Pulmonary hypertension resident survival guide

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

Oral Calcium channel blocker (CCB)

Lower risk

Higher risk

No

Sustained response

❑ ERAs or PDE-5 Is ((Oral) ❑ Epoprostenol or Treprostinil (IV) ❑ Illoprost (inhaled) ❑ Treprostinil (SC)

❑ Epoprostenol or Treprostinil (IV) ❑ Illoprost (inhaled) ❑ ERAs or PDE-5 Is ((Oral) ❑ Treprostinil (SC)

Yes

Continue CCB

Reassess consider combo-therapy

Atrial septostomy Lung transplant

Investigational protocols