Mitral stenosis anatomy and pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Mohammed A. Sbeih, M.D. [2]; Cafer Zorkun, M.D., Ph.D. [3]

Overview

Mitral stenosis (MS) is most commonly secondary to acute rheumatic fever. Generally, the initial valvulits is associated with valvular regurgitation but over a period of 2 or more years, the commissures fuse and the valves thicken and calcify. The chordal supporting structure also calcifies and retracts. The result is the typical “fish mouth deformity”. 70% of the time; the mitral valve is involved in isolation, and 25% of the time; the aortic valve is involved as well. The tricuspid and pulmonic valves are involved less commonly.

The normal mitral valve orifice area is 4-6 cm2. Mitral stenosis occurs when the orifice area is reduced to at least 2.2 cm2. This result in a gradient across the mitral valve which is the haemodynamic hallmark of the disease.

Anatomy

The mitral valve is typically 4–6 cm² in area. It has two cusps or leaflets (the anteromedial leaflet and the posterolateral leaflet) that guard the opening. The opening is surrounded by a fibrous ring known as the mitral valve annulus [1]. The anterior cusp protects approximately two-thirds of the valve (imagine a crescent moon within the circle, where the crescent represents the posterior cusp). Note that although the anterior leaflet takes up a larger part of the ring and rises higher, the posterior leaflet has a larger surface area. These valve leaflets are prevented from prolapsing into the left atrium by the action of tendons attached to the posterior surface of the valve, chordae tendineae.

The inelastic chordae tendineae are attached at one end to the papillary muscles and the other to the valve cusps. Papillary muscles are fingerlike projections from the wall of the left ventricle. Chordae tendineae from each muscle are attached to both leaflets of the mitral valve. Thus, when the left ventricle contracts, the intraventricular pressure forces the valve to close, while the tendons keep the leaflets coapting together and prevent the valve from opening in the wrong direction (thus preventing blood to flow back to the left atrium). Each chord has a different thickness. The thinnest ones are attached to the free leaflet margin, whereas thickest ones are attached quite away from the free margin. This disposition has important effects on systolic stress distribution physiology [2].

Pathophysiology

When the mitral valve area goes below 2 cm2, the valve causes an impediment to the flow of blood into the left ventricle, creating a pressure gradient across the mitral valve. This gradient may be increased by increases in the heart rate or cardiac output. As the gradient across the mitral valve increases, the amount of time necessary to fill the left ventricle with blood increases. Eventually, the left ventricle requires the atrial kick to fill with blood. As the heart rate increases, the amount of time that the ventricle is in diastole and can fill up with blood (called the diastolic filling period) decreases. When the heart rate goes above a certain point, the diastolic filling period is insufficient to fill the ventricle with blood and pressure builds up in the left atrium, leading to pulmonary congestion.

When the mitral valve area goes less than 1 cm2, there will be an increase in the left atrial pressures (required to push blood through the stenotic valve). Since the normal left ventricular diastolic pressures is about 5 mmHg, a pressure gradient across the mitral valve of 20 mmHg due to severe mitral stenosis will cause a left atrial pressure of about 25 mmHg. This left atrial pressure is transmitted to the pulmonary vasculature and causes pulmonary hypertension. Pulmonary capillary pressures in this level cause an imbalance between the hydrostatic pressure and the oncotic pressure, leading to extravasation of fluid from the vascular tree and pooling of fluid in the lungs (congestive heart failure causing pulmonary edema).

Increases in the heart rate will allow less time for the left ventricle to fill, also causing an increase in left atrial pressure and pulmonary congestion.

The constant pressure overload of the left atrium will cause the left atrium to increase in size. As the left atrium increases in size, it becomes more prone to develop atrial fibrillation. When atrial fibrillation develops, the atrial kick is lost (since it is due to the normal atrial contraction).

In individuals with severe mitral stenosis, the left ventricular filling is dependent on the atrial kick. The loss of the atrial kick due to atrial fibrillation can cause a precipitous decrease in cardiac output and sudden congestive heart failure.

The mitral annulus changes in shape and size during the cardiac cycle. It is smaller at the end of atrial systole due to the contraction of the left atrium around it, like a sphincter. This reduction in annulus size at the end of atrial systole may be important for the proper coapting of the leaflets of the mitral valve when the left ventricle contracts and pumps blood [3].

The closing of the mitral valve and the tricuspid valve constitutes the first heart sound (S1). It is not actually the valve closure which produces a sound but rather the sudden cessation of blood flow caused by the closure of the mitral and tricuspid valves. The mitral valve opening is normally not heard except in mitral stenosis as the opening Snap. Flow of blood into the heart during rapid filling is not normally heard except in certain pathological states where it constitutes the third heart sound (S3).

Thickening and immobility of the mitral valve leaflets causes an obstruction in blood flow from the left atrium to left ventricle (Mitral stenosis). As a result, the pressure in the left atrium increases, also the pressure in the pulmonary vasculature and right side of the heart increases. Mitral stenosis may cause left ventricular dysfunction if it is associated with mitral regurgitation [4].

Almost all cases of mitral stenosis are due to disease in the heart secondary to rheumatic fever and the consequent rheumatic heart disease (a condition that may develop after strep throat or scarlet fever). Around 90% of cases of rheumatic heart disease are associated with mitral stenosis [5]. The valve problems develop 5 - 10 years after the rheumatic fever, a tiny nodules forms along the valve leaflets [6], the leaflets eventually thicken with deposition of fibrin. The cusps may become fibrosed, calcified and thickened over a span of a decade [7][8]. Chronic turbulent flow through a deformed valve appears to cause these changes and as a result the valve losses it's normal morphology [4]. The degree of leaflet thickening and calcification and the severity of chordal involvement are variable. Rheumatic fever is becoming rare in the United States, so mitral stenosis is also less common [9][10].

Severity of Mitral stenosis

The severity of mitral stenosis depends on the pressure gradient between the left atrium and ventricle which depends on the cross sectional area of the mitral valve. The normal mitral valve orifice has a cross sectional area of about 4.0 cm2.

  • Mitral stenosis is mild if the cross sectional area is about 2 cm2 and the pressure gradient is small.
  • Mitral stenosis is moderate if the cross sectional area is about 1.0 to 1.5 cm2.
  • Mitral stenosis is severe if the cross sectional area is ≤1.0 cm2 and the pressure gradient between the left atrium and left ventricle is significant.

Usually, the rate of decrement in the valve area is about 0.1 cm2/year once mitral stenosis is present [11][12].

In pregnancy, the pressure gradient between the left atrium and ventricle is usually increased due to the increase in the heart rate and cardiac output during pregnancy. This can lead to the diagnosis of previously asymptomatic case of mitral stenosis, or worsening of the symptoms of previously diagnosed case.

References

  1. ↑ Shinoda H, Stern PH (1992). "Diurnal rhythms in Ca transfer into bone, Ca release from bone, and bone resorbing activity in serum of rats". Am J Physiol. 262 (2 Pt 2): R235–40. PMID 1539731.
  2. ↑ Nazari S, Carli F, Salvi S, Banfi C, Aluffi A, Mourad Z; et al. (2000). "Patterns of systolic stress distribution on mitral valve anterior leaflet chordal apparatus. A structural mechanical theoretical analysis". J Cardiovasc Surg (Torino). 41 (2): 193–202. PMID 10901521.
  3. ↑ Pai RG, Varadarajan P, Tanimoto M (2003). "Effect of atrial fibrillation on the dynamics of mitral annular area". J Heart Valve Dis. 12 (1): 31–7. PMID 12578332.
  4. ↑ 4.0 4.1 Marcus RH, Sareli P, Pocock WA, Barlow JB (1994). "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. 120 (3): 177–83. PMID 8043061.
  5. ↑ BLAND EF, DUCKETT JONES T (1951). "Rheumatic fever and rheumatic heart disease; a twenty year report on 1000 patients followed since childhood". Circulation. 4 (6): 836–43. PMID 14879491.
  6. ↑ Selzer A, Cohn KE (1972). "Natural history of mitral stenosis: a review". Circulation. 45 (4): 878–90. PMID 4552598.
  7. ↑ Rajamannan NM, Nealis TB, Subramaniam M, Pandya S, Stock SR, Ignatiev CI; et al. (2005). "Calcified rheumatic valve neoangiogenesis is associated with vascular endothelial growth factor expression and osteoblast-like bone formation". Circulation. 111 (24): 3296–301. doi:10.1161/CIRCULATIONAHA.104.473165. PMID 15956138.
  8. ↑ Horstkotte D, Niehues R, Strauer BE (1991). "Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis". Eur Heart J. 12 Suppl B: 55–60. PMID 1936027.
  9. ↑ Chapter 1: Diseases of the Cardiovascular system > Section: Valvular Heart Disease in: Elizabeth D Agabegi; Agabegi, Steven S. (2008). Step-Up to Medicine (Step-Up Series). Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7153-6.
  10. ↑ "Mitral Stenosis: Heart Valve Disorders: Merck Manual Home Edition". Retrieved 2009-03-14.
  11. ↑ Gordon SP, Douglas PS, Come PC, Manning WJ (1992). "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. 19 (5): 968–73. PMID 1552121.
  12. ↑ Sagie A, Freitas N, Padial LR, Leavitt M, Morris E, Weyman AE; et al. (1996). "Doppler echocardiographic assessment of long-term progression of mitral stenosis in 103 patients: valve area and right heart disease". J Am Coll Cardiol. 28 (2): 472–9. doi:10.1016/0735-1097(96)00153-2. PMID 8800128.


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