Tricuspid stenosis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rim Halaby, M.D. [2] Fatimo Biobaku M.B.B.S [3]
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. Tricuspid stenosis is quite uncommon, it is usually caused by rheumatic heart disease and generally accompanies mitral and/or aortic valve involvement.[1] A majority of stenotic tricuspid valves are associated with evidence of regurgiation that has been clinically documented through a physicial examination (murmur), echocardiogram, or angiogram. Stenotic tricuspid valves are anatomically abnormal and usually take years to develop, with few exceptions such as congenital causes, active infective endocarditis.
Classification
Tricuspid stenosis is staged based on the valve anatomy and hemodynamics, and the hemodynamic consequences.[2]
| Stage | Definition | Valve anatomy | Valve hemodynamics | Hemodynamic consequences | Symptoms |
|---|---|---|---|---|---|
| C, D | Severe TS | Thickened, distorted, calcified leaflets |
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Right atrial / Inferior vena cava enlargement |
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Pathophysiology
TS is characterized by structural changes in the tricuspid valve. The pathophysiology of the 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.[3] 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.[2]
The pathophysiology of tricuspid stenosis depends on the underlying etiology:[3]
- 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
- Infective endocarditis:
- Stenosis may develop as a result of large infected vegetations obstructing the opening of the tricuspid valve.
- 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
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.[4][3]
Common Causes
- Rheumatic heart disease (majority of the cases)[3]
- Carcinoid syndrome[3]
- Congenital[3]
Causes by Organ System
| Cardiovascular | Congenital heart disease, cardiac tumor, saphenous vein bypass graft aneurysm,[5] Ebstein's anomaly, endomyocardial fibrosis, infective endocarditis, myxoma, 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[6], device closure of right coronary arteriovenous fistula.[7] |
| 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,[8] 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,[9] systemic lupus erythematosus |
| Sexual | No underlying causes |
| Trauma | No underlying causes |
| Urologic | No underlying causes |
| Miscellaneous | Giant blood cyst |
Causes by Alphabetical Order
- Amyloidosis[9]
- Carcinoid syndrome
- Cardiac tumor
- Congenital heart disease
- Ebstein's anomaly
- Endomyocardial fibrosis
- Fabry disease
- Giant blood cyst
- Infective endocarditis
- Intravenous leiomyomatous tumor[8]
- Metastatic tumor
- Myxoma
- Pacemaker infection
- Pacemaker leads
- Rheumatic heart disease
- Systemic lupus erythematosus
- Whipple's disease
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.[3] 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:
- 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:
Epidemiology and Demographics
TS is the least common valvular disease. TS is rarely an isolated disease, it is mostly associated with mitral valve abnormalities and/or concomitant tricuspid regurgitation. Approximately 8% of patients with rheumatic heart disease develop isolated TS, while up to 50% develop tricuspid regurgitation and TS.[10] The prevalence of TS is lower in developed countries compared to developing countries due to the low prevalence of rheumatic heart disease, the most common cause of TS.
Risk Factors
One of the most recognized risk factors of TS is rheumatic fever.
Natural History, Complications, and Prognosis
TS rarely exists in isolation, it is usually associated with existing mitral valve abnormality and/or tricuspid regurgitation. Complications of TS include heart failure, liver failure, and stroke.[11]
Diagnosis
History and Symptoms
TS is mostly associated with mitral valve abnormalities. Common symptoms include dyspnea, peripheral edema, and fatigue.
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 diastolic rumbling murmur that is localized to the lower left sternal border at the fourth intercostal space and it increases with inspiration.
Electrocardiogram
The electrocardiogram of patients with TS can demonstrate a sinus rhythm with or without right atrial hypertrophy.[11] Patients with TS experience frequent arrhythmias, particularly atrial flutter and/or atrial fibrillation due to the enlargement of the right atrium.
Chest X-Ray
The chest X-ray in a patient with TS may be significant for a pronounced right atrial enlargement. The heart size can range from a normal-sized heart to cardiomegaly.
Echocardiography
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.[4]
Cardiac MRI
While echocardiography remains the diagnostic imaging modality of choice, cardiac MRI is useful to evaluate TS when the results of the echocardiography are insufficient.
Cardiac Catheterization
While echocardiography remains the diagnostic imaging modality of choice, cardiac catheterization is useful to evaluate TS 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.[2] In the older pre-surgery population, cardiac catheterization may be necessary in order to assess concomitant artery disease.
Treatment
Medical Therapy
Medical therapy with diuretics and sodium restriction is the mainstay of treatment among patients with TS complicated by systemic and pulmonary congestion. Patients with TS should receive medical therapy for left heart failure, and/or pulmonary hypertension if case they are present.[12]
Surgery
Surgical tricuspid valve replacement in TS is recommended among patients undergoing surgical intervention for left valvular disease as well as among patients with severe symptomatic isolated TS.[12]
References
- ↑ Roguin A, Rinkevich D, Milo S, Markiewicz W, Reisner SA (1998). "Long-term follow-up of patients with severe rheumatic tricuspid stenosis". Am Heart J. 136 (1): 103–8. PMID 9665226.
- ↑ 2.0 2.1 2.2 Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA; et al. (2014). "2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines". J Am Coll Cardiol. 63 (22): e57–185. doi:10.1016/j.jacc.2014.02.536. PMID 24603191.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Waller BF, Howard J, Fess S (1995). "Pathology of tricuspid valve stenosis and pure tricuspid regurgitation--Part I." Clin Cardiol. 18 (2): 97–102. PMID 7720297.
- ↑ 4.0 4.1 Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP; et al. (2009). "Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice". Eur J Echocardiogr. 10 (1): 1–25. doi:10.1093/ejechocard/jen303. PMID 19065003.
- ↑ Jellis CL, Navia JL, Flamm SD, Rodriguez LL (2016). "Severe Functional Tricuspid Stenosis Secondary to a Giant Saphenous Vein Bypass Graft Aneurysm". Circulation. 133 (21): 2099–102. doi:10.1161/CIRCULATIONAHA.115.014772. PMID 27217436 PMID: 27217436 Check
|pmid=value (help). - ↑ Taira K, Suzuki A, Fujino A, Watanabe T, Ogyu A, Ashikawa K (2006). "Tricuspid valve stenosis related to subvalvular adhesion of pacemaker lead: a case report". J Cardiol. 47 (6): 301–6. PMID 16800373.
- ↑ Changchien C, Lin MT, Wang CC, Liu HM, Wang CC, Chiu SN; et al. (2015). "Neonatal tricuspid stenosis caused by device closure of a large coronary fistula". EuroIntervention. 11 (7): e1. doi:10.4244/EIJV11I7A162. PMID 26603866 PMID: 26603866 Check
|pmid=value (help). - ↑ 8.0 8.1 Nili M, Liban E, Levy MJ (1982). "Tricuspid stenosis due to intravenous leiomyomatosis--a call for caution: case report and review of the literature". Tex Heart Inst J. 9 (2): 231–5. PMC 351617. PMID 15226964.
- ↑ 9.0 9.1 Kim KH, Park CH, Park HS, Kim YR, Choi EY (2014). "Amyloidosis-induced tricuspid stenosis mimicking rheumatic heart disease". Eur Heart J Cardiovasc Imaging. 15 (10): 1167. doi:10.1093/ehjci/jeu075. PMID 24797117.
- ↑ Goswami KC, Rao MB, Dev V, Shrivastava S (1999). "Juvenile TS and rheumatic tricuspid valve disease: an echocardiographic study". Int J Cardiol. 72 (1): 83–6. PMID 10636636.
- ↑ 11.0 11.1 Diaof M, Ba SA, Kane A, Sarr M, Diop IB, Diouf SM (2004). "[Tricuspid valve stenosis. A prospective study of 35 cases]". Dakar Med. 49 (2): 96–100. PMID 15786615.
- ↑ 12.0 12.1 Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA; et al. (2014). "2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines". Circulation. doi:10.1161/CIR.0000000000000029. PMID 24589852.