Pulmonary Disorders II

Diuretics should be used for the symptomatic management of RV dysfunction and signs of fluid

overload; choice of diuretic is variable.

although data specifically in PAH are not available.

Anticoagulation is not generally recommended for patients with PAH and has been shown harmful

in patients with PAH associated with systemic sclerosis. Consider on an individualized basis if

appropriate.

Aggressive treatment of sleep apnea and hypertension

Angiotensin-converting enzyme inhibitors, angiotensin-receptor blockers, angiotensin-receptor/

neprilysin inhibitors, sodium-glucose cotransporter-2 inhibitors, β-blockers, or ivabradine are not

recommended in patients with PAH unless indicated for a different comorbidity.

Treatment of iron deficiency anemia is indicated, defined by serum ferritin less than 100 mcg/L, or

serum ferritin 100–299 mcg/L, and transferrin saturation less than 20%.

Patients with PAH should be vaccinated against influenza, Streptococcus pneumoniae and SARS-

CoV-2 at minimum.

Psychosocial support in the form of patient support groups and mental health professionals.

Pharmacists play a role in disease state and pharmacotherapy education to keep patients on

treatment.

Special populations

Pregnancy

Despite advances in treatment and management, for patients with PAH who become pregnant,

birthing parent and neonatal mortality rates are high.

ii.

People of childbearing potential should be provided with clear contraceptive recommendations.

Some oral contraceptives have significant drug–drug interactions with PAH therapy, which

should be considered and discussed with the patient.

Surgery

Surgical procedures pose a risk for patients with PAH. Risk increases with severity of PAH.

ii.

Decisions to perform surgery should be made by a multidisciplinary team familiar with the

risks and benefits of both the procedure and PAH.

Vasodilator therapy with calcium channel blockers (CCBs) (diltiazem, amlodipine, nifedipine)

Patients who should undergo vasoreactivity testing include those with idiopathic PAH, heritable

PAH, and PAH secondary to toxins or drugs who have a cardiac index above 2 L/min/m2 on right

heart catheterization.

Considered first-line treatment for patients who have a positive response to acute vasoreactivity

testing (reduced mPAP by 10 mm Hg or more to an mPAP of 40 mm Hg or less with increased or

unchanged cardiac output) (N Engl J Med 1992;327:76-81)

CCBs improve mPAP, PVR, WHO FC, and survival in patients with PAH and a positive

vasoreactivity test (Circulation 1987;76:135-41; N Engl J Med 1992;327:76-81). CCBs are initiated

at low doses and rapidly titrated to the maximum tolerated dose.

cardiac output, or WHO FC IV symptoms.

Marked hemodynamic improvement in patients on CCB therapy is defined as mPAP greater than

30 mm Hg and a PVR less than 4 Wood units. If patients do not reach this threshold of response,

CCB therapy can be continued or stopped according to patient-specific factors; some patients may

experience deterioration when CCBs are withdrawn.