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 (PH) is defined by mean pulmonary artery pressure > 25 mm Hg at rest.

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: ❑ Progressive dyspnea ❑ Exertional dizziness and syncope ❑ Edema of the extremities ❑ Anginal pain ❑ Palpitations

Examine the patient: ❑ Loud P2 (Pulmonary second heart sound) ❑ Systolic murmur from Tricuspid regurgitation ❑ Raised JVP (Jugular venous pressure) ❑ Peripheral edema ❑ Ascites

Consider alternative diagnosis: ❑ Left sided heart failure ❑ Coronary artery disease ❑ Liver disease ❑ Budd-chiari syndrome

❑ Anticoagulation ± ❑ Diuretics ± ❑ Oxygen ± ❑ Digoxin

Acute vasoreactivity testing

Positive

Negative

Oral Calcium channel blocker (CCB)

Lower risk

Higher risk

No

Sustained response

❑ Endothelin receptor antagonsists (ERA's) or Phospodiesterase-5 inhibitors (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