questions 2

Pulmonary Hypertension

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Topic updated on 09/03/17 12:53pm

Introduction
  •  Definition
    • pulmonary artery pressure ≥ 25 mmHg or > 30 mmHg during exercise
      • normal = 10 - 14 mmHg
  • Causes
    • idiopathic or familial pulmonary artery hypertension (formerly primary pulmonary hypertension)
      • a mutation in the BMPR2 gene predisposes to abnormal vasculature 
        • normally acts to inhibit smooth muscle growth
        • when presented with an insult (e.g. drugs and infection), the disease process begins (two-hit hypothesis)
          • increased secretion of vasoconstrictors
          • decreased secretion of vasodilators
      • results in vascular hyperactivity and hyperplasia of smooth muscle
        • may also involve fibrosis, thombosis of pulmonary vasculature, and endothelial cell growth
      • poor prognosis
      • more common in women
    • secondary pulmonary hypertension
      • endothelial cell dysfunction
        • an increase in vasoconstrictors or reduction in vasodilators
      • COPD
        • destruction of lung parenchyma results in hypoxia
        • reduction in O2 tension results in pulmonary vasoconstriction
      • mitral stenosis or left heart failure
        • increased pressure due to backflow of blood from the left heart into the pulmonary circulation
      • autoimmune disease (e.g. SLE)
        • inflammation results in intimal fibrosis
      • left-to-right shunt (e.g. ASD/VSD)
        • increased blood flow results in endothelial injury
      • sleep apnea or living at high altitude
        • reduction in O2 tension results in pulmonary vessel constriction
      • recurrent thromboemboli
        • reduced pulmonary vasculature
  • Pathology
    • atherosclerosis
      • result of injury to lumen as a result of ↑ pressure
    • proliferation of medial smooth muscle
Presentation
  • Symptoms
    • dyspnea on exertion
    • fatigue
    • angina
  • Physical examination
    • increased P2 on auscultation
    • right ventricular heave
Evaluation
  • Echocardiogram
    • elevated right ventricular systolic pressure
  • Chest radiograph
    • enlarged pulmonary arteries
    • pruning of peripheral vessels
  • CT angiography
    • enlarged pulmonary trunk and PA's
  • Right heart catheterization
    • gold-standard, definitive test
Complications
  • Chronic hypoxemic respiratory failure
  • Right ventricular failure 


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