ACQUIRED VALVULAR HEART DISEASESDefinition, pathophysiology, clinical condition, treatment, prevention

Schematic representation of transthoracic and transesophageal echocardiographic views

Apical four-chamber view recorded in systole in a normal patient

Schematic representation of mitral valve inflow

Transmitral Doppler tracings

Mitral Valve Disease Diseases directly affecting the

mitral apparatus   Rheumatic heart disease   Congenital mitral stenosis   Congenital cleft mitral valve   Infectious endocarditis   Marantic endocarditis   Libman-Sacks endocarditis   Hypereosinophilic heart disease   Coronary artery disease   Diet drug valvulopathy   Mitral anular calcification   Degenerative   Infiltrative   Carcinoid

Indirect effect on mitral valve function   Dilated cardiomyopathy   Hypertrophic cardiomyopathy   Left atrial myxoma   Myocardial ischemia or infarction

The pathophysiologic consequences of acute, severe MR such as that observed in patients following rupture of a papillary muscle differ from those of chronic MR.

With acute MR, a sudden volume overload is imposed on the nondilated, nonhypertrophied left ventricle and left atrium causing a sudden increase in left atrial pressure.

Preload is increased, whereas afterload decreases as a result of the newly developed low-pressure runoff to the left atrium during systole

Overall LV pump function declines in acute MR. The clinical impact of acute MR is largely determined by the

compliance of the left atrium

Acute Mitral Regurgitation.Pathophysiology

The main etiology factors of the acute regurgitant lesion onset Structural

Trauma Dysfunction or rupture of a papillary muscle (ischemic heart disease) Prosthetic valve malfunction with paravalvular leak or leaflet

malfunction or disruption

Infectious Infectious endocarditis Acute rheumatic fever

Degenerative Myxomatous degeneration with chordal rupture

Acute Mitral RegurgitationDiagnosis Clinical condition

Acute MR is usually associated with the sudden development of severe LV failure.

Patients are often in pulmonary edema when initially seen, and cardiogenic shock is common.

Severe acute MR secondary to acute inferoposterior MI is seen more often in women

The murmur is usually loud if normal LV function is present. The murmur may be soft if LV function is markedly reduced

Transesophageal and transthoracic echocardiography Right heart catheterization (measurement of right-sided and

capillary wedge pressures, as well as cardiac index)

Acute Mitral Regurgitation. Transesophageal echocardiogram

Treatment The nitroprusside infusion is continued until the patient is

stabilized by either surgical intervention or long-term oral medical therapy (e.g., ACE inhibitors, digoxin, and diuretics)

IV nitroglycerin is often effective in reducing pulmonary vascular congestion in these patients, particularly if the underlying etiology for MR is ischemic heart disease.

Surgical therapy is almost always required in patients with acute severe MR.

Intraaortic balloon counterpulsation can be helpful in stabilizing the patient before definitive therapy is instituted

Chronic Mitral Regurgitation The vast majority of patients present with

chronic MR at the time of diagnosis with LV and left atrial adaptation to volume overload.

The causes of Chronic Mitral Regurgitation

Structural   Ruptured chordae tendineae (acute MR that can become chronic)   Dysfunction of a papillary muscle: ischemia (acute MR that can become chronic)   Dilatation of mitral valve annulus secondary to LV dilatation (cardiomyopathy)   Hypertrophic cardiomyopathy   Paravalvular prosthetic valve leak

Congenital   Cleft mitral valve (associated with other congenital heart diseases [e.g., primum atrial septal defect])   Parachute mitral valve (associated with other congenital heart diseases [e.g., endocardial cushion defect, endocardial fibroelastosis, transposition of the great arteries])

Inflammatory   Rheumatic heart disease   Systemic lupus erythematosus   Progressive systemic sclerosis   Methysergide therapy

Degenerative   Myxomatous degeneration of the valve   Calcification of the mitral valve annulus   Marfan syndrome   Ehlers-Danlos syndrome   Pseudoxanthoma elasticum   Ankylosing spondylitis   Infiltrative amyloidosis

Infectious   Infectious endocarditis

Pathophysiology of Chronic Mitral Regurgitation MR causes LV and left atrial volume overload. The degree of volume overload depends on the regurgitant volume Initially, as the severity of MR increases there is progressive enlargement

of the left atrium and left ventricle to compensate for the regurgitant volume.

LV dilatation occurs as a result of remodeling of the extracellular matrix with rearrangement of myocardial fibers, in association with the addition of new sarcomeres in series and the development of eccentric LV hypertrophy

In patients with severe, chronic MR, the left atrium is markedly dilated and atrial compliance is increased.

There is fibrosis of the atrial wall, left atrial pressure can be normal or only slightly elevated

Diagnosis Echocardiography is the main diagnostic tool in patients with

MR The complete mitral valve apparatus must be interrogated by

two-dimensional echo and Doppler. Transesophageal echocardiography offers a better definition

of the anatomy, and is indicated in cases with suboptimal transthoracic windows or unclear etiology, mechanism, or severity of the MR

Four imaging planes to assess the location of prolapsed or flail segments

Treatment All patients with MR should receive antibiotic prophylaxis before dental and other

invasive procedures according to American Heart Association/American College of Cardiology guidelines. Without antibiotic prophylaxis patients with mitral valve prolapse have a three- to eightfold higher risk of developing infective endocarditis

Optimal blood pressure control is important Restriction of physical activity is recommended only in symptomatic patients and in

those with LV dysfunction Management of underlying disease is fundamental in patients with ischemic heart

disease or infective endocarditis The most important goals of surgical intervention are improvement of symptoms,

preservation of LV function, and increased longevity Other considerations include preservation of the native mitral valve apparatus,

avoidance of chronic anticoagulation, maintenance and/or restoration of sinus rhythm, and prevention/improvement of pulmonary hypertension and right ventricular dysfunction.

Degenerative Mitral Valve Disease Degenerative mitral valve disease includes

myxomatous mitral disease, mitral leaflet flail or prolapse, and Barlow syndrome

The Framingham Heart Study employed strict echocardiographic criteria to determine the prevalence of mitral valve prolapse.

They noted a prevalence of 2.4% in their population; 60% of the patients with mitral valve prolapse were women

Pathology Pathologic changes associated with degenerative mitral valve disease may

include annular dilatation, leaflet thickening, myxoid degeneration, chordal elongation, chordal rupture, and annular and leaflet calcification

There are lower collagen concentrations in the leaflets compared with normals.

The biochemical effects are more pronounced in chordae than in leaflets. Disruption of the collagen bundles may explain why degenerative leaflets

and chordae exhibit enhanced extensibility and decreased stiffness compared to normal valves.

Chordal rupture is one of the causes of severe MR in degenerative disease and is secondary to mechanical weakening of the chordae and the abnormal stresses imparted by the redundant leaflet

Mitral valve prolapse Mitral valve prolapse has been associated with a number of different

conditions, including Marfan syndrome, Ehlers-Danlos syndrome, acute rheumatic carditis, and a variety of congenital cardiac anomalies.

A number of these entities are the result of genetic defects in connective tissue structure that produce, among other anatomic abnormalities, mitral valve prolapse.

Although most cases of degenerative mitral valve disease are sporadic, a familial basis for the condition has been recognized.

This form of mitral valve prolapse has an autosomal dominant mode of inheritance with variable penetrance and is influenced by age and gender.

There is marked heterogeneity of clinical presentation

Natural History The clinical spectrum of patients with degenerative valve disease is wide. Two distinct clinical groups appear to exist. One consists of young women with a

midsystolic click and mild echocardiographic prolapse; they often have multiple complaints.

The other group consists of middle-aged men with thickened valves, more severe prolapse, and MR.

The majority of patients with mitral valve prolapse and no or mild MR are asymptomatic.

Many patients have symptoms that seem unrelated to the cardiac pathophysiology: anxiety, easy fatigue, palpitations, and orthostatic hypotension.

Some patients report chest discomfort that, at times, is obviously musculoskeletal in origin and at other times seems anginal in nature

The majority of patients with mitral valve prolapse and no or mild MR have a benign prognosis, but there are a number of complications that have been associated with this entity including arrhythmias, sudden death, cerebrovascular events, and MR.

Diagnosis Physical Examination Echocardiography

(Color Doppler echocardiography)

Multislice CT scanning Coronary angiography

(In patients at high risk for coronary artery disease)

M-mode echocardiograms recorded in two patients with mitral valve prolapse

Treatment Medical Treatment

patients with MR or thickened leaflets should receive antibiotic prophylaxis

The routine use of vasodilators in patients with MR remains controversial

Surgical Treatment Surgery should be considered in all patients with severe

MR caused by degenerative mitral valve disease

Ischemic and Cardiomyopathic Mitral Regurgitation Ischemic and cardiomyopathic MR occurs in patients

with normal mitral leaflets and chordae and no significant intrinsic mitral valve disease.

Ischemic MR occurs as a consequence of coronary artery disease and should be distinguished from coexisting organic mitral valve disease and coronary artery disease

Ischemic MR may be caused by papillary muscle rupture or elongation, which cause leaflet flail or prolapse

Diagnosis Clinical condition

Whereas the patient with a ruptured papillary muscle caused by MI usually presents in extremis with pulmonary edema and shock, most patients with ischemic and cardiomyopathic MR present with symptoms of congestive heart failure.

The degree of congestive heart failure dominates physical exam findings

Echocardiography Magnetic resonance imaging (in patients with functional MR, regional

remodeling with thinning and hypokinesis/akinesis of the inferior wall ) Cardiac Catheterization (if considere revascularization) Ventriculography (in cases where noninvasive determination of the

severity of the MR is inconclusive or inconsistent with the history or clinical findings)

Mitral regurgitation

Apical four-chamber view recorded in a patient with prominent pulmonary vein flow in the left atrium in systole

Spectral Doppler image recorded in a patient with mitral regurgitation using pulsed wave Doppler

Schematic demonstration of the principle involved in calculating mitral regurgitation severity from the proximal isovelocity surface area method.

Treatment Medical treatment

optimal blood pressure control use of vasodilators beta-blockers at maximal tolerated dosages

Surgical Treatment coronary artery bypass grafting (in patients with

mild to moderate ischemic MR ) Annuloplasty (optionally)

Mitral Stenosis Rheumatic cardiac disease is an immunologic phenomenon

that may affect any of the heart valves and the myocardium and is by far the most common cause of mitral stenosis.

Pathologic features of rheumatic mitral valve disease include thickening of the leaflets with fusion of the edges at the commissures.

The initial valvular pathologic lesion in acute rheumatic fever consists of inflammation manifesting as a series of translucent nodules along the line of closure of the mitral valve.

Rarely, mitral stenosis is the result of entities other than rheumatic heart disease

Classification of the Severity of Mitral Stenosis

Natural History The progression of mitral stenosis is generally slow. Recent serial hemodynamic and Doppler

echocardiographic studies have demonstrated annual mitral valve area loss ranging from 0.09 to 0.32 cm2

The rate of progression of mitral valve narrowing is variable among individuals, and difficult to predict based on initial valve area or gradients.

Complications of rheumatic mitral disease are attributable to the abnormal hemodynamic state produced by severe mitral stenosis.

Complications of rheumatic mitral disease

Pulmonary edema Heart failure Arrhytjmia Sudden death Atrial fibrillation (as result of left atrial dilatation and hypertrophy) Systemic embolism (occurs as a result of left atrial thrombosis and fibrillation) Liver cyrrosis due to congestive heart failure Hemoptysis ( as result from rupture of small venules secondary to sudden

increases in pulmonary venous pressure) Ortner syndrome (hoarseness) due to secondary to compression of the left

recurrent laryngeal nerve by a dilated left pulmonary artery, leading to vocal cord paralysis

Dysphagia, resulting from esophageal compression by a dilated left atrium

Diagnosis Physical Examination

characteristic physical findings (Jugular venous distension, the second or third left intercostal space, classic auscultatory findings of mitral stenosis with diastolic rumble etc.)

Electrocardiogram Chest X-Ray Cardiac Catheterization

Parasternal short-axis view recorded in a patient with rheumatic heart disease and mitral stenosis

Rheumatic heart disease and mitral stenosis. Parasternal long-axis view.

Apical four-chamber view recorded in a patient with rheumatic mitral stenosis. Focal calcification of the anterior mitral valve leaflet

Rheumatic mitral stenosis. M-mode

echocardiogram.

Recorded in a patient with severe mitral stenosis

Transmitral Doppler image recorded in a patient with concurrent mitral stenosis and mitral regurgitation

Treatment Relieving the mitral valvular obstruction with balloon valvuloplasty or

surgical intervention is the best treatment for symptomatic mitral stenosis (inc. percutaneous balloon mitral commissurotomy, metallic valvulotome, mitral valve replacement )

Prophylactic antibiotics are administered to patients with mitral stenosis before dental work and other interventions to minimize the risk of developing infective endocarditis

Antiagregants Warfarine Vasodilators Diuretics Antiarrhythmic drug (inc. beta-blockers, amiodarone)

Aortic Stenosis

Etiology The main causes are

senile calcific valve Bicuspid form Rheumatic processes congenital varieties.

The most common cause of adult-acquired aortic stenosis is a chronic inflammatory and fibrotic process of the aortic valve similar to arterial atherosclerosis

The progressive fibrosis and calcification can occur on a bileaflet or a trileaflet valve, it just occurs earlier in life in the bicuspid valve

The rheumatic aortic stenosis occurs rarely today

Timing of onset of symptoms and significantly calcified and stenotic valves versus the major etiologies of AS, including (A) congenital, (B) bicuspid, (C) rheumatic, and (D) senile

CLINICAL CLASSIFICATION OF AORTIC STENOSIS

Rheumatic Valvular Disease (less than 20% of the total)

Radiation-Induced Aortic Stenosis (5%) Congenital Aortic Stenosis (15%) Senile Valvular Disease (59%) Other causes accounted less that 1%

Natural History

Clinical Presentation Asymptomatic (65% patients) Symptomatic (35% patients)

Angina Syncope sudden cardiac death Congestive Heart Failure Gastrointestinal Bleeding (Heyde syndrome) due to

Angiodysplasia or arteriovenous malformations of the intestines

Mean Systolic Pressure Gradient and Orifice Area in Aortic Stenosis

Progression of Aortic Stenosis reduction in aortic orifice area from its normal 3.0 to 4.0 cm2 to

approximately 1.5 cm2 results in only a small gradient. After that degree of stenosis has developed, small additional

reductions in the orifice area lead to progressively more dramatic increases in gradient.

The average increase in gradient is 7 to 10 mm Hg per year but with a large and unpredictable individual variation, as much as 25 mm Hg in 1 year in occasional patients

A rapid rate of progression of stenosis, as reflected by an increase in aortic-jet maximum velocity of more than 0.3 m/second in the course of a year, occurs more often in patients who have clinical cardiac events

Diagnosis Physical Examination (the murmur typically peaks in early to

midsystole and is associated with a systolic thrill, carotid upstroke becomes reduced in amplitude and delayed in timing)

ECG Chest X-Ray Echo- Doppler Stress Testing Cardiac Catheterization Coronary arteriography

Typical ECG from a patient with severe symptomatic AS showing LV hypertrophy

Echocardiographic images (A) and schematics (B) taken from the parasternal short-axis view from a patient with a bicuspid aortic valve in diastole (left) and in systole (right).

Echo-gram in mild aortic stenosis patient

Chest X-ray examination A 17-year-old boy with

congenital aortic valve stenosis. Note dilatation of the ascending aorta, increased convexity of the left ventricle, and normal pulmonary vascularity. The systolic aortic pressure gradient was 100 mmHg.

Transcatheter Aortic Valve Implantation – ТАVIEdwards SAPIEN valve.

Transcatheter Aortic Valve Implantation – ТАVICoreValve ReValving

Chronic Aortic Regurgitation.Etiologies

Common causes of Chronic Aortic Regurgitation

congenital bicuspid aortic valve, rheumatic heart disease, infective endocarditis, all causes of fibrosis and calcification of the

leaflets

Hemodynamics of the clinical stages of aortic regurgitation

(A) Normal conditions.

(B) Severe acute aortic regurgitation, although total stroke volume (SV) is increased, forward SV is reduced. LV end-diastolic pressure (LVEDP) rises dramatically.

(C) Chronic, compensated aortic regurgitation. Eccentric hypertrophy produces increased end-diastolic volume (EDV), which permits an increase in total as well as forward SV. The volume overload is accommodated, and LVEDP is normalized. Ventricular emptying and end-systolic volume (ESV) remain normal.

(D) Chronic, decompensated aortic regurgitation. Impaired LV emptying produces an increase in ESV and a decrease in EF, total SV, and forward SV.

(E) Immediately after AVR, preload estimated by EDV decreases, as does LVEDP. ESV is also decreased, but to a lesser extent, resulting in an initial decrease in EF.

Signs of Aortic insufficiency

Aortic regurgitation in normal and thoracic aortic aneurysms

Diagnosis Physical Examination ECG Chest X-Ray Echo- Doppler Stress Testing Cardiac Catheterization Coronary arteriography

Echo- Doppler in patient with aortic regurgitation

Diseases Causing Acquired Tricuspid Valve Stenosis Rheumatic heart disease (usually with mitral stenosis) Carcinoid heart disease Fabry's disease Whipple's disease Endocardial fibroelastosis Endomyocardial fibrosis Methysergide therapy Systemic lupus endocarditis RA myxoma or thrombus Prosthetic valve thrombosis Prosthetic valve infective endocarditis Paraprosthetic valve degeneration and calcification

Parasternal views of the normal tricuspid valve

Tricuspid stenosis

Tricuspid Regurgitation

Acquired Lesions of the Pulmonic Valve

Pulmonary hypertension with pulmonic regurgitation

Mitral stenosis Chronic lung disease Pulmonary emboli Inflammatory lesions Endocarditis Rheumatic fever Tuberculosis Tumors Sarcoma

Myxoma Previous surgery or angioplasty for

congenital lesions Mediastinal lesions Tumor Aneurysm Constrictive pericarditis Miscellaneous Carcinoid syndrome

Subcostal short-axis view of the base of the heart

Schematic representation of M-mode echocardiograms of normal and abnormal pulmonary valves

Pulmonary valve stenosis