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. | |
| 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:
•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