Case of the Month #51 Pulmonary Hypertension

• Treat underlying precipitant 

• Treat RH failure 

  • Optimise preload 
  • Reduce afterload 
  • Improve contractility 
  • Maintain coronary perfusion 
  • Rhythm control 

• In practice: 

  • Avoid hypoxia, hypercarbia, acidosis 
  • Diuretics to reduce RV preload 
  • IV or nebulised pulmonary vasodilators (epoprostenol, iloprost), other PAH drugs, iNO to reduce RV afterload 
  • Inotropes / inodilators: Dobutamine / Milrinone 
  • Vasopressors (noradrenaline improves RV:PA coupling) 
  • Consider RRT (offload, normalise metabolic) 
  • Intubation and mechanical ventilation high risk 
  • Titrate PEEP (avoid excess but need to balance against hypoxia) 

• MDT approach: Close collaboration with PH physicians. Contact PH centre for advice / potential transfer 

The patient survives her admission with pneumonia, and subsequently a formal diagnosis of idiopathic PAH is made. 

Two years later the patient is re-admitted to ICU for decompensated RV failure with significant hypotension and AKI. She is deteriorating rapidly. By this time, she has been established on maximum combination therapy in the community. Her recent RHC data and cardiac MRI show significant progression of her PH and she is now breathless at rest. She has been referred for transplant assessment, but a decision is pending.