Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary arterial hypertension (PAH) is a severely disabling disorder characterized by sustained elevations of pulmonary vascular resistance, ultimately leading to right-heart failure and death. Intravenous epoprostenol has been widely used in patients with PAH, leading to long-term clinical benefits and improved survival. Epoprostenol has to be delivered through a permanently implanted intravenous catheter, with the potential of life-threatening complications. Thus, treprostinil, a stable prostacyclin analog suitable for subcutaneous administration, has been developed. Treprostinil is chemically stable at room temperature, has a long half-life (2-4 hours), and has similar pharmacologic properties with comparable hemodynamic effects as epoprostenol.A large, double-blind, placebo-controlled, multicenter, 12-week study confirmed the efficacy of subcutaneous treprostinil, by improving exercise capacity, Borg Dypnea Score (BDS), New York Heart Association (NYHA) class, and clinical signs and symptoms in patients with PAH. Subsequently, multiple observational studies reported the long-term effects of subcutaneous treprostinil. The long-term survival of patients treated with subcutaneous treprostinil was similar to that reported with intravenous epoprostenol. Pain at the infusion site has been a major drawback of subcutaneous treprostinil, hampering dose titration and leading to an 8-10% discontinuation rate. In addition, studies have examined the efficacy of intravenous treprostinil in the treatment of patients with PAH. An open-label study demonstrated that intravenous treprostinil improved exercise capacity, BDS, NYHA functional class, and hemodynamics at week 12 compared with baseline. Transitioning from intravenous epoprostenol to intravenous treprostinil is safe and effective. The dose of intravenous treprostinil has to be adjusted to approximately twice the dose of intravenous epoprostenol. Most patients have reported less severe adverse effects with intravenous treprostinil compared with intravenous epoprostenol. The assessment of the long-term efficacy and safety of continuous intravenous treprostinil requires further studies.
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PMID:The role of treprostinil in the management of pulmonary hypertension. 1869 Jul 55

Patients with Eisenmenger syndrome form a small percentage of congenital heart disease patients. The rarity of this syndrome, combined with its complex pathophysiology, account for the insufficient understanding of the principles underlying its proper treatment. The main clinical symptoms are: cyanosis due to secondary erythrocytosis, resulting in increased blood viscosity, iron deficiency anemia (enhanced by unnecessary phlebotomies), blood clotting disturbances, heart failure and serious supraventricular and ventricular arrhythmias. Recent decades have seen developments in pulmonary hypertension pathophysiology which have led to the introduction of new groups of drugs: prostacycline analogs (Epoprostenol, Treprostinil, Beraprost, Illoprost), phosphodiesterase inhibitors (Sildenafil, Tadalafil), endothelin receptor antagonists (Bosentan, Sitaxantan, Ambrisentan) and nitric oxide. These drugs should be administered to patients in III-IV NYHA class. Despite successful early results, the therapeutic effect on patients with Eisenmenger syndrome has not been conclusively established. Our therapeutic efforts should be directed mainly towards preventing complications. As a rule, we should avoid agents with no established therapeutic efficacy and try to alleviate symptoms without any additional risk, so as not to disrupt the existing clinical balance.
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PMID:Therapeutic methods used in patients with Eisenmenger syndrome. 1995 85