Mitral Valve Prolapse

Overview

Epidemiology

• Most common cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) valve anomaly
• Affects 3%–7% of the global population
• Women > men 
• Most common between 15–30 years of age
• Complications are more often seen in men.

Etiology

• Primary/ idiopathic Idiopathic Dermatomyositis (most common)
• Secondary:
  • Connective tissue Connective tissue Connective tissues originate from embryonic mesenchyme and are present throughout the body except inside the brain and spinal cord. The main function of connective tissues is to provide structural support to organs. Connective tissues consist of cells and an extracellular matrix. Connective Tissue: Histology disorders:
    • Marfan’s syndrome 
    • Ehlers-Danlos syndrome Ehlers-Danlos syndrome Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders that are characterized by hyperextensible skin, hypermobile joints, and fragility of the skin and connective tissue. Ehlers-Danlos Syndrome 
    • Osteogenesis imperfecta Osteogenesis imperfecta Osteogenesis imperfecta (OI), or “brittle bone disease,” is a rare genetic connective tissue disorder characterized by severe bone fragility. Although OI is considered a single disease, OI includes over 16 genotypes and clinical phenotypes with differing symptom severity. Osteogenesis Imperfecta
    • Pseudoxanthoma elasticum
  • Cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) abnormalities:
    • Atrial septal defect Atrial Septal Defect Atrial septal defects (ASDs) are benign acyanotic congenital heart defects characterized by an opening in the interatrial septum that causes blood to flow from the left atrium (LA) to the right atrium (RA) (left-to-right shunt). Atrial Septal Defect (ASD)
    • Hypertrophic cardiomyopathy Cardiomyopathy Cardiomyopathy refers to a group of myocardial diseases associated with structural changes of the heart muscles (myocardium) and impaired systolic and/or diastolic function in the absence of other heart disorders (coronary artery disease, hypertension, valvular disease, and congenital heart disease). Cardiomyopathy: Overview and Types

Pathophysiology

Mitral valve Mitral valve The valve between the left atrium and left ventricle of the heart. Heart: Anatomy prolapse (MVP) is the bulging of the mitral valve Mitral valve The valve between the left atrium and left ventricle of the heart. Heart: Anatomy (MV) leaflets into the atrium by ≥ 2 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma above the plane of the MV annulus during ventricular systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle:

• Most commonly characterized by myxomatous degeneration (Barlow’s syndrome): accumulation of glycosaminoglycans (GAGs) and alterations in collagen Collagen A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of skin; connective tissue; and the organic substance of bones (bone and bones) and teeth (tooth). Connective Tissue: Histology structure → expansion of the spongiosa layer of the MV leaflets and chordae tendineae Chordae tendineae The tendinous cords that connect each cusp of the two atrioventricular heart valves to appropriate papillary muscles in the heart ventricles, preventing the valves from reversing themselves when the ventricles contract. Heart: Anatomy leading to:  
  • Thickened, stretchy leaflets with excess tissue
  • Elongated chordae tendineae Chordae tendineae The tendinous cords that connect each cusp of the two atrioventricular heart valves to appropriate papillary muscles in the heart ventricles, preventing the valves from reversing themselves when the ventricles contract. Heart: Anatomy (rupture less common)
  • MV annulus enlargement
  • Endothelial disruption of the MV leaflets → possible sites for endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis and thrombus formation
• Fibroelastic deficiency is another potential cause (usually older patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship): ↓ collagen Collagen A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of skin; connective tissue; and the organic substance of bones (bone and bones) and teeth (tooth). Connective Tissue: Histology, elastin, and proteoglycans Proteoglycans Glycoproteins which have a very high polysaccharide content. Basics of Carbohydrates leads to:
  • Thin leaflets, no excess tissue
  • Some annulus dilation
  • Thin, elongated chordae → ↑ risk of rupture
• MVP may progress to mitral regurgitation Mitral regurgitation Mitral regurgitation (MR) is the backflow of blood from the left ventricle (LV) to the left atrium (LA) during systole. Mitral regurgitation may be acute (myocardial infarction) or chronic (myxomatous degeneration). Acute and decompensated chronic MR can lead to pulmonary venous congestion, resulting in symptoms of dyspnea, orthopnea, and fatigue. Mitral Regurgitation ( 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):
  • Billowing of leaflets into the atrium during systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle → mild 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
  • Ruptured chordae tendineae Chordae tendineae The tendinous cords that connect each cusp of the two atrioventricular heart valves to appropriate papillary muscles in the heart ventricles, preventing the valves from reversing themselves when the ventricles contract. Heart: Anatomy → flail leaflets → severe 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

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. MVP 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 Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) 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 MVP. 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.
• Mitral stenosis Mitral stenosis Mitral stenosis (MS) is the narrowing of the mitral valve (MV) orifice, leading to obstructed blood flow from the left atrium (LA) to the left ventricle (LV). Mitral stenosis is most commonly due to rheumatic heart disease. Mitral stenosis leads to impaired LV diastolic filling, increased LA pressure, and LA dilation. Mitral Stenosis: narrowing of the MV, which results in obstruction of blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure from the LA to the LV. 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 the most common cause. 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 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. The exam may reveal a low-pitched, rumbling, diastolic murmur at the cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) apex, which will differentiate mitral stenosis Mitral stenosis Mitral stenosis (MS) is the narrowing of the mitral valve (MV) orifice, leading to obstructed blood flow from the left atrium (LA) to the left ventricle (LV). Mitral stenosis is most commonly due to rheumatic heart disease. Mitral stenosis leads to impaired LV diastolic filling, increased LA pressure, and LA dilation. Mitral Stenosis from MVP. Diagnosis is made by 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), and treatment includes diuretics Diuretics Agents that promote the excretion of urine through their effects on kidney function. Heart Failure and Angina Medication, beta blockers, and surgery for severe disease.
• Aortic stenosis Aortic stenosis Aortic stenosis (AS), or the narrowing of the aortic valve aperture, is the most common valvular heart disease. Aortic stenosis gradually progresses to heart failure, producing exertional dyspnea, angina, and/or syncope. A crescendo-decrescendo systolic murmur is audible in the right upper sternal border. Aortic Stenosis: narrowing of the aortic valve Aortic valve The valve between the left ventricle and the ascending aorta which prevents backflow into the left ventricle. Heart: Anatomy, which obstructs blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure from the LV to the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship may develop the classic triad of syncope Syncope Syncope is a short-term loss of consciousness and loss of postural stability followed by spontaneous return of consciousness to the previous neurologic baseline without the need for resuscitation. The condition is caused by transient interruption of cerebral blood flow that may be benign or related to a underlying life-threatening condition. Syncope, angina, and 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 as aortic stenosis Aortic stenosis Aortic stenosis (AS), or the narrowing of the aortic valve aperture, is the most common valvular heart disease. Aortic stenosis gradually progresses to heart failure, producing exertional dyspnea, angina, and/or syncope. A crescendo-decrescendo systolic murmur is audible in the right upper sternal border. Aortic Stenosis progresses. The exam will reveal a systolic crescendo-decrescendo murmur at the upper right sternal border, which will differentiate aortic stenosis Aortic stenosis Aortic stenosis (AS), or the narrowing of the aortic valve aperture, is the most common valvular heart disease. Aortic stenosis gradually progresses to heart failure, producing exertional dyspnea, angina, and/or syncope. A crescendo-decrescendo systolic murmur is audible in the right upper sternal border. Aortic Stenosis from MVP. The diagnosis is confirmed by 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), and valvuloplasty or valve replacement is performed for severe disease.
• Tricuspid stenosis Tricuspid stenosis Tricuspid stenosis (TS) is a valvular defect that obstructs blood flow from the right atrium to the right ventricle during diastole. This condition most commonly results from rheumatic heart disease or a congenital defect, and is usually found in conjunction with other valvular disease. A mid-diastolic murmur is best heard at the lower left sternal border. Tricuspid Stenosis: 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 Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure from the right atrium (RA) to the right ventricle (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 Tricuspid stenosis Tricuspid stenosis (TS) is a valvular defect that obstructs blood flow from the right atrium to the right ventricle during diastole. This condition most commonly results from rheumatic heart disease or a congenital defect, and is usually found in conjunction with other valvular disease. A mid-diastolic murmur is best heard at the lower left sternal border. Tricuspid Stenosis from MVP. 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. 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 Tricuspid regurgitation Tricuspid regurgitation (TR) is a valvular defect that allows backflow of blood from the right ventricle to the right atrium during systole. Tricuspid regurgitation can develop through a number of cardiac conditions that cause dilation of the right ventricle and tricuspid annulus. A blowing holosystolic murmur is best heard at the left lower sternal border. Tricuspid Regurgitation: 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 Tricuspid regurgitation Tricuspid regurgitation (TR) is a valvular defect that allows backflow of blood from the right ventricle to the right atrium during systole. Tricuspid regurgitation can develop through a number of cardiac conditions that cause dilation of the right ventricle and tricuspid annulus. A blowing holosystolic murmur is best heard at the left lower sternal border. Tricuspid Regurgitation from MVP. 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. 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.