Clinical Presentation In infancy, bicuspid aortic valve disease is often asymptomatic. By adolescence an estimate 1 of 50 children born with these abnormalities will have clinically significant obstruction or regurgitation.[24] Complications of bicuspid aortic valve disease are common in adulthood.[32] The abnormal shear stress leads to valve calcification and further aortic root dilation has been reported. [33] The most common complication is aortic stenosis, caused by premature fibrosis, stiffening, and calcium deposition. The majority of patients under 65 years of age with significant aortic valve stenosis have bicuspid aortic valve disease. A more rare complication of bicuspid aortic valve disease is aortic regurgitation. 15% of all cases https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 15/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology of aortic regurgitation in the Euro Heart survey had bicuspid aortic valve disease. On auscultation, an ejection sound can be audible, best heard at the apex. There may be associated murmurs of aortic stenosis, incompetence, or coarctation of the aorta when these lesions are present. Diagnostic options Echocardiography Echocardiography is used to confirm the diagnosis of bicuspid aortic valve disease. Reported sensitivities and specificities of echocardiography for detecting BAV anatomy are 92% and 96% respectively. To establish the diagnosis, visualization of the aortic valve in systole in the short-axis view is essential. During diastole, the raphe can make the valve appear trileaflet. In the long-axis view, the valve often has an eccentric closure line and there is doming of the leaflets. Transesophageal echocardiography may improve visualization of the leaflets in case of inconclusive transthoracic echocardiography. In all patients, serial transthoracic echocardiography should be performed to evaluate the valve and disease progression. Annual cardiac imaging is recommended for patients with significant valve lesions or with aortic root diameters >40 mm. Complete imaging of the thoracic aorta should be performed periodically for surveillance.[34] Cardiac Magnetic Resonance Imaging and Computed Tomography The thoracic aorta is visualized by alternative imaging modalities such as cardiac magnetic resonance imaging (MRI) or computer tomography (CT). Both cardiac MRI and CT images can help to confirm the bicuspid anatomy of the aortic valve. Treatment Medical treatment In patients with bicuspid aortic valve disease, high blood pressure should be treated aggressively. The ACC/AHA guidelines for the management of adult congenital heart disease and guidelines for the management of patients with valvular heart disease suggest that it is reasonable to use beta-blockers in this population (Class IIa recommendation).[35] This is in accordance with the standard of care at many centers to slow the progression in Marfan-associated aortopathy. Surgery Indications for surgery are similar to indications for patients with degenerative aortic valve disease ; intervention is indicated for severe valvular dysfunction, symptomatic patients, and patients with evidence of abnormal left ventricular dimensions and function. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 16/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology In children and young adults, the bicuspid valve is not calcified and balloon valvuloplasty is recommended. A prosthetic valve implantation would be suboptimal due to the continuing growth of the child. Indications for valvuloplasty in children include peak-to peak gradients >50 mm Hg with ST- or T-wave changes at rest or with exercise. Valvuloplasty is also indicated for symptomatic children with peak-to- peak gradients >60 mm Hg. [35] Surgical options for adult bicuspic aortic valve disease include valve replacement (bioprosthetic or mechanical valves), Ross procedure or valve repair (for those with aortic incompetence) Surgical aortic valve replacement is the most common procedure in adults with bicuspid aortic valve disease, for either aortic valve stenosis or regurgitation. Indications of interventions are similar to those described for tricuspid aortic valve disease in the ACC/AHA guidelines for the management of patients with valvular heart disease.[33] Approximately 30% of adults with bicuspid aortic valve disease undergoing aortic valve replacement will need aortic root surgery. Surgical attention for dimensions of the aortic root is essential because of the risk for further root dilation. The ascending aorta in patients with bicuspid aortic valve disease increases 0.2 to 1.2 mm/year.[36] Guidelines suggest that changes in root size more than 0.5 cm/year are an indication for root replacement. Aortic root dimensions of 5.0 cm require intervention and aortic root dimensions of 4.5 cm require intervention if surgery is performed for valvular indications according to current guidelines. Prognosis Life expectancy in adult patients with bicuspid aortic valve disease is not shortened when compared to the general population. 10-year survival in asymptomatic adults with bicuspid aortic valve disease with a spectrum of valve function, was 96%.[32] In asymptomatic adults with bicuspid aortic valve disease without significant valve dysfunction the 20-year survival was 90%.[37] Aortic Regurgitation A variety of aetiologies can cause aortic regurgitation by preventing proper coaptation of the aortic valve leaflets with a subsequent diastolic reflux of blood from the aorta into the left ventricle. Etiology of aortic regurgitation can be primary valvular, or it can be primarily caused by aortic root or disease. The origin of primary valve disease may be calcific aortic disease, idiopathic degenerative disease, endocarditis, rheumatic disease, a biscuspid aortic valve, or myxomatous proliferation of valvular tissue. In the majority of patients the disease is caused by rheumatic disease. However, in Western countries the disease is most often of degenerative origin. In the Euro Heart Survey degenerative aortic regurgitation accounted for approximately half of the cases of aortic regurgitation, 15% of cases had a bicuspid aortic valve.[11] Accelerated degeneration of valve leaflets, resulting in regurgitation similar carcinoid syndrome related regurgitation, can be caused by certain anorectic medications, such as fenfluramine and phentermine.[22] Aortic annulus dilation, without primary involvement of the leaflets may result in aortic regurgitation due to leaflet separation. Aortic regurgitation of primary aortic root or annulus aetiology includes idiopathic aortic root dilatation, aortic dissection, trauma, and chronic severe systemic hypertension. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 17/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Aortitis represents less than 5% of the aetiologies of aortic regurgitation and may be due to inflammatory disease, such as giant cell, Takayasu and Behcet syndrome. Syphilis and ankylosing spondylitis may affect the aortic valve, but may also be associated with aortic dilatation. Other systemic arteritides and connective tissue disorders such as Marfan syndrome, Reiter disease, Ehlers-Danlos syndrome, osteogenesis imperfecta, and rheumatoid arthritis can lead to annular dilatation and valvular insufficiency. In patients without generalized tissue disease the same pattern of ascending aortic enlargement is known as annuloaortic ectasia. Chronic aortic regurgitation itself may lead to progressive aortic root dilatation. Aortic valve regurgitation is often accompanied by other valvular abnormalities. Aortic valvular insufficiency is most commonly seen in combination with aortic stenosis. Aortic insufficiency due to rheumatic aetiology is often associated with mitral valve disease. The valve leaflets are retracted by fused commissures and by fibrotic scarring of the leaflets itself. Aortic cusp prolapse can be isolated, or due to myxomatous degeneration, sometimes with associated mitral or tricuspid valve involvement. In 15% of patients with ventricular septum defect, prolapse of an aortic cusp leads to aortic insufficiency. Isolated aortic regurgitation is often caused by a primary aortic annular etiology. Rheumatic origin is much less common in patients with pure aortic regurgitation. In approximately 10% of cases, aortic regurgitation results from infective endocarditis, with perforation or erosion of leaflets. It is of considerable clinical importance to distinguish between acute aortic regurgitation and chronic regurgitation since acute aortic regurgitation can be life-threatening if not treated immediately, in contrast to chronic regurgitation which can be tolerated for years. Pathophysiology In patients with aortic insufficiency the regurgitating volume increases the total stroke volume. This volume might equal the effective forward stroke volume in patients with severe aortic regurgitation. In chronic aortic regurgitation, several compensatory mechanisms ensure cardiac output. Acute aortic regurgitation Acute aortic regurgitation is a sudden hemodynamically significant aortic incompetence, which can often be catastrophic. An increase in left ventricular end diastolic volume with absence of ventricular remodelling may lead to elevated left atrial and pulmonary artery wedge pressure and decreased effective cardiac output, with compensatory tachycardia to maintain sufficient output. Acute severe aortic regurgitation typically occurs with infective endocarditis, trauma, and aortic dissection. The left ventricle cannot dilate sufficiently. The patient may present with chest pain due to increased myocardical oxygen consumption combined with decreased coronary bloodflow from changes in diastolic perfusion. Coronary bloodflow in acute aortic insufficiency occurs during stystolic cardiac phase. Other symptoms of acute aortic regurgitation are tachypnea, tachycardia and rapidly progressive pulmonary edema and/or cardiogenic shock. Chronic Aortic regurgitation https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 18/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Aortic root dilatation, annular dilation and congenital bicuspid valve are, in developed countries, the most common causes of severe chronic aortic valve regurgitation. The slow process of chronic aortic regurgitation allows adaptation of the ventricle to the increased preload and afterload. The left ventricular compensates to the regurgitant flow, the increased volume and pressure by enlargement. The left ventricle end diastolic pressure remains relatively low and does not approach the aortic diastolic pressure. The additional stroke volume is responsible for increased systolic pressure and eventually the wide pulse pressure. The systolic hypertension further increases left ventricle afterload. In contrast to the compensatory mechanism in mitral valve regurgitation, a modest concentric left ventricular hypertrophy accompanies the eccentric hypertrophy, with a normal mass-to-volume ratio. [38] In a chronic state, progressive left ventricle dilatation leads to pre- and afterload mismatch. With gradually decompensation and deterioration of systolic function, the ventricle is not able to sustain perfusion. Causes of chronic aortic regurgitation Aortic root/annular dilation Congenital bicuspid valve Previous infective endocarditis Rheumatic In association with other diseases Clinical presentation Patients with aortic regurgitation typically present with symptoms of left sided heart failure including dyspnea on exertion, orthopnea, fatigue, and occasionally paroxysmal nocturnal dyspnea. Angina is less common in patients with aortic regurgitation compared to aortic stenosis. The reduced aortic diastolic blood pressure reduces the coronary blood flow, resulting in angina. The same mechanism is presumed to cause syncope. Diagnostics Chest Radiography Chest Radiography in acute aortic regurgitation reveals minimal cardiac enlargement, with normal aortic root and arch. In acute aortic regurgitation, signs of left heart failure are frequent. Cardiomegaly with left ventricular enlargement is the main feature on chest radiography in chronic aorta regurgitation. The ascending aorta may be enlarged in case of an aortic aneurysm or aortic dissection but Chest X-ray is not a sensitive examination to detect ascending aortic aneurysm. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 19/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Pulmonary congestion is noted at advanced stages of chronic AR, when heart failure has developed. Electrocardiography Electrocardiography in patients with aortic regurgitation may be normal early in the disease. Left ventricular hypertrophy is the main feature of aortic regurgitation, with or without associated repolarization abnormalities. Left axis deviation may be present. Echocardiography Echocardiography is used to evaluate the anatomy of the aortic valve and other valves, as well as aortic leaflets and the aortic root. The regurgitation mechanism and aetiology can be assessed. Three types of mechanisms can be identified; enlargement of aortic root with normal cusps, cusp prolapse or fenestration and poor cusp tissue quality or quantity. Doppler is used for quantifying the aortic regurgitation by the width of regurgitant jet and its extension into the LV, the rate of decline of aortic regurgitant flow and diastolic flow reversal in the descending aorta. Severe aortic regurgitation is defined as effective regurgitant orifice (ERO) area of >0.30cm2, regurgitant volume >60mL, or a regurgitant fraction of >50% Preoperatively transoesophageal echocardiography is performed to more accurately evaluate the anatomy and mechanism of the aortic valve regurgitation Treatment The only direct method to reduce aortic regurgitation is surgical treatment. However, some patients may benefit from medical treatment. Medical treatment The relative reduction of myocardial blood supply due to increased demand and/or associated obstructive coronary artery disease may cause angina. Angina may be treated by reducing aortic regurgitation, reduction of myocardial demand of revascularization of the myocardium. Clinical heart failure is treated with traditional therapy, including digitalis, diuretics, and ACEI. In severe heart failure, parenteral inotropic and vasodilator therapy may be needed. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 20/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Current Guidelines: Medical treatment of Aortic Regurgitation Class I Vasodilator therapy is indicated for chronic therapy in patients with severe AR who have symptoms or LV dysfunction when surgery is not recommended because of additional cardiac or noncardiac factors. (Level of Evidence: B) Class IIa the Vasodilator hemodynamic profile of patients with severe heart failure symptoms and severe LV dysfunction before proceeding with AVR. (Level of Evidence: C) therapy is reasonable for short-term therapy to improve Class IIb Vasodilator therapy may be considered for long-term therapy in asymptomatic patients with severe AR who have LV dilatation but normal systolic function. (Level of Evidence: B) Class III 1. Vasodilator therapy is not indicated for long-term therapy in asymptomatic patients with mild to moderate AR and normal LV systolic function. (Level of Evidence: B) 2. Vasodilator therapy is not indicated for long-term therapy in asymptomatic patients with LV systolic dysfunction who are otherwise candidates for AVR. (Level of Evidence: C) 3. Vasodilator therapy is not indicated for long-term therapy in symptomatic patients with either normal LV function or mild to moderate LV systolic dysfunction who are otherwise candidates for AVR. (Level of Evidence: C) Surgical treatment Surgical treatment in case of isolated Aortic valve regurgitation is aortic valve replacement. Aortic valve replacement is indicated in all symptomatic patients with severe Aortic regurgitation and in all asymptomatic patients with severe Aortic regurgitation with left ventricular ejection fraction (LVEF) <50% or left ventricular dilatation (end-diastolic diameter >75 mm or end-systolic diameter >55 mm). When an aneurysm of the aortic root is associated with severe aortic regurgitation a Bentall procedure can be indicated. The ascending aorta is replacement by a composite graft comprising an aortic prosthesis, with re-implantation of the coronary arteries. When aortic enlargement is localized in the supra-coronary part of ascending aorta, replacing the supra-coronary section of the ascending aorta may be sufficient which is considered technically easier. Although the prosthetic valve replacement remains the standard for aortic valve regurgitation, aortic valve repair procedures are performed with a combination of different surgical techniques. The quality of the cusps is essential for repair. The annulus and sinotubular junction can be surgically readapted to the cusps, eliminating the regurgitation. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 21/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Current Guidelines Class I 1. An adolescent or young adult with chronic severe AR* with onset of symptoms of angina, syncope, or dyspnea on exertion should receive aortic valve repair or replacement. (Level of Evidence: C) 2. Asymptomatic adolescent or young adult patients with chronic severe AR* with LV systolic dysfunction (ejection fraction less than 0.50) on serial studies 1 to 3 months apart should receive aortic valve repair or replacement. (Level of Evidence: C) 3. Asymptomatic adolescent or young adult patients with chronic severe AR* with progressive LV enlargement (end-diastolic dimension greater than 4 standard deviations above normal) should receive aortic valve repair or replacement. (Level of Evidence: C) 4. Coronary angiography is recommended before AVR in adolescent or young adult patients with AR in whom a pulmonary autograft (Ross operation) is contemplated when the origin of the coronary arteries has not been identified by noninvasive techniques. (Level of Evidence: C) Class IIb 1. An asymptomatic adolescent with chronic severe AR* with moderate AS (peak LV to peak aortic gradient greater than 40 mm Hg at cardiac catheterization) may be considered for aortic valve repair or replacement. (Level of Evidence: C) 2. An asymptomatic adolescent with chronic severe AR* with onset of ST depression or T-wave inversion over the left precordium on ECG at rest may be considered for aortic valve repair or replacement. (Level of Evidence: C) Mitral Stenosis Etiology and pathology The leading cause of mitral stenosis is rheumatic fever, causing postrheumatic deformities. Other rare aetiologies of left atrial outflow obstruction include congenital and degenerative mitral valve stenosis, severe mitral annular and/or leaflet calcification carcinoid disease, neoplasm, left atrial thrombus, infective endocarditis with large vegetations, certain inherited metabolic disorders such as Fabry s disease, mucopolysaccharidosis, Whipple s disease, gout, rheumatic arthritis, lupus erythematosus, methysergide therapy, and cases related to previous implanted prosthesis or commisurotomy. Rheumatic valvular disease causes diffuse thickening of the valve leaflets by fibrous, or fibrocalcific distortion, with fusion of one or more commisures valve commisures, and fusion and shortening of the subvalvular apparatus. This combined with increasingly rigid cusps results in narrowing of the valve. The area of the normal mitral valve orifice is 4-6 cm2. In patients with mitral stenosis, when the valve area approaches 2 cm2 or less, an early, mid and late diastolic transvalvular gradient is present between the left atrium and ventricle. With progressive mitral stenosis, transvalvular pressure gradient increases. Mitral transvalvular flow depends on cardiac output and heart rate. Shortening of diastolic phase in increased heart rate causes symptoms by reducing forward cardiac output. Mitral stenosis develops gradually, and may be asymptomatic for years. Clinical presentation https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 22/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Patients with mitral stenosis may be asymptomatic for years. Mean age of presentation of symptoms is fifty to sixty years old. The presenting symptom in patients with mild mitral stenosis is typically dyspnea precipitated by stress or atrial fibrillation. Progression of disease with increasing left atrial and pulmonary venous pressures will cause progressive dyspnea. At advanced stage, patients are often thin and frail and complain of weakness and fatigue due to low cardiac output. When pulmonary hypertension and right ventricular failure develop, signs of tricuspid regurgitation, abdominal discomfort due to hepatomegaly and ascites can be present. On physical examination, heart size is usually normal. An apical diastolic thrill can be palpated. A holodiastolic murmur can be auscultated at the apex with a presystolic accentuation in sinus rhythm. The intensity of this murmur is determined by the transmitral gradient. In patients with severe stenosis, calcified leaflets, or low cardiac output no diastolic murmur may be audible. An opining snap may be present due to sudden tensing of the pliable leaflets during opening. S1 may be loud when the mitral leaflets are pliable. Thickening and calcification will diminish S1 in more advanced stages. With pulmonary hypertension, S2 becomes prominent and a murmur of tricuspid regurgitation located at the xyphoid can be present. In severe mitral valve stenosis with pulmonary hypertension, pulmonary rales are audible. Intermittent malar flushes, jugular distension, and peripheral cyanosis may be present Diagnostics Chest Radiography Signs of left atrial enlargement are often the earliest changes on chest radiography. Straightening of the left heart border by prominent pulmonary arteries coupled with left atrial enlargement can be observed as well as a double contour of the left atrium and elevation of the left main stem bronchus. Distension of the pulmonary arteries and veins in the upper lung fields and pleural effusions indicate elevated pulmonary pressures. Kerley B lines may be observed in severe mitral stenosis. Electrocardiography Electrocardiography is in many cases normal and cannot asses the severity of mitral stenosis accurately. Atrial arrhythmias are more common in patients with advanced mitral stenosis. In sinus rythm signs of left atrial enlargement may be present with a prolonged P wave and a negative deflection in lead V1 and left axial deviation of P wave. Signs of right ventricular hypertrophy with right-axis deviation, a tall R wave in V1, and secondary ST-T-wave changes may be present in cases of severe pulmonary hypertension Echocardiography The primary diagnostic method for assessing mitral valve pathology and pathophysiology is echocardiography. The severity and consequences of the mitral stenosis can be evaluated. The valvular anatomy and morphology including valve thickening, mobility, calcification, subvalvular deformity and anatomic lesions can be assessed Rheumatic mitral stenosis can cause reduced diastolic excursion of the leaflets and thickening or calcification of the valvular and subvalvular apparatus. Doppler is used to determine the peak and mean transvalvular pressure gradients. The valve area can be measured accurately by three-dimensional https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 23/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology echocardiogram. Transesophageal echocardiography is able to provide more detailed information of the mitral valvular pathology than transthoracic echocardiography. Cardiac Catheterization Cardiac catheterization can provide information regarding coronary artery status. This diagnostic tool is not necessary to establish the diagnosis of mitral stenosis. Prognosis The progression of mitral stenosis ranges from 0.1 0.3cm2/year.[39] Survival rates of 80% at 10 years have been reported with asymptomatic mitral valve stenosis. Once symptoms develop related to pulmonary hypertension 10-year survival is 0 15%. Progressive heart failure is the most common cause death in the untreated patients with mitral stenosis. Treatment Medical treatment No medical treatments will relief mitral stenosis. Dyspnea may be transiently relieved by diuretics or long acting nitrates. In order to slow the heart rate, Beta-blockers or calcium-channel blockers can be prescribed. Patients with atrial fibrillation should be on anticoagulants with a target INR of 2-3. In patients with severe mitral stenosis, cardioversion should not be performed prior to intervention since it does not durably restore sinus rhythm. After successful intervention cardioversion is indicated if atrial fibrillation is of recent onset and the left atrium only moderately enlarged. Percutaneous mitral commissurotomy Mitral stenosis can be relieved by percutaneous mitral commisurotomy. This procedure was introduced in 1980 and used worldwide ever since [40][41]. The results of balloon valvotomy are comparable to open commissurotomy.[42] The commissural splitting of percutaneous mitral commissurotomy substantially increases the valvular area. Pulmonary pressures decrease immediately. Procedural mortality has been reported 0 3%. In 2- 10% leaflet tearing results in severe mitral regurgitation. Preprocedural condition of the patients and the experience of the operator and operating team are of major influence on the complication rate of percutaneous mitral commissurotomy. Longterm results of a successful procedure have been reported with event-free survival ranges from 35 70% after 10 15 years. Surgical intervention Open commissurotomy or valve replacement is indicated if balloon valvotomy is unfeasible. The concept of surgical repair of the mitral valve was first introduced in 1902 by Sir Thomas Lauder Brunton, a Scottish physician.( Brunton L, Edin MD: Preliminary note on the possibility of treating mitral stenosis by surgical methods. Lancet 1902; 1:352[43]). The first successful mitral valve operation https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 24/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology was a transventricular commissurotomy by Elliot Cutler in 1923. The following transventricular valvultomies performed with the cardiovalvulotome resulted in regurgitation and Cutler abandoned the procedure. In 1948, Dwight Harken reported his series of patients with mitral stenosis successfully treated with a valvuloplasty procedure.[44] After development of cardiopulmonary bypass, the closed commissurotomy has been replaced by an open mitral commissurotomy. This allows not only correction of commissural fusion, but also chordal and papillary fusion. Surgical intervention can improve the functional capacity and long-term survival of patients with mitral stenosis substantially. Survival rates of 96% and freedom from valve-related complications of 92% at 15 years have been reported.[45] Surgery should be performed before New York Heart Association (NYHA) class III symptoms are present. Mitral regurgitation Mitral valve regurgitation results from inadequate mitral leaflet coaptation during systole. This allows the systolic regurgitation of blood from the high-pressure LV to the normally low-pressure LA. The regurgitating volume depends on both the size of the regurgitant orifice and the pressure gradient between the left ventricle and the left atrium. In primary mitral regurgitation, inadequate mitral leaflet coaptation results from an abnormality in any of the functional components of the mitral apparatus. Secondary or functional mitral regurgitation results from left ventricle disease and remodeling. During systole, combined papillary muscle contraction and contraction of the dynamic annulus promote leaflet coaptation. Calcification of the annulus may hinder the sphincter-like contraction of the annulus allowing regurgitation. Secondary mitral regurgitation due to annulus dilation may be caused by ischemic or dilated cardiomyopathy. The regurgitant volume causes left ventricular enlargement and contractile dysfunction. Left ventricle dilation may cause enlargement of the mitral annulus and the regurgitant orifice, increasing the mitral regurgitation. Positive inotropes, diuretics and vasodilators reduce the size of the left ventricle and the regurgitant orifice, and decrease the regurgitant flow. Etiology Three different types of primary mitral regurgitation can be defined; leaflet retraction from fibrosis and calcification, annular dilatation and chordal abnormalities (including rupture, elongation, or shortening). Functional mitral regurgitation results from LV dysfunction with or without annular dilation. Mitral regurgitation was classified by Carpentier into three types based on leaflet and chordal motion: normal leaflet motion (type I), leaflet prolapse or excessive motion (type II), and restricted leaflet motion (type III).[46] Chronic mitral valve regurgitation Degenerative mitral valve disease is the most common cause of mitral regurgitation in Europe. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 25/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Myxomatous mitral valve degeneration is also known as floppy mitral valve or mitral valve prolapse. Prolapse is defined as excursion of one or both leaflets above the plane of the annulus during systole.[47] Prolapse of the middle portion of the posterior leaflet is the most common finding in degenerative MR. [48] Mitral regurgitation in Barlow syndrome or parachute mitral valve is due to annular dilatation and extensive hooding of leaflets with large amounts of excessive leaflet tissue. In mitral regurgitation, the mass-to-volume ratio of the enlarged, thin walled left ventricle is less than one.[38] Clinical Presentation Patients with mild to moderate compensated chronic mitral regurgitation may remain asymptomatic for many years. The adapted left ventricle maintains normal forward cardiac output. The left ventricle ejection fraction in chronic mitral regurgitation may be greater than normal due to the compensatory cardiac adaptations. Progression of severity depends on etiology of regurgitation; in patients with connective tissue disease regurgitation tends to progress more rapidly than patients with mitral valve prolapse or rheumatic mitral regurgitation. Progression in acute rheumatic fever is often rapid. Acute progression may by caused by endocarditis or chordae rupture. Gradual progression and eventually decompensation results in decreased cardiac output with physical activity and pulmonary congestion. Patients present with weakness, fatigue, palpitations, dyspnea on exertion. Hepatomegaly, peripheral edema and ascites due to right sided heart failure can be associated with rapid clinical deterioration. Acute mitral regurgitation is associated with sudden pulmonary congestion and edema. On physical examination the apical impulse is displaced laterally, indicating left ventricular enlargement. S1 is normal or diminished. S2 may be single, closely split, normally split, or even widely split as a consequence of the reduced resistance to LV ejection. A widely split S2 is often audible, due to shortening of LV systole and early closure of the aortic valve. The P2 component of the second heart sound may be increased if pulmonary hypertension has developed. The apical systolic murmur is typically holosystolic and radiates to the axilla, depending on the direction of the regurgitant jet. It can be blowing, moderately harsh, or even soft. An S3 gallop often is present, reflecting the transmitral diastolic flow during the rapid filling phase. Early in the disease process of patients with Barlow syndrome, a characteristic midsystolic click can be appreciated, followed by a late systolic murmur; with disease progression the murmur becomes holosystolic, and the midsystolic click may become inaudible.[46] Acute mitral regurgitation Immediate intervention is often necessary in acute mitral regurgitation. Etiology can be organic or functional. Organic causes include rupture of a major chorda tendinea (in myxomatous mitral valve disease) or papillary muscle (due to myocardial infarction), leaflet perforation (of endocarditic origin), and dysfunction of a prosthetic valve due to endocarditis or paravalvular regurgitation. Regurgitation of functional etiology results from left ventricular abnormalities such as dyskinetic wall due to ischemia or dilated ventricle due to cardiomyopathy. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 26/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Acute mitral regurgitation is associated with dyspnea and orthopnea, caused by sudden pulmonary congestion and edema. Acute papillary muscle rupture may mimic the presentation of a patient with a postinfarction ventricular septal defect.[49] On physical examination no signs of cardiac compensatory mechanisms are present. The increase in left atrial pressure diminishes the pressure gradient between the left ventricle and left atrium by midsystole. The murmur of mitral regurgitation is shortened and of decreased intensity, it may be soft, short of even inaudible. An S3 gallop usually is present. The volume overload is increasing the severity of MR over time, and leads to a greater percentage of the LV stroke volume being ejected in a retrograde fashion. Diagnostic Options Chest Radiography In chronic mitral regurgitation chest radiography demonstrates enlargement of the left ventricle and atrium. The mitral annulus may be calcified. In acute mitral regurgitation, no adaptive left atrium or ventricle enlargement has developed. Signs of interstitial or alveolar pulmonary edema may be present. Electrocardiography Signs of left ventricular hypertrophy and left atrial enlargement due to chronic volume overload may be seen on the electrocardiogram. Atrial fibrillation is a common finding late in the natural history of the disease. Q-waves may be seen in ischemic mitral regurgitation. In patients with acute mitral regurgitation, left atrial and ventricular enlargement may not be evident and the electrocardiogram may be normal or show only nonspecific findings. The resting electrocardiogram of patients with asymptomatic mitral valve prolaps is normal. A variety of ST-T-wave changes, including T-wave inversion and sometimes ST-segment depression, particularly in the inferior leads, can be found in patients with symptomatic mitral valve prolapse. Echocardiography Mitral valve pathology and pathophysiology is primary assessed by echocardiography. Left atrial and ventricular dimensions can be quantified. The etiology and mechanism of mitral regurgitation can be identified by echocardiography. Leaflet abnormalities and chordal morphology and function is assessed by echocardiography. Fused subvalvular apparatus due to rheumatic valvulitis and leaflet destruction due to endocarditis can be visualized by echocardiography. Myxoid degeneration of the mitral valve is characterized by an excess of tissue and by leaflets of more than 5mm thickness. Annular dilatation is characterized by a ratio of >1.3 anterior posterior diameter of the annulus to the length of the anterior leaflet in diastole. The regurgitation caused by annular dilatation and incomplete leaflet coaptation is directed straight back into the left atrium. In ischaemic MR, the apical displacement of the leaflets can be quantified by measuring the tenting area and the leaflet opening angles. https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 27/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology Color doppler analysis can be used to grade the severity of the regurgitation and permits visualization of the origin, extent, direction, duration, and velocity of disturbed backward flow of the regurgitant leak or leaks into the left atrium.[46] MR is considered severe when the jet area is >10cm2 or >40% of the left atrial area. Cardiac Catheterization Coronary ischemic causes of mitral regurgitation can be identified by cardiac catheterization. Treatment Surgical The hemodynamic overload on the heart caused by mitral regurgitation can ultimately only be corrected by surgically restoring valve competence. For all valve surgery timing of surgery is essential. Irreversible left ventricular dysfunction will result in suboptimal results indelayed surgery. Due to the operative risk and risk of valve prosthesis surgery should however be delayed as long as possible Mitral valve regurgitation is surgically corrected by mitral valve replacement or repair. Mitral valve repair is generally found to be superior to replacement, with preservation of left ventricular function and part of the mitral valve apparatus [50] [51] [52] [53] and without the use of a prosthesis. In mitral valve regurgitation indication for valve surgery is influenced by symptomatic status, ventricular functional status, and the procedure to be performed. Repair might be considered in asymptomatic patients with normal left ventricular function or patients with severe impairment of left ventricular function who might not be candidates for mitral valve replacement. For most patients, mitral valve surgery is performed for the relief of symptoms or to prevent worsening of asymptomatic left ventricular dysfunction. Tricuspid stenosis Tricuspid stenosis (TS) is most commonly of rheumatic origin and combined with tricuspid regurgitation. The anatomical characteristics are similar to those of mitral stenosis, including fibrous leaflet thickening and fusion and shortening of the subvalvular apparatus. The preponderance of cases is in young women. Other aetiologies of right atrial obstruction are rare and include congenital tricuspid atresia, right atrial tumors and carcinoid syndrome The normal valve area of the tricuspid valve is 7 8cm2. Reduction of valve area to <2 cm2 causes a pressure gradient. A small diastolic pressure gradient (<5 mmHg), gradient between the right atrium and ventricle can be present due to tricuspid stenosis. The gradient is increasing on inspiration. A mean pressure gradient >5mmHg is considered indicative of significant TS and is usually associated with symptoms Clinical presentation https://www.textbookofcardiology.org/wiki/Valvular_Heart_Disease 28/39 7/4/23, 12:36 AM Valvular Heart Disease - Textbook of Cardiology A tricuspid opening snap and a characteristic mid-diastolic murmur may be audible along the left sternoid border on auscultation. Carvallo s sign, an increase of murmur intensity on inspiration, may be present. Distention of jugular veins, ascites, pleural effusion and peripheral edema may be present due to increased right atrial pressures. Reduced cardiac output causes symptoms of fatigue and malaise. The pulmonary congestion of mitral stenosis may be masked in severe tricuspid stenosis. Diagnostic Options Chest radiography Cardiomegaly with an increase in right atria and pulmonary artery size is demonstrated on chest radiography. Electrocardiography An increased P-wave amplitude is seen on the electrocardiogram if the patient is in normal sinus rhythm. Echocardiography