Table 1. Summary of Conventional Therapies in PAH4
Treatment Measure
Intervention
Comments
Physical activity
Encouraged to avoid sedentary lifestyle due to muscular deconditioning.
Watch for anginal chest pain and syncope.
Avoidance of aggressions
Avoid acute stresses and invasive medical procedures.
Due to an increase in sympathetic nervous system activation, an increase in PVR is poorly tolerated.
Avoid pregnancy
Low-dose hormonal contraception.
N/A
Avoid hypoxia
Avoid high altitudes; treat pulmonary infections.
Due to superimposed hypoxic pulmonary vasoconstriction, hypoxia aggravates PAH. Supplemental oxygen rarely indicated.
Avoid appetite-suppressant drugs
Absolute contraindication. Agents include dexfenfluramine.
These agents are known to cause PAH.
Anticoagulation
Maintain an INR between 2 to 3 with warfarin (Coumadin®).
These patients are at risk for venous thromboembolism.
Diuretics
Various doses are required depending on volume status of the patient.
Diuretics optimize preload by adjusting volume status and venous return.
Inotropes
Agents include dobutamine, dopamine (Inotropin®), and digoxin (Lanoxin®).
To increase contractility; RV contractility is usually preserved in these patients.
Vasodilators
  • Calcium channel blockers:
    - Nifedipine 20 mg three times daily, increased as tolerated.
    - Diltiazem 60 mg three times daily, increased as tolerated.
  • Epoprostenol 2 ng/kg/min and increased by 2 ng/kg/min every 10 to 15 minutes, as tolerated (see text).
Vasodilators decrease afterload by decreasing PVR.

PVR: peripheral vascular resistance, INR: international normalized ratio, RV: right ventricular

Return to article