What treatment is available for primary pulmonary hypertension ?
• Oral anticoagulants: warfarin is the anticoagulant of choice, in doses adjusted to achieve an INR of
approximately 2.0. Anticoagulants nearly double the 3-year survival rate (Circulation 1984; 70: 580-7).
* Calcium channel blockers: nifedipine, diltiazem. Patients who respond to calcium channel blockers
have a 5-year survival rate of 95% (N Engl J Med 1992; 327: 76-81).
• Intravenous epoprostenol (formerly prostacyclin or prostaglandin I2), which is a potent short-acting
vasodilator and inhibitor of platelet aggregation that is produced by the vascular endothelium (N Engl
J Med 1996; 334: 296-301; N Engl J Med 1998; 338: 273-7).
• Atrial septostomy: the creation of a right-to-left shunt by blade-balloon atrial septostomy has been
reported to improve forward output and alleviate right-sided heart failure by providing blood with a
low-resistance channel, thereby decompressing the right atrium and improving filling of the left side of
the heart (Circulation 1995; 91: 2028-35).
• Lung transplantation and combined heart-lung transplantation: survival rates after the two
procedures are similar. Even markedly depressed right ventricular function improves considerably
with single- or double-lung transplantation.
• Possible future drugs: (a) UT-15, a prostaglandin 12 analogue, has been shown to have sustained
and favourable effects in patients when administered sub-cutaneously (Circulation 2000; 102(18):
11-101); (b) Sitaxsentan, an oral selective endothelin-A receptor blocker, has been shown to produce
sustained improve-ments in pulmonary artery pressure (Circulation 2000; 101(25): 2922-7).
Graham Steell (1851-1942), assistant physician to the Manchester Royal Infirmary, described the murmur in a paper titled The murmur of high pressure in the pulmonary artery (Med Chron (Manchester) 1888; 9: 182-8).