Pulmonary Hypertension Management

1. See Also
   1. Pulmonary Hypertension
   2. Pulmonary Hypertension Causes
   3. Pulmonary Hypertension Diagnosis
2. Management: General
   1. Treat underlying cause
      1. Treat Mitral Stenosis
      2. Afterload reduction for Left Ventricular Dysfunction
      3. Avoid predisposing medications (weight loss drugs)
      4. Prevent and treat respiratory infections
   2. Evaluate WHO functional class status
      1. Six minute walk test
   3. Determine if patient responds to vasodilation (vasoreactivity study)
      1. Perform right heart catheterization (mandatory prior to using CCB)
      2. Administer vasodilator (e.g. Adenosine, epoprostenol)
      3. Responder criteria
         1. Pulmonary Artery Pressure decreases >10 mmHg and <40 mmHg
         2. Cardiac output does not change or increases
      4. Use responder status to direct therapy
         1. Responder: Calcium Channel Blocker
         2. Non-responder: Use other vasodilators listed below
   4. General measures
      1. Regular symptom-limited Exercise
      2. Avoid pregnancy (50% mortality)
3. Management: Protocol
   1. Starting medications
      1. Anticoagulation (see Warfarin below)
      2. Diuretics
      3. Oxygen supplementation to keep Oxygen Saturation >90%
      4. Consider Digoxin
   2. Obtain vasoreactivity test (see above)
   3. Vasoreactivity Test positive
      1. Calcium Channel Blockers (see below)
   4. Vasoreactivity Test negative (or Calcium Channel Blocker trial fails)
      1. Low risk patient (or Calcium Channel Blocker ineffective)
         1. See Vasodilators below
         2. PDE-5 Inhibitors (Sildenafil) or
         3. Endothelin receptor antagonists (Bosentan, Ambrisentan)
      2. High risk patient (or low risk patient protocol fails)
         1. Epoprostenol (Flolan) IV or
         2. Treprostinil (Remodulin) IV or SQ or
         3. Iloprost (Ventavis) Inhaled or
   5. Above measures do not control symptoms
      1. See Surgical options below
4. Management: Medications
   1. Calcium Channel Blockers
      1. Effective longterm in only 20%
      2. Preparations (use high dose)
         1. Diltiazem
         2. Nifedipine
         3. Amlodipine
      3. Requires vasodilation test as above (use if responder)
         1. If non-responder, then do not use Calcium Channel Blocker
         2. Use other vasodilators listed below
   2. Vasodilators (reduce vascular resistance)
      1. Low flow Supplemental Oxygen (as above)
      2. Endothelin receptor antagonists
         1. Precautions
            1. FDA pregnancy category X (and reduce OCP efficacy)
            2. Liver Function Testing monthly
         2. Preparations
            1. Bosentan (Tracleer)
            2. Ambrisentan (Letairis)
            3. Sitaxsentan
      3. PDE-5 Inhibitors
         1. Precautions: Do not use with nitrates
         2. Preparations
            1. Sildenafil (Revatio)
            2. Tedalafil (Adcirca)
      4. Prostenoids (Prostacyclin)
         1. Efficacy: Highly effective
         2. Preparations
            1. Epoprostenol (Flolan)
            2. Iloprost (Ventavis)
            3. Treprostinil (Remodulin)
      5. Nitric oxide (experimental)
   3. Anticoagulation
      1. Coumadin to keep INR between 1.7 to 2.2
   4. Cardiac Output maximization
      1. Parenteral inotropic medications
      2. Digoxin
   5. Decrease preload
      1. Follow low-salt diet
      2. Diuretics
5. Management: Surgery
   1. Lung Transplantation
   2. Balloon Atrial Septoplasty
6. References
   1. Gaine (2000) JAMA 284:3160-8
   2. Nauser (2001) Am Fam Physician 63(9):1789-98
   3. Rubin (1997) N Engl J Med 336:111-7
   4. Rubin (1993) Chest 104:236-50
   5. McLaughlin (2009) Circulation 119(16): 2250-94
   6. Stringham (2010) Am Fam Physician 82(4): 370-7