Tricuspid Valve Disease

oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

The majority of serious heart valve problems affect the mitral and aortic valves; disease of the tricuspid and pulmonary valves is fairly rare but more common in India, Pakistan and other developing countries than in North America or Western Europe:

  • Tricuspid regurgitation (TR) is more common than tricuspid stenosis and usually develops in association with pulmonary hypertension in patients with mitral stenosis or mitral regurgitation.
  • TR may occur as a result of trauma or infective endocarditis.
  • Tricuspid stenosis is nearly always rheumatic in origin. However, a similar clinical presentation may occur with:
    • Congenital tricuspid atresia.
    • Tumours in the right atrium.
    • Obstruction of right ventricular inflow due to endomyocardial fibrosis in carcinoid syndrome.
    • Tricuspid valve vegetations.
    • The presence of a pacemaker lead.
    • Compression due to extracardiac tumours.
  • Tricuspid atresia is the third most common form of cyanotic congenital heart disease.[1]

Tricuspid stenosis is rare, occurring in less than 1% of the population. Tricuspid stenosis is almost always rheumatic in origin and is usually associated with involvement of the mitral and aortic valves.[2]

  • Valve leaflets become thickened and undergo sclerosis with narrowing of the opening area of the valve.
  • This leads to enlargement of the right atrium, and reduced flow into the ventricle. Blood flow into the pulmonary circulation is impaired, and is accompanied by peripheral oedema and hepatomegaly.
  • Chronic rheumatic heart disease is usually the underlying cause and tricuspid stenosis is almost invariably associated with mitral valve disease.[2]
  • Other causes include carcinoid syndrome, systemic lupus erythematosus, right atrial myxoma, congenital atresia or infiltrating tumours.

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Presentation

See also separate articles on Heart Auscultation and Heart Murmurs in Children.

Detection requires careful evaluation, as it is almost always associated with left-sided valve lesions that dominate the presentation. Clinical signs are often masked by associated valve lesions, especially mitral stenosis.[3] 

  • History of rheumatic fever.
  • Low output symptoms of fatigue, anorexia, wasting, peripheral cyanosis and cold skin.
  • Usually those of accompanying left-sided rheumatic valve lesions.
  • Right hypochondrial discomfort from liver distension, hepatic pulsation, ascites and peripheral oedema, which are severe compared to the degree of dyspnoea.
  • In sinus rhythm - an 'a' wave in the jugular venous pulse.
  • A low rumbling diastolic murmur along the left sternal border, which increases with inspiration.
  • Tricuspid regurgitation is often also present, causing a pansystolic murmur in a similar location.
  • The first heart sound may be split widely and the second heart sound may be single (inaudible closure of the pulmonary valve).

Investigations

  • ECG may show evidence of right atrial hypertrophy with tall peaked P waves; arrhythmias (eg, atrial fibrillation, atrial flutter) occur frequently.
  • CXR may show a dilated right atrium without an enlarged pulmonary-artery segment.
  • Echocardiography: to detect and quantify tricuspid stenosis, and for assessment of the dimensions of cardiac chambers, determination of right ventricular and pulmonary pressures, and detection of associated other heart valve abnormalities.[4]
  • Cardiac catheterisation: may be required prior to surgery to assess for possible coronary artery disease. Right heart catheterisation can help to determine the severity of the stenosis and associated congenital defects. Assessment of aortic and mitral valves via left heart catheterisation is useful in patients with rheumatic disease.[2]

Management[2]

  • Treatment of the underlying condition - eg, antibiotics for infective endocarditis.
  • Treatment of associated arrhythmias.
  • Management of heart failure.
  • Surgery:[3] 
    • Surgery is usually carried out at the time of intervention for the other valves in patients who are symptomatic despite medical therapy.
    • Conservative surgery or valve replacement is preferred to balloon commissurotomy, which can only be considered as a first approach in the rare cases of isolated tricuspid stenosis.
    • The lack of pliable leaflet tissue is the main limitation for valve repair.
    • Biological prostheses for valve replacement are usually preferred over mechanical ones because of a lower risk of thrombosis.
    • Percutaneous balloon tricuspid dilatation has been performed but frequently induces significant regurgitation.

Prognosis

  • Prognosis is generally good but dependent on the prognosis of the underlying disease, associated other heart abnormalities and associated arrhythmias.

The incidence of TR is less than 1%.[5] Trivial TR is frequently detected by echocardiography in normal subjects.[3] 

  • TR is more often secondary, rather than due to a primary valve lesion. Secondary TR is due to annular dilatation and increased tricuspid leaflet tethering in relation to right ventricular pressure overload (eg, pulmonary hypertension, cor pulmonale, idiopathic pulmonary arterial hypertension and/or volume overload (eg, atrial septal defect, intrinsic disease of the right ventricle).[3] 
  • Other causes include rheumatic heart disease,[5] infective endocarditis (eg, intravenous drug abuse), papillary muscle dysfunction (right ventricular infarction), following heart transplantation,[6] carcinoid syndrome and rare congenital causes (including Ebstein's anomaly).
  • Iatrogenic causes include latent regurgitation unmasked following mitral valvotomy, and fenfluramine.

Presentation

See also separate articles on Auscultation of the Heart and Heart Murmurs in Children.

Predominant symptoms are those of other associated valve diseases. Even severe TR may be well tolerated for a long period of time. Clinical signs of right heart failure are useful in evaluating the severity of TR.[3] 

  • It is often asymptomatic in the absence of pulmonary hypertension.
  • Development of pulmonary hypertension leads to reduction of cardiac output and features of right heart failure with dyspnoea, fatigue, cyanosis, cold skin, oedema and discomfort in the right hypochondrium.
  • Jugular venous pressure is elevated with a prominent systolic 'v' wave.
  • Right ventricular impulse is hyperdynamic and may be thrusting in quality.
  • Systolic pulsations of an enlarged, tender liver.
  • Ascites and oedema.
  • Heart sounds and murmurs:
    • High-pitched pansystolic murmur, most prominent in the fourth intercostal space in the left parasternal region. In the absence of pulmonary hypertension, the murmur is usually low-intensity and early systolic.
    • The murmur is accentuated during inspiration, with exercise, with legs raised, and with direct liver compression.
    • The pulmonary component of the second heart sound becomes louder in the presence of pulmonary hypertension.
    • Tricuspid valve prolapse may present with a midsystolic click and a late systolic murmur, most prominent at the lower left sternal border.

Investigations

  • ECG: findings are usually nonspecific; they may show right atrial hypertrophy (tall peaked 'p' waves), incomplete right bundle-branch block, Q waves in lead V1, and atrial fibrillation.
  • CXR: shows marked cardiomegaly with right heart enlargement, and pleural effusions. Ascites with diaphragmatic elevation may be present. Pulmonary arterial and venous hypertension are common.[5]
  • Echocardiography: to detect and quantify tricuspid regurgitation, and for assessment of the dimensions of cardiac chambers, determination of right ventricular and pulmonary pressures, and detection of other associated heart valve abnormalities.[4]
  • When available, cardiac magnetic resonance (CMR) is the preferred method for evaluating right ventricular size and function.
  • Cardiac catheterisation: may be required prior to surgery to assess for possible coronary artery disease, and help determine the severity of the regurgitation and associated congenital defects.

Management

  • Treatment of the underlying condition - eg, antibiotics for infective endocarditis.
  • Treatment of associated arrhythmias.
  • Management of heart failure.
  • Surgery:[3] 
    • Patients with mild TR do not require intervention.
    • Asymptomatic patients with severe primary TR should be followed carefully to detect progressive right ventricular enlargement and development of early right ventricular dysfunction. Surgery, if required, should be carried out early enough to avoid irreversible right ventricular dysfunction.
    • Tricuspid valve surgery is indicated in patients with severe TR. Tricuspid surgery should be considered in patients with moderate primary TR, as well as in patients with mild or moderate secondary TR and significant dilatation of the annulus.
    • Ring annuloplasty is currently favoured for severe TR related to isolated tricuspid annular dilatation. Better long-term results are seen with prosthetic rings than with the suture annuloplasty.
    • In more advanced forms of tethering and right ventricular dilatation, valve replacement should be considered. However, if technically possible, valve repair is preferable to valve replacement.
    • The need for correction of TR is usually considered at the time of surgical correction of associated left-sided valve lesions..

Prognosis[3] 

  • Severe TR has a poor prognosis, even if it may be well tolerated functionally for years.
  • Prolonged volume overload may result in ventricular dysfunction and irreversible myocardial damage.
  • Flail tricuspid valve (classically associated with severe TR) is associated with decreased survival and increased risk of heart failure.
  • TR may persist even after successful correction of left-sided lesions.
  • Pulmonary hypertension, increased right ventricular pressure and dimension, reduced right ventricular function, atrial fibrillation, pacemaker leads, and the severity of tricuspid valve deformation are important risk factors for persistence or late worsening of TR.

Venous blood returning to the right atrium can only exit via an intra-atrial communication. Other cardiovascular anomalies occur in 15-20% of patients with tricuspid atresia - eg, transposition of the great vessels or a persistent anomaly of the left superior vena cava.[1]

Presentation[1]

  • Tricuspid atresia is usually detected in infancy with cyanosis, heart failure and growth restriction.
  • There is a history of feeding difficulties.
  • Bacterial endocarditis and brain abscess are common and may present with headaches, seizures or neurological deficits.
  • Finger clubbing is common in infants older than three months.
  • Raised jugular venous pressure.
  • The left ventricular impulse is prominent because of volume overload. The apical impulse is displaced to the left of the mid-clavicular line.
  • The liver may be large and pulsatile.
  • There is usually a single first heart sound that may be increased in intensity. The second heart sound may be single or normally split.
  • Cardiac murmurs are present in 80% of patients, including:
    • A pansystolic murmur, which may signify blood flow through the ventricular septal defect.
    • Systemic-to-pulmonary arterial collaterals or arterial-to-pulmonary arterial anastomoses surgically created to improve pulmonary blood flow, which may cause a continuous murmur.
    • A murmur indicating mitral regurgitation, which may be present.

Investigations[1]

  • FBC may show polycythaemia.
  • Blood gases may show hypoxaemia and acidosis.
  • CXR: cardiomegaly is usually present, with a prominent right heart border (enlargement of the right atrium). Pulmonary vascular markings are usually diminished (but may be increased when pulmonary flow is not obstructed).
  • Other investigations include ECG, echocardiography and cardiac catheterisation.

Management[1]

  • Infants with obstructed pulmonary blood flow and severe hypoxaemia require urgent prostaglandin E infusions in order to maintain patency of the ductus arteriosus.
  • Other non-surgical management includes oxygen therapy, prevention of bacterial endocarditis and management of heart failure.
  • Most patients with tricuspid atresia require some form of surgical treatment during the first year of life, usually involving a palliative shunt procedure.
  • Fontan's operation involves the formation of a right atrial-to-pulmonary artery connection or an extracardiac vena cava-to-pulmonary artery anastomosis using a synthetic graft. The child should be at least 4 years old to have the operation.

Complications[1]

  • Paradoxical emboli, stroke, brain abscess.
  • Polycythaemia.
  • Progressive cardiac dilatation.
  • Ventricular dysfunction.
  • Mitral valve insufficiency.
  • Arrhythmias.

Prognosis[1]

The one-year survival rate after Fontan's operation is 85%; the five-year survival rate is 78%.

Further reading & references

  1. Mancini MC; Tricuspid Atresia, Medscape, May 2011
  2. Mancini MC; Tricuspid Stenosis, Medscape, Jul 2011
  3. Management of Valvular Heart Disease, European Society of Cardiology (2012)
  4. Shah PM, Raney AA; Tricuspid valve disease. Curr Probl Cardiol. 2008 Feb;33(2):47-84.
  5. Mancini MC; Tricuspid Regurgitation, Medscape, Sep 2011
  6. Wong RC, Abrahams Z, Hanna M, et al; Tricuspid regurgitation after cardiac transplantation: an old problem revisited. J Heart Lung Transplant. 2008 Mar;27(3):247-52.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Colin Tidy
Current Version:
Peer Reviewer:
Dr Hannah Gronow
Last Checked:
30/10/2012
Document ID:
1686 (v23)
© EMIS