Pathophysiology and natural history of mitral stenosis
- Theo E Meyer, MD, PhD
Theo E Meyer, MD, PhD
- Professor of Medicine
- Chief, Clinical Cardiology
- Director, Advanced Heart Failure Program
- University of Massachusetts Medical School
- UMassMemorial Medical Center
- William H Gaasch, MD
William H Gaasch, MD
- Section Editor — Valvular Disease
- Professor of Medicine
- University of Massachusetts Medical School
- Tufts University School of Medicine
- Senior Consultant in Cardiology
- Lahey Clinic
The hemodynamic consequence of mitral stenosis (MS) is manifested by increased impedance or resistance to transmitral flow. MS attenuates the atrial contribution to left ventricular filling and it also poses a hydraulic opposition or resistance to early filling. Unlike other valvular lesions, which are often attributable to many etiologies, MS alone, or in combination with other valvular pathology, is almost exclusively secondary to rheumatic heart disease. Progression of MS eventually leads to the development of disabling symptoms (eg, dyspnea, hemoptysis, thromboembolism, and right-sided heart failure). (See "Clinical manifestations and diagnosis of mitral stenosis".)
The natural history and pathophysiology of MS will be reviewed here. Given the widespread use of surgical and percutaneous interventions, information on the natural history of MS comes from older studies and other limited observations of patients in developing countries who did not receive a corrective procedure.
The roles of medical and surgical therapy and the use of percutaneous balloon valvotomy are discussed separately. (See "Medical management and indications for intervention for mitral stenosis" and "Surgical management of mitral stenosis" and "Percutaneous mitral balloon valvotomy for mitral stenosis".)
Rheumatic heart disease (RHD) is the most common cause of mitral stenosis (MS); other causes are much less frequent [1,2]. However, only 50 to 70 percent of patients with MS report a history of rheumatic fever [3-5]. RHD remains a major public health problem in developing countries [6-8]. Rheumatic MS has become less common in developed countries given marked reductions in the incidence of rheumatic fever . Occasional outbreaks of rheumatic fever in the United States appear to be the result of either increased virulence of a streptococcal strain or enhanced immigration from areas where RHD is prevalent . Involvement of the mitral valve is present in approximately 90 percent of individuals with RHD . Since rheumatic MS is a chronic condition it is not seen during the first episode of acute rheumatic carditis. In many populations, RHD is more common in females than in men . Rheumatic MS is a continuously progressive lifelong disease. (See "Acute rheumatic fever: Epidemiology and pathogenesis".)
The following are other causes of mitral stenosis:
- Olson LJ, Subramanian R, Ackermann DM, et al. Surgical pathology of the mitral valve: a study of 712 cases spanning 21 years. Mayo Clin Proc 1987; 62:22.
- Horstkotte D, Niehues R, Strauer BE. Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis. Eur Heart J 1991; 12 Suppl B:55.
- WOOD P. An appreciation of mitral stenosis. I. Clinical features. Br Med J 1954; 1:1051.
- Selzer A, Cohn KE. Natural history of mitral stenosis: a review. Circulation 1972; 45:878.
- ROWE JC, BLAND EF, SPRAGUE HB, WHITE PD. The course of mitral stenosis without surgery: ten- and twenty-year perspectives. Ann Intern Med 1960; 52:741.
- Rheumatic fever and rheumatic heart disease. World Health Organ Tech Rep Ser 2004; 923:1.
- Sani MU, Karaye KM, Borodo MM. Prevalence and pattern of rheumatic heart disease in the Nigerian savannah: an echocardiographic study. Cardiovasc J Afr 2007; 18:295.
- Zühlke L, Engel ME, Karthikeyan G, et al. Characteristics, complications, and gaps in evidence-based interventions in rheumatic heart disease: the Global Rheumatic Heart Disease Registry (the REMEDY study). Eur Heart J 2015; 36:1115.
- Gordis L. The virtual disappearance of rheumatic fever in the United States: lessons in the rise and fall of disease. T. Duckett Jones memorial lecture. Circulation 1985; 72:1155.
- Feldman T. Rheumatic mitral stenosis. On the rise again. Postgrad Med 1993; 93:93.
- BLAND EF, DUCKETT JONES T. Rheumatic fever and rheumatic heart disease; a twenty year report on 1000 patients followed since childhood. Circulation 1951; 4:836.
- Nishimura RA, Otto CM, Bonow RO, et al. 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 2014; 63:e57.
- Adabag AS, Dykoski R, Ward H, Anand IS. Critical stenosis of aortic and mitral valves after mediastinal irradiation. Catheter Cardiovasc Interv 2004; 63:247.
- Hull MC, Morris CG, Pepine CJ, Mendenhall NP. Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy. JAMA 2003; 290:2831.
- Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet 2005; 366:155.
- Marcus RH, Sareli P, Pocock WA, Barlow JB. The spectrum of severe rheumatic mitral valve disease in a developing country. Correlations among clinical presentation, surgical pathologic findings, and hemodynamic sequelae. Ann Intern Med 1994; 120:177.
- Rajamannan NM, Nealis TB, Subramaniam M, et al. Calcified rheumatic valve neoangiogenesis is associated with vascular endothelial growth factor expression and osteoblast-like bone formation. Circulation 2005; 111:3296.
- Hernandez R, Bañuelos C, Alfonso F, et al. Long-term clinical and echocardiographic follow-up after percutaneous mitral valvuloplasty with the Inoue balloon. Circulation 1999; 99:1580.
- Wang A, Krasuski RA, Warner JJ, et al. Serial echocardiographic evaluation of restenosis after successful percutaneous mitral commissurotomy. J Am Coll Cardiol 2002; 39:328.
- Chen CR, Cheng TO. Percutaneous balloon mitral valvuloplasty by the Inoue technique: a multicenter study of 4832 patients in China. Am Heart J 1995; 129:1197.
- Tantchou Tchoumi JC, Butera G. Rheumatic valvulopathies occurence, pattern and follow-up in rural area: the experience of the Shisong Hospital, Cameroon. Bull Soc Pathol Exot 2009; 102:155.
- Tadele H, Mekonnen W, Tefera E. Rheumatic mitral stenosis in children: more accelerated course in sub-Saharan patients. BMC Cardiovasc Disord 2013; 13:95.
- Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med 2007; 357:470.
- Sliwa K, Carrington M, Mayosi BM, et al. Incidence and characteristics of newly diagnosed rheumatic heart disease in urban African adults: insights from the heart of Soweto study. Eur Heart J 2010; 31:719.
- Hakim FA, Kendall CB, Alharthi M, et al. Parachute mitral valve in adults-a systematic overview. Echocardiography 2010; 27:581.
- Ruckman RN, Van Praagh R. Anatomic types of congenital mitral stenosis: report of 49 autopsy cases with consideration of diagnosis and surgical implications. Am J Cardiol 1978; 42:592.
- Moore P, Adatia I, Spevak PJ, et al. Severe congenital mitral stenosis in infants. Circulation 1994; 89:2099.
- Rao N, Gajjar T, Desai N. Accessory mitral valve tissue: an unusual cause of congenital mitral stenosis. Interact Cardiovasc Thorac Surg 2012; 14:110.
- Pressman GS, Agarwal A, Braitman LE, Muddassir SM. Mitral annular calcium causing mitral stenosis. Am J Cardiol 2010; 105:389.
- HUGENHOLTZ PG, RYAN TJ, STEIN SW, ABELMANN WH. The spectrum of pure mitral stenosis. Hemodynamic studies in relation to clinical disability. Am J Cardiol 1962; 10:773.
- Shrestha NK, Moreno FL, Narciso FV, et al. Two-dimensional echocardiographic diagnosis of left-atrial thrombus in rheumatic heart disease. A clinicopathologic study. Circulation 1983; 67:341.
- Hao xh, Lai YQ, Li JH, et al. Analysis of risk factors for left atrial thrombosis in patients with rheumatic mitral stenosis. Chin J Thorac Cardio Surg 2011; 27:293.
- Goldsmith I, Kumar P, Carter P, et al. Atrial endocardial changes in mitral valve disease: a scanning electron microscopy study. Am Heart J 2000; 140:777.
- Luo ZQ, Hao XH, Li JH, et al. Left atrial endocardial dysfunction and platelet activation in patients with atrial fibrillation and mitral stenosis. J Thorac Cardiovasc Surg 2014; 148:1970.
- Nunes MC, Handschumacher MD, Levine RA, et al. Role of LA shape in predicting embolic cerebrovascular events in mitral stenosis: mechanistic insights from 3D echocardiography. JACC Cardiovasc Imaging 2014; 7:453.
- WOOD P, BESTERMAN EM, TOWERS MK, MCILROY MB. The effect of acetylcholine on pulmonary vascular resistance and left atrial pressure in mitral stenosis. Br Heart J 1957; 19:279.
- Mahoney PD, Loh E, Blitz LR, Herrmann HC. Hemodynamic effects of inhaled nitric oxide in women with mitral stenosis and pulmonary hypertension. Am J Cardiol 2001; 87:188.
- Kulik TJ. Pulmonary hypertension caused by pulmonary venous hypertension. Pulm Circ 2014; 4:581.
- Snopek G, Pogorzelska H, Rywik TM, et al. Usefulness of endothelin-1 concentration in capillary blood in patients with mitral stenosis as a predictor of regression of pulmonary hypertension after mitral valve replacement or valvuloplasty. Am J Cardiol 2002; 90:188.
- Reyes VP, Raju BS, Wynne J, et al. Percutaneous balloon valvuloplasty compared with open surgical commissurotomy for mitral stenosis. N Engl J Med 1994; 331:961.
- Sagie A, Freitas N, Padial LR, et al. Doppler echocardiographic assessment of long-term progression of mitral stenosis in 103 patients: valve area and right heart disease. J Am Coll Cardiol 1996; 28:472.
- Gordon SP, Douglas PS, Come PC, Manning WJ. Two-dimensional and Doppler echocardiographic determinants of the natural history of mitral valve narrowing in patients with rheumatic mitral stenosis: implications for follow-up. J Am Coll Cardiol 1992; 19:968.
- Rinkevich D, Lessick J, Mutlak D, et al. Natural history of moderate mitral valve stenosis. Isr Med Assoc J 2003; 5:15.
- WHO Technical Report, Series. Rheumatic fever and rheumatic heart disease: Report of a WHO expert panel. Geneva 29 October - 1 November 2001. Geneva: WHO; 2004.
- Strasser T, Dondog N, El Kholy A, et al. The community control of rheumatic fever and rheumatic heart disease: report of a WHO international cooperative project. Bull World Health Organ 1981; 59:285.
- OLESEN KH. The natural history of 271 patients with mitral stenosis under medical treatment. Br Heart J 1962; 24:349.
- Ward C, Hancock BW. Extreme pulmonary hypertension caused by mitral valve disease. Natural history and results of surgery. Br Heart J 1975; 37:74.
- CASELLA L, ABELMANN WH, ELLIS LB. PATIENTS WITH MITRAL STENOSIS AND SYSTEMIC EMBOLI; HEMODYNAMIC AND CLINICAL OBSERVATIONS. Arch Intern Med 1964; 114:773.
- Abernathy WS, Willis PW 3rd. Thromboembolic complications of rheumatic heart disease. Cardiovasc Clin 1973; 5:131.
- Pathoanatomic considerations
- - Rheumatic heart disease
- - Congenital MS
- - Mitral annular calcification
- Hemodynamic and structural sequelae
- - Altered filling dynamics
- - Left atrial remodeling
- Pulmonary hypertension
- NATURAL HISTORY
- Rate of progression
- Asymptomatic phase
- Symptomatic phase
- Effect of pregnancy
- Causes of death