Rapid control of blood pressure
ACEI drug of choice, e.g. captopril 6.25 to 12.5mg PO, TID.
Avoid diuretics
Consult renal, as 50% of patients will require dialysis.
ACEI drug of choice, e.g. captopril 6.25 to 12.5mg PO, TID.
Consult renal, as 50% of patients will require dialysis.
Chronic management may include prostaglandin derivatives (e.g. epoprostenol), PDE-5 inhibitors (e.g. sildenafil), and/or endothelin receptor antagonists (e.g. bosentan)
Acute decompensation:
Optimize (usually reduce) RV preload:
Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure
Furosemide 20-40mg IV or drip at 5-20 mg/hr
If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start norepinephrine (MAP > 65 mmHg).
Increase cardiac output
Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
Improves inotropic support and theoretically decreases pulmonary vascular resistance
Reduce RV afterload
Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
Avoid hypercapnia (increases pulmonary vascular resistance)
Avoid acidosis
Treat arrhythmias:
Avoid calcium channel blockers and β-blockers
Optimize oxygenation
Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated
Optimize (usually reduce) RV preload:
Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure
Furosemide 20-40mg IV or drip at 5-20 mg/hr
If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start norepinephrine (MAP > 65 mmHg).
Increase cardiac output
Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
Improves inotropic support and theoretically decreases pulmonary vascular resistance
Reduce RV afterload
Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
Avoid hypercapnia (increases pulmonary vascular resistance)
Avoid acidosis
Treat arrhythmias:
Avoid calcium channel blockers and β-blockers
Optimize oxygenation
Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated
Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure
Furosemide 20-40mg IV or drip at 5-20 mg/hr
If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start norepinephrine (MAP > 65 mmHg).
Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
Improves inotropic support and theoretically decreases pulmonary vascular resistance
Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
Avoid hypercapnia (increases pulmonary vascular resistance)
Avoid acidosis
Avoid calcium channel blockers and β-blockers
Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated
Steroids
Cyclophosphamide
Ventilatory support prn
Airway stabilization if large volume hemoptysis or problems with oxygenation/ventilation (>8.0 ETT preferable)
IV corticosteroids - high dose Methylprednisolone Q6H or cyclophosphamide
Bronchoscopy for vessel localization
Vessel embolization if localizable
Management of Hemoptysis
Hemoptysis, infiltrates on CXR, anemia.
Emergent bronchoscopy
High dose corticosteroids, cyclophosphamide, local vessel embolization or plasma exchange
Hemoptysis, infiltrates on CXR, anemia.
Emergent bronchoscopy
High dose corticosteroids, cyclophosphamide, local vessel embolization or plasma exchange
Empiric treatment with a PPI to prevent reflux and stricture formation
Transfuse as needed for anemia due to mucosal telangiectasias
Ursodiol treatment  if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis
Scleroderma
Systemic Lupus Erythematosus
Dermatomyositis
Sjögren syndrome
Vitiligo
Acute arterial ischemia
Brash syndrome
Atheroembolism 