Fig. 2.1
JVP findings in tricuspid valve disease
Stages of TS: According to AHA/ACC (Table 2.1), only late two stages are identified (severe asymptomatic and severe symptomatic). The early two stages (at risk and progressive) were not defined [6].
Table 2.1
Stages of severe TS
Stage | Definition | Valve anatomy | Valve hemodynamics | Hemodynamic consequences | Symptoms |
|---|---|---|---|---|---|
C, D | Severe TS | • Thickened, distorted, calcified leaflets | • PHT > = 190 ms • Valve area = <1 cm2 | • RA/IVC enlargement | • None or variable presentations • Dependent on severity of valve associated disease and degree of obstruction |
Aetiology and Pathogenesis
At least four conditions can cause obstruction of the native TV. These include (1) rheumatic heart disease, (2) congenital abnormalities, (3) active infective endocarditis, and (4) metabolic or enzymatic abnormalities.
Rheumatic Heart Disease
In more than 90% of cases of TS, rheumatic heart disease is the cause. Rheumatic involvement of the TV is far less common than the mitral and the aortic valves. Isolated rheumatic TV disease is rare. Clinically significant TV disease almost always associated with rheumatic MS and up to 67% of patients with mitral valve disease showed involvement of TV with rheumatic process. Rheumatic process include diffuse thickening of the leaflets with contraction and fibrosis with or without fusion of the commissures. The chordae tendineae may be thickened and shortened. Calcification of the valve is rare [8, 9].
Congenital Tricuspid Stenosis
Congenital TS is characterized by a small but well-formed TV that joins an underdeveloped inlet portion of the RV. It represents a less severe form of tricuspid atresia. Abbott’s series of l000 cases of congenital heart disease identified only three cases of congenital TS (0.3% prevalence [3]). Hauck et al. reviewed the Mayo Clinic’s registry of excised tricuspid valves over 25 years. Out of 363 valves, only one showed TS without tricuspid insufficiency, and 3 showed combined stenosis and insufficiency. There was only one case of congenital TS. They may manifest as incompletely developed leaflets, shortened or malformed chordae, small annuli, abnormal size and number of the papillary muscles, or any combination of these defects [3, 10].
Infective Endocarditis
Infective endocarditis of the TV is not uncommon among intravenous drug abusers. It may also be observed in patients with long-term intravenous lines. Large infected vegetations obstructing the orifice of the TV may produce stenosis. This condition is relatively uncommon, even in those who abuse intravenous drugs. The clinical presentation is one of general systematic symptoms such as fever, weight loss, anemia, and fatigue; or of pulmonary embolism; or of right heart failure with hepatic congestion, peripheral edema, and ascites.
Carcinoid Heart Disease
Carcinoid tumors arising in the intestinal tract with secondary liver metastases are commonly associated with valvular pathology. The most commonly affected valve is the TV followed by the pulmonary valve. Occasionally, the left-sided heart valves may be affected when the primary carcinoid tumor is in the lung. Carcinoid valve lesions characteristically manifest as fibrous white plaques located on the valvular and mural endocardium. The valve leaflets are thickened, rigid, and reduced in area. Fibrous tissue proliferation is present on the atrial and ventricular surfaces of the valve structure. The valve leaflets are held partially open during systole and diastole leading to TS and TR. These lesions are observed more commonly in infants [11, 12].
Unusual Causes
Metabolic abnormalities: Fabry disease, Whipple disease and giant blood cysts.
Deposition abnormalities: Lofferler’s syndrome, endomyocardial fibroelastosis.
Drug-induced deposition (serotonin like substances): Methysegide, Ergotamine, Fenfuramine.
Differential Diagnosis
Diseases are mimicking TS due to delay of RA emptying in diastole [12–15] include:
Atrial Myxoma
Other intracardiac or extracardiac tumors,
Thrombosis or emboli,
Large endocarditis vegetations,
Supravalvular obstruction from congenital diaphragms,
Endomyocardial Fibrosis,
Tricuspid Atresia.
Diseases that impair right-sided filling can produce similar symptoms and physical findings. These conditions include:
Constrictive Pericarditis
Effusive-Constrictive Pericarditis
Restrictive Cardiomyopathy
Investigations
Electrocardiogram : ECG may show evidence of right atrial hypertrophy with tall peaked P waves. Atrial fibrillation and atrial flutter occur frequently. Absence of ECG abnormalities cannot be used to exclude significant tricuspid disease.
Chest Radiography: Chest X-ray eaxamination may show an enlarged right atrium without an enlarged pulmonary-artery segment. Cardiac size may range from normal to cardiomegaly. Calcification of the TV is rarely observed.
Echocardiography : Two-dimensional and three-dimensional echocardiography are used to detect and quantify TS and for quantification of cardiac chambers, determination of right ventricular and pulmonary pressures and detection of associated other heart valve abnormalities [7].
Cardiac catheterization : Due to accuracy of echocardiographic assessment of TV, catheterization is rarely necessary. It may be required prior to surgery to assess for possible coronary artery disease. Right heart catheterization can help to determine the severity of the stenosis and associated congenital defects. Assessment of aortic and mitral valves via left heart catheterization is useful in patients with rheumatic disease.
Management
- 1.
Life Style Modification
Fluid and sodium restriction is prudent if signs of venous congestion are present.
No specific dietary restrictions are necessary.
Activity is usually self-limited by the patient because of easy fatigability secondary to oxygen deprivation. No activity restrictions are necessary after invasive treatment.
- 2.
Pharmacotherapy: The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Treatment of the underlying condition—e.g., antibiotics for infective endocarditis.
Treatment of associated arrhythmias.
Management of heart failure. Loop diuretics may be useful to relieve systemic and hepatic congestion in patients with severe, symptomatic TS, although their use may be limited by worsening low-flow syndrome.
- 3.
Surgery: Surgery is usually carried out in severe TS at the time of intervention for the other left sided valves and in symptomatic isolated severe TS.
- 4.
Prognosis: Prognosis is generally good but dependent on the prognosis of the underlying disease, associated other heart abnormalities and associated arrhythmias.
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.
Tricuspid Regurgitation
Tricuspid regurgitation (TR) is a common valve lesion. Prevalence of moderate-to-severe TR is estimated to be 1.6 million in the United States [17]. Using echocardiography, the Framingham Heart Study investigators found a prevalence of moderate or severe TR of 0.8% and an increased prevalence with ageing. Overall, the prevalence of significant TR was 4.3 times greater in women than in men [18]. The pathophysiology is divided into three major categories:
- 1.
Physiologic TR: It is described as trivial to mild TR frequently detected by echocardiography in normal subjects with the absence of TV pathology and dilated chamber sizes. It is seen in over 70% of healthy adults [19].
- 2.
Primary (organic) TR: About 14% of significant TR may occur in the absence of structural TV alterations, pulmonary hypertension, or left heart dysfunction but due to local destruction of TV [20]. It can occur with or without TS. A long list of aetiologies account for organic TR.
- 3.
Secondary (functional) TR: is defined as TR without any identifiable structural damage of the TV leaflets or chordae. More than 80% of cases of significant TR are functional in nature with male predominance. It is related to one or more of the following mechanism [21, 22]:
Annular dilatation due to RV and/or RA dilatation results in lack of leaflet apposition.
Increased tricuspid leaflet tethering in relation to RV pressure and/or volume overload e.g., pulmonary hypertension.
Distortion of RV shape and papillary muscles e.g. RV infarction and intrinsic disease of the RV (Table 2.2).
Table 2.2
List of underlying aetiologies of primary and secondary TR
Primary TR: diseases of the TV leaflets or chordal structures, or both
Congenital disease
• Ebstein’s anomaly
• Tricuspid valve dysplasia, hypoplasia, or cleft
• Double orifice TV
• Unguarded tricuspid valve orifice
Acquired disease
• Infective Endocarditis (e.g., intravenous drug abuse).
• Marantic endocarditis
• Rheumatic heart disease
• Carcinoid syndrome, serotonin-active drugs
• Tricuspid valve prolapse, flail
• Mediastinal radiation
• Cardiac device (PPM, ICD) leads
• Blunt chest wall trauma
• Right ventricular endomyocardial biopsy
• Degenerated bioprosthesis
Secondary TR: Diseases affecting the right ventricle and tricuspid annulus
• Right ventricular and tricuspid annular dilatation,
• Left-sided valvular and/or myocardial disease,
• Pulmonary hypertension independent of left-sided cardiac pathology,
• Right ventricular infarction with remodeling,
• Papillary muscle dysfunction,
• Chronic right ventricular pacing (dyssynchrony),
• Atrial fibrillation.
Pathophysiology
The pathophysiology of secondary TR can be divided into three phases [13, 23, 24]. In the first phase, initial RV dilation results in tricuspid annulus dilation. Dilation of the tricuspid annulus occurs mainly in its posterior and anterior portions. Depending on the degree of annular dilation, TR may or may not be present. In the second phase, with progressive dilation of both RV and tricuspid annulus, malcoaptation of TV leaflets leads to significant TR. Finally, with progressive RV distortion and eccentricity, tethering of the leaflets occurs, in addition to tricuspid annulus dilation.
Presentation: AHA/ACC guidelines [6] categorized the with TR patients according to the stages of valve lesion (see Table 2.3).
Table 2.3
Stages of TR
Stage | Definition | Valve anatomy | Hemodynamic | Symptoms |
|---|---|---|---|---|
A | At risk | Primary • Mild rheumatic change Mild prolapse • Other (e.g., IE with vegetation, early carcinoid deposition, radiation) • Intra-annular RV pacemaker or ICD lead • Postcardiac transplant (biopsy related) Functional • Normal • Early annular dilation | None | None or in relation to other left heart or pulmonary/pulmonary vascular disease |
B | Progressive | Primary • Progressive leaflet deterioration/destruction • Moderate-to-severe prolapse, limited chordal rupture Functional • Early annular dilation • Moderate leaflet tethering | Mild TR • RV/RA/IVC size normal Moderate TR • No RV enlargement • No or mild RA enlargement • No or mild IVC enlargement with normal respirophasic variation • Normal RA pressure | None or in relation to other left heart or pulmonary/pulmonary vascular disease |
C | Asymptomatic severe | Primary • Flail or grossly distorted leaflets Functional • Severe annular dilation (>40 mm or 21 mm/m2) • Marked leaflet tethering | RV/RA/IVC dilated with decreased IVC respirophasic variation • Elevated RA pressure with “c-V” wave • Diastolic interventricular septal flattening may be present | None, or in relation to other left heart or pulmonary/pulmonary vascular disease |
D | Symptomatic severe | Primary • Flail or grossly distorted leaflets Functional • Severe annular dilation (>40 mm or >21 mm/m2) • Marked leaflet tethering | RV/RA/IVC dilated with decreased IVC respirophasic variation • Elevated RA pressure with “c-V” wave • Diastolic interventricular septal. flattening • Reduced RV systolic function in late phase | Fatigue, palpitations, dyspnea, abdominal bloating, anorexia, edema |
In primary TR, patients can be asymptomatic. Even severe TR may be well tolerated for a long period of time. With the development of RV systolic dysfunction, symptoms of systemic venous congestion appear. In secondary TR, the spectrum of presenting symptoms depends on underlying aetiology, chronicity of TR and presence or absence of pulmonary hypertension.
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.
Ascites and oedema.
Neck pulsation or eyeball pulsation.
Physical Examination
Findings on cardiovascular examination in patients with significant TR include the following:
Cachexia, peripheral oedema.
Cyanosis and jaundice.
Diminished peripheral pulse volume secondary to low cardiac output.
Atrial fibrillation.
Venous systolic thrill in the neck may present in severe TR.
Jugular venous pressure is elevated with a prominent systolic ‘v’ wave in 35% to 75% of patients (Fig. 2.1).
Ascites.
Hepatomegaly in 90% of patients. Palpable systolic pulsations of an enlarged and tender liver is less common.
Pulmonary rales, if the tricuspid regurgitation is associated with left ventricular dysfunction or mitral stenosis.
Right ventricular impulse is hyperdynamic and may be thrusting in quality.
Heart sounds and murmurs:
In presence of pulmonary hypertension, the pulmonary component of the second heart sound becomes accentuated and a high-pitched pansystolic murmur is audible most prominent in the fourth intercostal space in the left parasternal area. The murmur is accentuated during inspiration, with exercise, with legs raised, and with direct liver compression.
In the absence of pulmonary hypertension , the pulmonary component of the second heart sound is normal and the murmur is usually low-pitched and short (limited to first half of systole).
In RV failure, RV S3 and S4 gallop that increases with inspiration can be detected.
In tricuspid valve prolapse, murmur is late systolic and mid-systolic click may present.
In advanced stage of severe TR, the murmur is barely audible or absent due to ventricularization of RA.
Diastolic TR: It can occur if atrial contraction is not followed by ventricular contraction at regular interval e.g. heart block, restrictive cardiomyopathy, severe pulmonary regurgitation.
Differential Diagnosis
Atrial Fibrillation
Biliary Disease
Ebstein Anomaly
Cardiac Cirrhosis and Congestive Hepatopathy
Cor Pulmonale
Dilated Cardiomyopathy
Eisenmenger Syndrome
Heart Failure
Intestinal Carcinoid Tumor
Mitral Regurgitation
Investigations
ECG (Fig. 2.2): 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.
Fig. 2.2
ECG findings in Ebstein anomaly
Chest Radiography: Chest X ray has limited utility. It may show one or more of these findings:
Marked cardiomegaly
Evidence of elevated right atrial pressure may include distention of the azygous vein and pleural effusions
Ascites with diaphragmatic elevation may be present
Pulmonary arterial and venous hypertension is common
Echocardiography : is the main diagnostic modality to detect and quantify TR and for quantification of cardiac chambers, determination of right ventricular and pulmonary pressures and detection of other associated heart valve abnormalities (see Chaps. 5 and 6).
Cardiac magnetic resonance (CMR): CMR is the preferred method when available, for evaluating RV size and function (see Chap. 8).
Cardiac catheterisation : Catheterization may be required prior to surgery to rule out coronary artery disease and help determine the severity of the regurgitation and associated congenital defects (see Chap. 13).
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