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Risk factors
Tricuspid stenosis (TS) is a rare valvular lesion without an independent atherosclerotic-type risk-factor profile. Clinically relevant risk factors are therefore largely conditions or exposures that predispose to structural tricuspid valve disease. TS almost always occurs with tricuspid regurgitation (TR), and rheumatic TS commonly coexists with left-sided rheumatic valve disease, particularly mitral stenosis.^{[1]}^{[2]}
Established risk factors
Rheumatic heart disease
Rheumatic heart disease (RHD) is the dominant risk factor and cause of TS, accounting for approximately 90% of cases.^{[1]}
Important upstream risk factors include:
- Recurrent acute rheumatic fever.
- Untreated or inadequately treated group A streptococcal pharyngitis.
- Residence in or origin from regions with a high burden of RHD.
- Crowding and limited access to primary and secondary antibiotic prophylaxis.
- Established left-sided rheumatic valve disease, particularly mitral stenosis. The presence of rheumatic mitral disease should prompt assessment of the tricuspid valve for coexisting TS/TR.^{[3]}^{[4]}
Non-rheumatic predisposing conditions
Carcinoid heart disease
Carcinoid syndrome is an important non-rheumatic predisposition to right-sided valvular disease. More than 50% of patients with carcinoid syndrome develop carcinoid heart disease, predominantly involving the tricuspid and pulmonary valves. Tricuspid regurgitation is more common than TS, but stenotic involvement can occur.^{[5]}^{[6]}
Right-sided valvular involvement predominates because serotonin and other vasoactive mediators are normally inactivated during passage through the pulmonary circulation. Left-sided valvular involvement is uncommon, but its reported frequency varies substantially between clinical populations and sources. Some sources report left-sided involvement in <10% of cases, whereas expert consensus describes involvement in up to one-third of selected patients, particularly those with a patent foramen ovale with right-to-left shunting, bronchial carcinoid, or very high circulating levels of vasoactive substances. These estimates should therefore be interpreted according to the underlying population and clinical setting rather than expressed as a single fixed percentage.^{[5]}^{[7]}^{[8]}
Congenital tricuspid valve abnormalities
Congenital structural abnormalities that can predispose to TS include:
- Ebstein anomaly.
- Congenital tricuspid valve dysplasia.
- Congenital TS or tricuspid atresia.
Congenital forms generally present earlier in life and may occur as part of complex congenital heart disease.^{[2]}^{[9]}
Drug exposure
Exposure to recognized valvulopathic serotonergic or ergot-derived agents can predispose to tricuspid valve disease. Relevant exposures include:
- Fenfluramine and related anorectic agents.
- Methysergide and other ergot alkaloids.
A targeted medication and exposure history is appropriate when unexplained tricuspid valve disease is identified.^{[2]}^{[4]}
Prosthetic tricuspid valve dysfunction
Patients with a previous tricuspid valve replacement are at risk for acquired prosthetic TS due to:
- Bioprosthetic degeneration.
- Mechanical or bioprosthetic valve thrombosis.
Prosthetic TS is an increasingly relevant clinical category as the number of patients undergoing tricuspid valve replacement increases.^{[1]}^{[10]}
Cardiac implantable electronic devices
Indwelling right-heart hardware, including pacemaker and implantable cardioverter-defibrillator leads, can predispose to tricuspid inflow obstruction through:
- Device-associated thrombus.
- Lead-related fibrosis.
- Infective endocarditis with bulky vegetations.
New right-sided congestion in a patient with intracardiac hardware should prompt evaluation for device-related obstruction or endocarditis.^{[2]}
Right atrial or tricuspid orifice masses
Right atrial myxoma and other right-heart mass lesions can mechanically obstruct the tricuspid orifice and produce TS-like inflow obstruction. These lesions represent mechanical obstruction rather than intrinsic rheumatic leaflet disease.^{[10]}^{[11]}
Rare systemic and infiltrative diseases
Rare systemic or enzymatic disorders reported in association with tricuspid valve disease include:
Evidence for these associations is limited, and their magnitude of risk is not established.^{[2]}
Clinically actionable risk assessment
- In patients with rheumatic mitral or aortic valve disease, assess the tricuspid valve for coexisting TS/TR.
- In patients with carcinoid syndrome, incorporate appropriate cardiac surveillance for right-sided valvular involvement.
- Obtain a targeted history of serotonergic and ergot-derived valvulopathic drug exposure.
- In patients with a previous tricuspid valve replacement who develop right-heart congestion, consider prosthetic degeneration or thrombosis.
- In patients with pacemaker or ICD leads and new right-sided congestion, consider device-related obstruction or endocarditis.
- Prevention of acute rheumatic fever and appropriate secondary antibiotic prophylaxis remain important population-level strategies for reducing rheumatic valve disease burden.^3
Evidence limitations
The relative contribution of rare systemic diseases and individual case-report associations to TS risk is poorly quantified. Antiphospholipid syndrome, systemic lupus erythematosus/Libman-Sacks disease, metastatic tumors, intravenous leiomyomatosis, blunt trauma, and ventriculoatrial shunts should not be treated as established TS risk factors on the basis of isolated case reports.^{[2]}
High-yield clinical pearls
- Isolated TS is rare; identification of TS should prompt assessment for coexisting TR and left-sided rheumatic valve disease.^{[1]}
- Rheumatic TS typically involves commissural thickening and fusion with chordal shortening rather than the heavy leaflet calcification characteristic of rheumatic mitral stenosis.^{[1]}
- In carcinoid heart disease, right-sided valves are preferentially affected. Left-sided involvement is uncommon and should raise consideration of a patent foramen ovale with right-to-left shunting, bronchial carcinoid, or high circulating levels of vasoactive substances.^{[5]}^{[7]}
- New right-heart failure in a patient with a tricuspid bioprosthesis or pacemaker/ICD lead should prompt evaluation for prosthetic or device-related TS.^{[2]}
Common pitfalls
- Attributing right-heart congestion solely to TR and missing coexisting TS, particularly in patients with rheumatic or post-mitral valve disease.
- Overweighting rare single-case-report associations as established TS risk factors.
- Failing to obtain a history of serotonergic or ergot-derived valvulopathic drug exposure.
References
1. Asmarats L, Taramasso M, Rodés-Cabau J. (2019). "Tricuspid valve disease: diagnosis, prognosis and management of a rapidly evolving field". Nat Rev Cardiol. 16 (9): 538–554. doi:10.1038/s41569-019-0186-1. PMID 30988448.
2. Praz F, Borger MA, Lanz J, et al. (2025). "2025 ESC/EACTS Guidelines for the management of valvular heart disease". Eur Heart J. 46 (44): 4635–4736. doi:10.1093/eurheartj/ehaf194. PMID 40878295.
3. Kumar RK, Antunes MJ, Beaton A, et al. (2020). "Contemporary Diagnosis and Management of Rheumatic Heart Disease: Implications for Closing the Gap: A Scientific Statement From the American Heart Association". Circulation. 142 (20): e337–e357. doi:10.1161/CIR.0000000000000921. PMID 33073615.
4. Otto CM, Nishimura RA, Bonow RO, et al. (2021). "2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". J Am Coll Cardiol. 77 (4): e25–e197. doi:10.1016/j.jacc.2020.11.018. PMID 33342586.
5. Maleszewski JJ, Bois MC, Bois JP, Young PM, Stulak JM, Klarich KW. (2018). "Neoplasia and the Heart: Pathological Review of Effects With Clinical and Radiological Correlation". J Am Coll Cardiol. 72 (2): 202–227. doi:10.1016/j.jacc.2018.05.026. PMID 29976295.
6. Lyon AR, López-Fernández T, Couch LS, et al. (2022). "2022 ESC Guidelines on cardio-oncology developed in collaboration with the European Hematology Association (EHA), the European Society for Therapeutic Radiology and Oncology (ESTRO) and the International Cardio-Oncology Society (IC-OS)". Eur Heart J. 43 (41): 4229–4361. doi:10.1093/eurheartj/ehac244. PMID 36017568.
7. Davar J, Connolly HM, Caplin ME, et al. (2017). "Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement". J Am Coll Cardiol. 69 (10): 1288–1304. doi:10.1016/j.jacc.2016.12.030.
8. Hofland J, et al. (2022). "Synoptic reporting of echocardiography in carcinoid heart disease (ENETS Carcinoid Heart Disease Task Force)". J Neuroendocrinol. 34 (3): e13060. doi:10.1111/jne.13060.
9. Otto CM, Nishimura RA, Bonow RO, et al. (2021). "2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary". Circulation. 143 (5): e35–e71. doi:10.1161/CIR.0000000000000932. PMID 33332149.
10. Rodés-Cabau J, Taramasso M, O'Gara PT. (2016). "Diagnosis and treatment of tricuspid valve disease: current and future perspectives". Lancet. 388 (10058): 2431–2442. doi:10.1016/S0140-6736(16)00740-6. PMID 27048553.
11. Şaşkın H, Düzyol Ç, Özcan KS, Aksoy R. (2015). "Right atrial myxoma mimicking tricuspid stenosis". BMJ Case Rep. 2015. doi:10.1136/bcr-2015-211187. PMID 26272962.