How to perform supportive care in long-term management?
Oxygen therapy for patients with COPD and a PaO2 < 60 mm Hg
Diuretics for volume overload
Avoidance of the following, whenever possible:
Pregnancy: Offer counseling on contraception options
Consider referral to palliative care for patients with advanced PH and distressing symptoms.
What are the results of overdiuresis?
Overdiuresis may reduce right ventricular preload, resulting in decreased cardiac output and subsequent complications, e.g., prerenal failure
How to manage group 1 PH patients?
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.
What is vasoreactivity testing and how to interpret it?
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.
What is the first-line treatment for patients with PAH and positive vasoreactivity testing and no signs of RHF or other contraindications to CCBs?
Calcium channel blockers
What are other pulmonary vasodilator therapies?
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)
Name 2 surgical therapies for refractory PAH
Atrial septostomy (right-to-left shunt)
Heart-lung/bilateral lung transplantation
How to manage group 2 PH patients?
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.
How to manage group 3 PH patients?
Start long-term oxygen therapy.
Optimize treatment of underlying COPD or interstitial lung disease.
Consider lung transplantation.
How to manage group 4 PH patients?
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.
Extrinsic compression (rare): Consider treatment tailored to the individual extrinsic etiology.