Tricuspid stenosis

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]

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.

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

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:

• Rheumatic tricuspid stenosis:
  • 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.
  • 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:
  • Fibrous white plaques located on the valvular and mural endocardium are characteristic presentations of carcinoid valve lesions.
  • Valve leaflets become thick, rigid and smaller in area.
  • Atrial and ventricular surfaces of the valve structure contain fibrous tissue proliferation.
• Congenital tricuspid stenosis:
  • More common in infants
  • Lesions may present in a number of different ways, either singularly or in any combination of the following:
    • Incompletely developed leaflets
    • Shortened or malformed chordae
    • Small annuli
    • Papillary muscles of abnormal size and number
• Mechanical obstruction of flow through the tricuspid valve:
  • Supravalvular obstruction from congenital diaphragms
  • Intracardiac or extracardiac tumors
  • Thrombosis or emboli
  • Large endocarditis vegetations

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.

• Rheumatic heart disease (majority of the cases)
• Carcinoid syndrome
• Congenital

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

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

• Amyloidosis
• Carcinoid syndrome
• Cardiac tumor/Thrombus
• Congenital heart disease
• Ebstein’s anomaly
• Endomyocardial fibrosis
• Fabry disease
• Giant blood cyst
• Infective endocarditis
• Intravenous leiomyomatous tumor
• Metastatic tumor
• Myxoma
• Pacemaker infection
• Pacemaker leads
• Rheumatic heart disease
• Systemic lupus erythematosus
• Whipple’s disease

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:

• Aortic regurgitation: The 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 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, 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 atresia
• Constrictive pericarditis
• Restrictive cardiomyopathy
• Atrial myxoma

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.

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.

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

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

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.

• Right atrial enlargement
• Atrial fibrillation
• Heart failure
• Infective endocarditis
• Cerebrovascular accident
• Liver failure

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

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

• Fatigue
• Dyspnea
• Abdominal discomfort (due to hepatomegaly secondary to systemic venous congestion)
• Pedal edema
• Jugular venous distension
• Heart murmur

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.

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:

• Tricuspid valve thickening and calcification
• Chordal thickening and calcification
• Decreased valve mobility with diastolic doming
• Reduced leaflet separation at peak opening
• Immobility of the leaflets (‘frozen’ appearance seen in carcinoid syndrome)
• Atrial tumors or metastatic lesions
• Valvular vegetations (suggestive of infective endocarditis)
• Right atrial ball valve thrombus

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.

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

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

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)”

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.

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.

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.

“

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.

”

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:

• The gradient across the tricuspid valve
• Associated congenital defects

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.

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)”

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.

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.

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)”

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

Tricuspid stenosis (TS) staging sections into categories A, B, C, D. Stages C (without symptoms) and D (with symptoms). When valve and/or chordal thickening and calcification are evident, there are additional findings indicative of severe TS, for example, pressure gradient greater than or equal to 5 mm Hg, pressure half-time greater than or equal to 190 milliseconds, valve area less than or equal to 1.0 cm^2, associated moderate right atrial enlargement, and inferior vena cava dilatation.

Tricuspid stenosis (TS) is staged based on the valve anatomy and hemodynamics, and the hemodynamic consequences. No criteria for Stage A or B were included in the 2014 American Heart Association/American College of Cardiology valve guidelines. Stage C is defined as severe TS without symptoms. Stage D is defined as severe TS with symptoms.^[1][2]

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

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohammed Salih, M.D. Syed Musadiq Ali M.B.B.S.[2] Fatimo Biobaku M.B.B.S [3] Vamsikrishna Gunnam M.B.B.S [4]

Tricuspid stenosis (TS) is characterized by structural changes in the tricuspid valve. The pathophysiology of tricuspid valve depends on the underlying etiology. In rheumatic heart disease which is the most common cause of tricuspid stenosis, there is fibrous thickening of the valve leaflets and chordae tendineae with/without fusion of the commissures as a result of inflammation. The obstruction to right ventricular filling due to the stenotic tricuspid valve can result in systemic venous hypertension and congestion.

• The tricuspid disease is characterized by diffuse fibrous thickening of the leaflets and fusion of 2 or 3. ^{[1][2][3][4][5]}
• Leaflet thickening usually occurs in the absence of calcific deposits, and the anteroseptal commissure is most commonly involved.
• Incompletely developed leaflets, shortened or malformed chordae, a small annulus, or an abnormal number or size of papillary muscles may result in TS.
• The valves consist of an outer layer of valve endothelial cells (VECs) surrounding three layers of the extracellular matrix each with specialized function and interspersed with interstitial valve cells (VICs).
• Genetic or acquired/environmental causes that disrupt the normal organization and composition of the extracellular matrix and communication between VECs and VICs alter valve mechanics and interfere with the valve leaflet function, culminating in heart failure.
• As a result of valvular stenosis, there is a persistent diastolic pressure gradient between the right atrium and right ventricle. This gradient increases when blood flow across the tricuspid valve increases, as occurs with inspiration and exercise, and decreases when blood flow decreases, such as with expiration.^[6]
• The primary result of TS is right atrial pressure elevation and consequent right-sided congestion.^[7]
• During sinus rhythm, the right atrial “a” wave is increased and may approach the level of right ventricular systolic pressure.
• The resting cardiac output may be reduced and fails to increase with exercise.
• This may contribute to the only modestly elevated left atrial and pulmonary arterial pressures seen when mitral valve disease is also present.
• As a result, most patients with significant tricuspid stenosis have systemic venous congestion with jugular venous distension, ascites, and peripheral edema.
• The pathophysiology of tricuspid stenosis depends on the underlying etiology:^[5]

• Rheumatic tricuspid stenosis:^{[9][10][5][11]}
  • 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.
  • 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:
  • Fibrous white plaques located on the valvular and mural endocardium are characteristic presentations of carcinoid valve lesions.^[12][13][14]
  • Valve leaflets become thick, rigid and smaller in area.
  • Atrial and ventricular surfaces of the valve structure contain fibrous tissue proliferation.
• Congenital tricuspid stenosis:
  • More common in infants
  • Lesions may present in a number of different ways, either singularly or in any combination of the following:
    • Incompletely developed leaflets
    • Shortened or malformed chordae
    • Small annuli
    • Papillary muscles of abnormal size and number
• Infective endocarditis:
  • Stenosis may develop as a result of large infected vegetations obstructing the opening of the tricuspid valve.^[15]
• Other conditions may mimic tricuspid stenosis by the mechanical obstruction of flow through the tricuspid valve:
  • Supravalvular obstruction from congenital diaphragms
  • Intracardiac or extracardiac tumors
  • Thrombosis or emboli
  • Large endocarditis vegetations
  • Other conditions that impair right-sided filling
    • Constrictive pericarditis
    • Restrictive cardiomyopathy

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

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

• Rheumatic heart disease is one of the most common causes of TS and almost always occurs in conjunction with mitral stenosis.^[1][2][3]
• Large vegetations in infective endocarditis can cause relative stenosis.
• Carcinoid syndrome may cause isolated TS or mixed with the regurgitant lesion.^[4]
• Systemic diseases like systemic lupus erythematosus (SLE), antiphospholipid antibody (APLA) syndrome and the presence of permanent pacing and fusion of implantable cardioverter defibrillator leads to sub-valvular structures can cause tricuspid stenosis.^[5][6]
• Benign tumors like atrial myxomas can cause functional TS^[7].
• Blunt trauma has also been described as a risk factor. Renal and ovarian tumors can grow into the tricuspid orifice causing stenosis.

• Other less common causes of TS include congenital abnormalities (Ebstein’s anomaly), metabolic or enzymatic abnormalities (Fabry’s disease, Whipple’s disease).^[3][8]
• Sometimes described are intravenous leiomyomatosis, ventriculoatrial shunts causing TS.^[9][10]
• Valvulopathy associated with drugs like fenfluramine/phentermine and methysergide is characterized by thickened fibrotic and hypomobile tricuspid leaflets, with various degrees of valve stenosis and regurgitation.^[11]

Cardiovascular	Congenital heart disease, cardiac tumor, saphenous vein bypass graft aneurysm, Ebstein’s anomaly, endomyocardial fibrosis, infective endocarditis, myxoma, rheumatic heart disease.[12]
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[13], device closure of right coronary arteriovenous fistula.[14]
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,[10] 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,[15] systemic lupus erythematosus
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	Giant blood cyst

• Amyloidosis^[15]
• Carcinoid syndrome
• Cardiac tumor
• Congenital heart disease
• Ebstein’s anomaly
• Endomyocardial fibrosis
• Fabry disease
• Giant blood cyst
• Infective endocarditis
• Intravenous leiomyomatous tumor^[10]
• Metastatic tumor
• Myxoma
• Pacemaker infection
• Pacemaker leads
• Rheumatic heart disease
• Systemic lupus erythematosus
• Whipple’s disease

Template:WH

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohammed Salih, M.D. Syed Musadiq Ali M.B.B.S.[2] Rim Halaby, M.D. [3] Fatimo Biobaku M.B.B.S [4]

The differential diagnosis of tricuspid stenosis includes valvular abnormalities causing a similar clinical presentation, and other causes of systemic venous congestion such as constrictive pericarditis.

• The heart murmur of tricuspid stenosis must be differentiated from that of other valvular diseases. However, it should be noted that tricuspid stenosis can coexist with other valvular diseases such as tricuspid regurgitation, mitral valve and aortic valve abnormalities.^[1]
• 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.
  • Mitral regurgitation: The 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.
  • Tricuspid regurgitation: The murmur of tricuspid regurgitation is blowing, holosystolic, and best heard over the fourth intercostal area at the left sternal border.

• Tricuspid stenosis must be differentiated from diseases that can cause a similar clinical presentation, such as:
  • Tricuspid atresia
  • Constrictive pericarditis
  • Restrictive cardiomyopathy
  • Atrial myxoma

• Tricuspid stenosis must be differentiated from the following:^{[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]}

Diseases	History	Symptoms	Physical Examination	Murmur	Diagnosis				Other Findings
					ECG	CXR	Echocardiogram	Cardiac Catheterization
Mitral Stenosis	Age ( Mitral annular calcification in older patients) Rheumatic fever Endocarditis	Dyspnea on exertion Paroxysmal nocturnal dyspnea Orthopnea New onset atrial fibrillation	Mitral facies Heart murmur Jugular vein distension Apical impulse displaced laterally or not palpable Diastolic thrill at the apex Signs of heart failure in severe cases	Diastolic murmur Low pitched Opening snap followed by a decrescendo-crescendo rumbling murmur Best heard with the bell of the stethoscope at the apex at end-expiration in the left lateral decubitus position Intensity increases after a valsalva maneuver, after exercise and after increased after load (eg., squatting, isometric hand grip)	P mitrale Atrial fibrillation: No P waves and irregularly irregular rhythm Right axis deviation Right ventricular hypertrophy: Dominant R wave in V1 and V2	Straightening of the left border of the heart suggestive of enlargement of the left atrium Double right heart border (Enlarged left atrium and normal right atrium) Prominent left atrial appendage Splaying of subcarinal angle (>120 degrees) Calcification of mitral valve Kerley B lines	Reduced valve leaflet mobility Valve calcification Doming of mitral valve Valve thickening Enlargement of left atrium	Right heart catheterization: Pulmonary capillary wedge pressure (left atrial pressure) Left heart catheterization: Pressures in left ventricle Determines the gradient between the left and right atrium during ventricular diastole (a marker of the severity of mitral stenosis)	Hemoptysis (heart failure) Ortner’s syndrome
Mitral Regurgitation	CAD MI Rheumatic fever Endocarditis Mitral valve prolapse Cardiomyopathy Radiation therapy Trauma	Palpitations Symptoms of heart failure in severe cases	Palpation Brisk carotid upstroke and hyperdymanic carotid impulse on palpation Apical impulse is displaced to left S3 and a palpable thrill Auscultation Murmur	Holosystolic murmur High pitched, blowing Radiates to the axilla Best heard with the diaphragm of the stethoscope at the apex in left lateral decubitus position Intensity increases with hand grip or squatting Decrease in intensity on standing or valsalva maneuver	P mitrale in lead II Increased QRS voltage Right axis deviation Atrial fibrillation	Acute MR Kerley B lines No enlargement of cardiac silhouette Chronic MR Enlarged cardiac silhouette Straightening of left heart border Splaying of subcarinal angle Calcification of mitral annulus Double right heart border	Enlargement of left atrium and ventricle Identify valve abnormality Valve calcification Severity of regurgitation	Grading of MR is done with left ventriculography	Decompensated and acute MR may lead to heart failure
Atrial septal defect	Frequent respiratory or lung infections Dyspnea Tiring when feeding (Infants) Shortness of breath on exertion Palpitations Swelling of feet	Shortness of breath Fatigue Failure to thrive Swelling of feet and abdomen (Right heart failure) Palpitations Respiratory infections	Inspection Precordial bulge Precordial lift Palpation Right ventricular impulse Pulmonary artery pulsations Thrill Auscultation Murmur	Midsystolic (ejection systolic) murmur Widely split, fixed S2 Upper left sternal border	Normal Prolonged PR interval Right bundle branch block ECG findings varies according to the underlying type of ASD	Increased pulmonary markings Cardiomegaly Triangular appearance of heart Schimitar sign	Gold standard test for diagnosis of atrial septal defect (for more information click Atrial septal defect echocardiography)	Defect size Pulmonary venous return Pulmonary vascular resistance Pulmonary artery hypertension	Asymptomatic until later part of their life May be associated with migraine with aura
Left Atrial Myxoma	Dyspnea Orthopnea Pulmonary edema Hyperpigmentation of skin and endocrine activity Cerebral embolism	Symptoms may mimic mitral stenosis	Skin Signs of an embolic phenomenon Raynaud’s phenomenon Swelling Clubbing Auscultation: Lung: Fine crepitations Heart: Characteristic “tumor plop”	Early diastolic sound as “tumor plop” Low frequency diastolic murmur may be heard if the tumor obstructing mitral valve	Often normal	Often normal Rare findings: cardiomegaly Left atrial enlargement tumor calcification etc.,	Initial and most useful diagnostic study For more information click Myxoma echocardiography or ultrasound	Useful to detect vascular supply of the tumor by the coronary arteries	Associated with Carney complex (genetic predisposition)
Prosthetic Valve Obstruction	History of valve replacement Systemic embolism	Shortness of breath Fatigue	Ausculation Muffling of murmur	Muffling or disappearance of prosthetic sounds Appearance of new regurgitant or obstructive murmur			Degree of stenosis Assess thrombus size and location Differentiate between thrombus, pannus and vegetations		Causes: Thrombus Pannus formation
Cor Triatriatum	Dyspnea on exertion Recent onset of congestive heart failure	Dsypnea on exertion Orthopnea Tachypnea Palpitations Growth failure	Auscultation Murmur Other findings Signs of heart failure	Diastolic murmur with loud P2 No opening snap or a loud S1	Non specific but may have Right axis deviation Right atrial enlargement Right ventricular hypertrophy	Normal cardiac silhouette Hemodynamic changes similar to mitral stenosis (non specific findings)	Direct visualization of membrane through the atrium +/- visualization of accessory chamber	Normal left ventricular hemodynamic profile with a trans atrial gradient	Types Cor triatriatum sinistrum Cor triatriatum dextrum
Congenital Mitral Stenosis	Respiratory distress shortly after birth Recurrent severe pulmonary infections Other associated congenital cardiovascular anamolies Atrial fibrillation	Infants: Exhaustion and sweating on feeding Rapid breathing Failure to thrive Pulmonary infections Chronic cough Older patients: Dyspnea Orthopnea Paroxysmal nocturnal dyspnea Peripheral edema Fatigue	Auscultation Murmur Other findings Signs of heart failure	Mild-Moderate Loud S1 Loud P2 Low-frequency diastolic murmur best heard at the apex Severe Soft S1 Loud pulmonic component of S2 with the minimal respiratory splitting of S2 Holodiastolic murmur with presystolic accentuation best heard at the apex Early diastolic murmur of pulmonic valve regurgitation	Sharp P waves in leads I and II Inversion of P wave in lead III Marked Q waves in leads II and III	Left atrial dilation Moderate enlargement of right heart Pulmonary venous congestion Esophageal compression	Reduced valve leaflet mobility Left atrial size Severity of mitral stenosis		Very rare condition
Supravalvular Ring Mitral Stenosis	Other associated congenital heart defects Fatigue Frequent respiratory infections Failure to thrive Poor feeding Precocious congestive heart failure	Shortness of breath Tachypnea Dyspnea Nocturnal cough Heamoptysis Syncope	Auscultation: Lungs: Fine, crepitant rales and rhonchi or wheezes may be present Heart: Murmur	An apical mid diastolic murmur with presystolic accentuation No opening snap The murmur is more prominent if associated with VSD or PDA		Left atrial and ventricular enlargement Alveolar edema	Supramitral ring: Associated with normal mitral valve apparatus Intramitral ring: Hypomobility of the posterior leaflet Reduced interpapillary muscle distance Reduced chordal length Dominant papillary muscle Hypoplastic mitral annulus (Difficult to visualize membrane <1mm in size)	Persistently elevated pulmonary venous pressures Increased pulmonary artery pressure	Types Supramitral Intramitral It is attached between the opening of the atrial appendage and the mitral annulus which helps in differentiating with Cor triatriatum sinister. Intramitral type is associated with shone complex

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

Tricuspid stenosis (TS) is the least common valvular disease. TS is rarely an isolated disease, it is mostly associated with mitral valve and/or aortic valve abnormalities. Approximately 8% of patients with rheumatic heart disease develop isolated TS, while up to 50% develop tricuspid regurgitation and TS. The prevalence of TS is lower in developed countries compared to developing countries due to the low prevalence of rheumatic heart disease, which is the most common cause of TS.

• United States data shows that tricuspid stenosis is rare, occurring in less than 1% of the population.
• While found in approximately 15% of patients with rheumatic heart disease at autopsy, it is estimated to be clinically significant in only 5% of these patients.
• The incidence of the congenital form of the disease is less than 1%.^[1]
• Tricuspid stenosis is found in approximately 3% of the international population.
• Tricuspid stenosis is more prevalent in areas with a high incidence of rheumatic fever.^[2][3]

• Tricuspid stenosis can present as a congenital lesion or later in life when it is due to some other condition.
• The congenital form accounts for approximately 0.3% of all congenital heart disease cases.
• The frequency of tricuspid stenosis in the older population, due to secondary causes, ranges from 0.3-3.2%

• Tricuspid stenosis is observed more commonly in women than in men, similar to mitral stenosis of rheumatic origin.
• The congenital form of the disease has a slightly higher male predominance.

• No racial predisposition is apparent.

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

The most potent risk factor in the development of tricuspid valve stenosis is rheumatic fever or infective endocarditis. Other risk factors include congenital heart disease, pulmonary hypertension, heart failure, cardiac tumors, and heart attack.

• Common risk factors in the development of tricuspid valve stenosis include:^{[1][2][3][4][5][6]}
  • Rheumatic heart disease
  • Infective endocarditis
  • Carcinoid syndrome
  • Systemic lupus erythematosus (SLE)
  • Renal and ovarian tumors
  • Antiphospholipid antibody syndrome (APLA)
  • Atrial myxomas

• Less common risk factors in the development of tricuspid valve stenosis include:^{[7][8][9][10]}
  • Ebstein’s anomaly
  • Fabry’s disease
  • Whipple’s disease
  • Intravenous leiomyomatosis
  • Ventriculoatrial shunts
  • Blunt trauma

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

There is insufficient evidence to recommend routine screening for tricuspid stenosis.

There is insufficient evidence to recommend routine screening for tricuspid stenosis.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

• The prognosis of tricuspid stenosis depends on the precipitating/underlying cause.
• The general mortality rate is approximately 5%.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

The treatment is usually by surgery (tricuspid valve replacement) or percutaneous balloon valvuloplasty. The resultant tricuspid regurgitation from percutaneous treatment is better tolerated than insufficiency that occurs following mitral valvuloplasty

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