Pulmonary vascular disease (Pulmonary hypertension) Flashcards

1
Q

What is pumonary hypertension?

A

Defined as a mean pulmonary artery pressure (mPAP) of >25 mmHg at rest as measured on right heart catheterization.

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2
Q

What is normal pulmonary blood pressure at rest?

A

Approx 25 mmHg

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3
Q

What is regarded as mild PHT?

A

Pulmonary pressure between 25-45 mmHg

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4
Q

What is regarded as moderate PHT?

A

46-65 mmHg

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5
Q

What is regarded as severe PHT?

A

>65 mmHg

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6
Q

Which sex is PHT more common in?

A

Female

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7
Q

What are the main classification groups used in in PHT?

A
  1. Pulmonary arterial hypertension
  2. PHT due to left heart disease
  3. PHT due to lung disease +/-hypoxia
  4. PHT due to thromboembolism
  5. PHT due to Multifactorial mechanisms
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8
Q

What are causes of pulmonary arterial hypertension?

A
  • Idiopathic
  • Heritable
  • Drug
  • Persistent PH in newborns
  • Connective tissue disease
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9
Q

What are causes of PHT due to left heart disease?

A
  • Systolic Dysfunction
  • Diastolic dysfunction
  • Valvular Disease
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10
Q

What are causes of PHT due to lung disease +/- hypoxia?

A
  • COPD
  • Interstitial LD
  • Other diseases with restrictive/obstructive pattern
  • Alveolar hypoventilation disorders
  • Chronic high altitude exposure
  • Sleep apnoea
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11
Q

In terms of pulmonary vessel loss, what percentage loss needs to be exceeded for resting pulmonary arterial pressure to increase?

A

60%

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12
Q

What are symptoms of PHT?

A

Early

  • Unexplained SOB
  • Fatigue
  • Syncope
  • Chest tightness
  • (Haemoptysis)

Late

  • Ankle swelling
  • RUQ pain
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13
Q

What are signs of PHT?

A
  • Loud P2
  • Right Ventricular Heave
  • Pulmonary Flow murmur (Ejection systolic)
  • Murmers increased by inspiration
  • Low gas transfers/desaturation/ hypoxia out of keeping with spirometry
  • Signs of RHF - backing up into the system
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14
Q

Why do you get a loud P2 in PHT?

A

Increased pulmonary hypertension of any cause may slam shut the pulmonary valve and cause a louder than normal pulmonary component of the second heart sound.

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15
Q

Why might you get a right ventricular heave in PHT?

A

Increased pressure load causes right ventricular hypertrophy and displacement of the right ventricle closer to the chest wall.

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16
Q

Why might you get a diastolic pulmonary regurgitation murmur in PHT?

A

Dilatation of the pulmonary valve due to back pressure

17
Q

What are the mechanisms that contribute to dyspnoea in primary PHT?

A
  • Relative hypoperfusion of ventilated alveoli leading to increased V/Q mismatch and increased alveolar dead space
  • Lactic acidemia
  • Hypoxaemia
  • Inability to increase pulmonary blood flow (and therefore systemic blood flow) to meet exercise oxygen demand.
18
Q

How would you investigate suspected PHT?

A

PHT should be considered in patients with unresponsive respiratory symptoms:

  • Chest X-ray
  • ECG
  • PFTs
  • V/Q scan/ CTPA
  • Echo
  • HRCT chest (high resolution CT scan)
  • Referral to Specialist Centre
19
Q

What might you see on CXR in someone with PHT?

A
  • Enlargement of the pulmonary arteries
  • Marked tapering (pruning) of peripheral arteries
  • Lucent lung fields
  • Right atrial and ventricular enlargement.
20
Q

What might you see on ECG in someone with PHT?

A
  • Right ventricular hypertrophy
  • Right atrial enlargement (P pulmonale)
21
Q

What routine bloods might you do when investigating for suspected PHT?

A
  • FBC
  • U+E’s
  • LFTs
  • TFTs
  • Serology - underlying connective tissue diseases, HIV and hepatitis.
22
Q

How would you manage pulmonary hypertension?

A

Start Medical:

  • Warfarin
  • Diuretics
  • Amlodipine
  • Prostcyclin analogues
  • NO and phosphdiesterase inhibitors (viagra)
23
Q

What surgical options are available for the treatment of PHT?

A
  • Atrial septostomy - creates shunt that eases RVF and increases CO
  • Pulmonary endarterectomy - for patients with PH due to chronic multiple PE
  • Transplantation