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1

Coarctation of aorta

definition

aetiology

consequences

associated features

defined as a narrowing of the aorta, usually just distal to the origin of the left subclavian artery, close to the ductus arteriosus/ligamentum arteriosum.

nearly always a congenital lesion but may very rarely be acquired through trauma causing aortic dissection, reducing the diameter of the true lumen of the aorta.

results in hypertension in the upper body and hypoperfusion of the lower body

 often associated with other cardiovascular malformations such as bicuspid aortic valve and ventriculoseptal defect (VSD). It may be associated with another syndrome - for example, Turner's syndrome.

2

coarctation of aorta epidemiology

In adults coarctation is twice as common in men. However, this preponderance is not seen in infants where it is found equally in both sexes.
Affected parents have a higher risk of the abnormality affecting their child

3


coarctation of aorta

LATE presentation

  1.  usually asymptomatic and the diagnosis is usually made on examination, prompted often by the presence of, for example, a murmur or hypertension.
  2.     It can cause headache, nosebleeds and leg cramps, particularly with exercise, although claudication is unusual.
  3.     If the left subclavian circulation is affected the left arm may be smaller.
  4.     Lower-limb muscle weakness, cold feet or neurological symptoms in the legs (poor blood supply to the spinal cord) may be the presenting feature.
  5.     There may be physical features of Turner's syndrome when associated with this condition.
  6.     Pulses distal to the obstruction are diminished and delayed. Simultaneous palpation of upper and lower limb pulses reveals the clinical hallmark of radiofemoral delay with reduced pulse amplitude in the lower limbs.
  7.     BP may be higher in the upper limbs, but the left arm BP can be normal or low if coarctation involves the origin of the left subclavian artery.
  8.     Auscultation reveals a systolic or continuous murmur, usually heard in the left infraclavicular area and under the left scapula. An ejection click may signify associated bicuspid aortic valve (present in about 85% of cases). A thrill or hum due to flow in aberrant collateral vessels may be present over the chest or abdominal wall.

4

aortic coarctation prognosis

  • If untreated, outlook is poor with less than a fifth surviving beyond age 50 years.[2] Even after angioplasty or surgical treatment there are significant morbidity and mortality.[8]
  • The main determinants of long-term survival include factors such as age at operation and degree and duration of hypertension
  • Principal problems are recoarctation, late aneurysm formation, hypertension and/or premature coronary and cerebrovascular disease.
  • Long-term follow-up (with careful monitoring for development of complications and possible ongoing pharmacological therapy) is needed.
  • Patients need to avoid excessively vigorous physical activity, contact sports and exercise that involves straining, such as weightlifting. Those with good repair and BP control can improve their outlook by taking regular gentle aerobic exercise.

5

mitral regurgitation definition

Mitral regurgitation (MR) occurs when the mitral valve does not close properly, causing the abnormal leaking of blood from the left ventricle through the mitral valve and back into the left atrium when the left ventricle contracts.

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mitral regurgitation aetiology


    Primary MR:

  1.         Intrinsic lesions affect one or several components of the mitral valve.
  2.         With the reduced incidence of rheumatic fever, degenerative MR is now the most common cause.
  3.         Acute MR may be caused by papillary muscle rupture, infective endocarditis or trauma.
  4.      Coronary artery disease (papillary muscle dysfunction, chordae tendineae dysfunction or rupture).
  5.  Infective endocarditis.
  6. Following mitral valve surgery surgery, prosthetic mitral valve dysfunction.
  7. Myxomatous degeneration: mitral valve prolapse, Ehlers-Danlos syndrome, Marfan's syndrome.
        Systemic lupus erythematosus (Libman-Sacks lesion), scleroderma.
  8. Cardiac tumours, especially atrial myxoma.
  9.   Acute rheumatic fever.
  10. Acute LV dysfunction.
  11. Congenital heart disease.
  12. Drug-related - eg, ergotamine, methysergide, pergolide.

Secondary MR (unctional MR):

  1.         Valve leaflets and chordae are structurally normal and MR results from distortion of the subvalvular apparatus, secondary to left ventricular (LV) enlargement and remodelling.
  2.         Secondary MR may be due to idiopathic cardiomyopathy or ischaemic heart disease (aka ischaemic mitral regurgitation).

7

Epidemiology of MR

In Europe, MR is the second most frequent valve disease requiring surgery (after the aortic valve).


MR is independently associated with female sex, lower body mass index, advanced age, renal dysfunction, prior myocardial infarction, prior mitral stenosis and prior mitral valve prolapse. It is not related to dyslipidaemia or diabetes.

8


Presentation of mitral regurgitation

 

  •     Acute mitral regurgitation leads to rapid pulmonary oedema which is life-threatening and requires emergency valve repair.
  •     Chronic mitral regurgitation is well tolerated but dilatation of the left ventricle eventually causes heart failure and breathlessness.

Auscultation reveals a pansystolic murmur at the apex.

Acute MR due to papillary muscle rupture should be considered in patients presenting with acute pulmonary oedema or shock following an acute myocardial infarction. However, the murmur may be soft or inaudible.[1]

Chronic MR may remain asymptomatic for many years but patients should be investigated before the onset of disabling dyspnoea.

9

Mitral stenosis

definition

Mitral stenosis occurs when there is obstruction to flow through the mitral valve separating the left atrium and left ventricle of the heart.

    The obstruction occurs due to a structural abnormality of the valve.[1] Mitral stenosis increases left atrial and pulmonary arterial pressure (especially in tachycardia).
    Pulmonary hypertension can lead to right ventricular dilation and tricuspid regurgitation. Right ventricular failure results in raised jugular venous pressure, liver congestion, ascites and peripheral oedema.[1] Left ventricular function and cardiac output can be normal in isolated mitral stenosis.

    Static blood flow in the left atrium (worsened in atrial fibrillation) can cause thromboemboli.

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aetiology of mitral stenosis

  1.     Rheumatic fever (the most common cause).
  2.     Degenerative calcification (can occur in the elderly).
  3.     Congenital mitral stenosis (secondary to parachute mitral valve or Lutembacher's syndrome).
  4.     Inborn errors of metabolism (eg, Hurler-Scheie syndrome, Anderson-Fabry disease).
  5.     Systemic lupus erythematosus.
  6.     Rheumatoid arthritis.
  7.     Carcinoid syndrome.
  8.     Infective endocarditis with large vegetations.
  9.     Amyloid deposition in the mitral valve.

11

Mitral stenosis

presentation

 

Patients with mitral stenosis may feel asymptomatic for years and then present with a gradual decrease in activity.[3]

  1.     Breathlessness: progressive breathlessness is the main symptom. This can include shortness of breath on exertion, orthopnoea and paroxysmal nocturnal dyspnoea. Pulmonary oedema can be triggered by the onset of atrial fibrillation.
  2.     Atrial fibrillation: palpitations due to atrial fibrillation may be the presenting feature.
  3.     Systemic emboli: are a rarer presentation. Stroke, renal failure and myocardial infarction can occur.
  4.     Haemoptysis: this may occur secondary to rupture of the bronchial veins due to raised left atrial pressure.
  5.     During pregnancy: the increase in blood volume may make a previously asymptomatic woman develop symptoms.
     

12

signs of mitral stenosis

  1.     Malar flush on the cheeks. (severe)
  2.     Raised JVP
  3.     Laterally displaced apex beat.
  4.     Right ventricular heave.
  5.     Loud first heart sound with an opening snap in early diastole.
  6.     A mid-late diastolic murmur, best heard, with the patient in the left lateral position, with the bell of the stethoscope
  7.     Atrial fibrillation.
  8.     Signs of right ventricular failure including hepatomegaly, ascites and peripheral oedema.

13

aortic stenosis

definition

The normal aortic valve is composed of three thin cusps that project from the wall at the origin of the aorta. Aortic stenosis (AS) refers to a tight valve. Aortic sclerosis is usually considered to be the precursor of calcified, degenerative AS but sclerosis is rather more common than stenosis.

14

aortic stenosis epidemiology


    AS has become the most frequent type of valvular heart disease in Europe and North America. It most often presents as calcific AS in adults of advanced age (2–7% of the population aged over 65 years).[1]


    The second most frequent cause, which dominates in the younger age group, is congenital. Aortic valve disease occurs in 3 of every 1,000 births.[2]


    Rheumatic AS has become rare in developed countries.

15

aortic stenosis presentation

Symptoms include shortness of breath on exertion, angina, dizziness, or syncope.


The characteristic murmur may occasionally be faint and so the patient may present as heart failure of unknown cause.


The disappearance of the second aortic sound is specific to severe AS, although not a sensitive sign

16

aortic stenosis

signs


    Examination of the pulse in significant AS reveals a slow-rising, flat character called pulsus parvus et tardus.[6]

Blood pressure will show a narrow pulse pressure (difference between systolic and diastolic pressures). In the elderly, a rigid aorta may make this sign less obvious.


    Examination of the cardiovascular system includes palpation of the cardiac apex. There may be a thrill. In regurgitation the left ventricle is enlarged as each beat has to pump the required cardiac output plus that which regurgitates. In AS the left ventricle hypertrophies as more force is required to eject the blood past a tight valve. In laminar flow, the resistance to flow is proportional to the 4th power of the radius so that a small reduction in calibre has a marked effect on resistance.

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aortic stenosis MURMUR

  1.     A2 is soft in AS. In aortic sclerosis, A2 is normal or loud.
  2.     Both conditions are associated with an early, harsh systolic murmur that is transmitted to the carotids. A similar murmur may occur without stenosis if turbulence is due to aortic aneurysm causing dilation of the proximal aorta. Such pathology may cause leakage from the valve too and with it an early diastolic murmur.
  3.     The typical murmur of AS is a crescendo-decrescendo systolic ejection murmur shortly after the first heart sound that ends just before the second heart sound. It is a rough, low-pitched sound that is loudest at the base of the heart and most commonly heard in the second right intercostal space.
  4.      If congestive heart failure leads to a fall in cardiac output, the murmur will be quieter.
  5.     An ejection click may be present, especially with bicuspid valves. Ejection sounds are more obvious in children in whom the valves are more mobile than in older people.
  6.     A fourth heart sound indicates left ventricular hypertrophy (LVH) in severe AS. If the left ventricle dilates and fails, a third heart sound may be heard.

18

DDx of murmurs that appear to be from the aortic valve

Other causes of murmurs that are, or appear to be, from the aortic valve include:

    Aortic sclerosis.
    Aortic regurgitation.
    Subacute bacterial endocarditis.
    Dilatation of the root of the aorta (may also lead to a leaking valve).
    Flow murmurs (turbulence from high cardiac output in anaemia, thyrotoxicosis and marked aortic regurgitation).
    Murmurs originating from the pulmonary valve with disease of that valve or atrial septal defect (the pulmonary and aortic areas are very close)

19

mitral valve prolapse definition

Mitral valve prolapse (MVP) is an abnormal bulging of one or both of the mitral valve leaflets into the left atrium during ventricular systole.

20


pulmonary valve disease

description

The pulmonary valve normally has three cusps, and is responsible for regulating the flow of deoxygenated blood from the right ventricle to the lungs. Pulmonary valve disease is very uncommon and can be due either to stenosis or to insufficiency. The majority of pulmonary valve problems occur as the result of congenital heart disease but the pulmonary valve may rarely be damaged as result of infection (eg, infective endocarditis) or as a result of disease (eg, carcinoid disease, Marfan's syndrome or pulmonary hypertension)

21

Pulmonary stenosis

presentation and diagnosis


    PS may be diagnosed in utero or shortly after birth. Critical PS causes cyanosis and is potentially lethal in the neonate.
    Ultrasound images of the four chambers of the heart in utero may demonstrate isolated PS due to an abnormally thickened or bicuspid valve, or PS in association with other congenital anomalies - eg, Fallot's tetralogy, Noonan's syndrome, or as a result of intrauterine infection with rubella.
    In infancy the condition is usually diagnosed by auscultation of the heart and the presence of a murmur.

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Symptoms and signs in

PULMONARY STENOSIS

Symptoms

The symptoms of PS will vary with the severity of the stenosis. Mild PS may be asymptomatic.

  1.     Shortness of breath.
  2.     Chest pain.
  3.     Fainting or exertional syncope.
  4.     Sudden death.

Signs

    Soft pulmonary systolic murmurs are more easily heard with the patient lying down. They are often heard in healthy individuals and may be due to physiological changes associated with respiration.

  1.     Ejection systolic murmur along the left upper edge of the sternum.
  2.     Pulmonary ejection click.
  3.     Delayed 2nd heart sound with severe stenosis.
  4.     Parasternal thrill and heave.
  5.     'A waves' in the JVP.

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PULMONARY HYPERTENSION

Idiopathic pulmonary arterial hypertension is rare -  a sustained elevation in pulmonary artery pressure and pulmonary vascular resistance, with normal pulmonary artery wedge pressure, in the absence of a known cause. It is a diagnosis of exclusion after other possible causes of pulmonary hypertension have been excluded. It is a severe and often rapidly progressive illness in many cases.

The injury to the pulmonary endothelium causes a tendency to in situ thrombosis in the pulmonary arterial tree, the so-called thrombotic pulmonary arteriopathy.

The disease process continues through vascular scarring, endothelial dysfunction and proliferation of smooth muscle cells within the intima and media of the pulmonary arterial tree, causing progressive pulmonary arterial hypertension.

This stage is known as plexogenic pulmonary arteriopathy and causes intimal fibrosis replacing normal endothelial structure.[1] This leads to progressive right heart strain due to obliteration of small pulmonary arterial vessels, and eventually right heart failure.[2]

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definition of pulmonary hypertension

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure (PAP) ≥25 mm Hg at rest as assessed by right heart catheterisation.

25

Classification of pulmonary hypertension

  1. Pulmonary arterial hypertension (PAH)
  2. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis.
  3. PH due to left heart disease: systolic dysfunction, diastolic dysfunction, valvular disease.
  4. PH due to lung diseases and/or hypoxia
  5. Chronic thromboembolic pulmonary hypertension.
  6. PH with unclear and/or multifactorial mechanisms

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Aetiology of pulmonary arterial hypertension


   1.  Idiopathic.


   2. Heritable:

  •         There is a small subset (~6%) of cases that are inherited in an autosomal dominant fashion due to mutations in the BMPR2 gene (receptor in TGF-beta family).[4]
  •         Endoglin (with or without hereditary haemorrhagic telangiectasia).

   3. Drug- and toxin-induced.


4.    Associated with:

  •         HIV infection, portal hypertension, congenital heart disease, schistosomiasis, chronic haemolytic anaemia.
  •         There is also a relatively high rate in certain connective tissue disorders such as the Calcinosis, Raynaud's syndrome, (O)Esophageal dysmotility, Sclerodactyly and Telangiectasia (CREST) syndrome, progressive systemic sclerosis, Sjögren's syndrome, rheumatoid arthritis, systemic lupus erythematosus (SLE), mixed connective tissue disorder and polymyositis/dermatomyositis.[5]

    5. Persistent pulmonary hypertension of the newborn.

27

Pulmonary hypertension due to lung disease or hypoxia

aetiology

  1. COPD
  2.    Interstitial lung disease.
  3.     Other pulmonary diseases with mixed restrictive and obstructive pattern.
  4.     Sleep-disordered breathing.
  5.     Alveolar hypoventilation disorders.
  6.     Chronic exposure to high altitude.
  7.     Developmental abnormalities.

28

PH

risk factors

Risk factors

    Female gender (F:M ratio variable at 2-9:1, depending on particular centre surveyed).[1]
    Tends to affect women of childbearing age - may rarely affect older women.
    May also rarely affect children.
 

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PH

presentation

signs

symptoms

Presentation

The illness can have a very insidious onset and be hard to notice for both patients and doctors; as it cannot be diagnosed on clinical grounds alone and requires detailed investigation, it is often diagnosed late, when useful therapeutic options may be limited. It may take up to two years to diagnose the condition and it can progress very quickly in some cases, so it presents a significant challenge in terms of early detection.[1]


Symptoms

  1.     Dyspnoea.
  2.     Generalised weakness and tiredness.
  3.     Recurrent syncope or presyncope, often related to exertion.

Signs

  1.     Right ventricular (parasternal) heave may be visible/palpable.
  2.     JVP may be elevated with prominent a and v waves.
  3.     Loud pulmonary component of S2
  4.     May be fixed or paradoxical splitting of second heart sound - very difficult to detect for most clinicians.
  5.     Murmur of pulmonary regurgitation - Graham Steell murmur.
  6.     Tricuspid regurgitation murmur - if significant right ventricular hypertrophy and dilatation are present.
  7.     If significant associated tricuspid regurgitation - enlarged pulsatile liver with exaggerated hepato-jugular reflux.
  8.     Ascites and peripheral oedema - if there is significant right ventricular failure.
  9.     Lung fields are usually clear.

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DDX for pulmonary hypertension

  1.     Cor pulmonale causing secondary pulmonary hypertension.
  2.     Cardiomyopathies.
  3.     Primary right ventricular failure - eg, following myocardial infarction.
  4.     Congestive cardiac failure.
  5.     Recurrent pulmonary emboli.
  6.     Mitral or tricuspid stenosis.
  7.     Tricuspid regurgitation.
  8.     Pulmonary stenosis.
  9.     Portal hypertension.
  10.     Obstructive sleep apnoea.
  11.     Hypothyroidism.
  12.     Sickle cell disease.[6]

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Complications of PH

  1. Deteriorating right heart function and right-sided cardiac failure.
  2. Gross peripheral oedema.
  3. Hepatic congestion and cardiac cirrhosis.
  4. Pleural effusions.
  5. Gross exertional dyspnoea.
  6. Exertional syncope.
  7. Sudden cardiac death.
  8. Problems during childbirth including sudden death.

32

Prognosis of PH

  1.     For untreated PPH, the estimated three-year survival rate is approximately 41%.
  2.     One study of long-term continuous intravenous prostacyclin therapy found that three-year survival increased to approximately 63%.[1]
  3.     One meta-analysis showed a reduction in mortality of 43% in response to active treatment.[18]
  4.     The advent of pulmonary vasodilators has improved the outlook for children.[19]
  5.     Patients who do not respond to medical therapy tend to develop progressive right-sided heart failure if transplantation cannot be carried out.