Anticoagulants
Thrombosis in situ is believed to be integral to the pathogenesis of PPH. Anticoagulant therapy has been investigated in patients with PPH and is associated with improved survival. Although the effectiveness of warfarin anticoagulation in patients with PPH has never been tested in a randomized, prospective, long-term trial, based on the known pathogenesis of PPH and the available data. Experts recommend the use of low-dose warfarin with an international normalized ratio of 2.0 to 2.5 times control as therapy for PPH.

Calcium Channel Blockers
Vasoconstriction is important in the pathogenesis of PPH in some patients. Although many drugs have been used for vasodilation in the setting of PPH, calcium channel blockers have been the most widely tested class of drugs for this purpose, and they appear to produce a more consistent reduction in pulmonary artery pressure and pulmonary vascular resistance that other vasodilators. Studies show that approximately  25 percent of patients with PPH will respond to calcium channel blockers).  A drug called Flolan may help many severely ill patients who do not respond to treatment with calcium channel blockers such as Nifedipine. Flolan imitates the natural prostacyclin produced by the body to keep blood vessels healthy by removing the build up of lipids, lowering blood pressure.  Most PPH patients do respond to increased doses of prostaglandin which dilate, or open up, blood vessels in the lungs and throughout the body. This Flolan-Prostacyclin also appears to prevent blood clots from forming.

Flolan has been studied in clinical trials , and is one of only a hand full of FDA approved drug treatment for PPH. Flolan is administered intravenously directly into the bloodstream through a surgically implanted catheter by a portable, battery-operated pump. The pump is worn attached to a belt around the waist or carried in a small shoulder pack. Since the drug lasts only 3-5 minutes it must constantly be infused: it is slowly and continuously pumped into the body through the permanent catheter placed in a vein in the neck or chest. Additionally Tracleer, Remodulin, UT-15 and Bosentan are also being used to treat primary pulmonary hypertension.

Prostacyclin
Prostacyclin is a metabolite of arachidonic acid that is produced primarily in the vascular endothelium. Its major pharmacologic actions include potent vasodilatation of the pulmonary and systemic arterial and venous beds and inhibition of platelet aggregation. Prostacyclin is administered through a permanent intravenous catheter and delivered by an ambulatory infusion system. The delivery system is complex and requires patients to learn the techniques of sterile preparation, operation of the pump, and care of the intravenous catheter.

Others
Diuretics are frequently required to reduce excessive edema in patients with right heart failure. they are particularly useful when hepatic congestion, ascites, and edema are present. Digoxin can increase cardiac output and reduce circulating neurohormones. Patients with hypoxemia, either at rest or with exercise, should receive supplemental oxygen. Because of increased cardiovascular stress, pregnancy is generally poorly tolerated by patients with PPH.

Heart and Lung Transplantation
Bilateral lung transplantation and single-lung transplantation have been performed successfully in patients with PPH. The immediate reduction in pulmonary artery pressure and pulmonary vascular resistance is associated with an improvement in right ventricular function. Bilateral lung transplantation is preferred at most centers, because there is greater pulmonary vascular reserve if the patient sustains rejection or infection. Single lung transplantation may be preferred in some situations because the operation is technically less challenging and the waiting time is shorter. As with any type of organ transplantation, the major long-term morbidity and mortality are related to the high incidence of rejection and opportunistic infections. In addition, lung transplantation carries a high risk of the development of bronchiolitis obliterates. In the era of prostacyclin, lung transplantation should be considered a treatment of last resort for PPH.

NOTE: Talk with your Doctor and find out what PPH Treatment Options are available and best for you.