Pulmonary hypertension (CV) Flashcards

1
Q

Define pulmonary hypertension.

A

An elevated mean pulmonary arterial pressure (mPAP) >20mmHg at rest

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2
Q

What are the causes of pulmonary hypertension? (5)

A
  • idiopathic pulmonary arterial hypertension (group 1)
  • left heart disease - congestive HF, valvular diseases e.g. AS, MS (group 2)
  • chronic lung disease - COPD, OSA, ILD (group 3)
  • pulmonary artery obstruction e.g. chronic thromboembolic occlusion of pulmonary vessels - thromboses/emboli in lungs (group 4)
  • unclear multifactorial mechanisms (group 5)
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3
Q

What left heart diseases can cause pulmonary hypertension? (2+2)

A
  • congestive heart failure
  • valvular diseases
    • aortic stenosis
    • mitral stenosis
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4
Q

What chronic lung diseases can cause pulmonary hypertension? (3)

A
  • COPD
  • OSA
  • ILD
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5
Q

What is cor pulmonale?

A

Right-sided heart failure secondary to pulmonary artery hypertension caused by long-standing pulmonary disease

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6
Q

What are the five groups of pulmonary hypertension?

A
  • group 1 - idiopathic
  • group 2 - secondary to left heart disease, valvular disease, restrictive cardiomyopathy
  • group 3 - secondary to chronic lung disease and environmental hypoxaemia
  • group 4 - due to chronic thrombotic disease, embolic disease or both
  • group 5 - metabolic, systemic, haematological disorders and other miscellaneous causes
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7
Q

Describe idiopathic pulmonary artery hypertension.

A

Rare, frequently fatal, most commonly seen in women

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8
Q

What are the clinical features of pulmonary hypertension? (7)

A
  • progressive dyspnoea or syncope/dizziness on exertion
  • weakness/fatigue
  • late stage –> oedema and ascites
  • angina, tachyarrhythmia
  • parasternal heave (sign of RV hypertrophy)
  • loud S2
  • symptoms of RHF - raised JVP, peripheral oedema, hepatomegaly
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9
Q

What might you see on examination in pulmonary hypertension? (8)

A
  • parasternal heave (RV hypertrophy)
  • loud S2
  • murmur - pulmonary regurgitation, tricuspid regurgitation (high-pitched pansystolic murmur)
  • raised JVP
  • peripheral oedema
  • hepatomegaly
  • ascites
  • tachyarrhythmia
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10
Q

What are some risk factors for pulmonary hypertension? (4)

A
  • family history
  • female sex
  • bone morphogenetic protein receptor type 2 mutations (BMPR2)
  • appetite suppressants
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11
Q

What are the first-line investigations for pulmonary hypertension? (4)

A
  • CXR
  • ECG
  • transthoracic doppler echocardiography
  • right heart catheterisation
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12
Q

What is the 1st line investigation for all patients with suspected pulmonary hypertension?

A

Transthoracic echocardiogram

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13
Q

What is a confirmatory test for pulmonary hypertension?

A

Right heart catheterisation - directly measure pulmonary pressure: mPAP>20mmHg

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14
Q

What would an ECG show in pulmonary hypertension? (1+3)

A

Findings of RVH and strain:

  • tall R wave in V1
  • P-pulmonale –> peaked P wave (due to RA enlargement)
  • right axis deviation
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15
Q

What would a CXR show in pulmonary hypertension?

A

Right heart hypertrophy; exclude other lung diseases

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16
Q

What are some differential diagnoses for pulmonary hypertension? (12)

A
  • cor pulmonale –> secondary pulmonary hypertension
  • cardiomyopathies
  • primary RV failure (following MI)
  • congestive heart failure
  • recurrent PE
  • mitral/tricuspid stenosis
  • pulmonary stenosis
  • tricuspid regurgitation
  • portal hypertension
  • OSA
  • hypothyroidism
  • sickle cell disease
17
Q

How do we approach management of pulmonary hypertension?

A

Management of underlying cause

18
Q

What is the first-line pulmonary vasodilator therapy for patients with idiopathic pulmonary hypertension and no signs of right HF?

A

CCB - for fluid overload (nifedipine / amlodipine)

19
Q

What are some supportive treatments for pulmonary hypertension? (4)

A
  • digoxin
  • diuretics - furosemide
  • anticoagulants - warfarin
  • oxygen
20
Q

What lifestyle changes can be made in pulmonary hypertension? (3)

A
  • restrict salt intake
  • exercise
  • avoid pregnancy
21
Q

What anticoagulation should patients with pulmonary hypertension be on?

A

Warfarin - adjust dose according to INR

22
Q

What are some specialist treatments for pulmonary hypertension?

A
  • prostacyclins - epoprostenol, iloprost
  • Bosentan - non-selective endothelin receptor antagonist
  • Sitaxentan - selective endothelin A receptor antagonist
  • Sildenafil - PD-5 inhibitor
23
Q

When is atrial septostomy done for pulmonary hypertension?

A
  • palliative procedure
  • may provide benefit to patients who are deteriorating
24
Q

What are some complications of pulmonary hypertension? (4)

A
  • cor pulmonale - RHF resulting from chronic pulmonary hypertension, commonly due to COPD
  • supraventricular tachyarrhythmias
  • RV failure - diuretics, digoxin, oxygen
  • treatment-related headache - prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors