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ANATOMY

Valvular Heart Disease

AORTIC VALVE DISEASE TRICUSPID VALVE DISEASE PULMONARY VALVE DISEASE

4th Year Medical students Lecture - 2014

Mohammed Al-Kebsi

kibsi@hotmail.com

https://www.facebook.com/pages/Dr-Mohammed-Al-Kebsi/346930838760542?ref=hl

What Are the Types of Valve Disease?

There are several types of valvular heart disease, include: Valvular stenosis: When a valve opening is smaller than normal Valvular Insufficiency/REGURGITATION: occurs when a valve does not close tightly, thus allowing blood to leak backwards. Both valvular diseases can involve all four valves.

Valvular Heart Disease

Acute and Chronic

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What Are the Types of Valve Disease?

Mitral valve disease (MVD):
Mitral Stenosis Mitral Regurgitation

What Are the Types of Valve Disease?

Mitral valve disease (MVD):
Mitral Stenosis Mitral Regurgitation

Aortic valve disease (AVD)

Aortic Stenosis Aortic regurgitation

Aortic valve disease (AVD)

Aortic Stenosis Aortic regurgitation

Tricuspid valve disease

Tricuspid stenosis Tricuspid regurgitation

Tricuspid valve disease

Tricuspid stenosis Tricuspid regurgitation

Pulmonary valve disease

Pulmonary Stenosis Pulmonary Regurgitation

Pulmonary valve disease

Pulmonary Stenosis Pulmonary Regurgitation

Rheumatic Heart Disease

Inflammatory process that may affect the myocardium, pericardium and or endocardium Usually results in distortion and scarring of the valves Caused by inflammatory reaction secondary to sore throat caused by a bacteria called S.pyogenes

Aortic Valve Disease

Aortic Stenosis Aortic regurgitation

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Aortic Root - Anatomy

The AORTIC ROOT has four anatomic components: The aortic annulus or aortoventricular junction The leaflets SINGLE The sinotubular junction FUNCTIONAL The aortic sinuses or sinuses of Valsalva UNIT

Aortic Stenosis
Types Levels of stenosis

Supravalvular Valvular Subvalvular

Aortic Stenosis
VALVAR SUPRA VALVAR
WILLIAMS SYNDROME
Elfin-like facies Hypervitaminosis D Pulmonary or aortic stenosis Mesenteric artery stenosis Thoracic aneurysm

Aortic Stenosis

Rheumatic Aortic Stenosis

Calcific Aortic Stenosis (Bicuspid) Degenerative Aortic Stenosis

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Aortic Stenosis
SUB VALVAR

AS: Clinical Picture Symptoms

Asymptomatic Syncope
cerebral hypoperfusion vs. dysrhythmias

Sub-aortic membranous stenosis Hypertrophic Obstructive CardioMyopathy (HOCM)

Angina
myocardial O2 supply/demand imbalance

Congestive Heart Failure Sudden death

AS: CVS Examination

CVS:
CXR

AS: Diagnosis
Left ventricular prominence with possible cardiomegaly Calcifications at level of aortic valve

Loud harsh crescendodecrescendo murmur radiating to neck Split S2, possible S4 with atrial hypertrophy Prominent presystolic apical impulse Weak peripheral pulse.

Prominence of ascending aorta post stenotic dilatation

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AS: Diagnosis
ECG: LVH LA hypertrophy conduction abnormalities

AS Management

Echocardiogram with Doppler

MANAGEMENT - AS
ASYMPTOMATIC Mild moderate stenosis Medical follow up Regular ECHO Avoid strenuous exercise Endocarditis prophylaxis ? Role for statins Progress ~0.1cm2 per year SYMPTOMATIC

No drugs alter course

AVR
Angina, syncope, failure Moderate AS + CAD Reduced BP on exercise Severe AS & reduced LV function

AORTIC VALVE REPLACEMENT

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Aortic Regurgitation (Causes)

AORTIC REGURGITATION

ACQUIRED
VALVE

CONGENITAL
Cusp thickening
Bicuspid valve Supra-valvar stenosis Sinus of Valsalva aneurysm

Rheumatic fever Infective endocarditis Rheumatoid disease Idiopathic degenerative disease Aortic dissection Calcific aortic disease SLE

AORTIC ROOT -

Dilation of sinus aorta, failure of coaptation of cusps

Aortic Dissection Aortitis (Syphilis, viral syndromes; giant cell arteritis;Takayasu disease; Connective tissue disorders (Marfans syndrome; Reiters disease; EhlersDanlos syndrome; Osteogenesis imperfecta; Rheumatoid arthritis syndrome; Ankylosing Spondylitis; SLE;) Hypertension Trauma

Chronic AR Pathophysiology
Aortic regurgitation
effective Stroke volume

Acute AR Pathophysiology

Excess volume to the LV LV end diastolic pressure

Stretching of Myocardium

Myocardial O2 supply

wall stress

LV failure

(Ischemia)

Eccentric LV hypertrophy

Myocardial O2 demand (Ischemia)

Most common causes of acute aortic regurgitation is infective endocarditis and aortic dissection Acute AR of significant severity leads to increased blood volume in the LV during diastole. The LV does not have sufficient time to dilate in response to the sudden increase in volume. As a result, LV end-diastolic pressure increases rapidly, causing an increase in pulmonary venous pressure and altering coronary flow dynamics. As pressure increases throughout the pulmonary circuit, the patient develops dyspnea and pulmonary edema. In severe cases, heart failure may develop and potentially deteriorate to cardiogenic shock. Decreased myocardial perfusion may lead to myocardial ischemia. Early surgical intervention should be considered

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Aortic Regurgitation Clinical picture

Acute aortic regurgitation Cases of acute AR may be fulminant and lead to cardiogenic shock; patients who have CHF or shock associated with severe AR often appear gravely ill. Other symptoms of acute AR include the following: Tachycardia Peripheral vasoconstriction Cyanosis Pulmonary edema Arterial pulsus alternans; normal LV impulse Early diastolic at the right sternal border is associated more often with dissection than it is with any other cause of aortic regurgitation.

AR Clinical picture
Chronic aortic regurgitation Patients with chronic AR often have a long-standing asymptomatic period that may last for several years. A compensatory tachycardia may develop to maintain a large forward stroke volume, leading to a decreased diastolic filling period. As a result, patients may be asymptomatic even with exercise. Over time, however, chronic volume overload leads to LV dysfunction as the LV dilates. Significant deterioration of LV function may begin prior to the development of symptoms. Symptoms of severe chronic AR include the following: Palpitations - Often described as the sensation of having forceful heart beats, due to widened pulse pressure with hyperdynamic circulation Uncomfortable awareness of the heartbeat Shortness of breath - May not worsen with exertion in the early stages due to compensatory tachycardia with shortened diastole Chest pain - Occurs if increased LV end-diastolic pressure compromises coronary perfusion pressure gradients Sudden cardiac death - This is uncommon (< 0.2% per year), however, in asymptomatic patients with preserved LV function

AR - Diagnosis
Austin-Flint murmur vibrations of anterior mitral leaflet Duroziezs sign to and fro femoral artery murmur Quinckes pulse capillary pulsation in finger tips Traubes sign pistol shot sound at femoral artery De Mussets sign head bobbing

Aortic Regurgitation Clinical picture

Chronic aortic regurgitation Diastolic pressures are often lower than 60 mm Hg, with pulse pressures often exceeding 100 mm Hg. Becker sign - Visible systolic pulsations of the retinal arterioles Corrigan pulse ("water-hammer" pulse) - Abrupt distention and quick collapse on palpation of the peripheral arterial pulse de Musset sign - Bobbing motion of the patient's head with each heartbeat Hill sign - Popliteal cuff systolic blood pressure 40 mm Hg higher than brachial cuff systolic blood pressure Duroziez sign - Systolic murmur over the femoral artery with proximal compression of the artery, and diastolic murmur over the femoral artery with distal compression of the artery Mller sign - Visible systolic pulsations of the uvula Quincke sign - Visible pulsations of the fingernail bed with light compression of the fingernail Traube sign ("pistol-shot" pulse) - Booming systolic and diastolic sounds auscultated over the femoral artery The apex of the heart is diffuse or hyperdynamic but is often displaced inferiorly and toward the axilla. Peripheral pulses are prominent or bounding. Auscultation may reveal an S3 gallop if LV dysfunction is present. Auscultation reveals Diastolic murmur at the left sternal border. An Austin-Flint murmur may be present at the cardiac apex in severe AR. In many cases, physical examination also reveals findings relating to the underlying cause of AR. For example, Marfan syndrome.

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Natural History - Incompetence

a) Latent period to cardiac decompensation is long Sudden death is not common
Once deterioration begins, the LV fails rapidly

Aortic Regurgitation Differential diagnosis

Physical examination findings suggesting a widened pulse pressure may also be seen in other conditions of hyperdynamic circulation, including the following: Thyrotoxicosis Severe anemia Pregnancy Thiamine deficiency (wet beriberi) Arteriovenous fistula - Such as patent ductus arteriosus or peripheral arteriovenous malformations

b) Symptomatic patient with CHF, angina, syncope Prompt operation is indicated c) Asymptomatic patient
Follow carefully for LV enlargement or decreased LV function by ECHO Operate at an appropriate time

Aortic Regurgitation- Diagnosis CXR ECG

Aortic Regurgitation- Diagnosis

Enlargement of the entire aorta

Left Ventricular enlargement

Electrocardiographic findings are nonspecific but may include evidence of the following: LV hypertrophy Left axis deviation Left atrial enlargement LV conduction defects.

ECHOCARDIOGRAPHY

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Aortic Regurgitation Management

Acute aortic regurgitation Administer a positive inotrope (eg, dopamine, dobutamine). Administration of vasodilators may be appropriate to improve systolic function and to decrease afterload. Avoid beta-blockers in the acute setting. Valve replacement. Chronic aortic regurgitation The administration of pressors and/or vasodilators may be appropriate. Regular ECHO Avoid isometric exercise Endocarditis prophylaxis Monitor for symptoms Valve replacement Mechanical versus bioprosthetic aortic valves

Tricuspid Valve Disease

Tricuspid Stenosis Tricuspid regurgitation

The forgotten valve

TVD- Etiology
Tricuspid Valve Anatomy
Primary (organic) TVD stenosis or regurge: regards pathologic involvement of the leaflets and chordae. Secondary (functional) tricuspid regurgitation is referred to when simple annular dilation has occurred.

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Etiology of Secondary or Functional Tricuspid Valve Disease

Etiology of Primary Tricuspid Valve Disease

Congenital Cleft valve generally in association with atrioventricular canal defect Ebsteins anomaly Congenital tricuspid stenosis Tricuspid atresia Rheumatic valve disease, generally in association with rheumatic mitral valve disease Infective endocarditis Carcinoid heart disease Toxic (eg, Phen-Fen valvulopathy or methysergide valvulopathy) Tumors (eg, myxoma) Iatrogenicpacemaker lead trauma Traumablunt or penetrating injuries Degenerativetricuspid valve prolapse Pulmonary hypertension Left side valvular heart disease. Cardiomyopathy, myocarditis. Endomyocardial fibrosis, arrhythmogenic right ventricular dysplasia

Clinical Presentations
Pure or predominant tricuspid stenosis Pure or predominant tricuspid regurgitation Mixed

Tricuspid valve diseaseSymptoms

Fatigue Liver/gut congestion Right upper quadrant discomfort Dyspepsia Indigestion Fluid retention with leg edema Ascites Raised JVP.

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Tricuspid valve disease ausculatory findings

Stenosis Low-to medium-pitch diastolic rumble with inspiratory accentuation Regurgitation Soft, early, or holosystolic murmur Augmented with inspiratory effort (Caravallos sign)

Tricuspid valve disease Managment

As for medical therapy, diuretics are used to reduce venous congestion. Vasodilating agents (e.g., angiotensin-converting enzyme inhibitors and angiotensin receptor blockers) may be associated. This therapy should be started before surgery and continued afterwards to give the chance to the right ventricle to re-remodel and to reduce the diameter of the tricuspid annulus, further decreasing the grade of regurgitation. Restriction of fluid and dietary sodium intake. Therapy of the underlying disease. Surgery : tricuspid valve repair and rarely replacment This therapy should be started before surgery and continued afterwards to give the chance to the right ventricle to re-remodel and to reduce the diameter of the tricuspid annulus, further decreasing the grade of regurgitation

Substantial tricuspid regurgitation may exist without the classic ausculatory findings. Thus, clinical evaluation including cardiac auscultation cannot be used to exclude tricuspid valve disease.

Pulmonary stenosis

Pulmonary Valve Disease

Pulmonary Stenosis Pulmonary regurgitation

Pulmonic stenosis (PS) refers to a dynamic or fixed anatomic obstruction to flow from the right ventricle (RV) to the pulmonary arterial vasculature. Although most commonly diagnosed and treated in the pediatric population, individuals with complex congenital heart disease and more severe forms of isolated PS are surviving into adulthood and require ongoing assessment and cardiovascular care.

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Pulmonary stenosis
Etiology
Isolated (10% of all congenital heart disease)
valvular subvalvular, narrowing of the infundibular or subinfundibular region. peripheral (supravalvular) obstruction obstruction at the level of the main pulmonary artery, at its bifurcation, or at the more distal branches.

Pulmonary stenosis- Diagnosis

Clinical features
Most children and adults with mild-to-moderately severe pulmonic stenosis (PS) are asymptomatic. Those with severe PS may experience exertional dyspnea and fatigue. In extremely rare cases, patients present with exertional angina, syncope, or sudden death. Peripheral edema and other typical symptoms occur with right heart failure. Cyanosis is present in those with significant right-to-left shunt via a patent foramen ovale, atrial septal defect, or ventricular septal defect.

In association with more complicated congenital heart disorders Tetralolgy of Fallot Rarely rheumatic heart disease, and it follows involvement of the mitral and aortic valves. Carcinoid tumor Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction. Sinus of Valsalva aneurysms .

Pulmonary stenosis -diagnosis

Clinical examination

Pulmonary stenosis Investigations

CXR
Enlarged main pulmonary artery Normal to decreased peripheral pulmonary vasculature The overall heart size usually is normal unless RV failure or tricuspid regurgitation develops.

A precordial heave or a palpable impulse from the RV along the left parasternal border. A systolic thrill may be palpable at the level of the second intercostal space. Auscultation reveals a normal S1 and a widely split S2, systolic crescendo-decrescendo ejection murmur in the left upper sternal border that increases with inspiration and radiates diffusely. Mild-to-moderately severe desaturation or frank cyanosis may be noted with rightto-left shunting through a patent foramen ovale, atrial septal defect, or ventricular septal defect.

Echocardiography provides a definitive confirmation of the diagnosis of PS. Electrocardiogram: right ventricular hypertrophy and right axis deviation on the ECG

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Pulmonary stenosis Treatment

Pulmonary Regurgitation
Pulmonic regurgitation is seldom clinically significant. Very rare: symptoms of right-sided heart failure , Dyspnea on exertion is the most common complaint. Easy fatigability, light-headedness, peripheral edema, chest pain, palpitations, and frank syncope may occur. Other symptoms specific to the underlying disease process causing pulmonic regurgitation may occur.

Medical treatment has weak role in the management Balloon valvuloplasty is the treatment of choice in severe cases. Surgery is rarely an option nowadays.

pulmonary Regurgitation Causes

Significant pulmonic regurgitation occurs variably as a complication of the following: Primary pulmonary hypertension. Secondary pulmonary hypertension. Infective endocarditis. (rare) Rheumatic heart disease: Pulmonary valve affected following mitral, aortic, and tricuspid valve involvement. Carcinoid heart disease: Medications: Medications that act (fenfluramine). Dilatation of the pulmonary trunk in Marfan syndrome or Takayasu arteritis Idiopathic Complications to pulmonary balloon valvuloplasty). Complications of surgical repair of pulmonic stenosis

Pulmonary Regurgitation Physical exam

Jugular venous pressure (JVP) is usually increased. Palpable pulmonary artery pulsation at the left upper sternal border may be present in the setting of significant pulmonary artery dilatation. The pulmonic component of the second heart sound (P2) is inaudible in the absence of a pulmonic valve, brief, decrescendo early diastolic murmur at the upper left sternal border. The Graham Steell murmur of pulmonary hypertension is a high-pitched, early diastolic decrescendo murmur noted over the left upper-to-left midsternal area

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Pulmonary Regurgitation Diagnosis

Chest X-Ray Prominent central pulmonary arteries with enlarged hilar vessels and loss of vascularity in the peripheral lung fields ("pruning") suggests severe pulmonary hypertension. Echocardiography can reveal right ventricular hypertrophy and dilatation. Cardiac magnetic resonance (CMR) CMR has shown promise based on recent studies of pulmonary regurgitation.

Pulmonary Regurgitation Management

Pulmonary regurgitation is seldom severe enough to warrant special treatment Surgical reconstruction or replacement of the pulmonary valve in rare cases.

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