Valvular heart disease Flashcards
Best investigation for assessing valves
ECHO
Acute rheumatic fever
- What age is most commonly affected?
- Pathophysiology
- What pathogonomic features may be seen?
- presentation
- diagnosis
- Treatment
- Secondary prevention
- children (5-15) or young adults
Triggered by immune-mediated delayed response to infection with strains of group A streptococci. Antigens may cross react with cardiac myosin and sarcolemmal membrane protein, leading to inflammation in endocardium, myocardium, pericardium, joints/skin
- Aschoff nodules - occur only in heart. Multinucleated giant cells surrounded by macrophages & T lymphocytes. Not seen until subacute/chronic phase
Fever, lethargy, joint pain, 2-3 weeks post streptococcal pharyngitis
- joint pain characteristically responds to aspirin
Revised Jones criteria
- at least 2 major and at least 2 minor manifestations & evidence of preceding streptococcal infection
Major = JONES J - Joints O - carditis N - nodules E - erythema marginatum S - sydenham chorea
Minor = CAFEPAL C - raised CRP A - arthralgia F - Fever/ first degree AV block E - Elevated ESR P - prolonged PR interval / Previous rheumatic fever A - Anamnesis of rheumatism L - Leukocytosis
Evidence of preceding streptococcal infection
- Throat swab culture - group A beta-hemolytic streptococci
- Anti-steptolysin O antibodies (ASO tiered)
Evidence of carditis
- CXR - cardiomegaly, pulmonary congestion
- ECG - 1st degree AV block, features of pericarditis; T wave inversion, reduction in QRS voltage
- ECHO - cardiac dilatation, valve abnormalities
Management of acute attack
- single dose benzyl-penicillin or oral phenoxymethylpenicillin
- pen allergy –> erythromycin, cephalosporin
- bed rest
Aspirin - relieves arthritis rapidly
Corticosteroids - if have carditis/ severe arthritis. Continue until ESR normal.
Secondary prevention
- long term prophylaxis with penicillin
- benzathine penicillin if compliance doubt
- may be stopped after 21 years old/ needs to be extended if have another attack in last 5 years; high risk occupation or high prevalence
- Doesn’t protect against infective endocarditis - ONLY acute rheumatic fever
Jones criteria for diagnosing rheumatic fever
Revised Jones criteria
- at least 2 major and at least 2 minor manifestations & evidence of preceding streptococcal infection
Major = JONES J - Joints O - carditis N - nodules E - erythema marginatum S - sydenham chorea
Minor = CAFEPAL C - raised CRP A - arthralgia F - Fever/ first degree AV block E - Elevated ESR P - prolonged PR interval / Previous rheumatic fever A - Anamnesis of rheumatism L - Leukocytosis
Erythema marginatum
- what does this look like?
- where can they be found?
- red macules that fade in centre but remain red at edges
- mainly on trunk and proximal extremities - NOT FACE
- red rings
Subcutanous nodules
- Are they painful?
- where are they best felt?
- when do they appear?
Small, firm, painless
Best felt over extensor surfaces of bones/tendons
typically appear >3 weeks after onset of other manifestations
Chronic rheumatic heart disease
- who does it most commonly affect?
- how common is it?
- what valves are most commonly affected?
- pathology
- presentation
- affects 50% of those with rheumatic fever & carditis
- 2/3 occur in women
- > 90% affect mitral valve, then aortic, tricuspid, pulmonary
- mixed mitral stenosis and regurgitation more common than isolated mitral stenosis
Progressive fibrosis - mainly affect heart valves. Fusion of valve commissures shortening chord tendinae.
Damaged valve - altered hemodynamic stresses make damage worse
Valve disease may be symptomatic during fulminant forms of acute rheumatic fever, but may remain asymptomatic for many years
Causes of mitral stenosis
- rheumatic disease
- older - calcifications,
- congenital
- carcinoid syndrome
- fenfluramine
- SLE
- amyloidosis
Mitral stenosis pathophysiology
- flow of blood from LA to LV restricted
- increased left atrial pressure
- pulmonary venous congestion and breathlessness (increased pulmonary artery pressure, right ventricular hypertrophy)
- pulmonary hypertension
- right ventricular hypertrophy
LEFT VENTRICLE IS NORMAL (ISSUES ARISE FROM BLOOD NOT BEING PUSHED OUT THROUGH MITRAL VALVE -> backs up)
Any situation that demand an increase in cardiac output also increase left atrial pressure (therefore exercise, pregnancy poorly tolerated)
Mitral stenosis
- presentation
- examination findings
- auscultation
- investigations
- management
Effort-related dyspnoea fatigue (low cardiac output) edema, ascites (right heart failure) palpitations (if in AF) haemoptysis (pulmonary congestion, PE) Thromboembolic complications
Malar flush - due to CO2 retention (reduced cardiac output)
AF, HF
Auscultation
- loud 1st HS, opening snap (moves closer to 2nd HS as stenosis becomes more severe)
- S1 and opening snap may not be audible in heavily calcified valves
- Loud pitched mid-diastolic murmur (roll patients towards left and use bell). Accenutated by exercise and during atrial systole
Exam
- may feel tapping apex beat
Investigations
- ECG - right ventricular hypertrophy (tall R waves in V1-V3), AF, P mitrale
- CXR - Enlarged LA, signs of pulmonary venous congestion (distended pulmonary veins, enlarged pulmonary trunk); splaying of carina, Kerley B lines
Treatment
- Anticoagulation
- AF - ventricular rate control - digoxin, beta-blockers, Ca channel antagonists
- diuretic therapy - control pulmonary congestion
if still symptomatic or develop pulmonary hypertension - balloon valvuloplasty, mitral valvotomy or mitral valve replacement
Mitral regurgitation
- pathophysiology
- Presentation
- Examination findings
- Investigations
- Management
- gradual dilatation of LA
- dyspnoea
- fatigue
- palpitations (AF, increased stroke volume)
- sudden acute onset pulmonary edema
Apical systolic murmur - 1st HS normal/soft - merges with pansystolic murmur extending to 2nd HS (Soft S1, apical S3)
- heard best at apex and left sternal edge
- radiates to axilla
- +/- thrill
- Apex beat feels active/rocking due to left ventricular volume overload - usually displaced to left
- May have AF/ atrial flutter
ECG - left atrial and left ventricular hypertrophy; bided p wave
CXR - enlarged LA and LV; pulmonary venous congestion/pulmonary oedema
ECHO - Dilated LA and LV
Management
- Acute - surgery and pre-op diuretic (furosemide)
Moderate - treat medically - Diuretics; Vasodilators – e.g. ACEi
If in AF: digoxin & anticoagulants
Chronic (asymptomatic):
- <60% ventricular ejection fraction: surgery
- > 60%: rate control ACEi and beta-blocker
Chronic (symptomatic):
- <30%: surgery + medical treatment
>30%: medical treatment, adjunct intra-aortic balloon counterpulsation
Diuretics may aid symptoms:
- More needed in AKI
- IV bumetanide as bowel congestion affects absorption
If concurrent AF, use digoxin + bisoprolol
- s/e monitor in CKD due to accumulation
- s/e xanthopsia (yellow tinged vision)
Transition to ACEi (lisinopril/ramipril) to improve symptoms & reduce admission
- GTN + hydralazine if ACEi not tolerated
High afterload may worsen degree of regurgitation, and hypertension should be treated with vasodilators e.g. ACE inhibitors.
Mitral valve repair used to treat mitral valve prolapse
- Many advantages over mitral valve replacement
- Severe regurgitation - early repair prevents irreversible L ventricular damage.
Mitral regurgitation often accompanies ventricular dilatation and dysfunction that are concomitants of coronary artery disease
- If need to undergo bypass graft surgery – usually repair valve & restore function by inserting annuloplasty ring to overcome annular dilatation and to bring valve leaflets closer together.
•- Can be difficult to determine whether ventricular dilatation or mitral regurgitation is predominant problem.
If ventricular dilatation is underlying cause of mitral regurgitation → then mitral valve repair/replacement may actually worsen ventricular function, as ventricle can no longer empty into low-pressure LA.
Aortic stenosis
- aetiology
- Pathophysiology
- examination findings
- investigations
- management
Aetiology depends on age
Infants/children/adolescents
- Congenital aortic stenosis - Bicuspid leaflet, Williams syndrome
- Congenital subvalvular/ supravalvular aortic stenosis
Young adult to middle-aged
- Calcification and fibrosis of congenitally bicuspid aortic valve
- Rheumatic aortic stenosis
Middle-aged to elderly
- Senile degenerative aortic stenosis (70-90 y/o)
- Calcification of bicuspid valve
- Rheumatic aortic stenosis
- LV becomes increasingly hypertrophied and coronary blood flow may then be inadequate → symptoms of angina
- Eventually pulmonary oedema supervenes.
Typically asymptomatic for many years but deteriorate rapidly when symptoms develop
- Death usually ensues within 3–5 years of these.
Clinical features
- Commonly asymptomatic at routine exams
3 main symptoms: SAD
- Syncope
- Angina
- Dyspnoea
Others:
- Sudden death
- Episodes of acute pulmonary oedema
Harsh ejection systolic murmur
- Radiates to neck
- Soft 2nd HS - particularly if have calcific valves.
- Like saw cutting wood / musical quality / ‘mew’ of seagull
- Crescendo-decrescendo
- Heard best at aortic area and apex
- Ejection click may be heard in young patients with bicuspid aortic valve – NOT in older patients with calcified valves
- May lead to left ventricular hypertrophy with 4th sound at apex
- Post stenotic dilatation of aortic arch
- Slow-rising carotid pulse
- Thrusting apex beat (LV pressure overload)
- Narrow pulse pressure
- Slow rising pulse
- Signs of pulmonary venous congestion (e.g. crepitations)
Investigations
ECG
- Severe - Left ventricular hypertrophy
- QRS complexes in limb leads – increased amplitude
- Very large R wave in V6
- S wave in V2
- ST depression and T wave inversion – Leads II, III, aVF, V5, V6 “strain pattern” in leads reflecting LV
- Absent q waves, p-mitrale
- Left bundle branch block
Chest X-ray
- May be normal
- Enlarged LV
- Dilated ascending aorta - PA view
- Calcified valve – lateral view
ECHO
- Calcified valve
- Hypertrophied LV
Cardiac catheterization
- Measure gradient between LV and aorta
CT & MRI
- Rarely needed
- CT = assess valve calcification / MRI = assess valve stenosis
DDx: hypertrophic cardiomyopathy
Management
- Irrespective of severity, asymptomatic - good immediate prognosis → conservative management
- Moderate/severe → evaluated every 1–2 years with Doppler ECHO
- Symptomatic severe aortic stenosis → prompt aortic valve replacement. // Old age = not contraindication. Good results for people in 80s // Delay → risk of sudden death / irreversible deterioration in ventricular function.
Severe stenosis but deny symptoms → may be due to sedentary lifestyle → exercise test may reveal symptoms on modest exertion.
Aortic balloon valvuloplasty useful in congenital aortic stenosis
- No value in older patients with calcific aortic stenosis.
Anticoagulants - only required in AF, mechanical valve replacement
Stable: TAVI
- Bioprosthetic → no need for anticoagulation
- Mechanical → lasts longer, indicated in younger, need anticoagulation
Aortic regurgitation
- aetiology
- pathophysiology
- presentation
- Examination findings
- investigations
- management
- disease of aortic valve cusps or dilatation of aortic root
Congenital = bicuspid valve or disproportionate cusps
- Rheumatic disease
- Infective endocarditis
- Trauma
- Aortic dilatation (Marfan’s syndrome, aneurysm, dissection, syphilis, ankylosing spondylitis )
- LV dilates and hypertrophies to compensate for regurgitation.
- Aortic arch may become dilated
- Stroke volume of LV may eventually be doubled or trebled → major arteries are then conspicuously pulsatile.
- As progresses, increased L ventricular diastolic pressure and breathlessness develops.
Clinical features Mild to moderate - Often asymptomatic - palpitations - Paroxysmal nocturnal dyspnoea – may be 1st symptom,
Severe = Breathlessness & Angina
- Peripheral oedema
Murmur
- Early diastolic murmur
- Lean patient forward with breath held in expiration
- Best heard to left sternal edge
- May be accompanied by ejection systolic murmur - ‘To and fro’ murmur; Due to increased stroke volume common
If severe → partial closure of anterior mitral leaflet → functional mitral stenosis → soft mid-diastolic (Austin Flint) murmur.
Pulses
- Large-volume or ‘collapsing’ pulse
- Capillary pulsation in nail beds – Quincke’s signs
- Femoral bruit ‘pistol shot’ – Duroziez’s sign
- Head nodding with pulse – de Musset’s sign
Other signs
- Displaced, heaving apex beat (volume overload)
- Pre-systolic impulse
- 4th heart sound
- Crepitations (pulmonary venous congestion)
Investigations
ECG
- left ventricular hypertrophy & T-wave inversion
Chest X-ray
- Cardiac dilatation, maybe aortic dilatation
- Features of left heart failure
ECHO
- Dilated LV; Hyperdynamic LV
- Fluttering anterior mitral leaflet
Doppler ECHO - detects regurgitation
- Severe acute aortic regurgitation → rapid rise in left ventricular diastolic pressure may cause premature mitral valve closure.
Management
- Treat underlying conditions - endocarditis or syphilis.
- Aortic valve replacement indicated if have symptoms
Chronic can remain asymptomatic for years because compensatory ventricular dilatation and hypertrophy occur
- Advise report development of breathlessness or angina.
- Followed up annually - ECHO → ? increasing ventricular size.
Systolic BP controlled with vasodilating drugs e.g. nifedipine or ACEi
If aortic root dilatation is cause of regurgitation (e.g. Marfan’s syndrome) → usually need aortic root replacement
Tricupid stenosis
- aetiology
- presentation
- investigations
- management
Usually rheumatic origin - rare in developed countries
- Nearly always in association with mitral and aortic valve disease.
Tricuspid stenosis & regurgitation → features of carcinoid syndrome
Symptoms of right heart failure - hepatic discomfort & peripheral oedema
- raised JVP - prominent a wave & slow y descent due to loss of normal rapid right ventricular filling
Mid-diastolic murmur
- Best heard at lower left or right sternal edge.
- Generally higher-pitched than murmur of mitral stenosis
- increased by inspiration.
R heart failure → hepatomegaly with pre-systolic pulsation (large a wave), ascites and peripheral oedema.
Doppler ECHO, → valve similar appearances to rheumatic mitral stenosis.
If require surgery to other valves, either tricuspid valve is replaced or valvotomy is performed at surgery.
Balloon valvuloplasty used to treat rare cases of isolated tricuspid stenosis.
Tricuspid regurgitation
- aetiology
- pathophysiology
- signs
- investigations
- management
Usually during systole
- Severely increased RV filling pressure associated with diastolic TR
Most frequently ‘functional’ as a result of right ventricular dilatation
Causes Primary = abnormal valve morphology - Rheumatic heart disease - Endocarditis – esp. IVDU - Ebstein’s congenital anomaly – dysplastic tricuspid valve displaced into RV with consequent enlargement of RA. RV ‘atrialised’. Tricuspid regurgitation and RA-to-LA shunt. Arrhythmias. Surgical repair possible but risky - Marfan’s syndrome - Pacemaker lead entrapment - Valve prolapse - Toxin exposure e.g. fenfluramine - IVDU
Secondary
- Right ventricular dilatation due to chronic left heart failure (‘functional tricuspid regurgitation’)
- Right ventricular infarction
- Pulmonary hypertension (e.g. cor pulmonale)
Pathophysiology
- Can produce reduced cardiac output
Symptoms - usually non-specific
- Tiredness related to reduced forward flow
- Oedema and hepatic enlargement - due to venous congestion.
Signs
- raised JVP = ‘giant’ v wave, (a cv wave replaces normal x descent = y descent).
- Pansystolic murmur at the left sternal edge
- Pulsatile liver
ECHO - dilatation of RV
Tricuspid regurgitation due to right ventricular dilatation often improves when cause of right ventricular overload is corrected → diuretic & vasodilator treatment of congestive cardiac failure.
Patients undergoing mitral valve replacement, who have tricuspid regurgitation due to marked dilatation of tricuspid annulus, benefit from valve repair with an annuloplasty ring to bring leaflets closer together.
Those with rheumatic damage may need tricuspid valve replacement.
Management
- Diuretics for pulmonary congestion
- Valve replacement
Pulmonary stenosis
- etiology
- examination
- investigations
- management
Can occur in carcinoid syndrome but usually congenital (may be isolated or associated with other abnormalities, e.g. Fallot’s tetralogy)
Examination
- Systolic murmur
- Loudest at left upper sternum
- Radiates towards left shoulder
- +/- thrill - best felt when patient leans forward and breathes out
- Murmur often preceded by ejection sound (click)
- Delay in R ventricular ejection may cause wide splitting of second HS
Severe pulmonary stenosis
- Loud harsh murmur
- Inaudible pulmonary closure sound (P2)
- Increased right ventricular heave
- Prominent a 2 waves in jugular pulse
ECG → right ventricular hypertrophy
Chest X-ray → post-stenotic dilatation in pulmonary artery
Doppler ECHO = definitive investigation.
Mild to moderate isolated pulmonary stenosis relatively common - does not usually progress or require treatment.
Severe = (resting gradient > 50 mmHg with a normal cardiac output) - treated by percutaneous pulmonary balloon valvuloplasty or, if this is not available, by surgical valvotomy.
- Long-term results very good
- Post-op pulmonary regurgitation common but benign.
