Primary pulmonary hypertension (PPH) is frequently a fatal disease.

Primary pulmonary hypertension (PPH) is frequently a fatal disease, with a majority of patients dying within 5 years.[1-3] It affects mainly young adults in their next to the first to fourth decades[4] and succeeds a progressively downhill course culminating in right ventricular failure. Various forms of vasodilator therapy have been used in the past,[5] moreover a large series of patients would be required to determine their long-term efficacy. Acetylcholine, phentolamine, tolazoline and isoprenaline were initially used with conflicting follows Oral vasodilators such as hydralazine and captopril have also been used with early succes on the other hand their long-term use has been disappointing.[6,7] lately Rich and Brundage[8] have shown sustained hemodynamic, symptomatic, and electrocardiographic improvement associated with the use of high-dose calcium channel blockers[8] and have also reported improved survival in a proportion of patients.[9] Long-term benefit with enhanced survival chance, however, strike one as beings to be confined to the form into groups of patients who had a significant vasodilatory responsiveness to acute challenge with oral calcium channel blocker and also had a relatively preserv cardiac output There remains a large proportion of patients with simple disease who have developed right ventricular dysfunction and are, therefore, not suitable for [CA.sup.++] channel blocker and require alternate therapies. greatly attention has been placed not long ago on lung transplantation and novel therapies which combine the couple vasodilatory and antithrombotic actions.

NATURAL HISTORY/PROGNOSIS

newly come large scale reviews of patients with PPH have provided a clearer idea of the natural history of the disease. From these data, strategies of care have been formulated.

In a retrospective analysis of 120 patients with PPH Fuster et al[1] reported barely 21 percent survival after 5 years indicating an overall poor prognosis of this disease. There is a significant proportion of patients who do smooth less well if their pulmonary artery oxygen saturation is les than 63 percent; in this clump 3 years' survival chance is below 20 percent[1] More lately a larger study from the NIH registry comprising prospective follow-up of 194 patients estimated a median survival of 28 years.[3] In this application of mind prognosis was most closely related to right ventricular hemodynamic function; thus, patients with elevated right artial squeezing (more than 20 mm Hg) impaired cardiac index (les than 2 L/min/[m.sup.2]), and mean pulmonary artery influence in excess of 85 mm Hg were associated with poor survival chance. recent York Heart Association (NYHA) class 3 or 4 vicinity of Raynauds phenomenon, and decreased diffusion capacity for carbon monoxide (DCO) were also associated with poor prognosis. This reflection however, did not record pulmonary artery oxygen saturation.

In spite of an overall bleak view in PPH, there is a small proportion of patients, particularly those with a maintained cardiac output where the disease progresse slowly leading to "spontaneous" regaining Active vasodilator treatment for like patients may not be indicated.

ANTICOAGULANTS IN PPH TREATMENT

The cause of PPH is unknown, therefore, treatment is mainly aimed at alleviating the validitys of pulmonary vasoconstriction and reasonable cardiac output state. However, the mien of endothelial injury in the pulmonary vascular bed in conjunction with a grave cardiac output makes these patients inclined to develop pulmonary thrombi. The enhanced risk of intravascular thrombosis explains the valuable part of long-term anticoagulants in the treatment of PPH There are now couple large-scale studies[1,9] which have clearly demonstrated improved survival when patients are treated with anticoagulants.

VASODILATORS

Calcium channel blocker are commonly the favored vasodilator treatment of PPH where right ventricular function is maintained with the cardiac index of more than 2 L/min/[m.sup.2] and right atrial urgency of less than 10 mm Hg Their use is restricted to patients with mild to moderate disease where there is a demonstrable proper state of pulmonary vasodilatation to acute vasodilator challenge. There is no clear evidence as to the optimum dose of [Ca.sup.++] blocker which can be used without having adverse results However, high doses of [Ca.sup.++] channel blocker of that kind as 720 mg of diltiazem by means of day or 240 mg of nifedipine daily have been reported to improve symptoms and hemodynamics, as well as survival chance through the whole extent of 5 years.[8,9] Rich et al[8,9] demonstrated that improved mortality with high dose [Ca.sup.++] channel blocker was confined to the assign places to of patients who dropped their pulmonary artery compressing and pulmonary vascular resistance by way of more than 20 percent when challenged with oral diltiazem or nifedipine. However, those patients who do not present to view significant acute vasodilatory responsiveness do not benefit from [Ca.sup.++] channel blockers[1011]

Acute Vasodilator Trial With Prostacyclin

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