Pulmonary Hypertension

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Pulmonary Hypertension

• Presented by
• Alaa AL-JEFRI..
• RWAYDA SAAD..
• FADAK JISHI..

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Definition

• Pulmonary hypertension (PH) defined as a mean
  pulmonary arterial (PA) pressure of greater than
  25 mmHg at rest or greater than 30 mmHg during
  exercise.
• It is characterized by a progressive and
  sustained increase in pulmonary vascular
  resistance that eventually leads
• to right ventricular (RV) failure.

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Causes of PH

• Pulmonary vascular disorders
• - Acute pulmonary thromboembolism, Primary
  pulmonary
• hypertension, Multiple pulmonary artery
  stenosis.
• Diseases of the lung and parenchyma
• - COPD
• Musculoskeletal disorders (causing chronic
  underventilation)
• - Kyphoscoliosis, Poliomyelitis,
  Myasthenia gravis.

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Cont..

• Disturbance of respiratory control
• - Obstructive sleep apnea, Morbid obesity,
  
• Cerebrovascular disease.
• Cardiac disorders
• - Mitral stenosis, Left ventricular
  failure.
• Miscellaneous
• - Appetite-suppressant drugs, (e.g.
  dexfenfluramine),
• Rheumatic autoimmune disease, (e.g.
  SLE), Sickle
• cell disease.

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Risk Factors

• The following factors increase the chance of
  developing PH
• Smoking
• Asthma or other chronic lung disease
• Recurring pulmonary emboli
• Obstructive sleep apnea
• Obesity
• Low thyroid (myxedema)
• Certain congenital and valvular heart conditions
• Muscle weakness diseases
• Home at high altitude (over 10,000 feet)
• Pectus excavatum or other severe chest deformity
  (eg, kyphoscoliosis)

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The causes of pulmonary hypertension can be
subdivided depending on whether their effects on
the lung circulation are

• Precapillary (in the pulmonary arteries and
  arterioles) e.g.
• - Primary pulmonary hypertension.
• - Congenital heart with Eisenmenger's
  syndrome.
• - Thromboembolic.
• Capillary disorders (causing damage to the
  alveolar capillary mechanisms) e.g.
• - Parenchymal lung diseases (e.g emphysema
  and pulmonary fibrosis
• hypoxia vasoconstriction
  PH, also they lead to decrease surface
• area of pulmonary vascular bed).
• Postcapillary (i.e lesions distal to the
  pulmonary capillary bed) e.g.
• - Mitral valve disease.
• - Left sided failure.
• - Pulmonary veno-occlusive disease.

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Pathophysiology

• Whatever the initial cause, pulmonary
  Hypertension results when the blood vessels
  constrict . Over time this constriction causes
  fibrosis of the vessel and higher pulmonary blood
  pressure.
• The chambers on the right side of the heart
  (right atrium and right ventricle) have
  difficulty pumping blood out to the pulmonary
  artery and through the lungs.
• The strain on the heart to overcome this high
  pressure and constriction causes the heart to
  become enlarged and weak.
• Eventually the heart cannot keep up with the
  bodys demands. This is known as heart failure.

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Cont..
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Pulmonary Hypertension can be classified into two
groups
2. Secondary Pulmonary Hypertension -
Pulmonary Hypertension that is associated with or
the result of another medical condition and it is
more common than PPH. .
1) primary pulmonary hypertension (idiopathic)
- not caused by any other disease or condition.
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Primary pulmonary hypertension

• Primary pulmonary hypertension is a condition of
  unknown cause characterized by clinical,
  radiological and electrocardiographic evidence of
  pulmonary hypertension and increased pulmonary
  artery pressure (PAP) and pulmonary vascular
  resistance (PVR) with normal Pulmonary capillary
  wedge pressure (PCWP).

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Incidence

• It is estimated that one to two individuals per
  million per year are diagnosed with PPH compared
  to five to six individuals per million per year
  with pulmonary hypertension secondary to
  rheumatic autoimmune disease.
• There is an unexplained predominance of women
  mainly presenting in the third decade and men in
  the fourth.
• Approximately 612 of cases are believed to have
  a familial origin with inheritance in an
  autosomal dominant fashion.

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CLINICAL PICTURE
Patients with pulmonary hypertension have an
insidious onset and indeed often their condition
comes to the attention of the physician late in
the course of their illness or when symptoms of
right ventricular failure develop. Physical
examination may reveal findings consistent with
pulmonary hypertension and right ventricular
overload. Clinical signs of right ventricular
dysfunction may be present.
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CLINICAL PICTURE
Pulmonary hypertension lead to dyspnea initially
on exertion and later at rest. Dull, retrosternal
chest pain resembling angina may be
present. Syncope or near syncope due to fixed
cardiac output.
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On examination

• Inspection- Prominent a wave in JVP
• - Cyanosis as a result of markedly reduced
  cardiac output.
• Palpation- Left parasternal heave due to right
  ventricular hypertrophy.
• - Systolic pulsation in 2nd left intercostal
  space due to dilated pulmonary artery.

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On examination
.
Auscultation - Loud pulmonary competent of
2nd heart sound - Systolic ejection click
and flow murmur in pulmonary area. - Right
ventricular S4 - In advanced cases,
tricuspid and pulmonary regurgitation and signs
of right heart failure are found.
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Investigations

• Blood Tests
• Arterial blood gas determinations should be
  performed in (PH) patients to assess for
  hypoxemia.
• A collagen-vascular disease screening should be
  performed. This includes measuring the
  erythrocyte sedimentation rate, rheumatoid factor
  levels, and antinuclear antibody levels.
• Synthetic liver function test results (ie,
  albumin levels, prothrombin time, bilirubin
  levels) may indicate liver disease associated
  with portal hypertension.
• HIV testing and hepatology serology tests should
  be performed on patients at risk.
• A complete blood cell count, biochemistry panel,
  prothrombin time, and activated partial
  thromboplastin time should be performed at
  baseline.

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• Imaging Studies
• Chest X-ray may demonstrate enlargement of the
  pulmonary arteries and the major branches, with
  marked tapering (pruning) of peripheral arteries.
  The lung fields are usually lucent and there may
  be right atrial and right ventricular
  enlargement.
• Chest radiograph of a patient with secondary
  pulmonary hypertension shows
• enlarged pulmonary arteries. This
  patient had an atrial septal defect.

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Cont..

• Chest CT scan Looks for blood clots and other
  lung conditions that may be contributing to or
  worsening pulmonary hypertension.
• Right heart catheterization Measures various
  heart pressures (ie, inside the pulmonary
  arteries, coming from the left side of the
  heart), the rate at which the heart is able to
  pump blood, and finds any leaks between the right
  and left sides of the heart.
• Echocardiography demonstrates enlarged right
  ventricular dimensions.

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Cont..

• Other tests
• ECG may show right ventricular hypertrophy and
  right atrial enlargement (P pulmonale).
• Pulmonary function tests should be done to
  exclude an underlying pulmonary disorder.
• 6 minute walk test Determines exercise tolerance
  level and blood oxygen saturation level during
  exercise.

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Management

• Since pulmonary hypertension is a condition
  caused by a heterogeneous group of diseases,
  treatment is directed at the primary cause, e.g.
• chest physiotherapy, good nutrition and early
  antibiotics to treat infection in patients with
  cystic fibrosis and bronchiectasis.
• Surgical or medical therapies to optimize cardiac
  and respiratory function,
• supplemental oxygen and anticoagulation are
  necessary together with newer specific therapies
  designed at modulating pulmonary vasomotor tone.
• Patients who have symptoms resulting in marked
  limitation of physical activity or those who are
  symptomatic at rest have a poor median survival
  (32 and 6 months, respectively).
• Adverse factors include right ventricular
  dysfunction and reduced 6-minute walk distance.

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Treatment

• The treatment for secondary pulmonary
  hypertension usually begins with treating the
  underlying cause. For instance, if a blood clot,
  or pulmonary embolism, is causing the
  hypertension, the clot may need to be surgically
  removed.
• Several treatments are used to manage primary
  pulmonary hypertension, although they don't cure
  the condition. These include
• Calcium Channel Blocking Drugs -- These help the
  heart pump better by relaxing muscles in the
  walls of blood vessels.
• Endothelin Receptor Antagonists (ERAs) -- These
  medications reverse the effects of endothelin, a
  substance in blood vessels that causes
  constriction.
• Continuously Infused Epoprostenol (Flolan) --
  This drug dilates and reverses thickening of the
  blood vessels of the lungs, and helps prevent
  platelets from clotting the blood. It has to be
  administered continuously via an implanted
  catheter and a portable, battery operated pump.
  The drug lasts less than six minutes, which is
  why it must be infused continuously into the
  bloodstream.

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Cont..

• Anticoagulants -- These drugs help keep blood
  from clotting, which makes it flow better.
• Diuretics -- Prescribed to minimize water
  retention, a condition that makes the heart work
  harder.
• Digoxin -- This drug can help the right side of
  the heart pump better.
• Lifestyle changes -- Adequate rest, a healthy
  diet, exercise and stress reduction can help
  control the disease. People with pulmonary
  hypertension should avoid smoking, pregnancy,
  birth control pills and high altitudes.
• Supplemental Oxygen This is used for patients
  with low oxygen level in the blood.
• Lung or Heart-Lung Transplantation -- These
  procedures are employed for patients with severe
  pulmonary hypertension and extremely poor quality
  of life. It is the procedure of last resort.

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Prognosis

• Several studies have reported a mean survival of
  23 years from the time of diagnosis.
• The cause of death is usually right ventricular
  failure or sudden death. Increased right atrial
  pressure above 15 mmHg and cardiac index below 2
  L/min/ m2 are haemodynamic predictors of poor
  prognosis.
• Heart and lung transplantation is used.

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Chronic Cor Pulmonale
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Definition
But still, presence of right ventricular failure
alone is not enough to diagnosis cor pulmonale.
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PathophysiologyCOPD is illustrative
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Clinical features

• Chest pain in the front of the chest
• exertional dyspnoea
• Syncope
• fatigue
• sudden death can occurs

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• systolic pulmonary ejection click
• mid-systolic ejection murmur
• early diastolic murmur due to pulmonary
  regurgitation (Graham Steell murmur).

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prominent 'a' wave in the jugular venous pulse.
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tricuspid regurgitationpan-systolic murmur and
a large jugular 'cv' venous wave.
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Investigations

• Chest X-ray
• right ventricular enlargement and right atrial
  dilatation.
• The pulmonary artery is usually prominent, then
  the proximal part will taper rapidly.
• Peripheral lung fields are oligaemic.

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• ECG
• right ventricular hypertrophy
• right axis deviation
• dominant R wave in lead V1
• inverted T waves in right precordial leads
• 2. right atrial abnormality
• tall peaked P waves in lead II

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• Echocardiography
• right ventricular dilatation and/or hypertrophy.
• measure the peak pulmonary artery pressure
  indirectly with Doppler echocardiography.
• reveal the cause of pulmonary hypertension, such
  as an intra-cardiac shunt.

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• With direct measurement of pulmonary artery
  pressure and pulmonary wedge pressure, cardiac
  catheterization is necessary in severe pulmonary
  hypertension of unknown cause.
• Pulmonary angiography indicated if multiple
  pulmonary emboli are suspected, but it is
  dangerous.
• If no other cause is found, then a diagnosis of
  primary pulmonary hypertension is made.

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Treatment

• Right ventricular failure Diuretics treatment,
  but careful to avoid excessive fluid depletion,
  as this will reduce output from the impaired
  right ventricle.
• Hypoxia oxygen therapy when safe and necessary.
• COPD long-term oxygen therapy.
• left ventricular function angiotensin-converting
  enzyme inhibitors may worse it.

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