Tricuspid stenosis

Highlights
Summary Overview

Basics
Definition Epidemiology Aetiology Pathophysiology

Prevention
Primary Secondary

Diagnosis
History & examination Tests Differential Step-by-step Criteria Guidelines Case history

Treatment
Details Step-by-step Guidelines

Follow Up
Recommendations Complications Prognosis

Resources
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History & exam

Key factors
history of acute rheumatic fever (ARF) during childhood dyspnoea elevated jugular venous pressure with prominent a-wave low-frequency pre-systolic (diastolic) murmur at lower left sternal border

Other diagnostic factors

age: 40s to 50s age: infancy or childhood exercise intolerance fatigue jugular pulsations abdominal swelling and discomfort oedema episodic facial flushing, watery diarrhoea, or bronchoconstriction cyanosis or hypoxaemia atrial fibrillation absent right ventricular lift or heave hepatomegaly ascites, oedema, anasarca opening snap endocarditis stigmata (e.g., splinter haemorrhages, Osler's nodes, Janeway lesions)

History & exam details

Diagnostic tests
1st tests to order
ECG CXR 2D transthoracic echocardiogram Doppler transthoracic echocardiogram LFTs blood biochemistry FBC blood cultures 24-hour urinary excretion of 5-hydroxy-indole acetic acid (5-HIAA)

Tests to consider
cardiac catheterisation cardiac MRI

Emerging tests
3D transthoracic echocardiogram Diagnostic tests details

Treatment details
Acute
congenital surgery + preoperative alprostadil postoperative antiplatelet therapy with carcinoid heart disease fluid and sodium restriction + loop diuretic + somatostatin analogue + valve replacement surgery or balloon dilatation with rheumatic fever sequelae o o mild to moderate disease fluid and sodium restriction + loop diuretic severe disease surgical valve repair or replacement with infective endocarditis o o o absolute indications for surgery appropriate antimicrobial therapy + surgery relative indications for surgery appropriate antimicrobial therapy + surgery no indications for surgery appropriate antimicrobial therapy Treatment details

Summary
Most common aetiology is a late complication of acute rheumatic fever. Rarely occurs in the absence of associated rheumatic mitral and/or aortic disease. Dyspnoea is a common finding and usually relates to concomitant mitral valve stenosis. Prominent a-waves in the jugular venous wave form are a hallmark of tricuspid stenosis in patients who are in sinus rhythm. Transthoracic echocardiogram with Doppler flow is accurate for diagnosis. Most effective therapy is valve replacement.

Definition
Tricuspid stenosis (TS) is a rare condition defined by an abnormally elevated pressure gradient across the tricuspid valve during diastolic filling of the right ventricle.

Epidemiology
Rheumatic heart disease (RHD) is by far the most common cause of tricuspid stenosis (TS) in the US and throughout the world. In developed countries, the relative incidence of acute rheumatic fever (ARF) has significantly decreased over the last 40 to 50 years; whereas, the burden of ARF and RHD remains high in developing countries. The estimated annual incidence of ARF in the US is 0.2 to 0.8 per 100,000 population compared to 100 per 100,000 in Sudan, and 150 per 100,000 in China. [4] [5] As a result, the overall prevalence of chronic RHD is much lower in the US with recent estimates as low as 0.05 per 1000. Socio-economic and environmental factors such as shortage of resources, lack of healthcare providers and low level of awareness all contribute to the continued high incidence of ARF and RHD in developing countries. In developed countries such as the US, it is estimated that up to 50% of those with a history of ARF and carditis will go on to have chronic RHD. [6] [7] Isolated tricuspid valve involvement is extremely rare and clinical TS is usually discovered concomitantly with mitral and often aortic valve disease. [8] [1] [9] The overall incidence of TS in patients with RHD has been estimated at 9% to 15%, with clinically significant and more severe forms occurring in only 5%. [1] [10] [11] [12] [13] This results in an overall estimated prevalence of rheumatic TS in the US of 0.003 per 1000 population. The incidence of ARF is equal between males and females; however, females tend to develop significant rheumatic TS more commonly. The only race in the US that has been shown to develop ARF and RHD at relatively higher rates is those of Polynesian descent living in Hawaii. The incidence of carcinoid heart disease is approximately 1 in 300,000 of the population. [14][15] The incidence of congenital TS is rare, occurring in <0.1% of the population.

Aetiology
Tricuspid stenosis (TS) most commonly occurs as a late complication of rheumatic heart disease (RHD). The aetiology of rheumatic TS is still not entirely understood. In nearly all cases, an antecedent case of group A streptococcal pharyngitis is required. Rare causes of TS include carcinoid syndrome, infective endocarditis, and congenital disease. Carcinoid heart disease usually only occurs in patients whose primary intestinal tumour has metastasised to the liver, at which time a paraneoplastic process causes the tricuspid valve abnormalities. Infective endocarditis affecting the tricuspid valve can also lead to TS and is most

common among those who are intravenous drug abusers or those who have anatomically abnormal tricuspid valves at baseline. The aetiology of congenital TS is not known, but is thought to be multi-factorial with genetics and environmental factors both playing a role.

Pathophysiology
Anatomical abnormalities of the stenotic tricuspid valve vary by root cause, but generally include fusion of commissural leaflets and foreshortened chordae tendineae. In rheumatic heart disease (RHD), antibody cross-reactivity between antigens of group A streptococcus (GAS) and host valve tissue leads to an inflammatory response. This ultimately causes progressive fibrin deposition within the valve leaflet and along the cusps. Eventual fusion of leaflet commissures ensues and the chordae tendineae become thickened, fused, and foreshortened, resulting in tricuspid stenosis (TS). GAS-specific immunogenicity, host genetic predisposition, and degree of molecular mimicry are all thought to play a role in the development of acute rheumatic fever (ARF) and subsequent RHD. In carcinoid syndrome, the metastatic carcinoid tumours produce paraneoplastic substances (e.g., serotonin), which are thought to lead to the characteristic endocardial fibrous white plaque formation within the valve leaflets and tricuspid valve chordal apparatus. These fibrous depositions lead to valve distortion, foreshortened leaflets, and the inability of the valve to coapt and open completely, resulting in TS and often tricuspid valve regurgitation. Infective endocarditis involving the tricuspid valve occurs when certain virulent strains of bacteria circulating in the blood stream adhere to the surface of the valve. The subsequent inflammatory process results in fibrin, platelet, leukocyte, and RBC deposition along the valve leaflet with resulting vegetation formation. Vegetations can then result in further valve thickening, valve perforation, failure of coaptation, and ultimately in a reduced valve orifice and stenosis. Congenital TS results in some degree of hypoplasia of the right ventricle, pulmonary outflow, and an atrial level shunt. View image It usually results in annular hypoplasia, incompletely developed leaflets, abnormal chordae tendineae, and dysplastic papillary muscles.

Distortion of the tricuspid valve apparatus and deficiency of the effective valve orifice are pathognomonic findings for all forms of TS, regardless of aetiology. This reduction in the size of the valve orifice results in obstruction of blood flow from the right atrium into the right ventricle during diastole. Because the pressures in the right side of the heart are normally low, a pressure gradient as low as 5 mmHg can result in elevated right atrial pressures, depending on the atrial compliance. [9] [16] Chronically elevated right atrial pressures lead to signs and symptoms of systemic venous congestion including prominent jugular venous pulsations with an exaggerated a-wave. In more severe forms of TS, pulmonary blood flow is significantly decreased and cardiac output can become limited, especially at times of increased requirements (i.e., exercise). Right atrial enlargement and hypertrophy can contribute to the development of atrial fibrillation, which significantly exaggerates the symptoms of venous congestion due to the lack of atrial systole.

Primary prevention
Primary prevention of rheumatic tricuspid stenosis includes prompt and complete antibiotic therapy in symptomatic patients with suspected group A streptococcal pharyngitis. [20] [4] [21][22] Similarly, in patients with a documented history of acute rheumatic fever, secondary prevention, or prophylaxis with long-term antibiotic therapy should be instituted following existing guidelines and recommendations. [20] [4]

Secondary prevention
Endocarditis prophylaxis is recommended for all patients with prosthetic cardiac valves undergoing dental procedures that involve manipulation of gingival tissue or the peri-apical region of teeth or perforation of the oral mucosa.

History & examination

Key diagnostic factorshide all
history of acute rheumatic fever (ARF) during childhood (common)

Most common cause of rheumatic valvular disease and tricuspid stenosis (TS). A positive history should increase index of suspicion.

dyspnoea (common)

Usually due to concomitant mitral stenosis, which is present in >95% of cases of rheumatic TS. [8] [1] [9] [33] The absence or resolution of dyspnoea may indicate progression of TS with

severely limited pulmonary blood flow. elevated jugular venous pressure with prominent a-wave (common)

Prominent a-waves are hallmark of TS in patients in sinus rhythm. In patients with atrial fibrillation, a-wave is lost, but jugular venous pressures are still elevated.

low-frequency pre-systolic (diastolic) murmur at lower left sternal border (common)

Can be confused or attributed to similar murmur heard with mitral stenosis. Must have high index of suspicion for TS if other signs of right heart involvement are present. Murmur increases in intensity with inspiration and squatting.

Other diagnostic factorshide all

age: 40s to 50s (common)

In the US, this is the most common age for presentation of the condition. In patients immigrating or visiting from developing countries with higher prevalence of ARF, it may present earlier and with more sudden onset. [6] [25]

exercise intolerance (common) In severe forms, cardiac output may be limited leading to fatigue and exercise intolerance. fatigue (common) In severe forms, cardiac output may be limited leading to fatigue and exercise intolerance. jugular pulsations (common) Prominent venous pulsations in neck may cause fluttering and patient discomfort. abdominal swelling and discomfort (common) Due to hepatomegaly and ascites from chronic systemic venous congestion. oedema (common) Occurs especially in extremities. cyanosis or hypoxaemia (common) Due to right-to-left atrial level shunting. atrial fibrillation (common) TS caused by rheumatic heart disease can present with atrial fibrillation in 40% to 70% of patients. hepatomegaly (common) Can be pulsatile in severe forms of TS. ascites, oedema, anasarca (common) Related to degree of systemic venous hypertension. age: infancy or childhood (uncommon)

Congenital TS presents at an early age, and is often associated with cyanosis or other structural heart defects.

episodic facial flushing, watery diarrhoea, or bronchoconstriction (uncommon)

Symptoms of patients with carcinoid heart disease. These patients will also present with the symptoms of TS.

absent right ventricular lift or heave (uncommon)

The absence of this sign suggests the right ventricle is protected from volume or pressure overload. In patients with mitral stenosis and pulmonary hypertension, right heart pressures are elevated and a right ventricular lift is usually present.

opening snap (uncommon)

Can often be confused with opening snap of rheumatic mitral stenosis. However, the opening snap of TS will intensify during inspiration.

endocarditis stigmata (e.g., splinter haemorrhages, Osler's nodes, Janeway lesions) (uncommon)

Physical findings that relate to intra-cardiac vegetations embolising to systemic peripheral arterial beds. Most commonly found in left-sided vegetations or if an intra-cardiac communication is present (e.g., patent foramen ovale).

Risk factorshide all

Strong group A streptococcal (GAS) pharyngitis

Outbreaks of acute rheumatic fever (ARF) follow epidemics of GAS pharyngitis or scarlet fever. Antibiotic treatment programmes for documented GAS pharyngitis have markedly reduced the incidence of ARF and rheumatic heart disease (RHD). Most patients with ARF develop elevated antibody titres to anti-streptococcal antigens, including streptolysin O, hyaluronidase, and streptokinase. Human host antibodies directed against GAS antigens have been demonstrated to cross-react with antigens (e.g., myosin, tropomyosin, and laminin) of human heart. [4] [17] [18] [19]

metastatic carcinoid tumours

Carcinoid heart disease usually only occurs in patients whose primary intestinal tumour has metastasised to the liver. Metastatic carcinoid tumours produce paraneoplastic substances (e.g., serotonin), which are thought to lead to the characteristic endocardial fibrous white plaque formation within the valve leaflets and tricuspid valve chordal apparatus, which lead to valve distortion, foreshortened leaflets, and the inability of the valve to coapt and open completely.

Weak artificial tricuspid valve

Tricuspid valve replacement leads to abnormal flow characteristics that predispose to bacterial super-infection in rare cases.

intravenous drug use

Intravenous drug use provides a direct route for introducing bacteria into the bloodstream with secondary seeding of the tricuspid valve. Bacterial endocarditis secondary to intravenous drug use may rarely lead to leaflet thickening and fibrosis even in the presence of a previously normal valve.

pacemaker/defibrillator leads crossing tricuspid valve orifice Adhesions between lead and valve may lead to progressive fibrosis and restricted valve motion. genetic predisposition and environmental factors

Aetiology of congenital tricuspid stenosis is presumed to be multi-factorial with genetics and environmental factors both playing a role.

Diagnostic tests
1st tests to orderhide all
Test

ECG If sinus rhythm is present, a key finding is tall, peaked P-waves (>2.5 mm in II, III, V1) of right atrial enlargement

absence of RVH. [9]Atrial fibrillation is present in up to 40% to 70% of patients with rheumatic tricuspid stenosis (TS). [1] [2] [3] CXR Findings are non-specific.

2D transthoracic echocardiogram

Normal appearing valve by 2D echocardiogram does not exclude TS. Doppler echocardiography also needs to b

performed. The presence of an abnormally appearing tricuspid valve with normal appearing mitral and aortic valv

should prompt consideration for carcinoid heart disease and dissuade the clinician from rheumatic heart disease

the aetiology. Right atrial enlargement with dilated systemic and hepatic veins is consistent with more severe TS

Doppler transthoracic echocardiogram

Transthoracic echocardiogram with Doppler has largely replaced cardiac catheterisation as a means to diagnose well with severity and has not been validated for clinical use. In the presence of moderate to severe tricuspid regurgitation, the valve area can be underestimated. A calculated valve area based on continuity equation <4 to cm^2 is clinically significant TS. Others have indicated that a valve area <1 cm^2 indicates severe TS. [23] View LFTs Specificity for TS is poor. A normal value may also be obtained.

and correlates well with haemodynamic data obtained during catheterisation. [34][35] Valve area has not correla

blood biochemistry Minor metabolic abnormalities may be present depending on the extent of disease, such as elevated urea and

creatinine in renal insufficiency. FBC Polycythaemia may suggest chronic hypoxaemia secondary to diminished pulmonary blood flow or right-to-left shunting at the atrial level. Leukocytosis may indicate infective endocarditis.

blood cultures Bacterial or fungal cultures may indicate infective endocarditis. 24-hour urinary excretion of 5-hydroxy-indole acetic acid (5-HIAA) Twenty-four-hour urinary excretion of 5-HIAA is 10-fold higher than normal level in patients with carcinoid heart disease. [15]

Tests to considerhide all

Test

cardiac catheterisation

Simultaneous right atrial and right ventricular pressures must be obtained over 8 to 10 cardiac cycles while breat mean diastolic gradient >2 mmHg indicates TS. [12] [30] A gradient >7 to 10 mmHg is considered severe. [28]

after normal inspiration. Tricuspid valve gradient can be provoked with normal saline bolus infusions. Trans-valvu

cardiac MRI and right ventricle dysfunction. [31] [32]

May be useful in identifying valve abnormalities in carcinoid heart disease and reliably detecting progressive dila

Emerging testshide all

Test

3D transthoracic echocardiogram valve abnormalities in carcinoid heart disease. [32]

May provide better anatomical imaging and more consistent estimations of valve area. May also be useful in iden

Differential diagnosis
Condition Differentiating signs/symptoms Differentiating tests

Cardiac tumours

There are no

Echocardiogram will often show tumours and differentiate cause

specific differentiating signs/symptoms. SLE

Malar rash, arthritis/arthralgi as, pancytopenia, photosensitivity, renal involvement, alopecia, or Raynaud's phenomenon.

ANA, anti-dsDNA or anti-Sm antibodies may be positive in SLE.

Constrictive pericarditis (CP)

Usually have a previous history of tuberculosis, pericarditis, cardiac surgery, mantle radiation, chest trauma, or connective tissue disease. Murmurs are rare with CP and jugular venous pulsations are less prominent.

Calcification of pericardium sometimes seen on CXR.

Echocardiogram shows only mildly enlarged atrium and normal t gradient.

Cardiac catheterisation reveals absence of tricuspid valve gradie haemodynamic findings of CP.

Restrictive cardiomyopathy

History is usually unremarkable. Systolic murmurs of mitral and tricuspid regurgitation are most common.

Echocardiogram shows severe bi-atrial enlargement in the abse commissural fusion are rarely discovered.

valve gradient. Valve can sometimes be thickened, but shortene

Cardiac catheterisation reveals classic findings of restrictive card tricuspid valve gradient.

Diastolic murmurs are less common. Jugular venous pulsations can be prominent.

Step-by-step diagnostic approach

Clinically significant tricuspid stenosis (TS) can be suspected based on a history of acute rheumatic fever (ARF) as a child, presenting symptoms (fatigue, extremity swelling, fluttering neck discomfort, abdominal discomfort, or dyspnoea), and on classic physical examination findings. [23] However, further studies such as ECG, echocardiogram, and cardiac catheterisation are typically used to confirm the diagnosis. By far the most common presentation is as a late result of ARF, which typically occurs anywhere from 16 to 40 years after initial group A streptococcus (GAS) exposure. [24] [6] [25] [26] [27]

History
Although the age range can vary, patients usually present in their 40s to 50s. Most patients with symptomatic TS will present with fatigue, extremity swelling, fluttering neck discomfort, abdominal discomfort, or dyspnoea that is slowly progressive over a period of months to years. The fatigue usually relates to some degree of limited cardiac output. The dyspnoea usually results from concomitant rheumatic mitral stenosis, and is usually more pronounced when attempting exercise. The other symptoms relate to systemic venous congestion causing hepatomegaly, elevated jugular venous pressure (JVP), ascites, and oedema. A critical piece of information to acquire is whether or not there is an antecedent history of ARF. Many adult patients may not have been informed of their actual diagnosis during childhood or the disease may have never been discovered. These patients may only remember an illness with a prolonged fever, rash, or severe joint pain. Similarly, the patient may only remember having had to endure a prolonged period of bed rest and being away from school. A history of persistent heart murmur beginning in late childhood that has been passed-off as benign or innocent may also be the only historical clue to antecedent episode of ARF. If a history of ARF is denied or unknown, these historical items should be sought out specifically.

Features of flushing, diarrhoea, and bronchospasm should raise the possibility of carcinoid syndrome, and these patients have a 50% chance of cardiac involvement. The vast majority of patients with cardiac involvement present with signs of right heart failure secondary to severe dysfunction of the tricuspid and pulmonary valves. Patients may present with unremitting or recurrent fever with generalised malaise and other physical stigmata of bacterial endocarditis. The patient may have an antecedent history of tricuspid valve replacement or intravenous drug use. Congenital stenosis usually presents in infancy or at an early age with symptoms of right heart failure and cyanosis and is often associated with other structural heart defects.

Physical examination
The patient's vital signs are often normal. Jugular venous pulsations are often elevated and a prominent pre-systolic a-wave during atrial contraction is considered a classic finding. However, in the presence of atrial fibrillation (patients with rheumatic TS may present with atrial fibrillation) when atrial systole is lost, this classic finding is absent, resulting in a lower index of suspicion for TS. Hepatomegaly, ascites, and peripheral oedema are often observed in patients with moderate to severe TS. Focused cardiac examination reveals cardinal signs of TS. The precordium is usually quiet without a right ventricular lift or heave. A soft, low-frequency presystolic murmur can usually be auscultated at the lower left sternal border in the fourth intercostal space. The murmur can be intensified with manoeuvres that increase blood flow across the valve (e.g., inspiration, squatting, and isotonic exercise). An opening snap can sometimes be heard, but is often confused with the opening snap of a stenotic mitral valve, which is also often present. Similarly, the pre-systolic murmur of TS is often missed or attributed to mitral valve stenosis. Although there are characteristics of the 2 murmurs that can help differentiate one from the other, a high index of suspicion must be maintained for tricuspid valve involvement in a patient with poly-valvular rheumatic heart disease (RHD). Patients with endocarditis-associated TS will present with the same physical findings discussed above, occasionally with the physical stigmata of bacterial

endocarditis (e.g., splinter haemorrhages, Osler's nodes, and Janeway lesions). Hypoxaemia predominates with congenital TS secondary to right-to-left atrial level shunting. There are no specific physical examination findings in patients with carcinoid heart disease that distinguish it from other forms of TS.

Laboratory evaluation
FBC and blood cultures should be used to evaluate for bacterial endocarditis. Comprehensive metabolic profile should be obtained to evaluate for significant hepatic congestion associated with systemic venous hypertension and as a general estimate of end-organ dysfunction (e.g., renal insufficiency). If carcinoid heart disease is suspected, twenty-four-hour urinary excretion of 5-hydroxy-indole acetic acid (5-HIAA) may be used to determine whether it is present.

Consultant referral, ECG, and imaging

If a patient presents to a physician with a history concerning for ARF and many of the signs and symptoms described, it is appropriate to refer the patient to a cardiologist for further evaluation. The cardiology evaluation is likely to include repeat physical examination, CXR, ECG, echocardiogram, and possibly referral for cardiac catheterisation. Echocardiogram should be performed at the time the diagnosis is being considered; in general this is performed and interpreted by a consultant. Initial echocardiogram is usually transthoracic (2D or Doppler); however, trans-oesophageal or 3D studies may be necessary if adequate images cannot be obtained. Although there are no clearly defined criteria in the literature for TS severity, it is the opinion of the author that severe TS is usually defined by the onset of overt symptoms accompanied by a mean gradient by echocardiogram or cardiac catheterisation of 2 mmHg or greater, but is usually found to be in excess of 7 to 10 mmHg in severe TS. [28] [29] [30] Mild to moderate TS is usually asymptomatic, and has no clearly defined gradient range beyond the definition of TS diagnosed on a gradient of 2 mmHg or greater.

Haemodynamic tracings obtained during cardiac catheterisation from a woman with moderate to severe rheumatic tricuspid valve stenosisFrom the collection of Martin Bocks, used with permission.

Cardiac MRI may be useful in identifying valve abnormalities in carcinoid heart disease and reliably detecting progressive dilatation and right ventricle dysfunction. [31] [32]
Click to view diagnostic guideline references.

Diagnostic criteria

Tricuspid stenosis (TS) severity

Although there are no clearly defined criteria in the literature for tricuspid stenosis severity, it is the opinion of the author that severe TS is usually defined by the onset of overt symptoms accompanied by a mean gradient by echocardiogram or cardiac catheterisation of 2 mmHg or greater, but is usually found to be >7 to 10 mmHg in severe TS. [28] Mild to moderate TS is usually asymptomatic, and has no clearly defined gradient range beyond the definition of TS diagnosed on a gradient of 2 mmHg or greater.

Haemodynamic tracings obtained during cardiac catheterisation from a woman with moderate to severe rheumatic tricuspid valve stenosisFrom the collection of Martin Bocks, used with permission.Supportive findings for haemodynamically

significant TS include right atrial enlargement and a dilated inferior vena cava.

Case history
An otherwise healthy 35-year-old male presents with complaints of fatigue, lower extremity swelling, fluttering neck sensation, and a sense of abdominal fullness. Symptoms began to occur over the last few months and have been slowly progressive. The patient, who is an avid cyclist, reports increasing dyspnoea on exertion and the inability to exercise to his usual capacity. The patient's medical history is notable for an intermittent murmur, which was typically described as "innocent" when heard at routine childhood and adult examinations. He underwent tonsillectomy and adenoidectomy at age 7 years for recurrent pharyngitis. There is no family history of early CAD, cardiomyopathy, or sudden death. He does not smoke and rarely drinks alcohol.

Other presentations
Tricuspid stenosis (TS) can occur as a result of bacterial endocarditis, especially among patients with endocardial pacemaker leads, artificial tricuspid valves, or in intravenous drug abusers. Under these conditions, the typical presenting symptoms may also include fever and other signs of infective endocarditis. Congenital TS typically presents during infancy or very early childhood, as opposed to adulthood. It is often associated with other structural heart defects and often presents with cyanosis when an atrial level shunt is also present. Carcinoid heart disease can cause TS and should be considered in any patient with typical carcinoid features, including facial flushing, intractable secretory diarrhoea, and bronchoconstriction. TS caused by rheumatic heart disease can present with atrial fibrillation in 40% to 70% of patients. [1] [2] [3]

Treatment Options

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery

plus [?]

postoperative antiplatelet therapy

Aspirin is given postoperatively as an antiplatelet agent. The duration and choice of antiplatelet therapy varies by institution. However, the most common protocol is to continue low-dose aspirin until the shunt is either taken down surgically or allowed to thrombose over time as the patient grows. Primary Options aspirin : neonates: 5-10 mg/kg/day orally

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery

with carcinoid heart disease

1st

fluid and sodium restriction + loop diuretic + somatostatin analogue + valve replacement surgery or balloon dilatation

Fluid and sodium restriction as well as the use of diuretics treat right heart failure. Somatostatin analogues (e.g., octreotide) have been shown to provide symptomatic improvement and improved peri-operative survival. [37]

Percutaneous balloon tricuspid dilatation is safe and effective for treating isolated severe TS in carcinoid heart disease and should be considered in those who

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery are not operative candidates. [40]

Bioprosthetic valves are preferred and long-term results are promising. [41][42] Concomitant replacement of affected pulmonary valves may also result in less right ventricular dilatation. [43]

Tricuspid valve repair is usually not feasible as the leaflets are so restricted that residual post-repair stenosis is highly likely. Primary Options

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery furosemide : adults: 20-80 mg orally every 6-24 hours, maximum 600 mg/day and octreotide : adults: 100-600 micrograms/day subcutaneously given in 2-4 divided doses for 2 weeks, followed by 150 micrograms 3 times daily and surgery

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery

with rheumatic fever sequelae mild to moderate disease 1st

fluid and sodium restriction + loop diuretic

Mild rheumatic tricuspid valve disease is usually asymptomatic and no specific therapy is indicated. However, patients with more significant stenosis (or associated mitral and/or aortic valve disease) may present with symptoms of right heart failure (i.e., fatigue, dyspnoea, abdominal discomfort, and swelling).

Initial medical therapy for symptomatic patients is targeted towards relief of systemic venous congestion

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery and includes fluid and sodium restriction and diuretics to help decrease symptoms and improve hepatic function. Primary Options furosemide : adults: 20-80 mg orally every 6-24 hours, maximum 600 mg/day

severe disease

1st

surgical valve repair or replacement

Severe rheumatic tricuspid valve disease is characterised by intractable right heart failure or low

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery cardiac output that usually takes years to develop following the initial illness. In order to relieve symptoms and signs of right heart failure and diminished pulmonary blood flow due to obstruction to right ventricular inflow, surgical valve repair or replacement are the primary options.

With tricuspid valve replacement, the risk of thrombosis is significant and many clinicians advise warfarin therapy following either mechanical or bioprosthetic valve placement. Therefore, surgical

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery repair is preferable to valve replacement whenever feasible.

When the annulus size and sub-valvar structures are normal, successful percutaneous balloon valvotomy has been reported. The major adverse effect of balloon valvotomy is tricuspid regurgitation that may progress over time and ultimately require valve replacement.

with infective endocarditis

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery

absolute indications for surgery

1st

appropriate antimicrobial therapy + surgery

Absolute indications for tricuspid valve surgery include progressive heart failure, severe valve obstruction, peri-valvular abscess, non-candidal fungal infection, or pseudomonal infection.

Surgery typically involves a mid-line sternotomy approach, central cannulation for cardiopulmonary bypass, and tricuspid valve replacement (with a bioprosthetic or mechanical valve) in a beating or arrested heart depending on the preference of the surgeon. Many clinicians advise warfarin therapy following either mechanical or bioprosthetic valve

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery placement.

Appropriate antimicrobial therapy according to latest treatment guidelines is initiated at the time of diagnosis, and appropriate antibiotic or antifungal therapy is determined by the sensitivity of the organisms cultured. Effective therapy generally requires 4 to 6 weeks of appropriate intravenous therapy. For certain organisms, synergy and increased efficacy may be achieved with a second antibiotic for the first 2 weeks of therapy.

relative indications for surgery

1st

appropriate antimicrobial therapy + surgery

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery

Relative indications for surgery include persistent bacteraemia despite appropriate antibiotic therapy, candidal endocarditis, and very large vegetations (>10 mm).

Surgery typically involves a mid-line sternotomy approach, central cannulation for cardiopulmonary bypass, and tricuspid valve replacement (with a bioprosthetic or mechanical valve) in a beating or arrested heart depending on the preference of the surgeon. Many clinicians advise warfarin therapy following either mechanical or bioprosthetic valve

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery placement.

Appropriate antimicrobial therapy according to latest treatment guidelines is initiated at the time of diagnosis and appropriate antibiotic or antifungal therapy is determined by the sensitivity of the organisms cultured. Effective therapy generally requires 4 to 6 weeks of appropriate intravenous therapy. For certain organisms, synergy and increased efficacy may be achieved with a second antibiotic for the first 2 weeks of therapy.

no indications for surgery

1st

appropriate antimicrobial therapy

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery

Infective endocarditis can affect both structurally normal and abnormal tricuspid valves. Appropriate antimicrobial therapy according to latest treatment guidelines is initiated at the time of diagnosis, and appropriate antibiotic or antifungal therapy is determined by the sensitivity of the organisms cultured.

Effective therapy generally requires 4 to 6 weeks of appropriate intravenous therapy. For certain organisms, synergy and increased efficacy may be achieved with a second antibiotic for the first 2 weeks

Treatment Patient group congenital line 1st Treatmenthide all

surgery + preoperative alprostadil

This represents a wide spectrum of disease that usually presents in early infancy due to cyanosis and right heart failure with ductal-dependent pulmonary blood flow (requiring continuous prostaglandin therapy to maintain ductal patency).

Infant may require single ventricle palliation (initial systemic-to-pulmonary artery shunt, second-stage hemi-Fontan or bidirectional Glenn procedure, and third-stage Fontan operation) or cardiac transplantation.

Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). Primary Options alprostadil : neonates: 0.05 to 0.1 micrograms/kg/minute intravenous infusion initially, adjust according to response, usual maintenance dose 0.01 to 0.4 micrograms/kg/minute until surgery and surgery of therapy.

Acute

Treatment approach
The main goal of treatment is to relieve the symptoms that result from restricted diastolic filling of the right ventricle and diminished pulmonary blood flow.

Symptomatic management
Initial medical care consists of:

Assessment and treatment of the underlying aetiology for the valvular pathology (e.g., antibiotics for bacterial endocarditis or somatostatin analogues for carcinoid syndrome)

flutter)

Fluid and sodium restriction Medicines to treat cardiac arrhythmias secondary to right atrial distension (usually atrial fibrillation and/or

Pharmacotherapy with diuretics to reduce morbidity associated with systemic venous congestion.

Activity is usually self-limited by the patient due to easy tiring and diminished pulmonary blood flow.

Surgery
If right heart failure persists or if low cardiac output develops, surgical commissurotomy or valve replacement is indicated. Historically, the morbidity and mortality of tricuspid valve replacement have been very high, with most series reporting operative mortality in excess of 20%. [36] Risk factors for mortality include urgent/emergent status, age >50 years, functional classification of heart failure, and elevated pulmonary artery pressures. [36] Successful percutaneous balloon valvotomy has been reported when the annulus size and sub-valvar structures are normal. Once the pathology is corrected, dietary and activity restrictions are no longer necessary and medicines may be weaned.

Congenital tricuspid stenosis (TS)

Infants with congenital TS often have incompletely developed cardiac valve leaflets, shortened or malformed chordae, small annuli, abnormal size, and number of the papillary muscles or any combination of these defects. These patients usually have associated abnormalities of the pulmonary valve as well. In the most severe cases, the entire right heart is underdeveloped and incapable of handling an entire cardiac output. The infant with congenital TS may require single ventricle palliation (with an initial systemic-to-pulmonary artery shunt followed by a Fontan operation) or cardiac transplantation, plus preoperative prostaglandins and postoperative antiplatelet therapy. Alprostadil is given preoperatively. Infant should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). The duration and choice of antiplatelet therapy varies by institution. However, the most

common protocol is to continue low-dose aspirin until the shunt is either taken down surgically or allowed to thrombose over time as the patient grows.

TS with carcinoid heart disease

Right heart failure should be treated with fluid and sodium restriction and use of diuretics. Somatostatin analogues (e.g., octreotide) have been shown to provide symptomatic improvement and improved peri-operative survival. [37] Absolute and relative indications for intervention are not well established. Reviews have suggested that early valve intervention has better results compared to waiting for advanced disease. [38] However early disease has not been clearly defined in the literature. Most authors support considering intervention at the onset of any cardiac-related symptoms or with the development of right ventricular dysfunction. Furthermore, expert guidelines recommend intervention in the following settings: severe TS (plus tricuspid regurgitation) with symptoms despite medical therapy; and severe TS (plus tricuspid regurgitation) in a patient undergoing left-sided valve intervention. [39] Some case reports have demonstrated that percutaneous balloon tricuspid dilatation is safe and effective for treating isolated severe TS in carcinoid heart disease. [40] However, the surgical approach is preferred by most experts, especially when there is concomitant regurgitation. Bioprosthetic valves are preferred and long-term results are promising. [41] [42]Concomitant replacement of affected pulmonary valves may also result in less right ventricular dilatation. [43]

TS with rheumatic fever sequelae

Mild to moderate disease:
Medical therapy for symptomatic patients is targeted towards relief of systemic venous congestion secondary to right heart failure and includes fluid and sodium restriction and diuretics to help decrease symptoms and improve hepatic function.

Severe disease:
In severe rheumatic tricuspid valve disease, surgical valve repair or replacement are the primary options. Warfarin therapy may be advised following either mechanical or bioprosthetic valve placement. Therefore, surgical repair is preferable to valve replacement whenever feasible. Successful percutaneous balloon valvotomy has been reported in some cases in which the annulus size and sub-valvar structures were normal. [44] [45]

In rheumatic TS, the concomitant involvement of the mitral and aortic valve makes determining absolute and relative indications for tricuspid valve surgery very difficult. Usually the mitral valve is considered the most important valve to address as symptoms present much sooner and more frequently when it is affected. However, many studies have shown that patients who have their mitral valve surgically repaired or replaced without addressing the tricuspid valve simultaneously do poorly, compared to those who have surgery for both at the same operation. In these older studies, any significant residual tricuspid valve stenosis was reported to be associated with higher mortality and more frequent postoperative time in low cardiac output. However, more recently, evidence challenged this belief, as patients did well whether or not the tricuspid valve was surgically treated, or when treated still had residual gradient. [1] Such studies are limited in number due to the rare occurrence of isolated TS and are predominantly retrospective. It is the opinion of the author that research still supports tricuspid valve intervention for isolated severe TS (symptomatic) with a tricuspid valve echocardiogram or cardiac catheterisation gradient of 3 mmHg or greater. For isolated TS without symptoms, intervention should be considered when tricuspid valve gradient is >7 mmHg (but these patients will rarely be asymptomatic). When mitral stenosis is the predominant lesion but TS is present (whether or not symptoms are attributed to the tricuspid disease), most consultants would recommend intervening upon the tricuspid valve with a mean gradient 3 mmHg or greater to prevent postoperative low cardiac output and to potentially lower mortality.

TS with infective endocarditis

Absolute indications for surgery:
Absolute indications for tricuspid valve surgery include progressive heart failure, severe valve obstruction, peri-valvular abscess, non-candidal fungal infection, or pseudomonal infection. Tricuspid valve replacement using a bioprosthetic or mechanical valve is performed. Warfarin therapy may be advised following valve placement. Appropriate antimicrobial therapy is initiated at the time of diagnosis, and further determined by the sensitivity of the organisms cultured. Duration is usually 4 to 6 weeks.

Relative indications for surgery:

Relative indications for surgery include persistent bacteraemia despite appropriate antibiotic therapy, candidal endocarditis, and very large vegetations (>10 mm); treatment is as for those with absolute indications for surgery.

No indications for surgery:

Appropriate antimicrobial therapy is initiated at the time of diagnosis, and further determined by the sensitivity of the organisms cultured. Duration is usually 4 to 6 weeks.

Monitoring

ECGs, CXRs, and echocardiograms should be performed annually to monitor for development or worsening of tricuspid stenosis and/or regurgitation. Patients not requiring surgery should have a follow-up visit with a cardiologist annually. Patients who underwent tricuspid surgery are typically seen in an outpatient cardiology clinic at 2 to 4 weeks after hospital discharge, at 6 months, and annually thereafter. For patients with mechanical valve prostheses, warfarin dosing is adjusted on an outpatient basis according to the INR. Endocarditis prophylaxis is recommended for all patients with prosthetic cardiac valves undergoing dental procedures that involve manipulation of gingival tissue or the peri-apical region of teeth or perforation of the oral mucosa.

Patient Instructions
If a patient with prosthetic cardiac valve is due to undergo a dental procedure, this should be discussed with the dentist and physician to ensure antibiotic prophylaxis is administered if required. Patients are encouraged to lead an active lifestyle and exercise regularly under the supervision of their primary physician or cardiologist. Even in the absence of significant residual disease, exercise tolerance may be limited. Each patient should be encouraged to rest as needed during any exertional activity if worsening symptoms develop.

Complications
Complicationhide all

surgical complications Postoperative complications following tricuspid valve rep pneumothorax, haemothorax, pericardial tamponade, an third-degree atrioventricular block).

Appropriate therapy for pneumothorax or haemothorax is

Pericardial tamponade is relieved by re-opening the ches thrombus.

Arrhythmias are treated with appropriate anti-arrhythmic atrioventricular block is treated with atrioventricular sync temporary atrial and ventricular pacing wires (placed intr

bacterial endocarditis see our comprehensive coverage of Infective endocardit Dysplastic valves and prosthetic valves are prone to end associated with high-risk dental procedures).

If a patient develops endocarditis, appropriate antimicrob instituted immediately and continued for a full 6-week co

prosthetic valve failure Mechanical valves may fail due to thrombosis. In order to patients are treated with anticoagulant therapy (e.g., war

If a mechanical valve fails in the closed position, the pati and symptoms of acute, severe tricuspid stenosis (TS). I position, the patient will present with signs and symptom

regurgitation.

Bioprosthetic valves may fail due to progressive calcifica develop signs and symptoms of either TS or regurgitatio

Prognosis
Mild to moderate (asymptomatic) tricuspid stenosis
In the absence of progression to severe disease, prognosis is good. Isolated mild to moderate tricuspid stenosis (TS) is well tolerated and rarely requires intervention. Progression to severe TS is common in patients with carcinoid heart disease if the malignancy is not treated. The frequency of progression to severe TS in rheumatic valve disease is variable. Regardless of the aetiology, a follow-up visit with a cardiologist is recommended annually.

Severe (symptomatic) tricuspid stenosis

Once symptoms occur, medical therapy is instituted and valve intervention is considered. For most, surgery (valve repair or replacement) will be the most likely treatment option offered. Peri-operative mortality related to valve repair and replacement continues to be relatively high in the carcinoid group despite significant improvements in surgical and anaesthetic techniques. Studies demonstrate peri-operative mortality ranging from 9% to 20%. [46] [47] Right ventricular dilatation, systolic dysfunction, and advanced New York Heart Association (NYHA) class have been implicated in those who suffered poor peri-operative outcomes. Peri-operative mortality with valve replacement in patients with TS secondary to other causes is much lower, which is likely to be due to a decreased incidence of co-existing tricuspid regurgitation and right ventricular dilatation. Following successful tricuspid valve repair or replacement, symptoms of right heart failure will generally subside within 6 months and the overall prognosis for these patients is very good. Heart failure is the predominant cause of early

and late deaths, emphasising the importance of timely referral before the development of end-stage cardiac disease. [36] Long-term survival for tricuspid valve replacement in carcinoid heart disease has been reported to be as high as 100% at median follow-up of 37 months. [47] Other studies reporting results of valve replacement for various acquired heart conditions have shown only modest results with 50% survival at 10 and 15 years. [36] [41] [46] [47]