrial fibrillation are often noted.

Preferred Examination

For patients with tricuspid valve disease, echocardiography is the preferred examination.

Tricuspid stenosis (TS)

The echocardiographic changes of the tricuspid valve in TS include the following: diastolic doming of the valve; thickening of the leaflets and restriction in the motion of the leaflets; a reduction in the separation of the commissures of the leaflets; and a diminished tricuspid opening. With transesophageal echocardiography, depiction of the details of valvular structure is improved. On Doppler echocardiography, a delayed slope of antegrade flow is seen; such findings compare well with cardiac catheterization findings in the quantification of TS and in the assessment of associated TR.^7,8

Tricuspid regurgitation (TR)

The main purpose of echocardiographic imaging is to diagnose TR, assess its severity, and estimate pulmonary arterial pressure and the status of RV function. In secondary TR, clinically significant enlargement of the tricuspid annulus, right atrium, and right ventricle are found. In patients with TR caused by endocarditis, echocardiography may reveal vegetations on the valve or a flail valve leaflet. Transesophageal echocardiography may depict the TR vividly.^7,8

Limitations of Techniques

If acoustic windows are poor, transthoracic echocardiograms may be suboptimal. In such instances, transesophageal echocardiography is helpful.

Differential Diagnoses

Carcinoid, Gastrointestinal
Cardiomyopathy, Dilated
Congestive Heart Failure
Ebstein Anomaly
Pulmonary Hypertension

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