Valvular Heart Disease ADRIEL E. GUERRERO, MD, FPCP, FPCC Training Officer Section of Cardiology, Dept of Medicine The Medical City

Mitral Stenosis

Diagnostic Features of MS@ 2/3 are females; Pure MS are generally rheumatic History: Exertional Dyspnea, PND, Orthopnea and Hemoptysis PE: Opening snap, loud S1, diastolic rumble at the apex ECG and Chest Xray: Evidence of left atrial enlargement with normal left ventricular size; RVH in later stages 2DECHO

Chest Xray MS

Common Etiologies of MS Rheumatic Heart Disease Congenital Heart disease Congenital MS Lutembachers syndrome (MS and ASD) Mitral Annular Calcification (elderly)

Mitral Stenosis@ Pathophysiology Obstruction to LV filling Increase LA pressure RV Failure

Natural History of MS Pulmonary Hypertension Thrombi and Emboli Fibrous thickening of alveolar and pulmo capillary walls Thrombi and Emboli Left atrial appendage Increased: AF, older patients, reduced CO Pulmonary Infections, IE,

Natural History of Mitral Stenosis MS - blue MR - purple

Differential Diagnosis of MS Atrial Septal Defect RVE and accentuated pulmo markings Widely split S2 (fixed)VS Opening snap; diastolic flow across the TV No LAE Left Atrial Myxoma Obstructing LA emptying, tumor-plop Mitral Regurgitation Systolic murmur; LVH Aortic Regurgitation (Austin Flint) Apical middiastolic murmur of AR. Becomes louder on handgrip and decreases with amyl nitrate 

Treatment of MS Penicillin Prophylaxis of B-hemolytic Streptococcal Infections to prevent Rheumatic Fever and IE Sodium restriction, oral diuretics Oral Anticoagulation (Warfarin) INR target 2-3.1 (embolization, permanent AF) Heart Rate Controlling drugs To lengthen diastolic LV filling Digitalis in Atrial Fibrillation; Beta-blockers in sinus rhythm Nondihydropyridine Calcium Antagonists

Mitral Valvotomy Indicated in symptomatic patients with isolated MS (<1.0 cm2/m2) Ideal for mobile, thin leaflets with no or little calcium without extensive subvalvular thickening and with no or mild MR Open valvotomy – mortality rate is 2% 50% of all patients require reoperation by 10 years. Pregnant – carried out if pulmonary congestion occurs despite intensive medical treatment

Mitral Valve Replacement MS with significant MR Distorted valves from previous transcatheter or operative manipulation Operative Mortality is 6% Long term complications of valve replacement Overall 10 year survival is 70% Poor Recovery Old patients Marked disability Depressed Cardiac index

Mitral Stenosis

Percutaneous Trans-septal Mitral Commissurotomy

Star Edwards Caged Ball Valve

Medtronic Hall Valve (Bjork-Shiley)

St Jude Bileaflet Valve

Carpentier Edwards Porcine Valve

Carpentier Edwards Pericardial Valve

Mitral Regurgitation Frequent in males History: PE: Easy Fatigue then exertional Dyspnea PE: Characteristic holosystolic murmur at the apex with radiation to the axilla

Common Etiologies of MR MV leaflet abnormality Rheumatic heart disease Myxomatous alteration (including MVP) Infective Endocarditis Mitral annulus dilatation of any cause Dilated Cardiomyopathy IHD with dilated LV Ruptured chordae tendinae Trauma Myocardial Infarction Papillary muscle disorder Ischemic Heart Disease

Mitral Regurgitation

NORMAL

Pathophysiology of Mitral Regurgitation

Pathophysiology of Mitral Regurgitation

Pathophysiology of Mitral Regurgitation

Laboratory Examination La enlargement; RAE maybe present if pulmonary HPN is severe Atrial Fibrillation LVH ECG 2DECHO – most accurate non-invasive technique CXR – LAE and LVE

Medical Treatment for MR Restrict Physical activities Reduce sodium intake and enhance sodium excretion (diuretics) Increase forward cardiac output Vasodilators (ACEI) and digitalis Anticoagulants and leg binders to decrease likelihood of venous thrombi and pulmonary emboli Endocarditis prophylaxis

Surgical Treatment of MR Non-surgical candidates: asymptomatic, or exercise limited to strenuous exertion, normal LV function Surgery for severe MR even if asymptomatic or when LV dysfunction is progressive (declining <60%) and/or LV ESD on echo is >45mm MV replacement for markedly shrunken, deformed, calcified leaflets MV repair (reconstruction) with annuloplasty Lessens problem on long term anticoagulation and thromboembolism For ruptured chordae, annular dilatation and IE Not suitable for Mr due to myxomatous degeneration and patients with calcified annulus

Natural History of Unoperated MR

Late Survival Rates after Surgical Correction in MR (pre-op EF)

Mitral Valve Prolapse Barlow’s syndrome, floppy-valve syndrome, systolic click-murmur syndrome, billowing mitral leaflet syndrome Excessive or redundant mitral leaflet tissue. Posterior MV leaflet is more affected than the AMVL May lead to excessive stress on the papillary muscles leading to dysfunction. Rupture of chordae tendineae with progressive annular dilatation and calcification Ventricular arrythmias

Clinical Features Females (14-30 years old) Clinical course is often benign Increased familial incidence – autosomal dominant Most common cause of isolated severe MR requiring surgical treatment in North America Arrythmias (PVCs, SVTs, VTs) – palpitations, lightheadedness and syncope. Sudden death is rare Chest pain substernal, prolonged, unrelated to exertion

Mitral Valve Prolapse Mismatch between elongated MV and LV cavity

Laboratory Exams ECG – non specific STTW changes, PVCs Echo – demonstrates systolic displacement of MVL and quantifies Mitral Regurgitation and LV function

Treatment of MVP IE prophylaxis Beta-blockers sometimes relieve chest pain For severe symptomatic MR, MV repair or rarely replacement is indicated Antiplatelets for patients with TIA, anticoagulation if recurrent TIAs

Survival Rates of MVP patients at baseline risk factors Primary Risk Factors Mod-severe MR; EF <50% Secondary Risk Factors Mild-mod MR; LA > 40 Flail leaflet; AF; age > 50

Aortic Stenosis 80% with symptomatic AS are males Age-related degenerative calcific AS – most common cause of AS in Adults

Common Etiologies of AS Valvular (90% of all cases) Rheumatic heart disease Degenerative calcification (elderly) Bicuspid AV stenosis/ Congenital heart disease Subvalvular (9%) Hypertrophic Obstructive Cardiomyopathy (HOCM) Discrete Supravalvular (<1%) Infancy

NORMAL AORTIC VALVE

Aortic Stenosis - Rheumatic

Aortic Stenosis - Degenerative

Aortic Stenosis - Bicuspid

Pathophysio of AS

Pathophysio of AS

Aortic Stenosis History: PE Cardinal symptoms Exertional Dyspnea Angina Pectoris Syncope PE Carotid upstroke slowly rising and reduced in amplitude in severe cases; Systolic ejection murmur radiating to the carotid arteries

Laboratory Exam LV hypertrophy is the key finding ECG 2DECHO – estimate valve area, LV size and function Cardiac catheterization Presence or absence of concomittant CAD

Natural History of Severe AS Death most commonly occurs in the 7th and 8th decade Average time from onset of symptoms to death: Angina – 3 years Syncope – 3 years Heart Failure – 1.5 – 2 years Sudden death 10-20% in AS patients 60 years old and above

Medical Treatment for AS Severe AS (<0.5 cm2/m2)Avoid strenuous physical activities even if asymptomatic Sodium restriction Cautious administration of diuretics and digitalis in CHF Nitroglycerin to relieve angina Statins (HMGCoA reductase inhibitors) slows down progression of calcification (?) No effect on long term survival

Surgery in Aortic Stenosis Indications for Surgery Severe Aortic Stenosis ( < 0.6 cm2/m2) Symptomatic with LV dysfunction (LV Ejection Fraction <50%) Expanding poststenotic aortic root (even if asymptomatic) Those who undergo CABG even if asymptomatic Operative risk of Aortic Valve Replacement in asymptomatic severe AS is 4%. In frank CHF – 15 – 20% 10 year survival rate of patients with AVR = 60% 30% of bioprosthesis fail in 10 yrs Mechanical prosthesis – hemorrhage fm anticoagulation

Management Strategy for Severe Aortic Stenosis

Percutaneous Balloon Aortic Valvuloplasty Preferred in children and young adults with congenital, noncalcific AS High “restenosis” rate in calcific AS “bridge to operation”

Percutaneous Transcatheter Placement of AV prosthesis

Percutaneous Transcatheter Placement of AV prosthesis

Aortic Regurgitation Etiology Primary Valve Disease Rheumatic – 2/3 of patients Infective Endocarditis Trauma Bicuspid valve Primary Aortic Root Disease Degenerative heart disease Syphilis Marfan’s syndrome Ankylosing Spondylitis Aortic Aneurysm with dissection Systemic hypertension Giant Cell arteritis

Pathophysio of AR

Aortic Regurgitation History: PE: Easy fatigue then exertional dyspnea (diminished cardiac reserve) – orthopnea, PND and excessive diaphoresis PE: Wide pulse pressure with bounding pulses Diastolic decresendo murmur at the base of the heart Midsystolic ejection murmur at base of the heart Austin Flint murmur – soft, low pitched rumbling middiastolic bruit at the apex

Peripheral Signs of Chronic AR Corrigan’s pulse: Pulses with abrupt distension and quick collapse; water hammer pulse De Musset’s sign: head bobbing Traube’s sign: Pistol shot sound on the femoral artery Duroziez’s sign: Systolic murmur heard over the femoral artery when compressed proximally Muller’s sign: systoic pulsation of the uvula Quincke’s sign: Capillary pulsation Hill’s sign: Popliteal cuff SBP > brachial cuff SBP by 60mmHg

Differential Diagnosis of a Bounding Pulse Cardiac Causes Aortic Regurgitation Patent Ductus Arteriosus Non-Cardiac Causes Arteriovenous Fistula Fever Thyrotoxicosis Pregnancy

Symptomatology in AR Symptoms depend on the onset of AR (acute/chronic) Compensatory mechanisms LV dilatation Laplace Law ( myocardial wall tension = intracavitary pressure x LV radius) Deterioration of LV function precedes the development of symptoms Chronic Severe AR may have a long latent period. Patients remain asymptomatic for as long as 10-15 years.

Laboratory Exam LV Hypertrophy 2DECHO + myocardial contractility and function Cardiac catheterization and angiography Magnitude of AR and status of LV function

Survival without Surgery in Chronic AR

Medical Treatment of AR Salt restriction Diuretics Vasodilators (ACEI) Nitrates not as helpful in relieving angina Syphilitic aortitis – penicillin tx

Surgical Treatment of AR Definitive treatment Should be done before development of Heart Failure Operation should be carried out even in Asymptomatic patients with progressive LV dysfunction and a left Ventricular Ejection Fraction < 55% or a LV end-systolic volume > 55 mL/m2 ( 55/55 rule) AVR – mechanical or bioprosthesis Operative mortality for isolated AVR is 4.3% Patients with marked LVE and dysfunction OR mortality 10% Late operative mortality 5% per year

Post-operative Survival in AR

Post-operative Survival in AR

Tricuspid Stenosis

Treatment of TS Medical Tx Surgical Tx Intensive salt restriction, diuretic tx Surgical Tx Definitive tx Diastolic pressure gradient >4 mmHg Tricuspid orifice < 1.5 to 2.0 cm Open heart repair/prosthesis (preferably large bioprosthetic valve)

Tricuspid Regurgitation

Treatment Considerations in TR Isolated TR, in the absence of Pulmo HPN, is well tolerated and does not require operation Functional TR (secondary to PHPN with MV disease) resolves with effective correction of the MV disease) - annuloplasty TVR for severe valve deformity

Pulmonic Valve Disease Pulmonic Regurgitation – most common acquired abnormality of the PV secondary to dilatation of the PV ring as a consequence of PHPN Graham Steell murmur (high pitched decresendo, diastolic blowing murmur along the left sternal border- like AR) Of little hemodynamic significance.

Ex.1 Patient has MS mild, AS severe, MR mild Four Guidelines to Follow in Assessing Patients with Multiple Valvular Abnormalities Guideline 1: Determine the Predominant Lesion A. The most severe lesion is usually the predominant lesion Ex.1 Patient has MS mild, AS severe, MR mild Ans. AS is the dominant lesion B. The lesion that causes the most corresponding chamber enlargement is usually the predominant lesion Ex.2 Patient has AR moderate, MS moderate, Severely dilated LV, slightly dilated LA Ans. AR is the predominant lesion

Guideline No. 2: Left-sided lesions are more important that right-sided lesions. Therefore tailor your treatment more for the left-sided lesion. Guideline No. 3: Significant stenotic lesions (MS or AS) should be given more serious attention compared to regurgitant lesions (MR or AR) Guideline No. 4: In severe valvular disease, surgical correction of the mechanical defect should be given prime consideration. Response to medical treatment is poor.

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