Tricuspid stenosis (TS) is narrowing of the tricuspid orifice that obstructs blood flow from the right atrium to the right ventricle. Almost all cases result from rheumatic fever. Symptoms include a fluttering discomfort in the neck, fatigue, cold skin, and right upper quadrant abdominal discomfort. Jugular pulsations are prominent, and a presystolic murmur is often heard at the left sternal edge in the 4th intercostal space and is increased during inspiration. Diagnosis is by echocardiography. TS is usually benign, requiring no specific treatment, but symptomatic patients may benefit from surgery.

TS is almost always due to rheumatic fever; tricuspid regurgitation is almost always also present, as is rheumatic mitral valvulopathy (usually mitral stenosis). Rare causes of TS include SLE, right atrial (RA) myxoma, congenital malformations, and metastatic tumors. The RA becomes hypertrophied and distended, and sequelae of right heart disease–induced heart failure develop but without right ventricular (RV) dysfunction; the RV remains underfilled and small. Uncommonly, atrial fibrillation occurs.

Symptoms and Signs

The only symptoms of severe TS are fluttering discomfort in the neck (due to giant a waves in the jugular pulse), fatigue and cold skin (due to low cardiac output), and right upper quadrant abdominal discomfort (due to an enlarged liver).

The primary visible sign is a giant flickering a wave with gradual y descent in the jugular veins. Jugular venous distention may occur, increasing with inspiration (Kussmaul sign). The face may become dusky and scalp veins may dilate when the patient is recumbent (suffusion sign). Hepatic congestion and peripheral edema may occur.

On auscultation, TS may produce a soft opening snap and a mid-diastolic rumble with presystolic accentuation. The murmur becomes louder and longer with maneuvers that increase venous return (exercise, inspiration, leg-raising, Müller maneuver) and softer and shorter with maneuvers that decrease venous return (standing, Valsalva maneuver).

Findings of TS often coexist with those of mitral stenosis and are less prominent. The murmurs can be distinguished clinically (see Table 1: Valvular Disorders: Distinguishing Murmurs of Tricuspid and Mitral Stenosis).

Table 1
PrintOpen table
Distinguishing Murmurs of Tricuspid and Mitral Stenosis Feature Tricuspid Stenosis Mitral Stenosis Character Scratchy Rumbling, low-pitched Duration Short Long Timing Starts in early diastole and does not increase up to S1 Increases through diastole Augmenting factor Inspiration Exercise Site Lower right and left parasternal borders Cardiac apex with patient in left lateral decubitus position S1 = 1st heart sound.

Diagnosis

• Echocardiography

Diagnosis is suspected based on history and physical examination and confirmed by Doppler echocardiography showing a pressure gradient across the tricuspid valve. Significant TS is signified by a mean forward gradient across the valve > 5 mm Hg. Two-dimensional echocardiography shows thickened leaflets with reduced movement and RA enlargement. An ECG and chest x-ray are often obtained. ECG may show RA enlargement out of proportion to RV hypertrophy and tall, peaked P waves in inferior leads and V₁. Chest x-ray may show a dilated superior vena cava and RA enlargement, indicated by an enlarged right heart border. Liver enzymes are elevated because of passive hepatic congestion.

Cardiac catheterization (see Cardiovascular Tests and Procedures: Cardiac Catheterization) is rarely indicated for evaluation of TS. When catheterization is indicated (eg, to evaluate coronary anatomy), findings include elevated RA pressure with a slow fall in early diastole and a diastolic pressure gradient across the tricuspid valve.

Treatment

• Diuretics and aldosterone antagonists
• Rarely valve repair or replacement

Evidence to guide treatment is scarce. For all symptomatic patients, treatment should include a low-salt diet, diuretics, and aldosterone antagonists. Patients with hepatic congestion leading to cirrhosis or severe systemic venous congestion and effort limitation may benefit from interventions such as balloon valvotomy or valve repair or replacement. Comparative outcomes are unstudied.

Key Points

• Tricuspid stenosis is almost always due to rheumatic fever; tricuspid regurgitation and mitral stenosis are often also present.
• Heart sounds include a soft opening snap and a mid-diastolic rumble with presystolic accentuation; the murmur becomes louder and longer with maneuvers that increase venous return (eg, exercise, inspiration, leg-raising) and softer and shorter with maneuvers that decrease venous return (standing, Valsalva maneuver).
• Treatment includes diuretics and aldosterone antagonists; surgical repair or replacement is rarely needed.

Last full review/revision November 2012 by Guy P. Armstrong

Content last modified December 2012