Valvular heart disease

Valvular heart disease
Classification and external resources
Specialty	Cardiology
ICD-10	I05-I08, I34-I37, Q22-Q23
ICD-9-CM	394-396, 424, 746
MeSH	D006349

Valvular heart disease is any disease process involving one or more of the four valves of the heart (the aortic and mitral valves on the left and the pulmonary and tricuspid valves on the right). These conditions occur largely as a result of aging. Most people are in their late 50s when diagnosed, and more than one in ten people over 75 have it.

Early detection may improve outcomes from surgery.

Collectively and anatomically, the valves are part of the dense connective tissue makeup of the heart known as the cardiac skeleton. Valve problems may be congenital (inborn) or acquired (due to another cause later in life). Treatment may be with medication but often (depending on the severity) involves valve repair or replacement (insertion of an artificial heart valve). Specific situations include those where additional demands are made on the circulation, such as in pregnancy.^[1]

Types

Valve involved	Stenotic disease	Insufficiency/regurgitation disease
Aortic valve	Aortic valve stenosis	Aortic insufficiency/regurgitation
Mitral valve	Mitral valve stenosis	Mitral insufficiency/regurgitation
Tricuspid valve	Tricuspid valve stenosis	Tricuspid insufficiency/regurgitation
Pulmonary valve	Pulmonary valve stenosis	Pulmonary insufficiency/regurgitation

Aortic and mitral valve disorders

These are termed left heart diseases.

Pulmonary and tricuspid valve disorders

Pulmonary and tricuspid valve diseases are right heart diseases. Pulmonary valve diseases are the least common heart valve disease in adults.^[2]

The most common types of pulmonary valve diseases are:

• pulmonary valve stenosis
• pulmonary valve insufficiency
  • pulmonary valve incompetence
  • pulmonary valve regurgitation

The International Statistical Classification of Diseases classifies non rheumatic pulmonary valve diseases as I37.

Both tricuspid and pulmonary valve diseases are less common than aortic or mitral valve diseases due to the lower pressure those valves experience.^[2]

Dysplasia

Heart valve dysplasia is an error in the development of any of the heart valves, and a common cause of congenital heart defects in humans as well as animals; tetralogy of Fallot is a congenital heart defect with four abnormalities, one of which is stenosis of the pulmonary valve. Ebstein's anomaly is an abnormality of the tricuspid valve.^[1]

Rheumatic disorders

Valvular heart disease resulting from rheumatic fever is referred to as "rheumatic heart disease". While developed countries once had a significant burden of rheumatic fever and rheumatic heart disease, medical advances and improved social conditions have dramatically reduced their incidence. Many developing countries, as well as indigenous populations within developed countries, still carry a significant burden of rheumatic fever and rheumatic heart disease and there has been a resurgence in efforts to eradicate the diseases in these populations. Inflammation of the heart valves due to any cause is called endocarditis; this is usually due to bacterial infection but may also be due to cancer (marantic endocarditis), certain autoimmune conditions (Libman-Sacks endocarditis) and hypereosinophilic syndrome (Loeffler endocarditis). Certain medications have been associated with valvular heart disease, most prominently ergotamine derivatives pergolide and cabergoline.^[3]

In pregnancy

The evaluation of individuals with valvular heart disease who are or wish to become pregnant is a difficult issue. Issues that have to be addressed include the risks during pregnancy to the mother and the developing fetus by the presence of maternal valvular heart disease as an intercurrent disease in pregnancy. Normal physiological changes during pregnancy require, on average, a 50% increase in circulating blood volume that is accompanied by an increase in cardiac output that usually peaks between the midportion of the second and third trimesters.^[4] The increased cardiac output is due to an increase in the stroke volume, and a small increase in heart rate, averaging 10 to 20 beats per minute.^[4] Additionally uterine circulation and endogenous hormones cause systemic vascular resistance to decrease and a disproportionately lowering of diastolic blood pressure causes a wide pulse pressure.^[4] Inferior vena caval obstruction from a gravid uterus in the supine position can result in an abrupt decrease in cardiac preload, which leads to hypotension with weakness and lightheadedness.^[4] During labor and delivery cardiac output increases more in part due to the associated anxiety and pain, as well as due to uterine contractions which will cause an increases in systolic and diastolic blood pressure.^[4]

Valvular heart lesions associated with high maternal and fetal risk during pregnancy include:^[4]

1. Severe aortic stenosis with or without symptoms
2. Aortic regurgitation with NYHA functional class III-IV symptoms
3. Mitral stenosis with NYHA functional class II-IV symptoms
4. Mitral regurgitation with NYHA functional class III-IV symptoms
5. Aortic and/or mitral valve disease resulting in severe pulmonary hypertension (pulmonary pressure greater than 75% of systemic pressures)
6. Aortic and/or mitral valve disease with severe LV dysfunction (EF less than 0.40)
7. Mechanical prosthetic valve requiring anticoagulation
8. Marfan syndrome with or without aortic regurgitation

^[4]

In individuals who require an artificial heart valve, consideration must be made for deterioration of the valve over time (for bioprosthetic valves) versus the risks of blood clotting in pregnancy with mechanical valves with the resultant need of drugs in pregnancy in the form of anticoagulation.

Comparison

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[5]	Approximately 2% of people over the age of 65, 3% of people over age 75, and 4% percent of people over age 85[6]		2% of the population, equally in males and females.[7]
Main causes and risk factors	Almost always caused by rheumatic heart disease[8]	Calcification of tricuspid aortic valve with age[8] (>50%[9]) Calcification of bicuspid aortic valve[8] (30-40%[9]) Rheumatic fever[8] (<10%[9]) Hypertension, diabetes mellitus, hyperlipoproteinemia and uremia may speed up the process.[9]	Acute Infective endocarditis[8][9] Trauma[8] Chronic Primary valvular: rheumatic fever, bicuspid aortic valve, Marfan's syndrome, Ehlers–Danlos syndrome, ankylosing spondylitis, systemic lupus erythematosus[8] Disease of the aortic root: syphilitic aortitis, osteogenesis imperfecta, aortic dissection, Behçet's disease, reactive arthritis, systemic hypertension[8]	Acute Endocarditis, mainly S. aureus[8] Papillary muscle rupture or dysfunction,[8] including mitral valve prolapse[9] Chronic Rheumatic fever[8] Marfan's syndrome[8] Cardiomyopathy[8]	Usually secondary to right ventricular dilation[8] left ventricular failure is, in turn, the most common cause[8] Right ventricular infarction[8] Inferior myocardial infarction[8] Cor pulmonale[8] Other causes: Tricuspid endocarditis, rheumatic fever, Ebstein's anomaly, carcinoid syndrome and myxomatous degeneration[8]
Hemo dynamics / Patho- physiology	Progressive obstruction of the mitral ostium causes increased pressure in the left atrium and the pulmonary circulation.[9] Congestion may cause thromboembolism, and atrial hypertension may cause atrial fibrillation.[9]	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[8] Palpitations[8] Chest pain[8] Hemoptysis[8] Thromboembolism[8] Ascites and edema (if right-sided heart failure develops)[8] Symptoms increase with exercise and pregnancy[8]	Heart failure symptoms, such as dyspnea on exertion (most frequent symptom[9]), orthopnea and paroxysmal nocturnal dyspnea[8] Angina pectoris[8] Syncope, usually exertional[8]	Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea[8] Palpitations[8] Angina pectoris[8] In acute cases: cyanosis and circulatory shock[8]	Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea[8] Palpitations[8] Pulmonary edema[8]	Symptoms of right-sided heart failure, such as ascites, hepatomegaly, edema and jugular venous distension[8]
Medical signs	Opening snap followed by a low-pitched diastolic rumble with presystolic accentuation.[8][9] The opening snap follows closer to the S2 heart tone with worsening stenosis.[8] The murmur is heard best with the bell of the stethoscope[8] lying on the left side[9] and its duration increases with worsening disease.[8] Loud S1 - may be the most prominent sign[8] 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.[8] Signs increase with exercise and pregnancy[8]	Systolic murmur of a harsh crescendo-decrescendo type, heard in 2nd right intercostal space,[9] radiating to the carotid arteries[8] Pulsus parvus et tardus, that is, diminished and delayed carotid pulse[8][9] Fourth heart sound[8] Decreased A2 sound[9] Sustained apex beat[8] Precordial thrill[8]	Increased pulse pressure by increased systolic and decreased diastolic blood pressure,[8] but may not be significant if acute[9] Diastolic decrescendo murmur best heard at left sternal border[8] Water hammer pulse[8] Austin Flint murmur[8] Apex beat displaced down and to the left[8] Third heart sound may be present[8]	Holosystolic murmur at the apex, radiating to the back or clavicular area[8] Commonly atrial fibrillation[8] Third heart sound[8] Laterally displaced apex beat,[8] often with heave[9] Loud, palpable P2,[8] heard best when lying on the left side[9] In acute cases, the murmur and tachycardia may be only distinctive signs.[9]	Pulsatile liver Prominent V waves and rapid y descents in jugular venous pressure[8] Inspiratory third heart sound at left lower sternal border (LLSB)[8] Blowing holosystolic murmur at LLSB, intensifying with inspiration, and decreasing with expiration and Valsalva maneuver[8] Parasternal heave along LLSB[8] Atrial fibrillation is usually present[8]
Diagnosis	Chest X-ray showing left atrial enlargement[8] 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[8]	Chest X-ray showing calcific aortic valve, and in longstanding disease, enlarged left ventricle[8][9] and atrium[8] ECG showing left ventricular hypertrophy and left atrial abnormality[8] Echocardiography is diagnostic in most cases, showing left ventricular hyperthrophy, thickened and immobile aortic valve and dilated aortic root,[8] but may appear normal if acute[9] Cardiac chamber catheterization provides a definitive diagnosis, indicating severe stenosis in valve area of <0.8 cm^2 (normally 3 to 4 cm^2). It is useful in symptomatic patients before surgery.[8]	Chest X-ray showing left ventricular hypertrophy and dilated aorta[8] ECG indicating left ventricular hypertrophy[8] 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.[8] Cardiac chamber catetherization assists in assessing the severity of regurgitation and any left ventricular dysfunction[8]	Chest X-ray showing dilated left ventricle[8] Echocardiography to detect mitral reverse flow, dilated left atrium and ventricle and decreased left ventricular function[8]	Echocardiography identifying tricuspid prolapse or flail[8] ECG showing enlargement of right ventricle and atrium[8]
Treatment	No therapy is required for asymptomatic patients. Diuretics for any pulmonary congestion or edema.[8] If stenosis is severe, surgery is recommended.[8] Any atrial fibrillation is treated accordingly.[8] Medically, with diuretics, prophylaxis of infective endocarditis and chronic anticoagulant administration with warfarin (especially when there's atrial fibrillation)[8] Surgically, by mitral valvuloplasty with a percutaneously inserted balloon, unless significant mitral regurgitation or too much calcification.[9] Indicated in ostium area < 1-1.2 cm^2.[9] Other options include valvulotomy or mitral valve replacement by open surgery[8]	No treatment in asymptomatic patients.[8] If symptomatic, treated with aortic valve replacement surgery.[8] Medical therapy and percutaneous balloon valvuloplasty have relatively poor effect.[8] - Any angina is treated with short-acting nitrovasodilators, beta-blockers and/or calcium blockers[9] - Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers[9] - Any heart failure is treated with digoxin, diuretics, nitrovasodilators and, if not contraindicated, cautious inpatient administration of ACE inhibitors[9]	If stable and asymptomatic - conservative treatment such as low sodium diet,[8] diuretics,[8] vasodilators[8] (e.g. (hydralazin[9] or prazosin[9]), digoxin,[8] ACE inhibitors[8]/angiotensin II receptor antagonists,[9] calcium blockers[9] and avoiding very strenuous activity[8][9] Aortic valve replacement in symptomatic patients (NYHA II[9]-IV) or progressive left ventricular dilation[9] or systolic ventricular diameter >55 mm[9] on echocardiogrphy. Immediately if acute.[8] Also, endocarditis prophylaxis is indicated before dental, gastrointestinal or genitourinary procedures.[8]	Medically Afterload reduction with vasodilators[8][9] Any hypertension is treated aggressively,[9] e.g. by diuretics and low sodium diet[8] digoxin[8][9] Antiarrhythmics[8][9] Chronic anticoagulation in concomitant mitral valve prolapse[9] or atrial fibrillation[8] In acute cases - IABP as temporary solution until surgery[8] Surgery by either mitral valve repair or mitral valve replacement,[8] indicated if very symptomatic (NYHA III), ventricular dilation or decreasing ejection fraction[9]	Treatment of underlying cause[8] Surgery[8] Tricuspid valvular repair[8] Valvuloplasty[8] Valve replacement (rarely performed)[8]
Follow-up		In moderate cases, echocardiography every 1–2 years, possibly complemented with cardiac stress test.[9] Immediate revisit if new related symptoms appear.[9] In severe cases, echocardiography every 3–6 months.[9] Immediate revisit or inpatient care if new related symptoms appear.[9]	In mild to moderate cases, echocardiography and cardiac stress test every 1–2 years[9] In severe moderate/severe cases, echocardiography with cardiac stress test and/or isotope perfusion imaging every 3–6 months.[9]	In mild to moderate cases, echocardiography and cardiac stress test every 1–3 years.[9] In severe cases, echocardiography every 3–6 months.[9]

References

1. Bonow RO, Carabello BA, Kanu C, et al. (2006). "ACC/AHA 2006 guidelines 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 (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons". Circulation 114 (5): e84–231. doi:10.1161/CIRCULATIONAHA.106.176857. PMID 16880336.
2. Ragavendra R. Baliga, Kim A. Eagle, William F Armstrong, David S Bach, Eric R Bates, Practical Cardiology, Lippincott Williams & Wilkins, 2008, page 452.
3. Schade R, Andersohn F, Suissa S, Haverkamp W, Garbe E (2007). "Dopamine agonists and the risk of cardiac-valve regurgitation". N. Engl. J. Med. 356 (1): 29–38. doi:10.1056/NEJMoa062222. PMID 17202453.
4. Bonow, RO; Carabello, BA; Chatterjee, K; De Leon Jr, AC; Faxon, DP; Freed, MD; Gaasch, WH; Lytle, BW; et al. (2008). "2008 focused update incorporated into the ACC/AHA 2006 guidelines 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 (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease). Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology 52 (13): e1–142. doi:10.1016/j.jacc.2008.05.007. PMID 18848134.
5. Gelson, E.; Gatzoulis, M.; Johnson, M. (2007). "Valvular heart disease". BMJ (Clinical research ed.) 335 (7628): 1042–1045. doi:10.1136/bmj.39365.655833.AE. PMC 2078629. PMID 18007005.
6. Stewart BF, Siscovick D, Lind BK, Gardin JM, Gottdiener JS, Smith VE. Clinical factors associated with calcific aortic valve disease. Cardiovascular Health Study. J Am Coll Cardiol. 1997; 29: 630-634.
7. The Cleveland Clinic Center for Continuing Education > Mitral Valve Disease: Stenosis and Regurgitation Authors: Ronan J. Curtin and Brian P. Griffin. Retrieved September 2010
8. 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.
9. VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Björklund May 2008

External links

• RHD Action
• Heart Valve Voice