Pulmonary hypertension resident survival guide

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

Definition

Pulmonary hypertension (PH) is defined by mean pulmonary artery pressure > 25, pulmonary capillary wedge pressure (PCWP), left atrial pressure, or left ventricular end-diastolic pressure (LVEDP) ≤ 15 mm Hg; and a pulmonary vascular resistance (PVR) > than 3 Wood units.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

• Pulmonary embolism

Common Causes

• Congenital heart disease with left-to-right shunt (ASD, VSD, PDA)
• Congestive heart failure
• COPD
• Cor pulmonale
• Interstital lung disease
• Obstructive sleep apnea
• Thromboembolism

Management

Characterize the symptoms: ❑ Progressive dyspnea ❑ Exertional dizziness and syncope ❑ Edema of the extremities ❑ Angina ❑ Palpitations

Examine the patient: ❑ Loud pulmonary second heart sound (P2) ❑ Systolic murmur suggestive of tricuspid regurgitation ❑ Raised jugular venous pressure (JVP) ❑ Peripheral edema ❑ Ascites

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

❑ Anticoagulation +/- ❑ Diuretics +/- ❑ Oxygen therapy +/- ❑ Digoxin

Acute vasoreactivity testing

Positive

Negative

Oral calcium channel blocker (CCB)

Lower risk

Higher risk

❑ Follow closely for efficacy and safety Sustained response

❑ Endothelin receptor antagonsists (ERA's) or Phospodiesterase-5 inhibitors (PDE-5 Is) (oral) ❑ Epoprostenol or Treprostinil (IV) ❑ Iloprost (inhaled) ❑ Treprostinil (SC)

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

❑ Reassess

Continue CCB

In case of absence of response to initial monotherapy: ❑ Consider combo-therapy

In case of progress despite optimal medical treatment: ❑ Investigational protocols, OR ❑ Atrial septostomy, OR ❑ Lung transplant

The following guideline is based on Expert consensus document on pulmonary hypertension published by ACCF/AHA in 2009.

Follow up testing after etiology for pulmonary hypertension is established:

Do's

• The diagnosis of Pulmonary hypertension requires confirmation with a right heart catheterization.
• Objective assessment of treatment measures includes:

• Exercise capacity.
• Hemodynamics.
• Survival.

• Epoprostenol is the only therapy that has been shown to prolong survival in patients with pulmonary hypertension.
• Monitor liver function tests monthly in patients being treated with endothelin receptor antagonists.
• Patients presenting with advanced symptoms, right heart failure, advanced hemodynamics and those on parenteral or combination therapy must be seen every 3 months.

Don'ts

• Do not perform vasospastic testing for those with overt heart failure or hemodynamic instability.