Cardiology

Valvular heart disease

Aortic valve

The aortic valve has three leaflets, each having a cusp or cup-like configuration. These are known as the left coronary cusp (L), the right coronary cusp (R) and the posterior non-coronary cusp (N). Just above the aortic valves there are anatomic dilations of the ascending aorta, also known as the sinus of Valsalva. The ostia for the coronary arteries lie just above the valve.

Aortic Valve Pathology

  • Bicuspid valve
  • Aortic stenosis
  • Aortic regurgitation
  • Supravalvular stenosis

Aortic regurgitation

Introduction

  • 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.

Causes

  • Valve damage
    • Rheumatic heart disease
    • Infective endocarditis
    • Degeneration
    • Bicuspid valve.
  • Root dilation
    • Hypertension
    • Marfan's disease
    • Connective tissue disease
    • Ankylosing spondylitis
    • Systemic lupus erythematosus
    • Rheumatoid arthritis
    • Aortic dissection
  • AR is commoner in males

Clinical

  • 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

Investigations

  • 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

Management

  • 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.

Aortic Stenosis

Introduction

  • Narrowing of the aortic valve can lead to syncope and chest pain which is readily treatable

Aetiology

  • 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

Causes

  • 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

Clinical

  • 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)

Investigations

  • 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.

Management

  • 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

Mitral valve

  • 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.

Mitral Stenosis

Introduction

  • Progressive breathlessness.
  • Commoner in Women.

Aetiology

  • 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

Causes

  • Rheumatic heart disease 99%
  • Calcification of valve apparatus seen in elderly
  • Congenital

Clinical

  • 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
    • Pregnancy
    • Onset of AF
    • Fluid overload
    • Fever /Tachycardia

Investigations

  • 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

Management

  • Diuretics e.g. IV/oral furosemide.
  • Fast AF (rate control to prolong diastolic filling)
    • Medical - Digoxin, Beta blockers, Verapamil
    • DC cardioversion
  • Warfarin for AF.
  • Surgery
    • Mitral commissurotomy
    • Valvuloplasty
    • Mitral Valve replacement.
  • Oral penicillin if past rheumatic fever to prevent recurrent rheumatic fever if age < 30

Mitral regurgitation

Introduction

  • Common valve disease with multiple causes
  • Severe Acute MR will lead to rapid cardiac decompensation

Aetiology

  • 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

Causes

  • Valve
    • Rheumatic heart disease
    • Endocarditis
    • Myxomatous degeneration
    • Marfan's and Ehlers-Danlos syndrome
    • Osteogenesis imperfecta.
  • Valve ring
    • Functional (LV dilates)
    • Mitral valve prolapse
    • Connective tissue disease
    • Trauma
    • Hypertrophic Cardiomyopathy (HCM).
  • Papillary muscle
    • Myocardial infarction - inferior wall

Clinical

  • 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.

Investigations

  • FBC, CRP - elevated always consider endocarditis
  • Echocardiogram shows reflux of blood into MR.
  • Cardiac catheterisation

Management

  • 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
  • Surgery
    • Mitral valve repair and reconstruction is preferable.
    • Mitral valve replacement if end systolic dimension > 50 mmHg or LVEF < 50%

Pulmonary Stenosis

Introduction

  • Rare but with multiple causes often associated with a more extensive congenital syndrome

Causes

  • Congenital - Fallot's Tetraology, Turner's syndrome, Noonan syndrome
  • William's syndrome (high calcium + supraaortic stenosis, elfin facies)
  • Acquired - Rheumatic heart disease, Carcinoid syndrome

Clinical

  • 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

Investigations

  • 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.

Management

  • Medical treatment with Diuretics
  • Surgery e.g. Percutaneous pulmonary valve replacement or Pulmonary valvotomy and/or infundibular resection is advanced cases

Pulmonary regurgitation

Introduction

  • Rarely problematic
  • Valve ring dilates due to Pulmonary hypertension

Causes

  • Pulmonary hypertension (With Mitral stenosis known as Graham Stell murmur)

Clinical

  • Usually asymptomatic
  • Clinical EDM, Normal or reduced volume pulse
  • Signs of pulmonary hypertension

Investigations

  • ECG
  • Transthoracic Echocardiogram

Management

  • Treatment is rarely needed

Tricuspid Stenosis

Introduction

  • 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

Causes

  • Rheumatic disease, Carcinoid, Congenital disease
  • A right atrial myxoma could mimic TS

Clinical

  • 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

Investigations

  • ECG tall P waves > 3mm in lead II
  • CXR - RA Enlargement
  • Echo - Right atrial enlargment and stenosed valve

Management

  • Medical - Diuretics + Salt restriction, AF - Warfarin
  • Surgery e.g. Tricuspid valvotomy or Valve replacement in severe cases

Tricuspid regurgitation

Introduction

  • Usually functional due to RV enlargement causing reflux of RV blood into RA

Causes

  • 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

Clinical

  • 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

Investigations

  • ECG - AF
  • CXR - cardiomegaly

Management

  • Medical - Diuretics and digoxin can improve functional disease
  • AF - consider warfarin
  • Surgery - Valve replacement in severe cases
  • Annuloplasty has poor results long term.

Aortic Sclerosis

Introduction

  • Degeneration of aortic valve leads to audible turbulence

Clinical

  • A loud ejection systolic murmur.
  • Normal pulse and pulse pressure.
  • No related symptoms

Investigations

  • ECG - Normal
  • CXR - normal or some calcification
  • Echo - may show some changes around the valve. No significant gradient

Management

  • Reassurance

Carcinoid heart disease

Introduction

  • High levels of serotonin (5HT) cause fibrosis of leaflets and chordae

Clinical

  • Tricuspid valve disease
  • Pulmonary stenosis
  • Mitral stenosis if PFO and shunt

Management

  • Treat Carcinoid tumour

Mitral Valve prolapse

Introduction

  • Click murmur syndrome
  • Traditional 2D echo led to much over-diagnosis

Causes

  • 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

Clinical

  • 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

Investigations

  • ECG - non specific changes
  • CXR - normal
  • Echocardiogram shows bulging of mitral leaflets past the plane of the valve ring into the left atrium

Management

  • 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