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Chronic Cor Pulmonale

  • Cor pulmonale – right heart failure caused by chronic pulmonary arterial hypertension (PHTN), as a result of respiratory disease

Etiology

  • Cor pulmonale develops in response to changes in pulmonary vasculature/lung parenchyma that are sufficient to cause PHTN
  • COPD
  • ARDS – acute respiratory distress syndrome
  • PE
  • Kyphoscoliosis
  • Cystic fibrosis, Interstitial lung disease

Pathophysiology

  • PHTN is sufficient to alter RV function
  • Normal PA pressure is 15mmHg
    • in diseased lung, the pulmonary circulation bed undergoes remodelling, vasoconstriction, destruction
    • causes PA pressure to increase – >20mmHg
      • leads to ↑ RV afterload
  • RV wall is thin and compliant, therefore increase in RV afterload causes RV failure

Chronic cor pulmonale

  • Slow progressing PHTN – first leads to RVH, then RV dilation

Clinical features

  • Dyspnoea, fatigue, syncope
  • Cyanosis
  • ↑JVP, tachycardia
  • Systemic venous congestion – Hepatomegaly, peripheral edema, ascites

Diagnosis

Investigate underlying pulmonary disease

  • Alpha-1 antitrypsin levels
  • Spirometry
  • Bronchoscopy
  • VQ scan
  • Catheter/ CVP line – check pulmonary and heart chamber pressures (pulmonary vascular resistance)

Investigate right heart function

  • ECG – RVH
  • CXR – pulmonary artery enlargement, RVH
  • Doppler echo
  • ABGs
  • BNP

Treatment

  • Long term oxygen therapy – when SaO2 <88%
  • Diuretics – for peripheral oedema
  • Vasodilators – CCBs for hypertension
  • Bosentan – endothelin receptor antagonist. For PHTN
  • Epoprostenol
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