Tricuspid stenosis is a relatively uncommon valvular heart condition, comprising less than 1% of reported cases. This disorder involves the narrowing of the tricuspid valve, stemming from diverse causes. The primary clinical consequence is systemic congestion.
Notably, tricuspid stenosis frequently coexists with mitral valve disorders, particularly in individuals with rheumatic heart disease. The origins of this condition are multifaceted, encompassing systemic ailments and drug-related factors. The causative factors for tricuspid stenosis can be broadly categorized into three groups: acquired, congenital, and iatrogenic.
Epidemiology
Tricuspid stenosis is a relatively rare valvular heart disease, constituting less than 1% of reported cases. The prevalence of TS varies globally, with higher incidences observed in regions where rheumatic heart disease is more prevalent. Rheumatic heart disease remains a significant contributor to tricuspid stenosis, often coexisting with mitral valve pathology.
The acquired form of tricuspid stenosis is commonly associated with rheumatic fever, which results from untreated streptococcal infections. Congenital tricuspid stenosis is a less common but recognized entity, typically presenting early in life.
It can occur as an isolated anomaly or in conjunction with other congenital heart defects. Iatrogenic tricuspid stenosis may develop as a consequence of medical interventions or procedures, such as catheter-based treatments or surgeries involving the tricuspid valve.
Anatomy
Pathophysiology
Etiology
Acquired
Rheumatic Heart Disease
Systemic Lupus Erythematous
Antiphospholipid antibody syndrome
Hyper-eosinophilic syndrome
Endomyocardial fibrosis
Carcinoid Syndrome
Congenital
Ebstein’s anomaly
Fabry Disease
Whipple disease
Genetics
Prognostic Factors
The prognosis in cases of isolated tricuspid stenosis undergoing intervention is largely contingent upon the underlying cause and the extent of ventricular dysfunction. Generally, the prognosis is favorable for these patients.
However, due to the rarity of the condition, comprehensive evidence of its outcomes is limited. The scarcity of extensive data underscores the need for further research to understand the prognostic factors better and optimize management strategies for individuals affected by tricuspid stenosis.
Clinical History
Individuals with tricuspid stenosis often have a background of predisposing factors, with rheumatic heart disease being a common precursor. The onset of symptoms can vary, but patients typically present with signs of systemic congestion, such as peripheral edema, hepatomegaly, and ascites. Additionally, symptoms may include fatigue and exertional dyspnea.
The duration of tricuspid stenosis is variable, with some cases manifesting gradually over time, particularly in the context of chronic rheumatic heart disease. In contrast, congenital cases may present earlier in life. The comprehensive assessment of clinical history, signs, symptoms, and temporal aspects is crucial for accurate diagnosis and effective management of tricuspid stenosis.
Physical Examination
Clinical manifestations of systemic congestion in tricuspid stenosis encompass lower limb edema, anasarca, ascites, and congestive hepatopathy. Elevated jugular venous pressure, characterized by a prominent or giant ‘a’ wave notably exceeding the norm, is a classic finding. A distinctive “slow y descent,” attributed to slowed emptying of the right atrium into the right ventricle, is observable.
Kussmaul’s sign, manifested by the failure of jugular venous pressure to descend during inspiration, may also be present. Despite these signs of venous congestion, lung fields remain clear in cases of isolated tricuspid stenosis. Precordial examination reveals a mid-diastolic rumble at the left fourth intercostal space, preceded by an opening snap.
This sound is briefer and less intense than the murmur associated with mitral stenosis. The murmur’s intensity and the opening snap’s prominence can be accentuated by maneuvers that augment blood flow across the tricuspid valve, particularly during inspiration, amyl nitrate inhalation, leg raises, exercise, or squatting.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Constrictive pericarditis
Right ventricular dysfunction
Tricuspid regurgitation
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
The management of tricuspid stenosis encompasses a multifaceted approach, considering the underlying etiology and severity of the condition. In cases of mild cases, close monitoring and conservative management may be sufficient, focusing on addressing associated systemic conditions. Loop diuretics can be employed to relieve congestion, but caution is necessary to avoid excessive preload reduction in patients with a low output state.
Surgical correction, either through repair or replacement, is recommended for individuals with low to moderate surgical risk or significant regurgitation. Repair is preferred when possible, and if not, valve replacement through open surgery is a viable option. No conclusive differences in outcomes have been established between bioprosthetic and mechanical valves, except in carcinoid syndrome, where a mechanical valve is favored to prevent degeneration.
Tricuspid valve surgery is indicated for symptomatic severe tricuspid stenosis accompanied by tricuspid regurgitation, especially in cases of a rheumatic or carcinoid etiology, as percutaneous interventions may exacerbate regurgitation. The comprehensive treatment approach underscores the importance of individualized strategies based on the specific characteristics and circumstances of each patient with tricuspid stenosis.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Valvotomy
For individuals with low to moderate surgical risk or significant regurgitation, surgical correction, either through repair or replacement, is recommended. This recommendation extends to patients with concomitant valvular heart diseases, such as mitral stenosis. Valvotomy, a procedure involving the use of one, two, or three balloons, can be employed.
Nearly some stenosis may persist; the alteration in the valve area results in a notable reduction in the transvalvular pressure gradient and a decrease in right atrial pressure. Typically, the valve area increases by 1 to 2 cm^2. In cases of bioprosthetic valve stenosis, options include valve-in-valve replacement or balloon valvotomy.
Valve Surgery
Tricuspid valve surgery involves the options of repair or replacement, with a preference for attempting repair whenever feasible. In cases where repair is not a viable option, open surgery for valve replacement is a viable alternative. Notably, tangible differences in outcomes have yet to be established between bioprosthetic and mechanical valves.
However, in the context of carcinoid syndrome, a mechanical valve is favored over a bioprosthetic one to mitigate the risk of degeneration. Indications for tricuspid valve surgery include the treatment of symptomatic severe tricuspid stenosis coupled with tricuspid regurgitation, especially in instances of a rheumatic or carcinoid etiology. It is worth noting that percutaneous balloon tricuspid commissurotomy may exacerbate regurgitation in such cases, emphasizing the need for surgical intervention.
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
Administration of a pharmaceutical agent
Loop diuretics can be beneficial in alleviating systemic and hepatic congestion in individuals with severe and symptomatic tricuspid stenosis. However, caution is warranted as these diuretics have the potential to excessively decrease preload, particularly in patients with a low-output state.Â
It is important to note that not all cases of tricuspid stenosis necessitate invasive interventions. Treating underlying conditions such as systemic lupus erythematosus (SLE) and antiphospholipid antibody (APLA) syndrome may mitigate the “coating” over the valves and chordae, potentially reducing stenosis and regurgitation.
Additionally, discontinuation of fenfluramine or methysergide has been associated with valve normalization. Nevertheless, in advanced cases, surgical therapy may become necessary, complementing medical management efforts.Â
Tricuspid stenosis is a relatively uncommon valvular heart condition, comprising less than 1% of reported cases. This disorder involves the narrowing of the tricuspid valve, stemming from diverse causes. The primary clinical consequence is systemic congestion.
Notably, tricuspid stenosis frequently coexists with mitral valve disorders, particularly in individuals with rheumatic heart disease. The origins of this condition are multifaceted, encompassing systemic ailments and drug-related factors. The causative factors for tricuspid stenosis can be broadly categorized into three groups: acquired, congenital, and iatrogenic.
Tricuspid stenosis is a relatively rare valvular heart disease, constituting less than 1% of reported cases. The prevalence of TS varies globally, with higher incidences observed in regions where rheumatic heart disease is more prevalent. Rheumatic heart disease remains a significant contributor to tricuspid stenosis, often coexisting with mitral valve pathology.
The acquired form of tricuspid stenosis is commonly associated with rheumatic fever, which results from untreated streptococcal infections. Congenital tricuspid stenosis is a less common but recognized entity, typically presenting early in life.
It can occur as an isolated anomaly or in conjunction with other congenital heart defects. Iatrogenic tricuspid stenosis may develop as a consequence of medical interventions or procedures, such as catheter-based treatments or surgeries involving the tricuspid valve.
Acquired
Rheumatic Heart Disease
Systemic Lupus Erythematous
Antiphospholipid antibody syndrome
Hyper-eosinophilic syndrome
Endomyocardial fibrosis
Carcinoid Syndrome
Congenital
Ebstein’s anomaly
Fabry Disease
Whipple disease
The prognosis in cases of isolated tricuspid stenosis undergoing intervention is largely contingent upon the underlying cause and the extent of ventricular dysfunction. Generally, the prognosis is favorable for these patients.
However, due to the rarity of the condition, comprehensive evidence of its outcomes is limited. The scarcity of extensive data underscores the need for further research to understand the prognostic factors better and optimize management strategies for individuals affected by tricuspid stenosis.
Individuals with tricuspid stenosis often have a background of predisposing factors, with rheumatic heart disease being a common precursor. The onset of symptoms can vary, but patients typically present with signs of systemic congestion, such as peripheral edema, hepatomegaly, and ascites. Additionally, symptoms may include fatigue and exertional dyspnea.
The duration of tricuspid stenosis is variable, with some cases manifesting gradually over time, particularly in the context of chronic rheumatic heart disease. In contrast, congenital cases may present earlier in life. The comprehensive assessment of clinical history, signs, symptoms, and temporal aspects is crucial for accurate diagnosis and effective management of tricuspid stenosis.
Clinical manifestations of systemic congestion in tricuspid stenosis encompass lower limb edema, anasarca, ascites, and congestive hepatopathy. Elevated jugular venous pressure, characterized by a prominent or giant ‘a’ wave notably exceeding the norm, is a classic finding. A distinctive “slow y descent,” attributed to slowed emptying of the right atrium into the right ventricle, is observable.
Kussmaul’s sign, manifested by the failure of jugular venous pressure to descend during inspiration, may also be present. Despite these signs of venous congestion, lung fields remain clear in cases of isolated tricuspid stenosis. Precordial examination reveals a mid-diastolic rumble at the left fourth intercostal space, preceded by an opening snap.
This sound is briefer and less intense than the murmur associated with mitral stenosis. The murmur’s intensity and the opening snap’s prominence can be accentuated by maneuvers that augment blood flow across the tricuspid valve, particularly during inspiration, amyl nitrate inhalation, leg raises, exercise, or squatting.
Constrictive pericarditis
Right ventricular dysfunction
Tricuspid regurgitation
The management of tricuspid stenosis encompasses a multifaceted approach, considering the underlying etiology and severity of the condition. In cases of mild cases, close monitoring and conservative management may be sufficient, focusing on addressing associated systemic conditions. Loop diuretics can be employed to relieve congestion, but caution is necessary to avoid excessive preload reduction in patients with a low output state.
Surgical correction, either through repair or replacement, is recommended for individuals with low to moderate surgical risk or significant regurgitation. Repair is preferred when possible, and if not, valve replacement through open surgery is a viable option. No conclusive differences in outcomes have been established between bioprosthetic and mechanical valves, except in carcinoid syndrome, where a mechanical valve is favored to prevent degeneration.
Tricuspid valve surgery is indicated for symptomatic severe tricuspid stenosis accompanied by tricuspid regurgitation, especially in cases of a rheumatic or carcinoid etiology, as percutaneous interventions may exacerbate regurgitation. The comprehensive treatment approach underscores the importance of individualized strategies based on the specific characteristics and circumstances of each patient with tricuspid stenosis.
Valvotomy
For individuals with low to moderate surgical risk or significant regurgitation, surgical correction, either through repair or replacement, is recommended. This recommendation extends to patients with concomitant valvular heart diseases, such as mitral stenosis. Valvotomy, a procedure involving the use of one, two, or three balloons, can be employed.
Nearly some stenosis may persist; the alteration in the valve area results in a notable reduction in the transvalvular pressure gradient and a decrease in right atrial pressure. Typically, the valve area increases by 1 to 2 cm^2. In cases of bioprosthetic valve stenosis, options include valve-in-valve replacement or balloon valvotomy.
Valve Surgery
Tricuspid valve surgery involves the options of repair or replacement, with a preference for attempting repair whenever feasible. In cases where repair is not a viable option, open surgery for valve replacement is a viable alternative. Notably, tangible differences in outcomes have yet to be established between bioprosthetic and mechanical valves.
However, in the context of carcinoid syndrome, a mechanical valve is favored over a bioprosthetic one to mitigate the risk of degeneration. Indications for tricuspid valve surgery include the treatment of symptomatic severe tricuspid stenosis coupled with tricuspid regurgitation, especially in instances of a rheumatic or carcinoid etiology. It is worth noting that percutaneous balloon tricuspid commissurotomy may exacerbate regurgitation in such cases, emphasizing the need for surgical intervention.
Loop diuretics can be beneficial in alleviating systemic and hepatic congestion in individuals with severe and symptomatic tricuspid stenosis. However, caution is warranted as these diuretics have the potential to excessively decrease preload, particularly in patients with a low-output state.Â
It is important to note that not all cases of tricuspid stenosis necessitate invasive interventions. Treating underlying conditions such as systemic lupus erythematosus (SLE) and antiphospholipid antibody (APLA) syndrome may mitigate the “coating” over the valves and chordae, potentially reducing stenosis and regurgitation.
Additionally, discontinuation of fenfluramine or methysergide has been associated with valve normalization. Nevertheless, in advanced cases, surgical therapy may become necessary, complementing medical management efforts.Â
medtigo
Tricuspid stenosis
Updated :
June 20, 2024
Tricuspid stenosis is a relatively uncommon valvular heart condition, comprising less than 1% of reported cases. This disorder involves the narrowing of the tricuspid valve, stemming from diverse causes. The primary clinical consequence is systemic congestion.
Notably, tricuspid stenosis frequently coexists with mitral valve disorders, particularly in individuals with rheumatic heart disease. The origins of this condition are multifaceted, encompassing systemic ailments and drug-related factors. The causative factors for tricuspid stenosis can be broadly categorized into three groups: acquired, congenital, and iatrogenic.
Tricuspid stenosis is a relatively rare valvular heart disease, constituting less than 1% of reported cases. The prevalence of TS varies globally, with higher incidences observed in regions where rheumatic heart disease is more prevalent. Rheumatic heart disease remains a significant contributor to tricuspid stenosis, often coexisting with mitral valve pathology.
The acquired form of tricuspid stenosis is commonly associated with rheumatic fever, which results from untreated streptococcal infections. Congenital tricuspid stenosis is a less common but recognized entity, typically presenting early in life.
It can occur as an isolated anomaly or in conjunction with other congenital heart defects. Iatrogenic tricuspid stenosis may develop as a consequence of medical interventions or procedures, such as catheter-based treatments or surgeries involving the tricuspid valve.
Acquired
Rheumatic Heart Disease
Systemic Lupus Erythematous
Antiphospholipid antibody syndrome
Hyper-eosinophilic syndrome
Endomyocardial fibrosis
Carcinoid Syndrome
Congenital
Ebstein’s anomaly
Fabry Disease
Whipple disease
The prognosis in cases of isolated tricuspid stenosis undergoing intervention is largely contingent upon the underlying cause and the extent of ventricular dysfunction. Generally, the prognosis is favorable for these patients.
However, due to the rarity of the condition, comprehensive evidence of its outcomes is limited. The scarcity of extensive data underscores the need for further research to understand the prognostic factors better and optimize management strategies for individuals affected by tricuspid stenosis.
Individuals with tricuspid stenosis often have a background of predisposing factors, with rheumatic heart disease being a common precursor. The onset of symptoms can vary, but patients typically present with signs of systemic congestion, such as peripheral edema, hepatomegaly, and ascites. Additionally, symptoms may include fatigue and exertional dyspnea.
The duration of tricuspid stenosis is variable, with some cases manifesting gradually over time, particularly in the context of chronic rheumatic heart disease. In contrast, congenital cases may present earlier in life. The comprehensive assessment of clinical history, signs, symptoms, and temporal aspects is crucial for accurate diagnosis and effective management of tricuspid stenosis.
Clinical manifestations of systemic congestion in tricuspid stenosis encompass lower limb edema, anasarca, ascites, and congestive hepatopathy. Elevated jugular venous pressure, characterized by a prominent or giant ‘a’ wave notably exceeding the norm, is a classic finding. A distinctive “slow y descent,” attributed to slowed emptying of the right atrium into the right ventricle, is observable.
Kussmaul’s sign, manifested by the failure of jugular venous pressure to descend during inspiration, may also be present. Despite these signs of venous congestion, lung fields remain clear in cases of isolated tricuspid stenosis. Precordial examination reveals a mid-diastolic rumble at the left fourth intercostal space, preceded by an opening snap.
This sound is briefer and less intense than the murmur associated with mitral stenosis. The murmur’s intensity and the opening snap’s prominence can be accentuated by maneuvers that augment blood flow across the tricuspid valve, particularly during inspiration, amyl nitrate inhalation, leg raises, exercise, or squatting.
Constrictive pericarditis
Right ventricular dysfunction
Tricuspid regurgitation
The management of tricuspid stenosis encompasses a multifaceted approach, considering the underlying etiology and severity of the condition. In cases of mild cases, close monitoring and conservative management may be sufficient, focusing on addressing associated systemic conditions. Loop diuretics can be employed to relieve congestion, but caution is necessary to avoid excessive preload reduction in patients with a low output state.
Surgical correction, either through repair or replacement, is recommended for individuals with low to moderate surgical risk or significant regurgitation. Repair is preferred when possible, and if not, valve replacement through open surgery is a viable option. No conclusive differences in outcomes have been established between bioprosthetic and mechanical valves, except in carcinoid syndrome, where a mechanical valve is favored to prevent degeneration.
Tricuspid valve surgery is indicated for symptomatic severe tricuspid stenosis accompanied by tricuspid regurgitation, especially in cases of a rheumatic or carcinoid etiology, as percutaneous interventions may exacerbate regurgitation. The comprehensive treatment approach underscores the importance of individualized strategies based on the specific characteristics and circumstances of each patient with tricuspid stenosis.
Valvotomy
For individuals with low to moderate surgical risk or significant regurgitation, surgical correction, either through repair or replacement, is recommended. This recommendation extends to patients with concomitant valvular heart diseases, such as mitral stenosis. Valvotomy, a procedure involving the use of one, two, or three balloons, can be employed.
Nearly some stenosis may persist; the alteration in the valve area results in a notable reduction in the transvalvular pressure gradient and a decrease in right atrial pressure. Typically, the valve area increases by 1 to 2 cm^2. In cases of bioprosthetic valve stenosis, options include valve-in-valve replacement or balloon valvotomy.
Valve Surgery
Tricuspid valve surgery involves the options of repair or replacement, with a preference for attempting repair whenever feasible. In cases where repair is not a viable option, open surgery for valve replacement is a viable alternative. Notably, tangible differences in outcomes have yet to be established between bioprosthetic and mechanical valves.
However, in the context of carcinoid syndrome, a mechanical valve is favored over a bioprosthetic one to mitigate the risk of degeneration. Indications for tricuspid valve surgery include the treatment of symptomatic severe tricuspid stenosis coupled with tricuspid regurgitation, especially in instances of a rheumatic or carcinoid etiology. It is worth noting that percutaneous balloon tricuspid commissurotomy may exacerbate regurgitation in such cases, emphasizing the need for surgical intervention.
Loop diuretics can be beneficial in alleviating systemic and hepatic congestion in individuals with severe and symptomatic tricuspid stenosis. However, caution is warranted as these diuretics have the potential to excessively decrease preload, particularly in patients with a low-output state.Â
It is important to note that not all cases of tricuspid stenosis necessitate invasive interventions. Treating underlying conditions such as systemic lupus erythematosus (SLE) and antiphospholipid antibody (APLA) syndrome may mitigate the “coating” over the valves and chordae, potentially reducing stenosis and regurgitation.
Additionally, discontinuation of fenfluramine or methysergide has been associated with valve normalization. Nevertheless, in advanced cases, surgical therapy may become necessary, complementing medical management efforts.Â
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