One patient, later determined to have pulmonary veno-occlusive disease, developed pulmonary edema and was excluded. Systemic resistance and pressure also decreased significantly. During the 8 week trial period, three control patients and one investigational treatment patient died. Investigators have noted improvement in patients' subjective well-being during treatment with epoprostenol which appears to exceed expectations based on degree of hemodynamic improvement alone. Also, a degree of hemodynamic improvement may be noted upon subsequent assessment beyond that which was observed acutely.
Several observations suggest that anticoagulation is warranted.
Patients treated with anticoagulants appear to have longer survival than untreated patients. Finally, right ventricular failure may predispose to deep venous or intracardiac thrombus formation, possibly complicating the illness. Although no clear-cut survival or hemodynamic benefit has been shown to result from the use of oxygen in the patient with primary pulmonary hypertension, portable oxygen is an important adjunct to the symptomatic care of the hypoxemic patient. Despite the lack of directed investigation, experience suggests that at least some patients with clinical right ventricular failure experience symptomatic improvement with chronic administration of digoxin. Clearly, patients with peripheral edema and ascites require diuresis as aggressively as possible within the constraints of maintaining adequate right ventricular preload in the face of pulmonary hypertension.