Rheumatic valvular diseases - Dr. S. Srinivasan

Rheumatic Valvular Heart Disease

S . SrinivasanProfessor of Paediatrics

MGMCRI, PlillayaarkuppamPuducherry

MGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Learning Objectives• To discuss the common etiologies of valvular

stenosis and regurgitation.• To recognize the signs and symptoms of valvular

stenosis and regurgitation• To clinically recognize, identify clinical features

of Rh. Mitral and Aortic Valvular Diseases and their attendant complications

• To offer a plan of investigative work up and interpret a few important findings

• To offer preventive and treatment modalities recommended in treating and preventing complications

• To identify & refer children with RHD for further work up, medical and surgical management


Rheumatic fever • Inflammatory autoimmune response

triggered by Group A beta-hem. streptococcal pharyngitis

• Children & Adolescents

• Poor SE Status, overcrowding, poor sanitation, developing & underdeveloped countries


Acute rheumatic Carditis PancarditisENDOCARDITIS MV Insufficiency (65 -100%)

AV Regurgitation (20-30%) TV (10% - ass.with MR,AR or both)

MYOCARDITIS Marked Sinus tachycardia S3; changing Murmurs ; Carey-Coomb’s MDM

Pericarditis Chest pain over the left chest and axillaPericardial Rub on auscultationRarely affects cardiac functionRarely results in large effusions or constrictive pericarditis


PREVALENCE RHD IN INDIA ( ECHO proven)

0.67/1000 to 0.12/1000 children(Periwal et al Bikaner) 2006

0.5 per 1000 children(Misra et al. 2003 -2006)


Genetic studies in RF

Progression to Chronic RHD Strong correlation to:HLA antigen DR class II alleles

Inflammatory protein-encoding genes MBL2 and TNFAGenes


Chronic Rheumatic Heart Disease in Children & Young Adults

Worldwide occurrence of RHDEstimated occurrence

5-30 million Children & Young Adults

New Cases 2.5 - 3 Lakhs of RHD added every year

Deaths attributed to Chronic RHD

90 Thousands – 2.5 Lakhs / Year


Rheumatic heart disease

Valvular Stenosis and/or Insufficiency ( Regurgitation)

( structural damage due to fibrosis, thickening, shortening and fusion of valvular cusps and apparatus )

Post Infective ( GpA-ᵦ hemolytic Strep) Autoimmune mediated Cardiac inflammation and scarring

Pancarditis -Myocarditis-Endocarditis & -Pericarditis)

Acute RF Chronic RH Disease


RHD• More severe in females than in males

• Mitral Insufficiency of Ac.RF resolves in 60-80% of patients who adhere to antibiotic prophylaxis

• Aortic Regurgitation in 20-30 % of ARF persists in spite of strict adherence to Secondary Rheumatic Prophylaxis


Rheumatic ( Valvular ) Heart Disease

Permanent structural alterations to heart valve cusps and supporting structures caused by a single or more attacks of ARF.

RHD.: 40-60% of children with ARFValves involved :

-- Mitral > Aortic > TV>PV -- MR(MI) > MR+MS > MS -- MR+AR > MS+AR > AR

-- Rt. Heart Valves in RHD : Rare -- AS of Rheumatic etiology : Uncommon -- ( seen beyond Adolescent age group )


EtiologyPathophysiologyHistory & Physical ExamNatural HistoryInvestigative EvaluationComplications Treatment Prevention


Mitral Regurgitation


Most commonly observed Murmurs of ARF

Aortic Regurgitation

High-pitched, blowing, decrescendo, early diastolic murmur of Aortic Regurgitation ,heard best along the right upper and mid-left sternal border after deep expiration while the patient is leaning forward.

Apical high-pitched, blowing-quality murmur pansystolic murmur radiating to the left axilla

MV insufficiency

◦ Apical diastolic murmur (also known as a Carey-Coombs murmur) in active carditis and accompanies severe mitral insufficiency


Mild (physiological) MR: 80% of normal individuals

Mitral Regurgitation

Definition Backflow of blood from the LV to the LA during systole


Chronic Mitral RegurgitationAge Group

Etiologies

YOUNG Rheumatic MVP Her. Connective Tissue Disorders Ac. Collagen Vascular Disorders

Elderly Rheumatic heart disease Myxomatous degeneration (MVP)

Ischemic MR Infective Endocarditis


Mitral RegurgitationPathophysiology

Volume OverloadCompensatory Mechanisms

LA enlargementLVH Increased contractility

Progressively increasing VO in Chronic MR

Progressive LA dilation Pulm.Arterial Hypertension RV Dysfunction Progressive LV volume

overload LV dilation Progressive heart failureMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Mitral Regurgitation Physical Exam findingsSymptoms Exertion Dyspnea (exercise Intolerance)

- Worsening with severity Palpitation ( more marked in combined MS &MR)

Signs Pulse : NV/HV/LV; Irregularly irregular in AF

Precardial prominence, LV type of APEX AuscultationHeart Sounds soft S1; Loud S2 in PAH;

S3 (CHF/LA overload)Murmurs Holosystolic murmur at

the apex radiating to the axilla;-Flow Middiastolic murmur

Rheumatic Mitral Regurgitation: Natural History

Compensatory phase

10-15 years

Asymptomatic severe MR

5%/year mortality rate

Severe Symptomatic MR

Sharp Rise in mortality rate

MR with EF <60%Cause of Mortality

Progressive CHF, Complications of MR like Arrhythmias, Embolism


Mitral Regurgitation : imaging studies

Chest X-Ray

LA enlargement, Central Pulmonary Artery enlargement

ECG To look for : LA enlargement, Atrial Arrhythmias like Atrial flutter, fibrillation and LVH

ECHO To estimate LA, LV size and functionTo assess valve structure

TEE inconclusive transthoracic ECHO


Mitral Regurgitation-Medical Management

1. Hydralazine & other Vasodilators

2. -blockers, CCB, digoxin : To control Heart Rate in atrial fibrillation with MR

3. Anticoagulants in atrial fibrillation and flutter

4. Diuretics for fluid overloadMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Management of MR• Serial Echocardiography: – Mild: 2-3 years– Moderate: 1-2 years– Severe: 6-12 months


IE prophylaxis Dental procedures Prosthetic valves


MR: Surgical Indications


MR: Important Indications for MV Replacement

Symptomatic Severe MR• Any cardiac related Symptoms at rest

or exercise with (repair if feasible)

Asymptomatic MR EF <60%New onset Atrial Fibrillation


Mitral Stenosis


Mitral Stenosis

• Recurrent episodes: Progressive Valvular Damage • Residual and progressive valve deformity• 10-40% of older children 2-10 years after

previous ARF with MR• Fusion of the valve apparatus (at the level of the

valve commissures, cusps, chordal attachments, or any combination of these ) resulting in stenosis or a combination of stenosis and insufficiency

99% of MS in adults: Rheumatic etiology


Mitral Stenosis

DefinitionLV inflow Obstruction: Impaired LV diastolic filling

Normal MV Area : 4-6 cm2

Basic Facts Onset of Symptoms

MV Area < 2 cm2 With Increasing

Transmitral gradientsPredominant Cause

Rheumatic HD

Prevalence and incidence

Decreasing due to a reduction of RHD

MGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16MGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Etiology of Mitral Stenosis

Rheumatic heart disease

77-99% of all cases

Mitral annular calcification

2.7%

Infective endocarditis

3.3%


Mitral StenosisPathophysiology

Cause Physical Symptoms & Signs

impaired LA emptying resulting in Increasing PV Pressue in capillaries

Progressive Dyspnea (70%) due to pulmonary congestion

Palpitations(worsening with exercise,

fever, tachycardia, and pregnancy)

Increasing Transmitral Pressures

Haempotysis, Progressive Dyspnea , PND, Pedal Oedema, Increased JVP, Hepatomegaly LA enlargement; LA

DilatationPulmonary venous HTNRupture of bronchial vessels PAH RHF-CHF


Natural History of MS

• Disease of plateaus: –Mild MS: 1 - 10 years after initial ARF–Moderate: 5 -10 years later– Severe: Beyond 10 years

Mortality

Pulmonary OedemaInfections-BE,LRI, andThromboembolismPulmonary EmbolismMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Physical Exam Findings of MS

JVP prominent "a" wave

Signs of right-sided heart failure

in advanced disease

Mitral facies Severe MS & Cachexia with GRetdn


• Loud S1• Opening S1 snap: Apex when leaflets are

still mobile Due to the abrupt halt in leaflet motion in

early diastole, after rapid initial rapid opening, due to fusion at the leaflet tips

Shorter the S2 -OS interval, severer the MS

Heart Sounds in MS


Low-pitched Diastolic Rumble Most Prominent at the Apex Best heard in the Left lateral position Bell of the stethExpiration Mild Exercise when in doubt

Heart Murmur in Mitral Stenosis

MidDiastolic Murmur


Evaluation of MSCXR LA enlargement

Pulmonary congestionSigns of PAH

ECG LA enlargementAtrial Fibrillation

ECHO: GOLD STANDARD To Assess

MV Leaflet mobilityGradientMV AreaNature of damage


Complications of Chronic RHD (Established Valvular disease )


1. heart failure from valve insufficiency (acute rheumatic carditis)

2. Atrial Arrhythmias ( rare in children)

3. Pulmonary Edema4. Recurrent Pulmonary Emboli5. Infective Endocarditis6. Intracardiac Thrombus Formation 7. Systemic Emboli.

Management of MSSerial echocardiography

-blockers, CCBs, Digoxin which control heart rate and hence prolong diastole for improved diastolic fillingDuiretics for fluid overload

Mild: 3-5 YearsModerate:1-2 YearsSevere: Yearly

Medications

Medical therapy does not prevent progression as MS is a mechanical problem and MGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Management of MS• Identify patient early who might benefit

from percutaneous mitral balloon valvotomy

REMEMBER to Implement :

IE Prophylaxis & Secondary RHD Prophylaxis


Simplified Indications for Mitral valve replacement

• ANY SYMPTOMATIC Patient with NYHA Class III or IV Symptoms

• Asymptomatic moderate or Severe MS with a pliable valve suitable for PMBV


Aortic Regurgitation


Etiology of Aortic Regurgitation

• Physical Findings:Wide pulse pressureDiastolic murmurFlorid pulmonary edema

AcuteARF( 20-30% in children )EndocarditisAortic Dissection

Chronic ARBicuspid aortic valveRheumatic Infective endocarditisCollagen Vascular Disorders


Pathophysiology of AR• Combined pressure & volume

overload

• Compensatory Mechanisms• LV dilation, LVH• Progressive dilation• Heart Failure


Natural History of AR• Asymptomatic until 4th or 5th decade• Rate of Progression: 4-6% per year• Progressive Symptoms include:

- Dyspnea: exertional, orthopnea, and paroxsymal nocturnal dyspnea

- Nocturnal angina: due to slowing of heart rate and reduction of diastolic blood pressure

- Palpitations: due to increased force of contraction


Physical Exam findings of AR• Wide pulse pressure: most sensitive• Hyperdynamic and displaced apical

impulse• Auscultation- –Diastolic blowing murmur at the left

sternal border– Austin flint murmur (apex): Regurgitant jet

impinges on anterior MVL causing it to vibrate – Systolic ejection murmur: due to increased

flow across the aortic valve MGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Evaluation of AR

CXR enlarged cardiac silhouette and aortic root enlargement

ECHO Evaluation of the AV and aortic root with measurements of LV dimensions and function (cornerstone for decision making and follow up evaluation)

AortographyMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Management of AR• General: IE prophylaxis in dental

procedures with a prosthetic AV or history of endocarditis.

• Medical: Vasodilators (ACEI’s), Nifedipine improve stroke volume and reduce regurgitation only if pt symptomatic or HTN.

• Serial Echocardiograms: to monitor progression.

• Surgical Treatment: Definitive TxMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12 th Feb 16

Simplified Indications for Surgical Treatment of AR

• ANY Symptoms at rest or exercise• Asymptomatic treatment if:–EF drops below 50% or LV becomes

dilated


THANK YOU


Rheumatic valvular diseases - Dr. S. Srinivasan

Healthcare

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