Valvular heart disease
Encyclopedia
Valvular heart disease is any disease process involving one or more of the valves of the heart
Heart valve
A heart valve normally allows blood flow in only one direction through the heart. The four valves commonly represented in a mammalian heart determine the pathway of blood flow through the heart...

 (the aortic
Aortic valve
The aortic valve is one of the valves of the heart. It is normally tricuspid , although in 1% of the population it is found to be congenitally bicuspid . It lies between the left ventricle and the aorta....

 and mitral valve
Mitral valve
The mitral valve is a dual-flap valve in the heart that lies between the left atrium and the left ventricle...

s on the left and the pulmonary
Pulmonary valve
The pulmonary valve is the semilunar valve of the heart that lies between the right ventricle and the pulmonary artery and has three cusps. Similar to the aortic valve, the pulmonary valve opens in ventricular systole, when the pressure in the right ventricle rises above the pressure in the...

 and tricuspid valves on the right). 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
Valve replacement
Valve replacement surgery is the replacement of one or more of the heart valves with either an artificial heart valve or a bioprosthesis . It is an alternative to valve repair.There are four procedures:...

 (insertion of an artificial heart valve
Artificial heart valve
An artificial heart valve is a device implanted in the heart of a patient with heart valvular disease. When one of the four heart valves malfunctions, the medical choice may be to replace the natural valve with an artificial valve. This requires open-heart surgery.Valves are integral to the normal...

). Specific situations include those where additional demands are made on the circulation, such as in pregnancy.

Types

Valve involved Stenotic disease Insufficiency/regurgitation disease
Aortic valve Aortic valve stenosis
Aortic valve stenosis
Aortic valve stenosis is a disease of the heart valves in which the opening of the aortic valve is narrowed. The aortic valve is the valve between the left ventricle of the heart and the aorta, which is the largest artery in the body and carries the entire output of blood.-Pathophysiology:The...

Aortic insufficiency
Aortic insufficiency
Aortic insufficiency , also known as aortic regurgitation , is the leaking of the aortic valve of the heart that causes blood to flow in the reverse direction during ventricular diastole, from the aorta into the left ventricle....

/regurgitation
Mitral valve Mitral valve stenosis Mitral insufficiency/regurgitation
Tricuspid valve Tricuspid valve stenosis
Tricuspid valve stenosis
Tricuspid valve stenosis is a valvular heart disease which results in the narrowing of the orifice of the tricuspid valve of the heart. It is a relatively rare condition that causes stenosis- increased resistance to blood flow through the valve.-Causes:...

Tricuspid insufficiency
Tricuspid insufficiency
Tricuspid insufficiency , a valvular heart disease also called tricuspid regurgitation , refers to the failure of the heart's tricuspid valve to close properly during systole. As a result, with each heart beat some blood passes from the right ventricle to the right atrium, the opposite of the...

/regurgitation
Pulmonary valve Pulmonary valve stenosis
Pulmonary valve stenosis
Pulmonary valve stenosis is a heart valve disorder in which outflow of blood from the right ventricle of the heart is obstructed at the level of the pulmonic valve. This results in the reduction of flow of blood to the lungs. Valvular pulmonic stenosis accounts for 80% of right ventricular outflow...

Pulmonary insufficiency/regurgitation

Pulmonary and tricuspid valve disorders

Pulmonary and tricuspid valve diseases are right-side heart diseases. Pulmonary valve diseases are the least common heart valve disease in adults.

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
Aortic valve
The aortic valve is one of the valves of the heart. It is normally tricuspid , although in 1% of the population it is found to be congenitally bicuspid . It lies between the left ventricle and the aorta....

 or mitral valve
Mitral valve
The mitral valve is a dual-flap valve in the heart that lies between the left atrium and the left ventricle...

 diseases due to the lower pressure those valves experience.

Complications arise when the flow of blood is obstructed from leaving the right ventricle and making its way into the pulmonary artery, or once blood is in the pulmonary artery, the blood has the ability to flow back into the right ventricle. When blood has a difficult time making its way from the right ventricle into the pulmonary artery due to the pulmonary valve area being: Too narrow, deformed in some capacity (e.g., one of the leaflets of the valve is too thick, misshaped, or doesn't separate from another leaflet), or the vessel above or below the valve being deformed in such a way as to prevent the proper flow of blood, the term "pulmonary valve stenosis" is used.

Dysplasia

Heart valve dysplasia
Heart valve dysplasia
Heart valve dysplasia is a congenital heart defect which affects the aortic, pulmonary, mitral, and tricuspid heart valves. Pulmonary valve stenosis and aortic valve stenosis are discussed separately...

 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
Tetralogy of Fallot
Tetralogy of Fallot is a congenital heart defect which is classically understood to involve four anatomical abnormalities...

 is a congenital heart defect with four abnormalities, one of which is stenosis of the pulmonary valve. Ebstein's anomaly
Ebstein's anomaly
Ebstein anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.-Presentation:...

 is an abnormality of the tricuspid valve.

Rheumatic disorders

Valvular heart disease resulting from rheumatic fever
Rheumatic fever
Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...

 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
Endocarditis
Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves . Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or even on intracardiac devices...

; this is usually due to bacterial infection but may also be due to cancer
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...

 (marantic endocarditis
Marantic endocarditis
Marantic endocarditis, also known as non-bacterial thrombotic endocarditis , is the deposition of small sterile vegetations on valve leaflets....

), certain autoimmune conditions (Libman-Sacks endocarditis
Libman-Sacks endocarditis
Libman-Sacks endocarditis is a form of nonbacterial endocarditis that is seen in systemic lupus erythematosus. It is one of the most common cardiac manifestations of lupus ....

) and hypereosinophilic syndrome
Hypereosinophilic syndrome
The hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow.HES is a diagnosis of exclusion, after clonal...

 (Loeffler endocarditis
Loeffler endocarditis
Loeffler endocarditis, a form of endocarditis, is a form of hypereosinophilic syndrome.It is a restricive cardiomyopathy characterized eosinophilia and eosinophilic penetration leading to the fibrotic thickening of portions of the heart and commonly has large mural thrombi. Common symptoms include...

). Certain medications have been associated with valvular heart disease, most prominently ergotamine derivatives pergolide
Pergolide
Pergolide is an ergoline-based dopamine receptor agonist used in some countries for the treatment of Parkinson's disease....

 and cabergoline
Cabergoline
Cabergoline , an ergot derivative, is a potent dopamine receptor agonist on D2 receptors. In vitro, rat studies show cabergoline has a direct inhibitory effect on pituitary lactotroph cells...

.

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 are the risks during pregnancy to the mother and the developing fetus. In individuals who require an artificial heart valve
Artificial heart valve
An artificial heart valve is a device implanted in the heart of a patient with heart valvular disease. When one of the four heart valves malfunctions, the medical choice may be to replace the natural valve with an artificial valve. This requires open-heart surgery.Valves are integral to the normal...

, consideration must be made for deterioration of the valve over time (for bioprosthetic valves) versus the risks of anticoagulation during pregnancy
Anticoagulation in pregnancy
Hypercoagulability in pregnancy is the propensity of pregnant women to develop thrombosis . Pregnancy itself is a factor of hypercoagulability as a physiologically adaptive mechanism to prevent postpartum hemorrhage...

.

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
Mitral valve prolapse
Mitral valve prolapse is a valvular heart disease characterized by the displacement of an abnormally thickened mitral valve leaflet into the left atrium during systole. There are various types of MVP, broadly classified as classic and nonclassic. In its nonclassic form, MVP carries a low risk of...

, rheumatic heart disease and endocarditis
Endocarditis
Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves . Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or even on intracardiac devices...

.
Valvular disease Mitral stenosis
Mitral stenosis
Mitral stenosis is a valvular heart disease characterized by the narrowing of the orifice of the mitral valve of the heart.-Signs and symptoms:Symptoms of mitral stenosis include:...

 
Aortic stenosis  Aortic regurgitation  Mitral regurgitation
Mitral regurgitation
Mitral regurgitation , mitral insufficiency or mitral incompetence is a disorder of the heart in which the mitral valve does not close properly when the heart pumps out blood. It is the abnormal leaking of blood from the left ventricle, through the mitral valve, and into the left atrium, when...

 
Tricuspid regurgitation
Prevalence Most common valvular heart disease in pregnancy
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...

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 factor
Risk factor
In epidemiology, a risk factor is a variable associated with an increased risk of disease or infection. Sometimes, determinant is also used, being a variable associated with either increased or decreased risk.-Correlation vs causation:...

s
Almost always caused by rheumatic heart disease
  • Calcification of tricuspid aortic valve with age
    (>50%)
  • Calcification of bicuspid aortic valve
    Bicuspid aortic valve
    A bicuspid aortic valve is most commonly a congenital condition of the aortic valve where two of the aortic valvular leaflets fuse during development resulting in a valve that is bicuspid instead of the normal tricuspid configuration. Normally the only cardiac valve that is bicuspid is the mitral...


    (30-40%)
  • Rheumatic fever
    Rheumatic fever
    Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...

     
    (<10%)

Hypertension
Hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...

, diabetes mellitus
Diabetes mellitus
Diabetes mellitus, often simply referred to as diabetes, is a group of metabolic diseases in which a person has high blood sugar, either because the body does not produce enough insulin, or because cells do not respond to the insulin that is produced...

, hyperlipoproteinemia and uremia
Uremia
Uremia or uraemia is a term used to loosely describe the illness accompanying kidney failure , in particular the nitrogenous waste products associated with the failure of this organ....

 may speed up the process.
Acute
  • Infective endocarditis
    Infective endocarditis
    Infective endocarditis is a form of endocarditis, or inflammation, of the inner tissue of the heart, such as its valves, caused by infectious agents. The agents are usually bacterial, but other organisms can also be responsible....

  • Trauma
    Trauma (medicine)
    Trauma refers to "a body wound or shock produced by sudden physical injury, as from violence or accident." It can also be described as "a physical wound or injury, such as a fracture or blow." Major trauma can result in secondary complications such as circulatory shock, respiratory failure and death...


Chronic
  • Primary valvular: rheumatic fever
    Rheumatic fever
    Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...

    , bicuspid aortic valve
    Bicuspid aortic valve
    A bicuspid aortic valve is most commonly a congenital condition of the aortic valve where two of the aortic valvular leaflets fuse during development resulting in a valve that is bicuspid instead of the normal tricuspid configuration. Normally the only cardiac valve that is bicuspid is the mitral...

    , Marfan's syndrome, Ehlers–Danlos syndrome, ankylosing spondylitis
    Ankylosing spondylitis
    Ankylosing spondylitis , previously known as Bekhterev's disease, Bekhterev syndrome, and Marie-Strümpell disease is a chronic inflammatory disease of the axial skeleton with variable involvement of peripheral joints and nonarticular structures...

    , systemic lupus erythematosus
    Systemic lupus erythematosus
    Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...

  • Disease of the aortic root: syphilitic aortitis
    Syphilitic aortitis
    Syphilitic aortitis is a disease of the aorta associated with the tertiary stage of syphilis infection. SA begins as inflammation of the adventia, including the vessels that supply the aorta itself with blood, the vasa vasorum...

    , osteogenesis imperfecta
    Osteogenesis imperfecta
    Osteogenesis imperfecta is a genetic bone disorder. People with OI are born with defective connective tissue, or without the ability to make it, usually because of a deficiency of Type-I collagen...

    , aortic dissection
    Aortic dissection
    Aortic dissection occurs when a tear in the inner wall of the aorta causes blood to flow between the layers of the wall of the aorta and force the layers apart. The dissection typically extends anterograde, but can extend retrograde from the site of the intimal tear. Aortic dissection is a medical...

    , Behçet's disease
    Behçet's disease
    Behçet's disease is a rare immune-mediated systemic vasculitis that often presents with mucous membrane ulceration and ocular involvements...

    , reactive arthritis
    Reactive arthritis
    Reactive arthritis , is classified as an autoimmune condition that develops in response to an infection in another part of the body. Coming into contact with bacteria and developing an infection can trigger the disease. Reiter's syndrome has symptoms similar to various other conditions collectively...

    , systemic hypertension
    Hypertension
    Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...

Acute
  • Endocarditis
    Endocarditis
    Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves . Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or even on intracardiac devices...

    , mainly S. aureus
    Staphylococcus aureus
    Staphylococcus aureus is a facultative anaerobic Gram-positive coccal bacterium. It is frequently found as part of the normal skin flora on the skin and nasal passages. It is estimated that 20% of the human population are long-term carriers of S. aureus. S. aureus is the most common species of...

  • Papillary muscle
    Papillary muscle
    In anatomy, the papillary muscles are muscles located in the ventricles of the heart. They attach to the cusps of the atrioventricular valves via the chordae tendinae and contract to prevent inversion or prolapse of these valves.- Action :There are five total papillary muscles in the heart, three...

     rupture or dysfunction, including mitral valve prolapse
    Mitral valve prolapse
    Mitral valve prolapse is a valvular heart disease characterized by the displacement of an abnormally thickened mitral valve leaflet into the left atrium during systole. There are various types of MVP, broadly classified as classic and nonclassic. In its nonclassic form, MVP carries a low risk of...


  • Chronic
    • Rheumatic fever
      Rheumatic fever
      Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...

    • Marfan's syndrome
    • Cardiomyopathy
      Cardiomyopathy
      Cardiomyopathy, which literally means "heart muscle disease," is the deterioration of the function of the myocardium for any reason. People with cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both. Cardiomyopathy can often go undetected, making it especially dangerous to...

  • Usually secondary to right ventricular dilation
    • left ventricular failure is, in turn, the most common cause
    • Right ventricular infarction
    • Inferior myocardial infarction
    • Cor pulmonale
      Cor pulmonale
      Cor pulmonale or pulmonary heart disease is enlargement of the right ventricle of the heart as a response to increased resistance or high blood pressure in the lungs ....

  • Other causes: Tricuspid endocarditis
    Endocarditis
    Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves . Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or even on intracardiac devices...

    , rheumatic fever
    Rheumatic fever
    Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...

    , Ebstein's anomaly
    Ebstein's anomaly
    Ebstein anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.-Presentation:...

    , carcinoid syndrome
    Carcinoid syndrome
    Carcinoid syndrome refers to the array of symptoms that occur secondary to carcinoid tumors. The syndrome includes flushing and diarrhea, and, less frequently, heart failure and bronchoconstriction...

     and myxomatous degeneration
    Myxomatous degeneration
    Myxomatous degeneration refers to a pathological weakening of connective tissue. The term is most often used in the context of mitral valve prolapse, which is known more technically as "primary form of myxomatous degeneration of the mitral valve."...

  • Hemo
    dynamics
    Hemodynamics
    Hemodynamics, meaning literally "blood movement" is the study of blood flow or the circulation.All animal cells require oxygen for the conversion of carbohydrates, fats and proteins into carbon dioxide , water and energy in a process known as aerobic respiration...


    /
    Patho-
    physiology
    Pathophysiology
    Pathophysiology is the study of the changes of normal mechanical, physical, and biochemical functions, either caused by a disease, or resulting from an abnormal syndrome...

    Progressive obstruction of the mitral ostium causes increased pressure in the left atrium and the pulmonary circulation
    Pulmonary circulation
    Pulmonary circulation is the half portion of the cardiovascular system which carries Oxygen-depleted Blood away from the heart, to the Lungs, and returns oxygenated blood back to the heart. Encyclopedic description and discovery of the pulmonary circulation is widely attributed to Doctor Ibn...

    . Congestion may cause thromboembolism, and atrial hypertension may cause atrial fibrillation
    Atrial fibrillation
    Atrial fibrillation is the most common cardiac arrhythmia . It is a common cause of irregular heart beat, identified clinically by taking a pulse. Chaotic electrical activity in the two upper chambers of the heart result in the muscle fibrillating , instead of achieving coordinated contraction...

    .
    Obstruction through the aortic ostium causes increased pressure in the left ventricle and impaired flow through the aorta Insufficiency of the aortic valve
    Aortic valve
    The aortic valve is one of the valves of the heart. It is normally tricuspid , although in 1% of the population it is found to be congenitally bicuspid . It lies between the left ventricle and the aorta....

     causes backflow of blood into the left ventricle during diastole.
    Insufficiency of the mitral valve
    Mitral valve
    The mitral valve is a dual-flap valve in the heart that lies between the left atrium and the left ventricle...

     causes backflow of blood into the left atrium during systole.
    Insufficiency of the tricuspid valve
    Tricuspid valve
    The tricuspid valve, or right atrioventricular valve, is on the right dorsal side of the mammalian heart, between the right atrium and the right ventricle. The normal tricuspid valve usually has three leaflets and three papillary muscles. They are connected to the papillary muscles by the chordae...

     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
    Chest pain
    Chest pain may be a symptom of a number of serious conditions and is generally considered a medical emergency. Even though it may be determined that the pain is non-cardiac in origin, this is often a diagnosis of exclusion made after ruling out more serious causes of the pain.-Differential...

  • Hemoptysis
    Hemoptysis
    Hemoptysis or haemoptysis is the expectoration of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis is the expectoration (coughing up) of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis ...

  • Thromboembolism
  • Ascites
    Ascites
    Ascites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver...

     and edema
    Edema
    Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...

     (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
      Syncope (medicine)
      Syncope , the medical term for fainting, is precisely defined as a transient loss of consciousness and postural tone characterized by rapid onset, short duration, and spontaneous recovery due to global cerebral hypoperfusion that most often results from hypotension.Many forms of syncope are...

      , usually exertional
  • Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea
  • Palpitations
  • Angina pectoris
  • In acute cases: cyanosis
    Cyanosis
    Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...

     and circulatory shock
  • Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea
  • Palpitations
  • Pulmonary edema
    Pulmonary edema
    Pulmonary edema , or oedema , is fluid accumulation in the air spaces and parenchyma of the lungs. It leads to impaired gas exchange and may cause respiratory failure...

  • Symptoms of right-sided heart failure, such as ascites
    Ascites
    Ascites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver...

    , hepatomegaly
    Hepatomegaly
    Hepatomegaly is the condition of having an enlarged liver. It is a nonspecific medical sign having many causes, which can broadly be broken down into infection, direct toxicity, hepatic tumours, or metabolic disorder. Often, hepatomegaly will present as an abdominal mass...

    , edema
    Edema
    Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...

     and jugular venous distension
  • Medical sign
    Medical sign
    A medical sign is an objective indication of some medical fact or characteristic that may be detected by a physician during a physical examination of a patient....

    s
  • 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
    Parasternal heave
    A parasternal heave is a praecordial impulse that may be palpable in patients with cardiac or respiratory disease. A parasternal impulse may be felt when the heel of the hand is rested just to the left of the sternum with the fingers lifted slightly off the chest. Normally no impulse or a slight...

    , jugular venous distension, hepatomegaly
    Hepatomegaly
    Hepatomegaly is the condition of having an enlarged liver. It is a nonspecific medical sign having many causes, which can broadly be broken down into infection, direct toxicity, hepatic tumours, or metabolic disorder. Often, hepatomegaly will present as an abdominal mass...

    , ascites
    Ascites
    Ascites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver...

     and/or pulmonary hypertension
    Pulmonary hypertension
    In medicine, pulmonary hypertension is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion...

     (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
      Fourth heart sound
      The fourth heart sound or S4 is a rare extra heart sound that occurs immediately before the normal two "lub-dub" heart sounds . It occurs just after atrial contraction and immediately before the systolic S1...

    • Decreased A2 sound
    • Sustained apex beat
      Apex beat
      The apex beat, also called the point of maximum impulse , is the furthermost point outwards and downwards from the sternum at which the cardiac impulse can be felt...

    • Precordial thrill
  • Increased pulse pressure
    Pulse pressure
    Pulse Pressure is most easily defined as being the amount of pressure required to create the feeling of a pulse. Measured in millimeters of mercury , the pressure difference between the systolic and diastolic pressures give you the amount of pressure change to create the pulse, which is the pulse...

     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
    Austin Flint murmur
    In cardiology, an Austin Flint murmur is a mid-diastolic or presystolic murmur low-pitched rumbling murmur which is best heard at the cardiac apex...

  • Apex beat
    Apex beat
    The apex beat, also called the point of maximum impulse , is the furthermost point outwards and downwards from the sternum at which the cardiac impulse can be felt...

     displaced down and to the left
  • Third heart sound
    Third heart sound
    The third heart sound or S3 is a rare extra heart sound that occurs soon after the normal two "lub-dub" heart sounds .-Physiology:It occurs at the beginning of diastole approximately 0.12 to 0.18 seconds after S2...

     may be present
  • Holosystolic murmur at the apex, radiating to the back or clavicular area
  • Commonly atrial fibrillation
    Atrial fibrillation
    Atrial fibrillation is the most common cardiac arrhythmia . It is a common cause of irregular heart beat, identified clinically by taking a pulse. Chaotic electrical activity in the two upper chambers of the heart result in the muscle fibrillating , instead of achieving coordinated contraction...

  • Third heart sound
    Third heart sound
    The third heart sound or S3 is a rare extra heart sound that occurs soon after the normal two "lub-dub" heart sounds .-Physiology:It occurs at the beginning of diastole approximately 0.12 to 0.18 seconds after S2...

  • Laterally displaced apex beat, often with heave
    Parasternal heave
    A parasternal heave is a praecordial impulse that may be palpable in patients with cardiac or respiratory disease. A parasternal impulse may be felt when the heel of the hand is rested just to the left of the sternum with the fingers lifted slightly off the chest. Normally no impulse or a slight...

  • Loud, palpable P2, heard best when lying on the left side

  • In acute cases, the murmur and tachycardia
    Tachycardia
    Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...

     may be only distinctive signs.
    • Pulsatile liver
    • Prominent V waves and rapid y descents in jugular venous pressure
      Jugular venous pressure
      The jugular venous pressure is the indirectly observed pressure over the venous system...

    • Inspiratory third heart sound
      Third heart sound
      The third heart sound or S3 is a rare extra heart sound that occurs soon after the normal two "lub-dub" heart sounds .-Physiology:It occurs at the beginning of diastole approximately 0.12 to 0.18 seconds after S2...

       at left lower sternal border (LLSB)
    • Blowing holosystolic murmur
      Heart murmur
      Murmurs are extra heart sounds that are produced as a result of turbulent blood flow that is sufficient to produce audible noise. Most murmurs can only be heard with the assistance of a stethoscope ....

       at LLSB, intensifying with inspiration, and decreasing with expiration and Valsalva maneuver
      Valsalva maneuver
      The Valsalva maneuver or Valsalva manoeuvre is performed by moderately forceful attempted exhalation against a closed airway, usually done by closing one's mouth and pinching one's nose shut...

    • Parasternal heave
      Parasternal heave
      A parasternal heave is a praecordial impulse that may be palpable in patients with cardiac or respiratory disease. A parasternal impulse may be felt when the heel of the hand is rested just to the left of the sternum with the fingers lifted slightly off the chest. Normally no impulse or a slight...

       along LLSB
    • Atrial fibrillation is usually present
    Diagnosis
  • Chest X-ray
    Chest X-ray
    In medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...

     showing left atrial enlargement
    Left atrial enlargement
    Left atrial enlargement or left atrial dilation is a form of cardiomegaly.In the general population, obesity appears to be the most important risk factor for LAE. Also, a study found that LAE can occur as a consequence of atrial fibrillation , although another study found that AF by itself does...

  • Echocardiography
    Echocardiography
    An echocardiogram, often referred to in the medical community as a cardiac ECHO or simply an ECHO, is a sonogram of the heart . Also known as a cardiac ultrasound, it uses standard ultrasound techniques to image two-dimensional slices of the heart...

     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
    Chest X-ray
    In medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...

     showing calcific aortic valve, and in longstanding disease, enlarged left ventricle and atrium
  • ECG showing left ventricular hypertrophy
    Left ventricular hypertrophy
    Left ventricular hypertrophy is the thickening of the myocardium of the left ventricle of the heart.-Causes:While ventricular hypertrophy occurs naturally as a reaction to aerobic exercise and strength training, it is most frequently referred to as a pathological reaction to cardiovascular...

     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
    Left ventricular hypertrophy
    Left ventricular hypertrophy is the thickening of the myocardium of the left ventricle of the heart.-Causes:While ventricular hypertrophy occurs naturally as a reaction to aerobic exercise and strength training, it is most frequently referred to as a pathological reaction to cardiovascular...

     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
    Atrial fibrillation
    Atrial fibrillation is the most common cardiac arrhythmia . It is a common cause of irregular heart beat, identified clinically by taking a pulse. Chaotic electrical activity in the two upper chambers of the heart result in the muscle fibrillating , instead of achieving coordinated contraction...

     is treated accordingly.
    • Medically, with diuretics, prophylaxis of infective endocarditis
      Infective endocarditis
      Infective endocarditis is a form of endocarditis, or inflammation, of the inner tissue of the heart, such as its valves, caused by infectious agents. The agents are usually bacterial, but other organisms can also be responsible....

       and chronic anticoagulant
      Anticoagulant
      An anticoagulant is a substance that prevents coagulation of blood. A group of pharmaceuticals called anticoagulants can be used in vivo as a medication for thrombotic disorders. Some anticoagulants are used in medical equipment, such as test tubes, blood transfusion bags, and renal dialysis...

       administration with warfarin
      Warfarin
      Warfarin is an anticoagulant. It is most likely to be the drug popularly referred to as a "blood thinner," yet this is a misnomer, since it does not affect the thickness or viscosity of blood...

       (especially when there's atrial fibrillation
      Atrial fibrillation
      Atrial fibrillation is the most common cardiac arrhythmia . It is a common cause of irregular heart beat, identified clinically by taking a pulse. Chaotic electrical activity in the two upper chambers of the heart result in the muscle fibrillating , instead of achieving coordinated contraction...

      )
    • 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
      Valvulotomy
      Commissurotomy of cardiac valves is called valvulotomy, and consists of making one or more incisions at the edges of the commissure formed between two or three valves, in order to relieve constriction such as occurs in valvular stenosis, especially mitral valve stenosis....

       or mitral valve replacement
      Mitral valve replacement
      Mitral valve replacement is a cardiac surgery procedure in which a patient’s mitral valve is replaced by a different valve. Mitral valve replacement is typically performed robotically or manually, when the valve becomes too tight for blood to flow into the left ventricle, or too loose in which...

       by open surgery

    No treatment in asymptomatic patients.
    • If symptomatic, treated with aortic valve replacement
      Aortic valve replacement
      Aortic valve replacement is a cardiac surgery procedure in which a patient's failing aortic valve is replaced with an alternate healthy valve. The aortic valve can be affected by a range of diseases; the valve can either become leaky or partially blocked...

       surgery.

    Medical therapy and percutaneous balloon valvuloplasty have relatively poor effect.

    - Any angina is treated with short-acting nitrovasodilator
    Nitrovasodilator
    A nitrovasodilator is an agent which causes vasodilation by donation of nitric oxide.Examples include nitroprusside and nitroglycerine.-References:...

    s, beta-blockers and/or calcium blockers

    - Any hypertension
    Hypertension
    Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...

     is treated aggressively, but caution must be taken in administering beta-blockers

    - Any heart failure is treated with digoxin
    Digoxin
    Digoxin INN , also known as digitalis, is a purified cardiac glycoside and extracted from the foxglove plant, Digitalis lanata. Its corresponding aglycone is digoxigenin, and its acetyl derivative is acetyldigoxin...

    , diuretic
    Diuretic
    A diuretic provides a means of forced diuresis which elevates the rate of urination. There are several categories of diuretics. All diuretics increase the excretion of water from bodies, although each class does so in a distinct way.- Medical uses :...

    s, nitrovasodilator
    Nitrovasodilator
    A nitrovasodilator is an agent which causes vasodilation by donation of nitric oxide.Examples include nitroprusside and nitroglycerine.-References:...

    s and, if not contraindicated, cautious inpatient administration of ACE inhibitor
    ACE inhibitor
    ACE inhibitors or angiotensin-converting enzyme inhibitors are a group of drugs used primarily for the treatment of hypertension and congestive heart failure...

    s
    • If stable and asymptomatic - conservative treatment such as low sodium diet
      Low sodium diet
      A low sodium diet is a diet that includes no more than 1,500 to 2,400 mgs of sodium per day. People who follow a vigorous or moderate exercise schedule are usually advised to limit their sodium intake to 3,000 mg per day and those with moderate to severe heart failure are usually advised to...

      , diuretic
      Diuretic
      A diuretic provides a means of forced diuresis which elevates the rate of urination. There are several categories of diuretics. All diuretics increase the excretion of water from bodies, although each class does so in a distinct way.- Medical uses :...

      s, vasodilators (e.g. (hydralazin or prazosin
      Prazosin
      Prazosin, trade names Minipress, Vasoflex, Pressin and Hypovase, is a sympatholytic drug used to treat high blood pressure and Anxiety, PTSD and Panic Disorder. It belongs to the class of alpha-adrenergic blockers. Specifically, prazosin is selective for the alpha-1 receptors on vascular smooth...

      ), digoxin
      Digoxin
      Digoxin INN , also known as digitalis, is a purified cardiac glycoside and extracted from the foxglove plant, Digitalis lanata. Its corresponding aglycone is digoxigenin, and its acetyl derivative is acetyldigoxin...

      , ACE inhibitor
      ACE inhibitor
      ACE inhibitors or angiotensin-converting enzyme inhibitors are a group of drugs used primarily for the treatment of hypertension and congestive heart failure...

      s/angiotensin II receptor antagonist
      Angiotensin II receptor antagonist
      Angiotensin II receptor antagonists, also known as angiotensin receptor blockers , AT1-receptor antagonists or sartans, are a group of pharmaceuticals which modulate the renin-angiotensin-aldosterone system...

      s, calcium blockers and avoiding very strenuous activity
      Anaerobic exercise
      Anaerobic exercise is exercise intense enough to trigger anaerobic metabolism. It is used by athletes in non-endurance sports to promote strength, speed and power and by body builders to build muscle mass...

    • Aortic valve replacement
      Aortic valve replacement
      Aortic valve replacement is a cardiac surgery procedure in which a patient's failing aortic valve is replaced with an alternate healthy valve. The aortic valve can be affected by a range of diseases; the valve can either become leaky or partially blocked...

       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
        Afterload
        Afterload is the tension or stress developed in the wall of the left ventricle during ejection. Following Laplace's law, the tension upon the muscle fibers in the heart wall is the product of the pressure within the ventricle, multiplied by the volume within the ventricle, divided by the wall...

         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
      Intra-aortic balloon pump
      The Intra-aortic balloon pump ' is a mechanical device that increases myocardial oxygen perfusion while at the same time increasing cardiac output. Increasing cardiac output increases coronary blood flow and therefore myocardial oxygen delivery...

       as temporary solution until surgery
    • Surgery by either mitral valve repair
      Mitral valve repair
      Mitral valve repair is a cardiac surgery procedure performed by cardiac surgeons to treat stenosis or regurgitation of the mitral valve. The mitral valve is the "inflow valve" for the left side of the heart. Blood flows from the lungs, where it picks up oxygen, through the pulmonary veins, to the...

       or mitral valve replacement
      Mitral valve replacement
      Mitral valve replacement is a cardiac surgery procedure in which a patient’s mitral valve is replaced by a different valve. Mitral valve replacement is typically performed robotically or manually, when the valve becomes too tight for blood to flow into the left ventricle, or too loose in which...

      , indicated if very symptomatic (NYHA III), ventricular dilation or decreasing ejection fraction
      Ejection fraction
      In cardiovascular physiology, ejection fraction is the fraction of Blood pumped out of the Right Ventricle of the heart to the Pulmonary Circulation and Left Ventricle of the heart to the Systemic Circulation with each Heart beat or Cardiac cycle...

  • Treatment of underlying cause
  • Surgery
    • Tricuspid valvular repair
      Heart valve repair
      Heart valve repair is a surgical technique used to fix defects in heart valves in valvular heart diseases, and provides an alternative to valve replacement. Without further specification, it refers to native heart valve repair, rather than repair of an artificial heart...

    • Valvuloplasty
    • Valve replacement
      Valve replacement
      Valve replacement surgery is the replacement of one or more of the heart valves with either an artificial heart valve or a bioprosthesis . It is an alternative to valve repair.There are four procedures:...

       (rarely performed)
  • Follow-up
  • In moderate cases, echocardiography every 1–2 years, possibly complemented with cardiac stress test
    Cardiac stress test
    Cardiac stress test is a test used in medicine and cardiology to measure the heart's ability to respond to external stress in a controlled clinical environment....

    . 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.

  • External Links

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