Valvular heart disease
Aortic Valve Pathology
- Bicuspid valve
- Aortic stenosis
- Aortic regurgitation
- Supravalvular stenosis
- Aortic valve is incompetent so ejected aortic blood refluxes back into the left ventricle immediately
- This adds to LV diastolic filling creating a volume overloaded left ventricle.
- Valve damage
- Rheumatic heart disease
- Infective endocarditis
- Bicuspid valve.
- Root dilation
- Marfan's disease
- Connective tissue disease
- Ankylosing spondylitis
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Aortic dissection
- AR is commoner in males
- Acute severe progression usually with endocarditis or trauma or Type A dissection
- Chest pain: get urgent TOE or CT Aorta to look for a dissection
- Fever, acute onset: Blood cultures and urgent TTE/TOE for endocarditis
- Progressive breathlessness, palpitations, Sudden death.
- Large volume collapsing pulse with wide pulse pressure - water hammer.
- High pitched early diastolic murmur best heard at LSE sitting forwards in full expiration.
- Possible added systolic aortic flow murmur + S3.
- Austin flint murmur - low pitched diastolic AMVL vibrations
- Hyperdynamic signs
- Corrigan's sign - visible carotid pulsation.
- DeMussets - head nodding,
- Quincke's - nailbed pulsations on mild pressure on nail
- Traube's - pistol shot femorals
- Duroziez's - femoral diastolic murmur
- FBC, U&E, CRP, ESR
- Blood cultures if endocarditis suspected
- Cardiomegaly - aortic root dilation, LV volume overloaded.
- Elevated WCC/CRP suggests endocarditis and blood cultures
- TTE and TOE needed urgently if endocarditis suspected
- Cardiac catheterisation and angiography if valve replacement considered
- TOE / CT aorta if dissection suspected
- Is there endocarditis ? Is there a proximal aortic root dissection ?
- Vasodilator therapy e.g. ACE, ARB
- Nifedipine to try to reduce afterload
- Diuretics and Digoxin and consider warfarin if AF.
- Aortic Valve replacement if end systolic diameter > 50 mm or signs of LV dysfunction.
- Do not use Intra-aortic balloon pumping as this just worsens reflux.
- Narrowing of the aortic valve can lead to syncope and chest pain which is readily treatable
- Normal orifice is 3-4 cm2 but this is reduced and the LV needs to work hard to generate high pressures and develops concentric hypertrophy to overcome the stenosis.
- Eventually this will lead to ventricular dilation and failure. There is
a large blood pressure drop across valve - this is the gradient.
- The gradient will fall if the LV begins to fail. Pressure overloaded Left ventricle
- Congenital Bicuspid valve
- Rheumatic heart disease
- Senile Degeneration.
- William's syndrome (high calcium + supraaortic stenosis, elfin like face).
- By age
- Age > 80:Senile
- Age 50-80:RHD
- Age < 50: Congenital
- Angina - may be due to ischaemia due to high wall tension rather than Coronary disease.
- Dizziness and Syncope with exertion
- Breathlessness - suggests failing LV
- Endocarditis, Arrhythmias, Sudden cardiac death
- Symptoms mean surgery required
- Slow rising plateau pulse (pulsus tardus et parvus)
- Narrow pulse pressure
- Ejection systolic thrill and murmur
- Ejection click, Apex - Sustained forceful, Soft A2, Audible S4 (SR)
- ECG - SR Left atrial enlargement, LVH + strain, LBBB, AV block.
- CXR - normal heart size until LV begins to fail and dilate + post stenotic dilatation of aorta.
- Echo - LVH, thickened cusps, reduced mobility. Aortic area < 1cm2. Peak gradient > 50 mmHg is significant and suggests moderate stenosis.
- Coronary angiogram in those over 40 to look for Coronary artery disease. Valve replacement may be combined with CABG.
- Avoid exercise if symptoms or heart failure
- Ensure Endocarditis prophylaxis.
- Avoid vasodilators - ACEI, ARB etc in significant disease which will reduce preload and ventricular volume
- Tissue or Metal Valve replacement in all with symptoms or very high gradients without symptoms
- Aortic balloon valvuloplasty is a short term solution in those not fit for valve replacement
- Replace aortic valve before
decline in LV function
The mitral valve is bicuspid with a larger anterior valve leaflet and smaller
posterior mitral valve leaflet.
- It separates the left atrium and left ventricle preventing reflux of blood into the left atrium during ventricular systole.
- Progressive breathlessness.
- Commoner in Women.
- The normal valve cross sectional area is 4-6 cm2 is reduced to 1-2 cm2
- Narrowing obstructs and impairs LV inflow and raises Left atrial pressure
- LA dilates. AF. Pulmonary hypertension develops
- The Left ventricle is uninvolved
- Rheumatic heart disease 99%
- Calcification of valve apparatus seen in elderly
- Commoner in women. Fatigue, Exertional Dyspnoea
- PND, Palpitations, Haemoptysis, Thromboembolism
- Hoarseness (Ortner's syndrome) due to pressure on recurrent laryngeal nerve by enlarged LA
- Dysphagia (local pressure of enlarged LA)
- Frank pulmonary oedema
- Atrial fibrillation, Low volume pulse, Mitral facies, cardioembolic thrombus
- Loud S1 and tapping apex beat, loud opening snap
- Mid diastolic low pitched rumbling murmur louder with exercise.
- Ask patient to touch toes 5 times and listen if suspicious
- Presystolic accentuation(SR), Loud P2, Closer the A2-OS the more severe the disease
- Pulmonary hypertension - RV lift, Loud P2, TR
- Acute deterioration
- Onset of AF
- Fluid overload
- Fever /Tachycardia
- ECG - AF and P mitrale, RVH
- CXR - normal sized LV and straight left heart border due to increased Left atrial size, MV calcification, Pulmonary oedema.
- Echocardiogram - fish mouth appearance, Severe MS - Reduced MV area to 1 cm2
- Catheterisation - raised Left atrial pressure with pulmonary hypertension
- Diuretics e.g. IV/oral furosemide.
- Fast AF (rate control to prolong diastolic filling)
- Medical - Digoxin, Beta blockers, Verapamil
- DC cardioversion
- Warfarin for AF.
- Mitral commissurotomy
- Mitral Valve replacement.
- Oral penicillin if past rheumatic fever to prevent recurrent rheumatic fever if age < 30
- Common valve disease with multiple causes
- Severe Acute MR will lead to rapid cardiac decompensation
- Ventricular systole results in reflux of blood back into the left atrium during systole due to impaired valve function due to a defect due to dilation of valve ring or abnormal valve or papillary muscle function
- Acute MR leads to a more acute and severe deterioration than chronic MR where there is time for the LA to dilate protecting the pulmonary circulation from the increased back pressure and volume changes
- Rheumatic heart disease
- Myxomatous degeneration
- Marfan's and Ehlers-Danlos syndrome
- Osteogenesis imperfecta.
- Valve ring
- Functional (LV dilates)
- Mitral valve prolapse
- Connective tissue disease
- Hypertrophic Cardiomyopathy (HCM).
- Papillary muscle
- Myocardial infarction - inferior wall
- Dyspnoea, Palpitations, Fatigue, Stroke
- Atrial fibrillation, Displaced hyperdynamic apex
- PSM at apex often radiates to axilla and S3
- Soft S1 and Loud P2 and pulmonary hypertension with EDM.
- FBC, CRP - elevated always consider endocarditis
- Echocardiogram shows reflux of blood into MR.
- Cardiac catheterisation
- Standard Treatment of heart failure, reduce afterload to encourage forward flow. Diuretics, ACEI/ARB, Digoxin.
- Anticoagulate if in AF and consider digoxin or other anti arrhythmic for rate control
- Always ensure Endocarditis prophylaxis
- Mitral valve repair and reconstruction is preferable.
- Mitral valve replacement if end systolic dimension > 50 mmHg or LVEF < 50%
- Rare but with multiple causes often associated with a more extensive congenital syndrome
- Congenital - Fallot's Tetraology, Turner's syndrome, Noonan syndrome
- William's syndrome (high calcium + supraaortic stenosis, elfin facies)
- Acquired - Rheumatic heart disease, Carcinoid syndrome
- Dyspnoea and Fatigue, RV failure
- Oedema, Failure to thrive in children
- Cyanosis if ASD or PFO, Prominent 'a' wave
- Ejection systolic murmur 2nd LICS Increases on inspiration
- Ejection click, Soft P2
- ECG P pulmonale, RAD, RVH, RBBB
- CXR - post stenotic dilatation of PA, large RA and RV, Pulmonary oligaemia
- Echocardiogram - Doppler gradient across the Pulmonary valve
- Cardiac catheterisation - determine size of PA and look for ASD, VSD, PFO.
- Medical treatment with Diuretics
- Surgery e.g. Percutaneous pulmonary valve replacement or Pulmonary valvotomy and/or infundibular resection is advanced cases
- Rarely problematic
- Valve ring dilates due to Pulmonary hypertension
- Pulmonary hypertension (With Mitral stenosis known as Graham Stell murmur)
- Usually asymptomatic
- Clinical EDM, Normal or reduced volume pulse
- Signs of pulmonary hypertension
- Transthoracic Echocardiogram
- Treatment is rarely needed
- There is impaired filling of RV due to a narrowed tricuspid valve.
- It is commoner in females (usually coexists with rheumatic mitral valve disease)
- Left heart rheumatic valve disease may be the predominant symptomatic lesion
- Rheumatic disease, Carcinoid, Congenital disease
- A right atrial myxoma could mimic TS
- Fatigue, Oedema, Ascites, AF
- JVP - Large 'a' wave in JVP (unless AF), Slow 'y' descent in JVP
- Loud S1, Low pitched tricuspid MDM + PSA at LSE and loudest with inspiration
- Right heart failure - pulsatile hepatomegaly
- Jaundice, Ascites, Pedal oedema
- ECG tall P waves > 3mm in lead II
- CXR - RA Enlargement
- Echo - Right atrial enlargment and stenosed valve
- Medical - Diuretics + Salt restriction, AF - Warfarin
- Surgery e.g. Tricuspid valvotomy or Valve replacement in severe cases
- Usually functional due to RV enlargement causing reflux of RV blood into RA
- Functional, Ischaemic heart disease, Cor pulmonale,
- Pulmonary hypertension primary/secondary
- Valve damage e.g. Rheumatic heart disease, Staphylococcal Endocarditis in IV drug users, Carcinoid
- Ebstein's anomaly
- Fatigue, Hepatic RUQ discomfort
- Atrial fibrillation, Ascites, Ankle oedema,
- JVP Systolic V wave in JVP, Rapid y descent
- RV heave, Inspiratory PSM at LSE
- Pulsatile liver, Ankle oedema, Ascites, Peripheral cyanosis
- ECG - AF
- CXR - cardiomegaly
- Medical - Diuretics and digoxin can improve functional disease
- AF - consider warfarin
- Surgery - Valve replacement in severe cases
- Annuloplasty has poor results long term.
- Degeneration of aortic valve leads to audible turbulence
- A loud ejection systolic murmur.
- Normal pulse and pulse pressure.
- No related symptoms
- ECG - Normal
- CXR - normal or some calcification
- Echo - may show some changes around the valve. No significant gradient
Carcinoid heart disease
- High levels of serotonin (5HT) cause fibrosis of leaflets and chordae
- Tricuspid valve disease
- Pulmonary stenosis
- Mitral stenosis if PFO and shunt
- Treat Carcinoid tumour
Mitral Valve prolapse
- Click murmur syndrome
- Traditional 2D echo led to much over-diagnosis
- Congenital - 2% of men and 5% of women
- Autosomal dominant trait
- Connective tissue disease e.g. Marfan's syndrome
Complications - some may just represent population levels
- Endocarditis - give antibiotics
- Sudden cardiac death - very rare
- Stroke - very rare
- Mitral regurgitation may develop
- AF and other arrhythmias - rare
- Nonspecific - causality difficult to prove.
- Dyspnoea, chest pain and palpitations all reported.
- Mid systolic click and mid to late systolic murmur which increases on standing and with valsalva which both reduce preload
- ECG - non specific changes
- CXR - normal
- Echocardiogram shows bulging of mitral leaflets past the plane of the valve ring into the left atrium
- Endocarditis prophylaxis for those with associated MR has been reviewed and now only those with a prosthetic valve or history of endocarditis need them (AHA recommendations)
- Beta blockers
- Warfarin if AF develops