Tricuspid stenosis overview: Difference between revisions

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{{Tricuspid stenosis}}
{{Tricuspid stenosis}}
{{CMG}} ; {{AE}}
{{CMG}} ; {{AE}} {{VKG}} [[User:Mohammed Salih|Mohammed Salih, M.D.]] {{sali}}
==Overview==
==Overview==
Tricuspid stenosis (TS) is a type of [[valvular heart disease]] where there is narrowing of the orifice of the [[tricuspid valve]] of the [[heart]]. A majority of stenotic tricuspid valves are associated with evidence of  [[tricuspid regurgitation]]. Tricuspid stenosis is quite uncommon in developed countries due to the low prevalence of [[rheumatic heart disease]], which is the commonest cause of TS.<ref name="pmidpmid19065003" />  It is the least common valvular stenosis lesion,<ref name="pmidpmid19065003" /> and generally accompanies mitral and/or [[aortic valve]] involvement. It is extremely rare to have isolated acquired tricuspid stenosis. [[Rheumatic disease|Rheumatic]] tricuspid valve disease seldom receives much attention and can be easily overlooked on routine clinical and [[echocardiographic]] examination, which may lead to postoperative problems after successfully relieving left-sided valvular disease. The clinical findings associated with [[Rheumatic disease|rheumatic]] [[mitral valve disease]] are also more severe than that of rheumatic [[tricuspid valve disease]], making it rather easy to miss the diagnosis of concomitant tricuspid stenosis (TS). There is a paucity of literature on the prevalence and management of rheumatic [[tricuspid valve disease]].<ref name="pmidPMID: 20583402" /> Most of the literature on rheumatic tricuspid stenosis are old, which may be reflective of the low prevalence of [[rheumatic heart disease]] in developed countries. However, developing countries and the Indian subcontinent still have a significant prevalence of rheumatic [[tricuspid valve disease]], occurring mostly in young women.<ref name="pmidPMID: 24495168" /> Stenotic tricuspid valves are usually anatomically abnormal, and often take years to develop, with few exceptions such as congenital causes, active [[endocarditis]].
[[Tricuspid stenosis]] (TS) is a type of [[valvular heart disease]] where there is a narrowing of the orifice of the [[tricuspid valve]] of the [[heart]]. A majority of [[Stenosis|stenotic]] [[Tricuspid valve|tricuspid]] valves are associated with evidence of  [[tricuspid regurgitation]]. [[Tricuspid stenosis]] is quite uncommon in developed countries due to the low prevalence of [[rheumatic heart disease]], which is the commonest cause of TS. It is the least common [[Valvular Diseases|valvular]] stenosis lesion, and generally accompanies [[Mitral valve|mitral]] and/or [[aortic valve]] involvement. It is extremely rare to have isolated acquired [[tricuspid stenosis]]. [[Rheumatic disease|Rheumatic]] [[tricuspid valve]] disease seldom receives much attention and can be easily overlooked on routine clinical and [[echocardiographic]] examination, which may lead to [[Postoperative complication|postoperative]] problems after successfully relieving left-sided valvular disease. The clinical findings associated with [[Rheumatic disease|rheumatic]] [[mitral valve disease]] are also more severe than that of [[rheumatic]] [[tricuspid valve disease]], making it rather easy to miss the diagnosis of concomitant [[tricuspid stenosis]] (TS). There is a paucity of literature on the [[prevalence]] and management of [[rheumatic]] [[tricuspid valve disease]]. Most of the literature on [[rheumatic]] [[tricuspid stenosis]] is old, which may be reflective of the low [[prevalence]] of [[rheumatic heart disease]] in developed countries. However, developing countries and the Indian subcontinent still have a significant prevalence of [[rheumatic]] [[tricuspid valve disease]], occurring mostly in young women. Stenotic [[Tricuspid valve|tricuspid valves]] are usually anatomically abnormal, and often take years to develop, with few exceptions such as [[congenital]] causes, active [[endocarditis]].
 
==Classification==
==Classification==
Tricuspid stenosis is staged based on the valve anatomy and hemodynamics, and the hemodynamic consequences.
[[Tricuspid stenosis]] is staged based on the [[valve]] [[anatomy]] and [[hemodynamics]], and the [[hemodynamic]] consequences.
{| class="wikitable"
{| class="wikitable"
!Stage
!Stage
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|C, D
|C, D
|Severe TS
|Severe TS
|Thickened, distorted, calcified leaflets
|Thickened, distorted, [[Calcification|calcified]] leaflets
|
|
*T ½ ≥190 ms 
*T ½ ≥190 ms 
*Valve area ≤1.0 cm2
*[[Valve]] area ≤1.0 cm2
|Right atrial / [[Inferior vena cava]] enlargement
|Right atrial / [[Inferior vena cava]] enlargement
|
|
*Stage C-No symptoms
*Stage C-No [[symptoms]]
*Stage D-Symptoms variable and dependent on the severity of associated valve disease and degree of obstruction
*Stage D-[[Symptom|Symptoms]] variable and dependent on the severity of associated valve disease and degree of obstruction
|}
|}
== Pathophysiology ==
== Pathophysiology ==
TS is characterized by structural changes in the [[tricuspid valve]]. The pathophysiology of [[tricuspid valve stenosis]] depends on the underlying etiology. In [[rheumatic heart disease]] which is the most common cause of TS, there is diffuse scarring and fibrosis of the valve leaflets, fusion of the [[commissures]], and shortening of the [[Chordae tendinae|chordae tendineae]] as a result of [[inflammation]].<ref name="pmid7720297" /> These abnormalities limit leaflet mobility and reduce the size of the tricuspid orifice, increasing the transtricuspid diastolic gradient, which can eventually result in systemic venous hypertension and congestion.<ref name="pmid24603191" />
TS is characterized by structural changes in the [[tricuspid valve]]. The [[pathophysiology]] of [[tricuspid valve stenosis]] depends on the underlying etiology. In [[rheumatic heart disease]] which is the most common cause of TS, there is diffuse scarring and fibrosis of the valve leaflets, fusion of the [[commissures]], and shortening of the [[Chordae tendinae|chordae tendineae]] as a result of [[inflammation]]. These abnormalities limit leaflet mobility and reduce the size of the [[Tricuspid valve|tricuspid]] orifice, increasing the transtricuspid diastolic gradient, which can eventually result in systemic [[venous]] [[hypertension]] and [[congestion]].


The pathophysiology of tricuspid stenosis based on the underlying etiology:<ref name="pmid7720297" />
The [[pathophysiology]] of [[tricuspid stenosis]] based on the underlying etiology:


*'''Rheumatic tricuspid stenosis''':
*'''Rheumatic tricuspid stenosis''':
**Diffuse scarring and fibrosis of the valve leaflets from inflammation. Fusion of the [[commissures]] may or may not occur.
**Diffuse [[scarring]] and [[fibrosis]] of the [[valve]] leaflets from [[inflammation]]. Fusion of the [[commissures]] may or may not occur.
**[[Chordae tendineae]] may become thickened and shortened.
**[[Chordae tendineae]] may become thickened and shortened.
**As a result of the dense collagen and elastic fibers that make up leaflet tissue, the normal leaflet layers become significantly distorted.
**As a result of the dense [[collagen]] and [[elastic fibers]] that make up leaflet tissue, the normal leaflet layers become significantly distorted.
*'''Carcinoid heart disease''':
*'''Carcinoid heart disease''':
**Fibrous white plaques located on the valvular and mural [[endocardium]] are characteristic presentations of [[carcinoid]] valve lesions.
**[[Fibrous]] white [[Plaque|plaques]] located on the [[valvular]] and mural [[endocardium]] are characteristic presentations of [[carcinoid]] [[valve]] [[Lesion|lesions]].
**Valve leaflets become thick, rigid and smaller in area.
**Valve leaflets become thick, rigid and smaller in area.
**Atrial and ventricular surfaces of the valve structure contain fibrous tissue proliferation.
**Atrial and [[ventricular]] surfaces of the valve structure contain [[fibrous tissue]] [[proliferation]].
*'''Congenital tricuspid stenosis''':
*'''Congenital tricuspid stenosis''':
** More common in infants
** More common in [[Infant|infants]]
**Lesions may present in a number of different ways, either singularly or in any combination of the following:
**[[Lesions]] may present in a number of different ways, either singularly or in any combination of the following:
***Incompletely developed leaflets
***Incompletely developed leaflets
***Shortened or malformed [[Chordae tendineae|chordae]]
***Shortened or malformed [[Chordae tendineae|chordae]]
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***[[Papillary muscle|Papillary muscles]] of abnormal size and number
***[[Papillary muscle|Papillary muscles]] of abnormal size and number
*'''Mechanical obstruction''' '''of flow''' through the [[tricuspid valve]]:
*'''Mechanical obstruction''' '''of flow''' through the [[tricuspid valve]]:
**Supravalvular obstruction from congenital diaphragms
**Supravalvular obstruction from [[congenital]] [[Diaphragm|diaphragms]]
**Intracardiac or extracardiac tumors
**[[Intracardiac]] or extracardiac [[Tumor|tumors]]
**[[Thrombosis]] or [[emboli]]
**[[Thrombosis]] or [[emboli]]
** Large [[endocarditis]] vegetations
** Large [[endocarditis]] [[Vegetation (pathology)|vegetations]]


== Causes==
== Causes==
The most common cause of TS is [[rheumatic heart disease]].  Other causes of [[TS]] include [[carcinoid syndrome]], [[congenital]] abnormalities, [[endocarditis]], [[lupus]], and mechanical obstruction by a [[tumor]].<ref name="pmid7720297" />
The most common cause of TS is [[rheumatic heart disease]].  Other causes of [[TS]] include [[carcinoid syndrome]], [[congenital]] abnormalities, [[endocarditis]], [[lupus]], and mechanical obstruction by a [[tumor]].<br />
<br />
===Common Causes===
===Common Causes<ref name="pmid7720297" />===


*[[Rheumatic heart disease]] (majority of the cases)
*[[Rheumatic heart disease]] (majority of the cases)
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*[[Congenital]]
*[[Congenital]]


Etiology of tricuspid stenosis in operatively excised valves in patients >15years<ref name="pmid7720297" />
Etiology of [[tricuspid stenosis]] in operatively excised valves in patients >15years
{| class="wikitable"
{| class="wikitable"
! colspan="5" |Etiology of tricuspid stenosis in 97 operatively excised stenotic tricuspid valves
! colspan="5" |Etiology of tricuspid stenosis in 97 operatively excised stenotic tricuspid valves
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|-
|-
|}
|}
===Causes by Alphabetical Order<ref name="pmid19065003" /><ref name="pmid7720297" />===
===Causes by Alphabetical Order===


*[[Amyloidosis]]<ref name="pmid24797117" />
*[[Amyloidosis]]
*[[Carcinoid syndrome]]
*[[Carcinoid syndrome]]
*[[Cardiac tumor]]/[[Thrombus]]
*[[Cardiac tumor]]/[[Thrombus]]
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*[[Cyst|Giant blood cyst]]
*[[Cyst|Giant blood cyst]]
*[[Infective endocarditis]]
*[[Infective endocarditis]]
*[[Intravenous leiomyomatous tumor]]<ref name="pmid15226964" />
*[[Intravenous leiomyomatous tumor]]
*[[Metastatic tumor]]
*[[Metastatic tumor]]
*[[Myxoma]]
*[[Myxoma]]
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==Differential Diagnosis==
==Differential Diagnosis==
The differential diagnosis of [[tricuspid stenosis]] include valvular abnormalities causing a similar clinical presentation, and other causes of systemic venous congestion. The [[heart murmur]] of tricuspid stenosis must be differentiated from that of other valvular diseases. However, it should be noted that tricuspid stenosis often co-exist with other valvular pathologies such as [[tricuspid regurgitation]], [[mitral valve]] and aortic valve abnormalities.<ref name="pmid7720297" /> Tricuspid stenosis is characterized by a [[mid diastolic murmur]] best heard over the left sternal border. It has a rumbling character, a tricuspid opening snap with wide splitting of [[S1]]. The differential diagnosis of tricuspid stenosis includes:
The differential diagnosis of [[tricuspid stenosis]] includes valvular abnormalities causing a similar clinical presentation, and other causes of systemic venous congestion. The [[heart murmur]] of [[tricuspid stenosis]] must be differentiated from that of other [[Valvular Diseases|valvular]] diseases. However, it should be noted that [[tricuspid stenosis]] often co-exists with other valvular pathologies such as [[tricuspid regurgitation]], [[mitral valve]] and [[aortic valve]] abnormalities. [[Tricuspid stenosis]] is characterized by a [[mid diastolic murmur]] best heard over the left [[sternal]] border. It has a rumbling character, a [[tricuspid]] opening snap with the wide splitting of [[S1]]. The differential diagnosis of [[tricuspid stenosis]] includes:


*[[Aortic regurgitation]]: The diastolic murmur of [[aortic regurgitation]] decreases with respiration, which is in contrast to that of [[tricuspid stenosis]].
*[[Aortic regurgitation]]: The [[Diastolic murmurs|diastolic murmur]] of [[aortic regurgitation]] decreases with [[respiration]], which is in contrast to that of [[tricuspid stenosis]].
*[[Mitral regurgitation]]: The murmur of [[mitral regurgitation]] is blowing, soft and best heard at the apex.
*[[Mitral regurgitation]]: The [[Heart murmur|murmur]] of [[mitral regurgitation]] is blowing, soft and best heard at the [[apex]].
*[[Mitral stenosis]]: The murmur of [[mitral stenosis]] is mid-diastolic, rumbling, and best heard after the opening snap.
*[[Mitral stenosis]]: The murmur of [[mitral stenosis]] is mid-diastolic, rumbling, and best heard after the opening snap.
*[[Tricuspid regurgitation]]: The murmur of [[tricuspid regurgitation]] is blowing, [[Holosystolic murmur|holosystolic]], and best heard over the fourth intercostal area at the left sternal border.
*[[Tricuspid regurgitation]]: The murmur of [[tricuspid regurgitation]] is blowing, [[Holosystolic murmur|holosystolic]], and best heard over the fourth intercostal area at the left sternal border.


Tricuspid stenosis should also be differentiated from diseases causing a similar clinical presentation,such as:
[[Tricuspid stenosis]] should also be differentiated from [[Disease|diseases]] causing a similar clinical presentation, such as:


*[[Tricuspid atresia (patient information)|Tricuspid atresia]]
*[[Tricuspid atresia (patient information)|Tricuspid atresia]]
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==Epidemiology and Demographics==
==Epidemiology and Demographics==
TS is the least common valvular disease.<ref name="pmid19065003" /> TS is rarely an isolated disease, it is mostly associated with mitral and/or aortic valve abnormalities with/without concomitant [[tricuspid regurgitation]].<ref name="pmid9665226" />
TS is the least common valvular disease. TS is rarely an isolated disease, it is mostly associated with mitral and/or aortic valve abnormalities with/without concomitant [[tricuspid regurgitation]].
====Prevalence====
====Prevalence====
A prospective study of the [[echocardiographic]] profile of tricuspid valve disease in 788 patients with rheumatic heart disease in India was done. 9% of the patients had [[tricuspid valve disease]] and half of these patients with tricuspid valve disease had tricuspid stenosis with/without [[tricuspid regurgitation]]. The prevalence of TS is lower in developed countries compared to the developing countries due to the low prevalence of [[rheumatic heart disease]].
A prospective study of the [[echocardiographic]] profile of tricuspid valve disease in 788 patients with rheumatic heart disease in India was done. 9% of the patients had [[tricuspid valve disease]] and half of these patients with tricuspid valve disease had tricuspid stenosis with/without [[tricuspid regurgitation]]. The prevalence of TS is lower in developed countries compared to the developing countries due to the low prevalence of [[rheumatic heart disease]].
====Gender====
====Gender====
Most patients with [[Rheumatic Fever|rheumatic]] tricuspid stenosis are young women with mitral and/or [[aortic valve disease]].<ref name="pmid9665226" />
Most patients with [[Rheumatic Fever|rheumatic]] tricuspid stenosis are young women with mitral and/or [[aortic valve disease]].
==Risk Factors==
==Risk Factors==
One of the most recognized risk factors for TS is [[rheumatic fever]].
One of the most recognized risk factors for TS is [[rheumatic fever]].
==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
====Natural history====
====Natural history====
The natural course of tricuspid stenosis is not well defined. It is extremely rare for TS to occur in isolation, it is usually associated with existing [[mitral valve disease]] with/without concomitant [[tricuspid regurgitation]]. The most common cause of TS is rheumatic heart disease and it is usually associated with coexisting mitral valve and/or aortic valve abnormality.<ref name="pmid9665226​" />  TS of rheumatic etiology usually occurs with [[tricuspid regurgitation]]. Tricuspid stenosis often takes years to develop<ref name="pmid7720297" /> ,with  some exceptions such as congenital causes and active [[infective endocarditis]].<ref name="pmid7720297" /> Complications of tricuspid stenosis include [[heart failure]], [[liver failure]], and [[stroke]].
The natural course of tricuspid stenosis is not well defined. It is extremely rare for TS to occur in isolation, it is usually associated with existing [[mitral valve disease]] with/without concomitant [[tricuspid regurgitation]]. The most common cause of TS is rheumatic heart disease and it is usually associated with coexisting mitral valve and/or aortic valve abnormality. TS of rheumatic etiology usually occurs with [[tricuspid regurgitation]]. Tricuspid stenosis often takes years to develop, with  some exceptions such as congenital causes and active [[infective endocarditis]]. Complications of tricuspid stenosis include [[heart failure]], [[liver failure]], and [[stroke]].
====Complications of TS<ref name="pmid9665226​" /><ref name="pmid15786615" />====
====Complications of TS====


*[[Right atrial enlargement]]
*[[Right atrial enlargement]]
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====Prognosis====
====Prognosis====
With medical intervention, severe [[tricuspid stenosis]] appears well tolerated over several years of follow-up.<ref name="pmid9665226​" />
With medical intervention, severe [[tricuspid stenosis]] appears well tolerated over several years of follow-up.<br />
<br />
==Diagnosis==
==Diagnosis==
===History and Symptoms===
===History and Symptoms===
Tricuspid stenosis is mostly associated with [[mitral valve]] abnormalities. Common symptoms include [[dyspnea]], [[peripheral edema]], and [[fatigue]].
[[Tricuspid stenosis]] is mostly associated with [[mitral valve]] abnormalities. Common symptoms include [[dyspnea]], [[peripheral edema]], and [[fatigue]].
===Signs and Symptoms<ref name="pmidPMID: 9665226" />===
===Signs and Symptoms===


*[[Fatigue]]
*[[Fatigue]]
*[[Dyspnea]]
*[[Dyspnea]]
*[[Abdominal discomfort]] (due to [[hepatomegaly]] secondary to systemic venous congestion)
*[[Abdominal discomfort]] (due to [[hepatomegaly]] secondary to systemic [[venous]] congestion)
*[[Pedal edema]]
*[[Pedal edema]]
*[[Jugular venous distension]]
*[[Jugular venous distension]]
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===Physical Examination===
===Physical Examination===
Tricuspid stenosis often co-exists with [[mitral stenosis]], thus depending on the severity of mitral valve pathology, symptoms differ. The diagnosis of TS may also be missed when they coexist. Patients can lay flat without any symptoms in the absence of serious mitral valve pathology and thus, not present with any signs of [[dyspnea]]. Characteristic findings of TS include an opening snap and a low to medium pitch diastolic rumbling murmur, usually localized to the lower left sternal border (fourth intercostal space) with inspiratory accentuation.
[[Tricuspid stenosis]] often co-exists with [[mitral stenosis]], thus depending on the severity of [[mitral valve]] pathology, [[Symptom|symptoms]] differ. The diagnosis of TS may also be missed when they coexist. Patients can lay flat without any [[Symptom|symptoms]] in the absence of serious [[mitral valve]] pathology and thus, not present with any signs of [[dyspnea]]. Characteristic findings of TS include an opening snap and a low to medium pitch diastolic rumbling [[murmur]], usually localized to the lower left sternal border (fourth [[intercostal]] space) with inspiratory accentuation.
===Echocardiogram===
===Echocardiogram===
[[Transthoracic echocardiography]] ([[TTE]]) should be performed among patients with suspected TS to confirm the diagnosis, determine the etiology, and establish the baseline severity. [[TTE]] commonly reveals findings associated with other valvular diseases, such as [[tricuspid regurgitation]] and/or [[mitral stenosis]]. TS is mainly characterized by an elevated transvalvular gradient.<ref name="pmid19065003" /> [[TTE]] helps in the determination of the anatomic and hemodynamic characteristics of the [[tricuspid valve]]. [[TTE]] allows the detection of the following:<ref name="pmid19065003" />
[[Transthoracic echocardiography]] ([[TTE]]) should be performed among patients with suspected TS to confirm the [[diagnosis]], determine the [[etiology]], and establish the baseline severity. [[TTE]] commonly reveals findings associated with other [[Valvular Diseases|valvular]] diseases, such as [[tricuspid regurgitation]] and/or [[mitral stenosis]]. TS is mainly characterized by an elevated transvalvular gradient. [[TTE]] helps in the determination of the [[anatomic]] and [[Hemodynamics|hemodynamic]] characteristics of the [[tricuspid valve]]. [[TTE]] allows the detection of the following:


*[[Tricuspid valve]] thickening and calcification
*[[Tricuspid valve]] thickening and [[calcification]]
*Chordal thickening and [[calcification]]
*Chordal thickening and [[calcification]]
*Decreased valve mobility with diastolic doming
*Decreased [[valve]] mobility with diastolic doming
*Reduced leaflet separation at peak opening
*Reduced leaflet separation at peak opening
*Immobility of the leaflets ('frozen' appearance seen in [[carcinoid syndrome]])
*Immobility of the leaflets ('frozen' appearance seen in [[carcinoid syndrome]])
*Atrial tumors or metastatic lesions
*[[Atrial tumor|Atrial tumors]] or [[metastatic]] [[Lesion|lesions]]
*Valvular  vegetations (suggestive of [[infective endocarditis]])
*Valvular  [[Vegetation (pathology)|vegetations]] (suggestive of [[infective endocarditis]])
*Right atrial ball valve [[thrombus]]
*Right atrial ball valve [[thrombus]]


[[Doppler echocardiography]]: The evaluation of the severity of tricuspid stenosis is primarily done using the haemodynamic information provided by continuous wave Doppler (CWD). Doppler echocardiography is useful to assess the severity of TS through the evaluation of the transvalvular gradient (the hallmark of a stenotic valve is an increase in transvalvular velocity recorded by CWD).<ref name="pmidPMID: 19065003" /><ref name="pmid19065003" /> The assessment of the [[tricuspid valve]] area is limited by the common association of TS with [[tricuspid regurgitation]]. The coexistence of [[tricuspid regurgitation]] causes the underestimation of the tricuspid valvular area.  A tricuspid valve area  < 1.0 cm<sup>2</sup> is associated with increased severity of the TS.<ref name="pmid19065003" />
[[Doppler echocardiography]]: The evaluation of the severity of [[tricuspid stenosis]] is primarily done using the hemodynamic information provided by continuous-wave Doppler ([[CWD]]). Doppler [[echocardiography]] is useful to assess the severity of TS through the evaluation of the transvalvular gradient (the hallmark of a stenotic valve is an increase in transvalvular velocity recorded by CWD). The assessment of the [[tricuspid valve]] area is limited by the common association of TS with [[tricuspid regurgitation]]. The coexistence of [[tricuspid regurgitation]] causes the underestimation of the tricuspid valvular area.  A tricuspid valve area  < 1.0 cm<sup>2</sup> is associated with increased severity of the TS.
===Findings Associated with Increased Severity===
===Findings Associated with Increased Severity===
TTE findings that are associated with increased severity of tricuspid stenosis include:<ref name="pmid19065003" />
[[TTE]] findings that are associated with increased severity of [[tricuspid stenosis]] include:


*Mean pressure gradient >5 mm Hg,
*Mean pressure gradient >5 mm Hg,
*Pressure half-time >190 milliseconds
*Pressure half-time >190 milliseconds
*Tricuspid valve area  < 1.0 cm2
*[[Tricuspid valve]] area  < 1.0 cm2
*Enlargement of the [[right atrium]]
*Enlargement of the [[right atrium]]
*Dilation of the [[inferior vena cava]]
*[[Dilation]] of the [[inferior vena cava]]


==2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary==
==2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary==
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|}<br />
|}<br />
===Electrocardiogram===
===Electrocardiogram===
The [[electrocardiogram]] of patients with TS can demonstrate a sinus rhythm with or without right atrial hypertrophy.<ref name="pmid15786615" />  Patients with TS experience frequent arrhythmias, particularly [[atrial flutter]] and/or [[atrial fibrillation]] due to the enlargement of the [[right atrium]]. EKG findings suggestive of coexisting mitral valve disease can also be seen.
The [[electrocardiogram]] of patients with TS can demonstrate a [[sinus rhythm]] with or without right [[atrial]] [[hypertrophy]]. Patients with TS experience frequent [[arrhythmias]], particularly [[atrial flutter]] and/or [[atrial fibrillation]] due to the enlargement of the [[right atrium]]. [[The electrocardiogram|EKG]] findings suggestive of coexisting [[mitral valve]] disease can also be seen.<br />
<br />
===Chest X ray===
===Chest X ray===
The chest X-ray in a patient with [[tricuspid stenosis]] may show right atrial enlargement. The heart size can range from a normal-sized heart to [[cardiomegaly]], with additional findings suggestive of coexisting valvular pathology such as [[mitral stenosis]].
The chest [[X-rays|X-ray]] in a patient with [[tricuspid stenosis]] may show right [[atrial]] enlargement. The heart size can range from a normal-sized heart to [[cardiomegaly]], with additional findings suggestive of coexisting valvular pathology such as [[mitral stenosis]].<br />
<br />
===Cardiac MRI===
===Cardiac MRI===
While echocardiography remains the diagnostic imaging modality of choice, [[cardiac MRI]] is useful to evaluate tricuspid stenosis when the results of the [[echocardiography]] are insufficient.
While [[echocardiography]] remains the diagnostic imaging modality of choice, [[cardiac MRI]] is useful to evaluate [[tricuspid stenosis]] when the results of the [[echocardiography]] are insufficient.
==ACC/AHA Guidelines- ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance (DO NOT EDIT)==
==ACC/AHA Guidelines- ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance (DO NOT EDIT)==
{{cquote|
{{cquote|
CMR may be used for assessing individuals with valvular heart disease in which evaluation of valvular stenosis, regurgitation, para- or perivalvular masses, perivalvular complications of infectious processes, or prosthetic valve disease are needed. CMR may be useful in identifying serial changes in LV volumes or mass in patients with valvular dysfunction.
CMR may be used for assessing individuals with valvular heart disease in which evaluation of valvular stenosis, regurgitation, para- or perivalvular masses, perivalvular complications of infectious processes, or prosthetic valve disease are needed. CMR may be useful in identifying serial changes in LV volumes or mass in patients with valvular dysfunction.
}}<br />
}}
===Cardiac Catheterization===
 
While [[echocardiography]] remains the diagnostic imaging modality of choice, [[cardiac catheterization]] is useful to evaluate tricuspid stenosis when the results of the non-invasive testing are insufficient, particularly among patients who are being evaluated for other conditions such as [[mitral stenosis]] and [[pulmonary hypertension]].<ref name="pmid24603191" />  In the older pre-surgery population, [[cardiac catheterization]] may be necessary in order to assess concomitant artery disease.
=== Cardiac Catheterization ===
While [[echocardiography]] remains the diagnostic imaging modality of choice, [[cardiac catheterization]] is useful to evaluate tricuspid stenosis when the results of the non-invasive testing are insufficient, particularly among patients who are being evaluated for other conditions such as [[mitral stenosis]] and [[pulmonary hypertension]]. In the older pre-surgery population, [[cardiac catheterization]] may be necessary in order to assess concomitant artery disease.


Catheterization of the right heart is useful for the evaluation of:
Catheterization of the right heart is useful for the evaluation of:


*The gradient across the [[tricuspid valve]]
*The gradient across the [[tricuspid valve]]
*Associated congenital defects
*Associated [[congenital defects]]


Catheterization of the left heart is useful for the assessment of hemodynamic changes related to the [[Aortic valve|aortic]] and [[mitral valve]]s in patients with [[rheumatic heart disease]].
[[Catheterization]] of the left heart is useful for the assessment of hemodynamic changes related to the [[Aortic valve|aortic]] and [[mitral valve]]s in patients with [[rheumatic heart disease]].
==2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary<ref name="pmid24589852" />==
==2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary==
{| class="wikitable"
{| class="wikitable"
|-
|-
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| bgcolor="lemonchiffon" |<nowiki>"</nowiki>'''1.''' Invasive hemodynamic assessment of severity of [[TS]] may be considered in symptomatic patients when clinical and noninvasive data are discordant.  ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
| bgcolor="lemonchiffon" |<nowiki>"</nowiki>'''1.''' Invasive hemodynamic assessment of severity of [[TS]] may be considered in symptomatic patients when clinical and noninvasive data are discordant.  ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|-
|}<br />
|}
==Treatment==
 
== Treatment ==
 
===Medical Therapy===
===Medical Therapy===
Medical therapy with [[diuretics]] and sodium restriction for patients with TS with [[systemic venous congestion]]. Patients with TS should receive medical therapy for [[left heart failure]], and/or [[pulmonary hypertension]] if they are present.<ref name="pmid24589852" /> Treatment of the underlying etiology and associated conditions/complications is necessary. [[Fibrinolytic therapy]] is the first line therapy for prosthetic tricuspid valve thrombosis resulting in tricuspid stenosis.
Medical therapy with [[diuretics]] and sodium restriction for patients with TS with systemic venous congestion. Patients with TS should receive medical therapy for [[left heart failure]], and/or [[pulmonary hypertension]] if they are present. Treatment of the underlying [[etiology]] and associated conditions/[[complications]] is necessary. [[Fibrinolytic therapy]] is the first line therapy for prosthetic [[tricuspid valve]] [[thrombosis]] resulting in [[tricuspid stenosis]].
===Surgery===
===Surgery===
[[Tricuspid valve]] surgery is recommended for patients undergoing surgical intervention for left valvular disease as well as among patients with severe symptomatic isolated TS.<ref name="pmid24589852" /> Tricuspid valve [[balloon valvuloplasty]] has a limited efficacy in the management of tricuspid stenosis.<ref name="pmid24589852" /><ref name="pmidPMID: 27048553" />
[[Tricuspid valve]] surgery is recommended for patients undergoing surgical intervention for left valvular disease as well as among patients with severe symptomatic isolated TS. Tricuspid valve [[balloon valvuloplasty]] has a limited efficacy in the management of [[tricuspid stenosis]].
==2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary<ref name="pmid24589852" />==
==2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary==
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Latest revision as of 04:44, 2 April 2020

Tricuspid stenosis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2] Mohammed Salih, M.D. Syed Musadiq Ali M.B.B.S.[3]

Overview

Tricuspid stenosis (TS) is a type of valvular heart disease where there is a narrowing of the orifice of the tricuspid valve of the heart. A majority of stenotic tricuspid valves are associated with evidence of tricuspid regurgitation. Tricuspid stenosis is quite uncommon in developed countries due to the low prevalence of rheumatic heart disease, which is the commonest cause of TS. It is the least common valvular stenosis lesion, and generally accompanies mitral and/or aortic valve involvement. It is extremely rare to have isolated acquired tricuspid stenosis. Rheumatic tricuspid valve disease seldom receives much attention and can be easily overlooked on routine clinical and echocardiographic examination, which may lead to postoperative problems after successfully relieving left-sided valvular disease. The clinical findings associated with rheumatic mitral valve disease are also more severe than that of rheumatic tricuspid valve disease, making it rather easy to miss the diagnosis of concomitant tricuspid stenosis (TS). There is a paucity of literature on the prevalence and management of rheumatic tricuspid valve disease. Most of the literature on rheumatic tricuspid stenosis is old, which may be reflective of the low prevalence of rheumatic heart disease in developed countries. However, developing countries and the Indian subcontinent still have a significant prevalence of rheumatic tricuspid valve disease, occurring mostly in young women. Stenotic tricuspid valves are usually anatomically abnormal, and often take years to develop, with few exceptions such as congenital causes, active endocarditis.

Classification

Tricuspid stenosis is staged based on the valve anatomy and hemodynamics, and the hemodynamic consequences.

Stage Definition Valve anatomy Valve hemodynamics Hemodynamic consequences Symptoms
C, D Severe TS Thickened, distorted, calcified leaflets
  • T ½ ≥190 ms 
  • Valve area ≤1.0 cm2
 Right atrial / Inferior vena cava enlargement
  • Stage C-No symptoms
  • Stage D-Symptoms variable and dependent on the severity of associated valve disease and degree of obstruction

Pathophysiology

TS is characterized by structural changes in the tricuspid valve. The pathophysiology of tricuspid valve stenosis depends on the underlying etiology. In rheumatic heart disease which is the most common cause of TS, there is diffuse scarring and fibrosis of the valve leaflets, fusion of the commissures, and shortening of the chordae tendineae as a result of inflammation. These abnormalities limit leaflet mobility and reduce the size of the tricuspid orifice, increasing the transtricuspid diastolic gradient, which can eventually result in systemic venous hypertension and congestion.

The pathophysiology of tricuspid stenosis based on the underlying etiology:

Causes

The most common cause of TS is rheumatic heart disease. Other causes of TS include carcinoid syndrome, congenital abnormalities, endocarditis, lupus, and mechanical obstruction by a tumor.

Common Causes

Etiology of tricuspid stenosis in operatively excised valves in patients >15years

Etiology of tricuspid stenosis in 97 operatively excised stenotic tricuspid valves
Rheumatic Carcinoid Congenital
Ebstein's anomaly Complex heart disease Shortened chordae and/or fused commissure
90 3 1 2 1

Causes by Organ System

Cardiovascular Congenital heart disease, cardiac tumor, saphenous vein bypass graft aneurysm, Ebstein's anomaly, endomyocardial fibrosis, infective endocarditis, myxoma, thrombus, rheumatic heart disease
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect Methysergide
Ear Nose Throat No underlying causes
Endocrine Carcinoid syndrome
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic No underlying causes
Hematologic No underlying causes
Iatrogenic Pacemaker infection, pacemaker leads, device closure of right coronary arteriovenous fistula.
Infectious Disease Infective endocarditis
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic Fabry disease, Whipple's disease
Obstetric/Gynecologic No underlying causes
Oncologic Carcinoid syndrome, cardiac tumor, intravenous leiomyomatous tumor, metastatic tumor, myxoma
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy Amyloidosis, systemic lupus erythematosus
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous Giant blood cyst

Causes by Alphabetical Order

Differential Diagnosis

The differential diagnosis of tricuspid stenosis includes valvular abnormalities causing a similar clinical presentation, and other causes of systemic venous congestion. The heart murmur of tricuspid stenosis must be differentiated from that of other valvular diseases. However, it should be noted that tricuspid stenosis often co-exists with other valvular pathologies such as tricuspid regurgitation, mitral valve and aortic valve abnormalities. Tricuspid stenosis is characterized by a mid diastolic murmur best heard over the left sternal border. It has a rumbling character, a tricuspid opening snap with the wide splitting of S1. The differential diagnosis of tricuspid stenosis includes:

Tricuspid stenosis should also be differentiated from diseases causing a similar clinical presentation, such as:

Epidemiology and Demographics

TS is the least common valvular disease. TS is rarely an isolated disease, it is mostly associated with mitral and/or aortic valve abnormalities with/without concomitant tricuspid regurgitation.

Prevalence

A prospective study of the echocardiographic profile of tricuspid valve disease in 788 patients with rheumatic heart disease in India was done. 9% of the patients had tricuspid valve disease and half of these patients with tricuspid valve disease had tricuspid stenosis with/without tricuspid regurgitation. The prevalence of TS is lower in developed countries compared to the developing countries due to the low prevalence of rheumatic heart disease.

Gender

Most patients with rheumatic tricuspid stenosis are young women with mitral and/or aortic valve disease.

Risk Factors

One of the most recognized risk factors for TS is rheumatic fever.

Natural History, Complications, and Prognosis

Natural history

The natural course of tricuspid stenosis is not well defined. It is extremely rare for TS to occur in isolation, it is usually associated with existing mitral valve disease with/without concomitant tricuspid regurgitation. The most common cause of TS is rheumatic heart disease and it is usually associated with coexisting mitral valve and/or aortic valve abnormality. TS of rheumatic etiology usually occurs with tricuspid regurgitation. Tricuspid stenosis often takes years to develop, with some exceptions such as congenital causes and active infective endocarditis. Complications of tricuspid stenosis include heart failure, liver failure, and stroke.

Complications of TS

Prognosis

With medical intervention, severe tricuspid stenosis appears well tolerated over several years of follow-up.

Diagnosis

History and Symptoms

Tricuspid stenosis is mostly associated with mitral valve abnormalities. Common symptoms include dyspnea, peripheral edema, and fatigue.

Signs and Symptoms

Physical Examination

Tricuspid stenosis often co-exists with mitral stenosis, thus depending on the severity of mitral valve pathology, symptoms differ. The diagnosis of TS may also be missed when they coexist. Patients can lay flat without any symptoms in the absence of serious mitral valve pathology and thus, not present with any signs of dyspnea. Characteristic findings of TS include an opening snap and a low to medium pitch diastolic rumbling murmur, usually localized to the lower left sternal border (fourth intercostal space) with inspiratory accentuation.

Echocardiogram

Transthoracic echocardiography (TTE) should be performed among patients with suspected TS to confirm the diagnosis, determine the etiology, and establish the baseline severity. TTE commonly reveals findings associated with other valvular diseases, such as tricuspid regurgitation and/or mitral stenosis. TS is mainly characterized by an elevated transvalvular gradient. TTE helps in the determination of the anatomic and hemodynamic characteristics of the tricuspid valve. TTE allows the detection of the following:

Doppler echocardiography: The evaluation of the severity of tricuspid stenosis is primarily done using the hemodynamic information provided by continuous-wave Doppler (CWD). Doppler echocardiography is useful to assess the severity of TS through the evaluation of the transvalvular gradient (the hallmark of a stenotic valve is an increase in transvalvular velocity recorded by CWD). The assessment of the tricuspid valve area is limited by the common association of TS with tricuspid regurgitation. The coexistence of tricuspid regurgitation causes the underestimation of the tricuspid valvular area. A tricuspid valve area < 1.0 cm2 is associated with increased severity of the TS.

Findings Associated with Increased Severity

TTE findings that are associated with increased severity of tricuspid stenosis include:

2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary

Class I
"1. TTE is indicated in patients with TS to assess the anatomy of the valve complex, evaluate severity of stenosis, and characterize any associated regurgitation and/or left-sided valve disease. (Level of Evidence: C)"


Electrocardiogram

The electrocardiogram of patients with TS can demonstrate a sinus rhythm with or without right atrial hypertrophy. Patients with TS experience frequent arrhythmias, particularly atrial flutter and/or atrial fibrillation due to the enlargement of the right atrium. EKG findings suggestive of coexisting mitral valve disease can also be seen.

Chest X ray

The chest X-ray in a patient with tricuspid stenosis may show right atrial enlargement. The heart size can range from a normal-sized heart to cardiomegaly, with additional findings suggestive of coexisting valvular pathology such as mitral stenosis.

Cardiac MRI

While echocardiography remains the diagnostic imaging modality of choice, cardiac MRI is useful to evaluate tricuspid stenosis when the results of the echocardiography are insufficient.

ACC/AHA Guidelines- ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance (DO NOT EDIT)

“

CMR may be used for assessing individuals with valvular heart disease in which evaluation of valvular stenosis, regurgitation, para- or perivalvular masses, perivalvular complications of infectious processes, or prosthetic valve disease are needed. CMR may be useful in identifying serial changes in LV volumes or mass in patients with valvular dysfunction.

”

Cardiac Catheterization

While echocardiography remains the diagnostic imaging modality of choice, cardiac catheterization is useful to evaluate tricuspid stenosis when the results of the non-invasive testing are insufficient, particularly among patients who are being evaluated for other conditions such as mitral stenosis and pulmonary hypertension. In the older pre-surgery population, cardiac catheterization may be necessary in order to assess concomitant artery disease.

Catheterization of the right heart is useful for the evaluation of:

Catheterization of the left heart is useful for the assessment of hemodynamic changes related to the aortic and mitral valves in patients with rheumatic heart disease.

2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary

Class IIb
"1. Invasive hemodynamic assessment of severity of TS may be considered in symptomatic patients when clinical and noninvasive data are discordant. (Level of Evidence: C)"

Treatment

Medical Therapy

Medical therapy with diuretics and sodium restriction for patients with TS with systemic venous congestion. Patients with TS should receive medical therapy for left heart failure, and/or pulmonary hypertension if they are present. Treatment of the underlying etiology and associated conditions/complications is necessary. Fibrinolytic therapy is the first line therapy for prosthetic tricuspid valve thrombosis resulting in tricuspid stenosis.

Surgery

Tricuspid valve surgery is recommended for patients undergoing surgical intervention for left valvular disease as well as among patients with severe symptomatic isolated TS. Tricuspid valve balloon valvuloplasty has a limited efficacy in the management of tricuspid stenosis.

2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary

Class I
"1.Tricuspid valve surgery is recommended for patients with severe TS at the time of operation for left-sided valve disease. (Level of Evidence: C)"
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