Pulmonary hypertension resident survival guide

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Abdurahman Khalil, M.D. [2]

Definition

Pulmonary hypertension (PHT) is defined by mean pulmonary artery pressure greater (mPAP) than 25 mmHg at rest,.Other hemodynamic characteristics include pulmonary capillary wedge pressure (PCWP),left atrial pressure or left ventricle end-diastolic pressure (LVEDP) less that 15 mmHg ,and pulmonary vascular resistance greater than 3 Wood units .

Causes

Life threatening causes

Life threatening conditions which may cause death or permanent disability within 24 hours if left untreated.

  • Pulmonary veno-occlusive disease (PE)

Common causes

  • Familial
  • LA/LV systolic/diastolic dysfunction.
  • Valvular heart disease in the left heart (MR,MS).
  • Congenital heart disease with left→right shunt (ASD,VSD,PDA).
  • Connective tissue diseases(CREST,SLE,MCTD,RA).
  • Lung diseases with chronic hypoxia (COPD,ILD,sleep apnea)
  • High altitude.
  • Idiopathic

Management

 
 
 
 
Characterize the symptoms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Examine the patient
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Consider alternative diagnosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Anticoagulation ± Diuretics ± Oxygen ± Digoxin
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acute vasoreactivity testing
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Positive
 
 
 
 
 
Negative
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Oral Calcium channel blocker
 
 
Lower risk
 
 
 
Higher risk
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Sustained response
 
 
ERAs or PDE-5 Is ((Oral)
Epoprostenol or Treprostinil (IV)
Illoprost (inhaled)
Treprostinil (SC)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Do's

Don'ts

[1]

References

  1. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR; et al. (2009). "ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association". Circulation. 119 (16): 2250–94. doi:10.1161/CIRCULATIONAHA.109.192230. PMID 19332472.