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 diseaseCongenital MSLutembachers 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 HypertensionFibrous thickening of alveolar and pulmo capillary

walls

Thrombi and EmboliLeft atrial appendage Increased: AF, older patients, reduced CO

Pulmonary Infections, IE,

Natural History of Mitral Stenosis

MS - blue

MR - purple

Differential Diagnosis of MSAtrial Septal Defect

RVE and accentuated pulmo markingsWidely split S2 (fixed)VS Opening snap; diastolic flow across the TVNo LAE

Left Atrial MyxomaObstructing LA emptying, tumor-plop

Mitral RegurgitationSystolic 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 drugsTo lengthen diastolic LV fillingDigitalis in Atrial Fibrillation; Beta-blockers in sinus

rhythmNondihydropyridine 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: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 outputVasodilators (ACEI) and digitalis

Anticoagulants and leg binders to decrease likelihood of venous thrombi and pulmonary emboli

Endocarditis prophylaxis

Surgical Treatment of MRNon-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 annuloplastyLessens problem on long term anticoagulation and

thromboembolismFor ruptured chordae, annular dilatation and IENot 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 diseaseDegenerative 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:Cardinal symptoms

Exertional Dyspnea Angina Pectoris Syncope

PECarotid 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 catheterizationPresence 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 yearsSyncope – 3 yearsHeart 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 SurgerySevere 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 yrsMechanical 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 RegurgitationEtiology

Primary Valve DiseaseRheumatic – 2/3 of patients Infective EndocarditisTraumaBicuspid valve

Primary Aortic Root DiseaseDegenerative heart diseaseSyphilisMarfan’s syndromeAnkylosing SpondylitisAortic Aneurysm with dissectionSystemic hypertensionGiant Cell arteritis

Pathophysio of AR

Aortic Regurgitation

History:Easy fatigue then exertional dyspnea (diminished

cardiac reserve) – orthopnea, PND and excessive diaphoresis

PE:Wide pulse pressure with bounding pulsesDiastolic decresendo murmur at the base of the

heartMidsystolic ejection murmur at base of the heartAustin Flint murmur – soft, low pitched rumbling

middiastolic bruit at the apex

Peripheral Signs of Chronic ARCorrigan’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 CausesAortic RegurgitationPatent Ductus Arteriosus

Non-Cardiac CausesArteriovenous FistulaFeverThyrotoxicosisPregnancy

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 angiographyMagnitude 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 ARDefinitive 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 Intensive salt restriction, diuretic tx

Surgical TxDefinitive txDiastolic pressure gradient >4 mmHgTricuspid orifice < 1.5 to 2.0 cmOpen 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.

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 mildAns. 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.

THANK YOU!!!