Condition- Pulmonary Hypertension Flashcards

1
Q

What is pulmonary hypertension and what value of mean Pulmoary Arterial pressure is defined as pulmoary hypertension?

A
  • It results in a progressive increase in pulmonary vascular resistance (PVR)
  • It is defined by a mean pulmonary arterial pressure ≥25 mmHg at rest
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2
Q

List some of the causes of PAH…

A
  • Idiopathic
  • Hereditary- genetic mutations
  • Drug and toxin induced
  • Left ventricular failure + Valvar diseases + outflow tract cardiomyopathies
  • Lung disease: COPD, Interstitial lung disease
  • PE
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3
Q

Describe some of the symptoms experienced by a patient with PAH

A
  • pregressive SOB esp on exertion
  • May have chest pain
  • Weakness/ tiredness
  • Exertional dizziness/ syncope
  • Late stage- oedema and ascites
  • Angina and tachyarrhythmia
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4
Q

List some of the signs of PAH…

A
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5
Q

State a gene mutation which increases the risk of developing PAH

A

Bone morphogenic protein receptor 1 (BMPR2)- changes in intracellular signalling pathways that result in proliferative and anti-apoptotic effects in the pulmonary vasculature

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6
Q

Which age group and gender is thought to be more at risk of PAH?

A

20-50 yr olds

Females> Males

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7
Q

List some of the signs of PAH upon examination

A
  • Cyanosis
  • Raised JVP
  • Right ventricular heave
  • Loud pulmonary second heart sound (P2)- because of delayed closure of pulmonic valve
  • MURMUR: High-pitched, holosystolic, best heard at the left sternal border- tricuspid regurgitation
  • MURMUR: An early diastolic high-pitched Graham Steell murmur- pulmonic regurgitation
  • Peripheral Oedema
  • Ascites
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8
Q

List some appropriate investigations for someone with PAH

A
  • CXR: to exlude other lung diseases
  • ECG: right ventricular hypertrophy + strain
  • Transthoracic doppler echocardiography: to check systolic pulmonary artery pressure and visualise right atrial and ventricular dilation
  • Right Heart Catheterisation: test to confirm PAH. Mean pulmonary arterial pressure >25 mmHg at rest
  • PFTs
  • LFTs: to exclude portal hypertension
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