Long-term management

• Oxygen therapy for patients with COPD and a PaO2 < 60 mm Hg

• Diuretics for volume overload

• Immunizations

• Psychosocial support

• Exercise training

• Avoidance of the following, whenever possible:

  • Pregnancy: Offer counseling on contraception options

  • High altitude

  • Nonessential surgeries

• Consider referral to palliative care for patients with advanced PH and distressing symptoms.

Overdiuresis may reduce right ventricular preload, resulting in decreased cardiac output and subsequent complications, e.g., prerenal failure

Refer all patients to a specialist PAH center for assessment and vasoreactivity testing prior to starting pulmonary vasodilator therapy aimed at decreasing pulmonary vascular resistance.

A test performed during right heart catheterization to identify responsiveness to calcium channel blocker (CCB) therapy; mPAP is measured after administration of a vasodilator (e.g., inhaled nitric oxide)

• Vasoreactive: Treat with CCBs.

• Nonvasoreactive: Consider other pulmonary vasodilator therapies.

Calcium channel blockers

typically second-line agents, the choice of which depends on symptom severity (e.g., WHO-FC) and should be guided by a PAH specialist

• Most patients (e.g., WHO-FC II-III) receive initial combination oral therapy (e.g., PDE-5 inhibitor PLUS endothelin receptor antagonist).

• Most high-risk patients (e.g., WHO-FC III-IV or rapidly progressive disease) are treated with parenteral prostacyclins (either as first-line or in combination)

• Atrial septostomy (right-to-left shunt)

• Heart-lung/bilateral lung transplantation

• Treat systemic hypertension

• Provide CHF treatment as needed.

  • Consider diuretics to manage volume overload.

  • Restrict sodium intake.

• Consider replacement or repair of damaged valves

• Aggressively treat any atrial fibrillation

• Treat comorbidities, e.g., diabetes, obesity, secondary prevention of ASCVD.

• Start long-term oxygen therapy.

• Optimize treatment of underlying COPD or interstitial lung disease.

• Consider lung transplantation.

• CTEPH

  • Pulmonary endarterectomy is the first-line treatment.

  • Consider balloon pulmonary angioplasty if the patient is ineligible for pulmonary endarterectomy or if PH persists or recurs.

  • Consider certain pulmonary vasodilators in select cases.

  • Treat associated hypoxia and heart failure.

  • Lifelong anticoagulation

• Extrinsic compression (rare): Consider treatment tailored to the individual extrinsic etiology.

• Direct initial therapy at the underlying cause of PH.

• Consider pulmonary vasodilator therapy on a case-by-case basis