Abstract | HISTORY: DIAGNOSTIC FEATURES, TREATMENT AND COURSE: She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 | PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome. CONCLUSION:
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Authors | S Rosenkranz, E Caglayan, F Diet, T Karasch, J Weihrauch, K Wassermann, E Erdmann |
Journal | Deutsche medizinische Wochenschrift (1946)
(Dtsch Med Wochenschr)
Vol. 129
Issue 33
Pg. 1736-40
(Aug 13 2004)
ISSN: 0012-0472 [Print] Germany |
Vernacular Title | Langzeiteffekte von Sildenafil bei Sklerodermie-assoziierter pulmonaler Hypertonie und Raynaud-Syndrom. |
PMID | 15295684
(Publication Type: Case Reports, English Abstract, Journal Article)
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Chemical References |
- Phosphodiesterase Inhibitors
- Piperazines
- Purines
- Sulfones
- Vasodilator Agents
- Atrial Natriuretic Factor
- Sildenafil Citrate
- Phosphoric Diester Hydrolases
- 3',5'-Cyclic-GMP Phosphodiesterases
- Cyclic Nucleotide Phosphodiesterases, Type 5
- PDE5A protein, human
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Topics |
- 3',5'-Cyclic-GMP Phosphodiesterases
- Aged
- Atrial Natriuretic Factor
(blood)
- Cyclic Nucleotide Phosphodiesterases, Type 5
- Female
- Fingers
(blood supply)
- Humans
- Hypertension, Pulmonary
(drug therapy, etiology)
- Laser-Doppler Flowmetry
- Phosphodiesterase Inhibitors
(pharmacology, therapeutic use)
- Phosphoric Diester Hydrolases
(drug effects)
- Piperazines
(pharmacology, therapeutic use)
- Prognosis
- Pulmonary Wedge Pressure
(drug effects)
- Purines
- Raynaud Disease
(complications, drug therapy)
- Regional Blood Flow
(drug effects)
- Scleroderma, Systemic
(complications, drug therapy)
- Sildenafil Citrate
- Sulfones
- Vasodilator Agents
(pharmacology, therapeutic use)
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