History and exam

Key diagnostic factors

common

history of rheumatic fever

While rheumatic fever is the usual cause of mitral stenosis, the history of a previous attack of rheumatic fever is notoriously unreliable.[26]

Many patients who claim a rheumatic fever history have no evidence of rheumatic heart disease, whereas many patients with clear evidence of rheumatic valvulopathy deny ever having had rheumatic fever.

dyspnea

As the mitral valve orifice narrows, increased left atrial pressure results in pulmonary congestion.

orthopnea

As a consequence of increased left atrial pressure.

opening snap on auscultation

The stiffened valve opens with a snap.

In mitral annular calcification-related disease, the leaflets move relatively normally and this finding is absent.

diastolic murmur

Flow through the narrowed valve produces a low pitched rumbling murmur.

Best heard with the patient in the left lateral decubitus position and may be accentuated by having the patient perform handgrip exercise.

In MAC-related disease the murmur may be absent, perhaps owing to different anatomy from rheumatic disease.


Mitral stenosis (severe)
Mitral stenosis (severe)

Auscultation sounds: Mitral stenosis (severe)


loud P2

Sign of pulmonary hypertension and/or right ventricular failure.

neck vein distension

Sign of pulmonary hypertension and/or right ventricular failure.

uncommon

paroxysmal nocturnal dyspnea

As a consequence of increased left atrial pressure.

hemoptysis

Sudden increases in pulmonary venous pressure, especially during exercise, may rupture bronchiolar vein anastomoses leading to hemoptysis.

hoarseness

The enlarged left atrium may encroach upon the left recurrent laryngeal nerve producing vocal cord paralysis and hoarseness. This is known as Ortner syndrome.

peripheral edema

As pulmonary hypertension becomes severe, right ventricular failure leads to right atrial hypertension, volume retention, and edema.

ascites

Sign of severe pulmonary hypertension leading to right ventricular failure and right atrial hypertension.

Other diagnostic factors

common

40-50 years old in rheumatic mitral stenosis, 70-90 years old in mitral annular calcification (MAC)-related disease

In high-income countries, the first symptoms of mitral stenosis related to rheumatic disease usually appear in the fourth and fifth decades of life. In low- and middle-income countries, symptoms occur at a younger age.[27] MAC-related disease is generally seen in older people.​

loud first heart sound (S1)

The pandiastolic transvalvular gradient holds the valve open until the ventricular systole closes it, producing a loud S1.

uncommon

irregularly irregular pulse

Left atrial enlargement and increased age commonly cause atrial fibrillation in patients with mitral stenosis.

flushed cheeks

The patient may appear with prominent reddish cheeks, known as mitral facies.

Risk factors

strong

streptococcal infection

Untreated streptococcal infection with strains containing an M antigen that is cross-reactive with, or binds to, the myocardium is the major risk factor for developing rheumatic fever and subsequent rheumatic heart disease.[8][16]

The most common infection is streptococcal pharyngitis. In the US, epidemic streptococcal infection occurs primarily in crowded situations such as schools, college dormitories, and military barracks.

Socioeconomic status must in some way influence the risk of contracting rheumatic fever, as there is a large difference in attack rate between developed and developing nations. How this risk factor translates to increased attack rates is uncertain.

female sex

Women are 3 times more likely than men to have mitral stenosis.

weak

ergot medications

Chronic ingestion of methysergide or ergotamine may result in endocardial thickening and valve dysfunction, commonly aortic and mitral stenosis.[17]

serotonergic medications

Such as fenfluramine and dexfenfluramine. Valvular abnormalities, mostly regurgitant lesions, resulted in the voluntary withdrawal from market of fenfluramine and dexfenfluramine in September 1997.[18]

systemic lupus erythematosus (SLE)

Mitral stenosis is a rare complication of SLE-related valvular disease.[19]

amyloidosis

Cardiac amyloidosis can cause valvular thickening.[20]

bronchial carcinoid syndrome

Carcinoid syndrome typically causes right heart valve disease (tricuspid and pulmonary valve) unless bronchial carcinoid is present, in which case left heart lesions may occur.[17]

atherosclerotic risk factors

Mitral stenosis due to mitral annular calcification (MAC) is a process related to atherosclerosis and shares many of the same risk factors including age, hypertension, and inflammation.[21]

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