The following table includes the main types of valvular stenosis and regurgitation. Major types of valvular heart disease not included in the table include mitral valve prolapse, rheumatic heart disease and endocarditis.
| Valvular disease |
Mitral stenosis |
Aortic stenosis |
Aortic regurgitation |
Mitral regurgitation |
Tricuspid regurgitation |
| Prevalence |
Most common valvular heart disease in pregnancy |
Approximately 2% of people over the age of 65, 3% of people over age 75, and 4% percent of people over age 85 |
|
2% of the population, equally in males and females. |
|
| Main causes and risk factors |
Almost always caused by rheumatic heart disease |
- Calcification of tricuspid aortic valve with age
(>50%)
- Calcification of bicuspid aortic valve
(30-40%)
- Rheumatic fever
(<10%)
Hypertension, diabetes mellitus, hyperlipoproteinemia and uremia may speed up the process.
|
Acute
- Infective endocarditis
- Trauma
Chronic
- Primary valvular: rheumatic fever, bicuspid aortic valve, Marfan's syndrome, Ehlers–Danlos syndrome, ankylosing spondylitis, systemic lupus erythematosus
- Disease of the aortic root: syphilitic aortitis, osteogenesis imperfecta, aortic dissection, Behçet's disease, reactive arthritis, systemic hypertension
|
Acute
- Endocarditis, mainly S. aureus
- Papillary muscle rupture or dysfunction, including mitral valve prolapse
Chronic
- Rheumatic fever
- Marfan's syndrome
- Cardiomyopathy
|
- Usually secondary to right ventricular dilation
- left ventricular failure is, in turn, the most common cause
- Right ventricular infarction
- Inferior myocardial infarction
- Cor pulmonale
- Other causes: Tricuspid endocarditis, rheumatic fever, Ebstein's anomaly, carcinoid syndrome and myxomatous degeneration
|
Hemo
dynamics
/
Patho-
physiology |
Progressive obstruction of the mitral ostium causes increased pressure in the left atrium and the pulmonary circulation. Congestion may cause thromboembolism, and atrial hypertension may cause atrial fibrillation. |
Obstruction through the aortic ostium causes increased pressure in the left ventricle and impaired flow through the aorta |
Insufficiency of the aortic valve causes backflow of blood into the left ventricle during diastole. |
Insufficiency of the mitral valve causes backflow of blood into the left atrium during systole. |
Insufficiency of the tricuspid valve causes backflow of blood into the right atrium during systole. |
| Symptoms |
- Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea
- Palpitations
- Chest pain
- Hemoptysis
- Thromboembolism
- Ascites and edema (if right-sided heart failure develops)
Symptoms increase with exercise and pregnancy
|
- Heart failure symptoms, such as dyspnea on exertion (most frequent symptom), orthopnea and paroxysmal nocturnal dyspnea
- Angina pectoris
- Syncope, usually exertional
|
- Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea
- Palpitations
- Angina pectoris
- In acute cases: cyanosis and circulatory shock
|
- Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea
- Palpitations
- Pulmonary edema
|
- Symptoms of right-sided heart failure, such as ascites, hepatomegaly, edema and jugular venous distension
|
| Medical signs |
- Opening snap followed by a low-pitched diastolic rumble with presystolic accentuation.
- The opening snap follows closer to the S2 heart tone with worsening stenosis.
- The murmur is heard best with the bell of the stethoscope lying on the left side and its duration increases with worsening disease.
- Loud S1 - may be the most prominent sign
- Advanced disease may present with signs of right-sided heart failure such as parasternal heave, jugular venous distension, hepatomegaly, ascites and/or pulmonary hypertension (presenting with a loud P2.
Signs increase with exercise and pregnancy
|
- Systolic murmur of a harsh crescendo-decrescendo type, heard in 2nd right intercostal space, radiating to the carotid arteries
- Pulsus parvus et tardus, that is, diminished and delayed carotid pulse
- Fourth heart sound
- Decreased A2 sound
- Sustained apex beat
- Precordial thrill
|
- Increased pulse pressure by increased systolic and decreased diastolic blood pressure, but may not be significant if acute
- Diastolic decrescendo murmur best heard at left sternal border
- Water hammer pulse
- Austin Flint murmur
- Apex beat displaced down and to the left
- Third heart sound may be present
|
- Holosystolic murmur at the apex, radiating to the back or clavicular area
- Commonly atrial fibrillation
- Third heart sound
- Laterally displaced apex beat, often with heave
- Loud, palpable P2, heard best when lying on the left side
In acute cases, the murmur and tachycardia may be only distinctive signs.
|
- Pulsatile liver
- Prominent V waves and rapid y descents in jugular venous pressure
- Inspiratory third heart sound at left lower sternal border (LLSB)
- Blowing holosystolic murmur at LLSB, intensifying with inspiration, and decreasing with expiration and Valsalva maneuver
- Parasternal heave along LLSB
- Atrial fibrillation is usually present
|
| Diagnosis |
- Chest X-ray showing left atrial enlargement
- Echocardiography is the most important test to confirm the diagnosis. It shows left atrial enlargement, thick and calcified mitral valve with narrow and "fish-mouth"-shaped orifice and signs of right ventricular failure in advanced disease
|
- Chest X-ray showing calcific aortic valve, and in longstanding disease, enlarged left ventricle and atrium
- ECG showing left ventricular hypertrophy and left atrial abnormality
- Echocardiography is diagnostic in most cases, showing left ventricular hyperthrophy, thickened and immobile aortic valve and dilated aortic root, but may appear normal if acute
- Cardiac chamber catheterization provides a definitive diagnosis, indicating severe stenosis in valve area of <0.8 cm2 (normally 3 to 4 cm2). It is useful in symptomatic patients before surgery.
|
- Chest X-ray showing left ventricular hypertrophy and dilated aorta
- ECG indicating left ventricular hypertrophy
- Echocardiogram showing dilated left aortic root and reversal of blood flow in the aorta. In longstanding disease there may be left ventricular dilatation. In acute aortic regurgitation, there may be early closure of the mitral valve.
- Cardiac chamber catetherization assists in assessing the severity of regurgitation and any left ventricular dysfunction
|
- Chest X-ray showing dilated left ventricle
- Echocardiography to detect mitral reverse flow, dilated left atrium and ventricle and decreased left ventricular function
|
- Echocardiography identifying tricuspid prolapse or flail
- ECG showing enlargement of right ventricle and atrium
|
| Treatment |
No therapy is required for asymptomatic patients. Diuretics for any pulmonary congestion or edema. If stenosis is severe, surgery is recommended. Any atrial fibrillation is treated accordingly.
- Medically, with diuretics, prophylaxis of infective endocarditis and chronic anticoagulant administration with warfarin (especially when there's atrial fibrillation)
- Surgically, by mitral valvuloplasty with a percutaneously inserted balloon, unless significant mitral regurgitation or too much calcification. Indicated in ostium area < 1-1.2 cm2. Other options include valvulotomy or mitral valve replacement by open surgery
|
No treatment in asymptomatic patients.
- If symptomatic, treated with aortic valve replacement surgery.
Medical therapy and percutaneous balloon valvuloplasty have relatively poor effect.
- Any angina is treated with short-acting nitrovasodilators, beta-blockers and/or calcium blockers
- Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers
- Any heart failure is treated with digoxin, diuretics, nitrovasodilators and, if not contraindicated, cautious inpatient administration of ACE inhibitors
|
- If stable and asymptomatic - conservative treatment such as low sodium diet, diuretics, vasodilators (e.g. (hydralazin or prazosin), digoxin, ACE inhibitors/angiotensin II receptor antagonists, calcium blockers and avoiding very strenuous activity
- Aortic valve replacement in symptomatic patients (NYHA II-IV) or progressive left ventricular dilation or systolic ventricular diameter >55 mm on echocardiogrphy. Immediately if acute.
Also, endocarditis prophylaxis is indicated before dental, gastrointestinal or genitourinary procedures.
|
- Medically
- Afterload reduction with vasodilators
- Any hypertension is treated aggressively, e.g. by diuretics and low sodium diet
- digoxin
- Antiarrhythmics
- Chronic anticoagulation in concomitant mitral valve prolapse or atrial fibrillation
- In acute cases - IABP as temporary solution until surgery
- Surgery by either mitral valve repair or mitral valve replacement, indicated if very symptomatic (NYHA III), ventricular dilation or decreasing ejection fraction
|
- Treatment of underlying cause
- Surgery
- Tricuspid valvular repair
- Valvuloplasty
- Valve replacement (rarely performed)
|
| Follow-up |
|
- In moderate cases, echocardiography every 1–2 years, possibly complemented with cardiac stress test. Immediate revisit if new related symptoms appear.
- In severe cases, echocardiography every 3–6 months. Immediate revisit or inpatient care if new related symptoms appear.
|
- In mild to moderate cases, echocardiography and cardiac stress test every 1–2 years
- In severe moderate/severe cases, echocardiography with cardiac stress test and/or isotope perfusion imaging every 3–6 months.
|
- In mild to moderate cases, echocardiography and cardiac stress test every 1–3 years.
- In severe cases, echocardiography every 3–6 months.
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