Mitral Stenosis

Overview

Definition

Mitral stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) ( MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis) is the narrowing of the mitral valve Mitral valve The valve between the left atrium and left ventricle of the heart. Heart: Anatomy (MV) orifice, which impedes blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure from the left atrium (LA) into the left ventricle (LV) during diastole Diastole Post-systolic relaxation of the heart, especially the heart ventricles. Cardiac Cycle.

Epidemiology

• Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency has decreased in the developed world. 
• Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency:
  • 1 in 100,000 in the United States
  • Higher in developing countries, where rheumatic heart disease Rheumatic Heart Disease Cardiac manifestation of systemic rheumatological conditions, such as rheumatic fever. Rheumatic heart disease can involve any part the heart, most often the heart valves and the endocardium. Rheumatic Fever is more prevalent
• Women > men
• Onset is usually in the 3rd and 4th decade of life.

Etiology

• Rheumatic heart disease Rheumatic Heart Disease Cardiac manifestation of systemic rheumatological conditions, such as rheumatic fever. Rheumatic heart disease can involve any part the heart, most often the heart valves and the endocardium. Rheumatic Fever (most common)
• Congenital Congenital Chorioretinitis 
• Mitral annular Annular Dermatologic Examination calcification
• Radiation Radiation Emission or propagation of acoustic waves (sound), electromagnetic energy waves (such as light; radio waves; gamma rays; or x-rays), or a stream of subatomic particles (such as electrons; neutrons; protons; or alpha particles). Osteosarcoma induced
• Fabry’s disease
• Whipple’s disease
• Systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus
• Rheumatoid arthritis Arthritis Acute or chronic inflammation of joints. Osteoarthritis
• Carcinoid disease
• Conditions that mimic the hemodynamic changes of MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis:
  • LA myxoma Myxoma A benign neoplasm derived from connective tissue, consisting chiefly of polyhedral and stellate cells that are loosely embedded in a soft mucoid matrix, thereby resembling primitive mesenchymal tissue. It occurs frequently intramuscularly where it may be mistaken for a sarcoma. It appears also in the jaws and the skin. Cardiac Myxoma
  • Large infective endocarditis Infective endocarditis Infective endocarditis (IE) is caused by infection or inflammation of the inner lining of the heart (endocardium), most commonly affecting the heart valves. Endocarditis
  • Degenerated bioprosthetic MV

Pathophysiology

• Rheumatic heart disease Rheumatic Heart Disease Cardiac manifestation of systemic rheumatological conditions, such as rheumatic fever. Rheumatic heart disease can involve any part the heart, most often the heart valves and the endocardium. Rheumatic Fever: molecular mimicry Molecular Mimicry The structure of one molecule that imitates or simulates the structure of a different molecule. Rheumatic Fever displayed by group A-hemolytic Streptococci after pharyngeal infection → acute pancarditis → chronic valvular inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation → 
  • Fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans and thickening of the valve leaflets and fusion of mitral commissures
  • Mitral calcification and stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) with narrowing of the valve orifice
• A narrowed MV orifice → restricted LV filling → ↑ LA pressure is required to propel blood →
  • LA dilation → atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation, LA thrombosis Thrombosis Formation and development of a thrombus or blood clot in the blood vessel. Epidemic Typhus 
  • ↑ Pulmonary capillary wedge pressure (PCWP) → pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension → right heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR)
  • ↓ Stroke volume Stroke volume The amount of blood pumped out of the heart per beat, not to be confused with cardiac output (volume/time). It is calculated as the difference between the end-diastolic volume and the end-systolic volume. Cardiac Cycle → ↓ cardiac output Cardiac output The volume of blood passing through the heart per unit of time. It is usually expressed as liters (volume) per minute so as not to be confused with stroke volume (volume per beat). Cardiac Mechanics → left heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR)

Clinical Presentation

Clinical manifestations

• Asymptomatic in the early stages
• Symptoms of heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR):
  • Progressive exertional dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
  • Fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia
  • Orthopnea Orthopnea Pulmonary Edema
  • Paroxysmal nocturnal dyspnea Paroxysmal nocturnal dyspnea A disorder characterized by sudden attacks of respiratory distress in at rest patients with heart failure and pulmonary edema. It usually occurs at night after several hours of sleep in a reclining position. Patients awaken with a feeling of suffocation, coughing, a cold sweat, and tachycardia. When there is significant wheezing, it is called cardiac asthma. Pulmonary Edema
  • Pulmonary edema Pulmonary edema Pulmonary edema is a condition caused by excess fluid within the lung parenchyma and alveoli as a consequence of a disease process. Based on etiology, pulmonary edema is classified as cardiogenic or noncardiogenic. Patients may present with progressive dyspnea, orthopnea, cough, or respiratory failure. Pulmonary Edema → rupture of small pulmonary vessels → hemoptysis Hemoptysis Hemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock. Hemoptysis
• Symptoms of atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation: 
  • Palpitations Palpitations Ebstein’s Anomaly
  • Sequelae from thromboembolism Thromboembolism Obstruction of a blood vessel (embolism) by a blood clot (thrombus) in the blood stream. Systemic Lupus Erythematosus
• Compression Compression Blunt Chest Trauma of the left recurrent laryngeal nerve by an enlarged LA → hoarseness Hoarseness An unnaturally deep or rough quality of voice. Parapharyngeal Abscess (Ortner syndrome)
• Compression Compression Blunt Chest Trauma of the bronchi Bronchi The larger air passages of the lungs arising from the terminal bifurcation of the trachea. They include the largest two primary bronchi which branch out into secondary bronchi, and tertiary bronchi which extend into bronchioles and pulmonary alveoli. Bronchial Tree: Anatomy by an enlarged LA → cough
• Compression Compression Blunt Chest Trauma of the esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy by an enlarged LA → dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming “stuck.” Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia

Physical exam

• Loud S1 S1 Heart Sounds → abrupt closure of stenotic MV leaflets ( S1 S1 Heart Sounds may be absent if the leaflets are heavily calcified.)
• Normal S2 S2 Heart Sounds split with an exaggerated P2 → pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension
• Early diastolic opening snap after S2 S2 Heart Sounds, followed by a low-pitched, decrescendo-crescendo rumbling murmur:
  • Heard best at the apex
  • Murmur ↑ with squatting, handgrip (↑ afterload Afterload Afterload is the resistance in the aorta that prevents blood from leaving the heart. Afterload represents the pressure the LV needs to overcome to eject blood into the aorta. Cardiac Mechanics)
  • Murmur ↓ with decreased blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure across the valve ( tachycardia Tachycardia Abnormally rapid heartbeat, usually with a heart rate above 100 beats per minute for adults. Tachycardia accompanied by disturbance in the cardiac depolarization (cardiac arrhythmia) is called tachyarrhythmia. Sepsis in Children, pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension)
  • Opening snap becomes closer to S2 S2 Heart Sounds as the stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) becomes more severe.
  • Snap may be absent if the valve is heavily calcified.
• Palpable P2 and right ventricle (RV) heave (parasternal lift)
• The point of maximal impulse is normal or ↓, due to ↓ LV filling.
• Signs of heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR) (usually right sided):
  • Jugular venous distension Jugular Venous Distension Cardiovascular Examination
  • Crackles
  • Hepatomegaly 
  • Peripheral edema Peripheral edema Peripheral edema is the swelling of the lower extremities, namely, legs, feet, and ankles. Edema
• Malar flush (“mitral facies”) → cutaneous vasodilation Vasodilation The physiological widening of blood vessels by relaxing the underlying vascular smooth muscle. Pulmonary Hypertension Drugs and hypoxemia Hypoxemia Neonatal Respiratory Distress Syndrome → low cardiac output Cardiac output The volume of blood passing through the heart per unit of time. It is usually expressed as liters (volume) per minute so as not to be confused with stroke volume (volume per beat). Cardiac Mechanics and severe pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension

Diagnosis

Echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA)

• Transthoracic echocardiography Transthoracic Echocardiography Imaging of the Heart and Great Vessels ( TTE TTE Imaging of the Heart and Great Vessels):
  • Diagnostic modality of choice
  • Findings:
    • Stenotic MV
    • Valve thickening and calcification
    • Commissural fusion
    • LA dilation
    • Evaluation of LV and RV function
  • Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) is used to evaluate: 
    • Transvalvular gradient Transvalvular gradient Tricuspid Stenosis
    • Mitral valve Mitral valve The valve between the left atrium and left ventricle of the heart. Heart: Anatomy area (MVA):
      • Moderate MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis: 1.5–2.5 cm²
      • Severe MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis: < 1.5 cm²
      • Very severe MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis: < 1 cm²
    • Pulmonary artery Pulmonary artery The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. Lungs: Anatomy (PA) pressure
    • Associated valve disease (mitral regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD) ( MR MR Calculated as the ratio of the total number of people who die due to all causes over a specific time period to the total number of people in the selected population. Measures of Health Status))
• Transesophageal echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA) ( TEE TEE Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues using a transducer placed in the esophagus. Imaging of the Heart and Great Vessels): 
  • Used to exclude LA thrombus before percutaneous balloon valvuloplasty
  • Can also evaluate for LA myxomas or large vegetations that may simulate MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis

Other workup

• Electrocardiography Electrocardiography Recording of the moment-to-moment electromotive forces of the heart as projected onto various sites on the body’s surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a cathode ray tube display. Electrocardiogram (ECG) ( ECG ECG An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG)):
  • Findings are non-specific.
  • Signs of LA enlargement: 
    • “Double-peaked” P waves (P mitrale)
    • Only seen when in sinus rhythm Sinus rhythm A heart rate and rhythm driven by the regular firing of the SA node (60–100 beats per minute) Cardiac Physiology
  • Atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation 
  • With severe pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension:
    • Right axis deviation
    • “Tall” P waves 
• Chest radiograph: 
  • Straightening of the upper left border of the cardiac silhouette → LA enlargement
  • Pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension → enlarged PA
  • Pulmonary vascular congestion:
    • Cephalization
    • Kerley B lines Kerley B Lines Pulmonary Edema 
• Cardiac magnetic resonance Cardiac magnetic resonance Aortic Regurgitation imaging (cMRI):
  • Used when an echocardiogram Echocardiogram Transposition of the Great Vessels is unsatisfactory or there is a discrepancy between findings and the clinical picture
  • Can provide information on valve anatomy
  • Can assess the severity of MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis
• Cardiac catheterization Cardiac Catheterization Procedures in which placement of cardiac catheters is performed for therapeutic or diagnostic procedures. Cardiac Surgery:
  • Part of the preoperative assessment
  • Evaluates for coronary artery Coronary Artery Truncus Arteriosus disease prior to surgery
  • Can confirm ↑ LA and PA pressures, MVA

Management

• Periodic monitoring for asymptomatic patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship
• Secondary prevention of rheumatic fever Rheumatic fever Acute rheumatic fever (ARF) is an autoimmune inflammatory process that usually follows Streptococcal pharyngitis. Acute rheumatic fever usually occurs 2-4 weeks after an untreated infection and affects the heart, skin, joints, and nervous system. Rheumatic Fever for patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with rheumatic MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis:
  • Penicillin Penicillin Rheumatic Fever G 
  • Duration of therapy depends on the number of previous attacks and duration since the last episode.
• Anticoagulation Anticoagulation Pulmonary Hypertension Drugs for moderate-to-severe MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis:
  • If atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation is present
  • If the patient had a prior embolic event
  • If there is an LA thrombus 
  • Consider anticoagulation Anticoagulation Pulmonary Hypertension Drugs for severe LA enlargement.
  • Note: Warfarin Warfarin An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. Warfarin is indicated for the prophylaxis and/or treatment of venous thrombosis and its extension, pulmonary embolism, and atrial fibrillation with embolization. It is also used as an adjunct in the prophylaxis of systemic embolism after myocardial infarction. Warfarin is also used as a rodenticide. Anticoagulants is typically used because the novel anticoagulants Anticoagulants Anticoagulants are drugs that retard or interrupt the coagulation cascade. The primary classes of available anticoagulants include heparins, vitamin K-dependent antagonists (e.g., warfarin), direct thrombin inhibitors, and factor Xa inhibitors. Anticoagulants have not been evaluated in valvular heart disease.
• Treatment of atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation:
  • Cardioversion Cardioversion Atrial Fibrillation in unstable patients Unstable Patients Blunt Chest Trauma
  • Beta blockers and calcium Calcium A basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. Electrolytes channel blockers in stable patients Stable Patients Blunt Chest Trauma
• Heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR) management:
  • Diuretics Diuretics Agents that promote the excretion of urine through their effects on kidney function. Heart Failure and Angina Medication
  • Beta blockers are used cautiously (↓ heart rate Heart rate The number of times the heart ventricles contract per unit of time, usually per minute. Cardiac Physiology can ↓ cardiac output Cardiac output The volume of blood passing through the heart per unit of time. It is usually expressed as liters (volume) per minute so as not to be confused with stroke volume (volume per beat). Cardiac Mechanics).
  • Salt restriction
• Percutaneous mitral balloon commissurotomy (PMBC): 
  • 1st line
  • Used in severe or symptomatic MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis
  • Requires favorable valve morphology with little or no MR MR Calculated as the ratio of the total number of people who die due to all causes over a specific time period to the total number of people in the selected population. Measures of Health Status and without LA thrombus
  • Can delay valve replacement
• Surgical MV replacement: 
  • Pursued if PMBC is contraindicated
  • Anticoagulation Anticoagulation Pulmonary Hypertension Drugs required:
    • 3–6 months for bioprosthetic valve
    • Lifelong for mechanical valve

Differential Diagnosis

• MR MR Calculated as the ratio of the total number of people who die due to all causes over a specific time period to the total number of people in the selected population. Measures of Health Status: valve disorder where blood refluxes from the LV to the LA during systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle. Mitral valve Mitral valve The valve between the left atrium and left ventricle of the heart. Heart: Anatomy prolapse ( MVP MVP Mitral valve prolapse (MVP) is the most common cardiac valvular defect, and is characterized by bulging of the mitral valve (MV) cusps into the left atrium (LA) during systole. Mitral valve prolapse is most commonly due to idiopathic myxomatous degeneration. Patients are typically asymptomatic. Mitral Valve Prolapse) is the most common etiology. Signs and symptoms are based on severity and can include exertional dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia, or edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema. The exam will be notable for a systolic murmur at the cardiac apex, and an echocardiogram Echocardiogram Transposition of the Great Vessels can establish the diagnosis and differentiate MR MR Calculated as the ratio of the total number of people who die due to all causes over a specific time period to the total number of people in the selected population. Measures of Health Status from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. Treatment includes sodium Sodium A member of the alkali group of metals. It has the atomic symbol na, atomic number 11, and atomic weight 23. Hyponatremia restriction, diuretics Diuretics Agents that promote the excretion of urine through their effects on kidney function. Heart Failure and Angina Medication, and surgery for severe cases.
• MVP MVP Mitral valve prolapse (MVP) is the most common cardiac valvular defect, and is characterized by bulging of the mitral valve (MV) cusps into the left atrium (LA) during systole. Mitral valve prolapse is most commonly due to idiopathic myxomatous degeneration. Patients are typically asymptomatic. Mitral Valve Prolapse: the most common cardiac valvular defect, characterized by bulging of the MV leaflets into the LA during systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle. Auscultation characteristically reveals a mid-systolic click followed by a possible regurgitant murmur. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship are generally asymptomatic. However, MVP MVP Mitral valve prolapse (MVP) is the most common cardiac valvular defect, and is characterized by bulging of the mitral valve (MV) cusps into the left atrium (LA) during systole. Mitral valve prolapse is most commonly due to idiopathic myxomatous degeneration. Patients are typically asymptomatic. Mitral Valve Prolapse can progress to MR MR Calculated as the ratio of the total number of people who die due to all causes over a specific time period to the total number of people in the selected population. Measures of Health Status in some patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship. Echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA) will establish the diagnosis and differentiate MVP MVP Mitral valve prolapse (MVP) is the most common cardiac valvular defect, and is characterized by bulging of the mitral valve (MV) cusps into the left atrium (LA) during systole. Mitral valve prolapse is most commonly due to idiopathic myxomatous degeneration. Patients are typically asymptomatic. Mitral Valve Prolapse from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. Most patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship do not require treatment.
• Severe aortic regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD): valve disorder allowing blood to reflux back into the ventricle from the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy during diastole Diastole Post-systolic relaxation of the heart, especially the heart ventricles. Cardiac Cycle. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship may present with exertional dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, orthopnea Orthopnea Pulmonary Edema, palpitations Palpitations Ebstein’s Anomaly, and chest pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways. An exam will show a blowing, early diastolic decrescendo murmur at the 3rd left intercostal space. Echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA) will establish the diagnosis and differentiate severe aortic regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD) from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. Treatment involves surgical repair or replacement.
• Tricuspid stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS): narrowing of the tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy, which prevents normal blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure from the RA to the RV. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship may be asymptomatic or may present with signs and symptoms of systemic venous congestion. A mid-diastolic murmur at the left lower sternal border distinguishes tricuspid stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. An echocardiogram Echocardiogram Transposition of the Great Vessels will establish the diagnosis. Management includes sodium Sodium A member of the alkali group of metals. It has the atomic symbol na, atomic number 11, and atomic weight 23. Hyponatremia restriction, diuretics Diuretics Agents that promote the excretion of urine through their effects on kidney function. Heart Failure and Angina Medication, and surgery for severe cases.
• Tricuspid regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD): valve disorder allowing blood to reflux into the RA from the RV during systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship may be asymptomatic or may present with signs and symptoms of systemic venous congestion. A holosystolic murmur Holosystolic Murmur Tricuspid Valve Atresia (TVA) at the left lower sternal border distinguishes tricuspid regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD) from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. An echocardiogram Echocardiogram Transposition of the Great Vessels will establish the diagnosis. Management involves treating the underlying cause, sodium Sodium A member of the alkali group of metals. It has the atomic symbol na, atomic number 11, and atomic weight 23. Hyponatremia restriction, diuretics Diuretics Agents that promote the excretion of urine through their effects on kidney function. Heart Failure and Angina Medication, and surgery for severe cases.
• ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder: an opening in the interatrial septum Interatrial Septum Atrial Septal Defect (ASD) that allows left-to-right shunting of blood. Complications include pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension, heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR), and atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation. Adults may present with dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia, and palpitations Palpitations Ebstein’s Anomaly. A mid-systolic murmur with a fixed and widely split S2 S2 Heart Sounds at the left sternal boarder may be heard, which differs from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. Diagnosis is made with an echocardiogram Echocardiogram Transposition of the Great Vessels. Surgical or transcatheter closure is used as treatment.
• LA myxoma Myxoma A benign neoplasm derived from connective tissue, consisting chiefly of polyhedral and stellate cells that are loosely embedded in a soft mucoid matrix, thereby resembling primitive mesenchymal tissue. It occurs frequently intramuscularly where it may be mistaken for a sarcoma. It appears also in the jaws and the skin. Cardiac Myxoma: a benign Benign Fibroadenoma, primary cardiac tumor Tumor Inflammation that can can cause MV outflow tract obstruction during diastole Diastole Post-systolic relaxation of the heart, especially the heart ventricles. Cardiac Cycle if located in the LA. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present with signs and symptoms of heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR). The murmur can mimic MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis, and a “ tumor Tumor Inflammation plop” is also heard as the myxoma Myxoma A benign neoplasm derived from connective tissue, consisting chiefly of polyhedral and stellate cells that are loosely embedded in a soft mucoid matrix, thereby resembling primitive mesenchymal tissue. It occurs frequently intramuscularly where it may be mistaken for a sarcoma. It appears also in the jaws and the skin. Cardiac Myxoma drops into the mitral orifice. Diagnosis is made with an echocardiogram Echocardiogram Transposition of the Great Vessels and a cMRI, which will differentiate LA myxoma Myxoma A benign neoplasm derived from connective tissue, consisting chiefly of polyhedral and stellate cells that are loosely embedded in a soft mucoid matrix, thereby resembling primitive mesenchymal tissue. It occurs frequently intramuscularly where it may be mistaken for a sarcoma. It appears also in the jaws and the skin. Cardiac Myxoma from MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis. Treatment includes surgical excision.