Marked Improvement with Sildenafil in a Patient with Primary Pulmonary Hypertension Unresponsive to Epoprostenol

• Published in: Internal Medicine Vol. 43; no. 10; pp. 945 - 950
• Main Authors: KATAOKA, Masaharu, SATOH, Toru, MANABE, Tomohiro, ANZAI, Toshihisa, YOSHIKAWA, Tsutomu, MITAMURA, Hideo, OGAWA, Satoshi
• Format: Journal Article
• Language: English
• Published: Tokyo The Japanese Society of Internal Medicine 01.10.2004; Japanese Society of Internal Medicine
• Subjects: Antihypertensive Agents - therapeutic use; Biological and medical sciences; Electrocardiography; epoprostenol; Epoprostenol - therapeutic use; Female; General aspects; Humans; Hypertension, Pulmonary - drug therapy; Lung - drug effects; Lung - pathology; Lung - physiopathology; Medical sciences; Middle Aged; Piperazines - therapeutic use; Pneumology; primary pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Purines; right heart failure; sildenafil; Sildenafil Citrate; Sulfones; Time Factors; Treatment Outcome; Vasodilator Agents - therapeutic use; Vasodilator agent; Prostaglandin I2; 3',5'-Cyclic-GMP phosphodiesterase; Cardiovascular disease; Esterases; Phosphoric diester hydrolases; primary pulmonary hypertension; Improvement; Primitive; Heart disease; Primary; Human; Heart failure; Respiratory disease; Enzyme; Arachidonic acid derivatives; Enzyme inhibitor; right heart failure; Pulmonary hypertension; Antiplatelet agent; Epoprostenol; Chemotherapy; Treatment; Hydrolases; Sildenafil; Prostaglandin derivatives
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.43.945

We report a 48-year-old woman with right heart failure due to primary pulmonary hypertension. Continuous infusion of epoprostenol (prostaglandin I2) for 1.5 years failed to control her condition, but she was later successfully treated with additional sildenafil for a few months. Her mean pulmonary artery pressure was originally 57 mmHg, increased to 62 mmHg with epoprostenol, and decreased to 45 mmHg with sildenafil. Additional sildenafil may be an effective and life-saving agent in patients with primary pulmonary hypertension who show a poor response to epoprostenol, which is considered to be very powerful medical treatment for the disease.