Efficacy of oral sildenafil in a beraprost-treated patient with severe pulmonary hypertension secondary to type I glycogen storage disease

Circulation Journal

Volumn 73, Issue 10, 2009, Pages 1965-1968

  Ueno, Michihiko ^a   Murakami, Tomoaki ^a   Takeda, Atsuhito ^a   Kubota, Mitsuru ^a  

^a HOKKAIDO UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

Author keywords

Beraprost sodium; Glycogen storage disease; Pulmonary arterial hypertension; Sildenafil

Indexed keywords

BERAPROST; BRAIN NATRIURETIC PEPTIDE; LOPRINONE; SILDENAFIL;

ADOLESCENT; ARTICLE; CASE REPORT; CLINICAL FEATURE; CREATINE KINASE BLOOD LEVEL; DIAGNOSTIC IMAGING; DRUG DOSE INCREASE; DRUG EFFICACY; DRUG TOLERANCE; DRUG TREATMENT FAILURE; DYSPNEA; FORWARD HEART FAILURE; GLYCOGEN STORAGE DISEASE TYPE 1; HEART RIGHT VENTRICLE PRESSURE; HUMAN; IMAGE ANALYSIS; MALE; PULMONARY HYPERTENSION; SIDE EFFECT; SYMPTOM; SYNCOPE;

ADMINISTRATION, ORAL; ADOLESCENT; ANTIHYPERTENSIVE AGENTS; DRUG TOLERANCE; ECHOCARDIOGRAPHY; ELECTROCARDIOGRAPHY; EPOPROSTENOL; GLYCOGEN STORAGE DISEASE TYPE I; HEMODYNAMICS; HUMANS; HYPERTENSION, PULMONARY; MALE; NATRIURETIC PEPTIDE, BRAIN; PIPERAZINES; PURINES; SEVERITY OF ILLNESS INDEX; SULFONES; SYNCOPE; TREATMENT OUTCOME; VASODILATOR AGENTS;

EID: 70349883809     PISSN: 13469843     EISSN: 13474820     Source Type: Journal    
DOI: 10.1253/circj.CJ-08-0181     Document Type: Article

References (25)

1. Chen YT. Glycogen storage disease. In: Behrman RE, Kliegman RM, Jenson HB, editors. Textbook of pediatrics. 17th edn. Philadelphia: Saunders; 2004; 469-475.
2. Pizzo CJ. Type I glycogen storage disease with focal nodular hyperplasia of the liver and vasoconstrictive pulmonary hypertension. Pediatrics 1980; 65: 341-344.
3. Furukawa N, Kinugasa A, Inoue F, Imashuku S, Takamatsu T, Sawada T. Type I glycogen storage disease with vasoconstrictive pulmonary hypertension. J Inher Metab Dis 1990; 13: 102-107. (Pubitemid 20141308)
4. Humbert M, Labrune P, Sitborn O, Le Gall C, Callebert J, Herve P, et al. Pulmonary arterial hypertension and type-I glycogen-storage disease: The serotonin hypothesis. Eur Respir J 2002; 20: 59-65. (Pubitemid 34822922)
5. Humbert M, Labrune P, Simonneau G. Severe pulmonary hypertension in type 1 glycogen storage disease. Eur J Pediatr 2002; 161: S93-S96.
6. Wanstall JC, Jeffery TK. Recognition and management of pulmonary hypertension. Drugs 1998; 56: 989-1007.
7. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hyperten- sion. N Engl J Med 1996; 334: 296-301. (Pubitemid 26033085)
8. Shapiro SM, Oudiz RJ, Cao T, Romano MA, Beckmann XJ, Georgiou D, et al. Primary pulmonary hypertension: Improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol 1997; 30: 343-349. (Pubitemid 27339311)
9. Miyata M, Ueno Y, Sekine H, Ito O, Sakuma F, Koike H, et al. Protective effect of beraprost sodium, a stable prostacyclin analogue, in development of monocrotaline-induced pulmonary hypertension. J Cardiovasc Pharmacol 1996; 27: 20-26. (Pubitemid 26010634)
10. 2 analogue, beraprost, in primary and secondary pulmonary hypertension. Am J Cardiol 1996; 78: 244-247. (Pubitemid 26244382)
11. Kukreja RC, Ockaili R, Salloum F, Yin C, Hawkins J, Das A, et al. Cardioprotection with phosphodiesterase-5 inhibition: A novel preconditioning strategy. J Mol Cell Cardiol 2004; 36: 165-173. (Pubitemid 38202344)
12. Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148-2157. (Pubitemid 41653106)
13. Lewis JF, Kuo LC, Nelson JG, Limacher MC, Quinones MA. Pulsed Doppler echocardiographic determination of stroke volume and cardiac output: Clinical validation of two new methods using the apical window. Circulation 1984; 70: 425-431.
14. Mclaughlin W, Genthner DE, Panella MM, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998; 338: 273-277. (Pubitemid 28065252)
15. Nishio S, Matsuura H, Kanai N, Fukatsu Y, Hirano T, Nishikawa N, et al. The in vitro and ex vivo antiplatelet effect of TRK-100, a stable prostacyclin analog, in several species. Jpn J Pharmacol 1988; 47: 1-10. (Pubitemid 18142601)
16. Galie N, Humbert M, Vachiery JL, Vizza CD, Kneussl M, Manes A, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: A randomized, double- blind, placebo-controlled. J Am Coll Cardiol 2002; 39: 1496-1502. (Pubitemid 34439066)
17. Barst RJ, McGoon M, McLaughlin V, Tapson V, Oudiz R, Shapir S, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 2119-2125. (Pubitemid 36724255)
18. Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J 2006; 151: e1-e5.
19. Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: Comparison with inhaled nitric oxide. Circulation 2002; 105: 2398-2403. (Pubitemid 34556392)
20. Bhatia S, Frantz RP, Severson CJ, Durst LA, McGoon MD. Immediate and long-term hemodynamics and clinical effects of sildenafil in patients with pulmonary arterial hypertension receiving vasodilator therapy. Mayo Clin Proc 2003; 78: 1207-1213. (Pubitemid 37193511)
21. Itoh T, Nagaya N, Fujii T, Iwase T, Nakanishi N, Hamada K, et al. A combination of oral sildenafil and beraprost ameliorates pulmonary hypertension in rats. Am J Respir Crit Care Med 2004; 169: 34-38. (Pubitemid 38233886)
22. Ikeda D, Tsujino I, Ohira H, Itoh N, Kamigaki M, Ishimaru S, et al. Addition of oral sildenafil to beraprost is a safe and effective therapeutic option for patients with pulmonary hypertension. J Cardiovasc Pharmacol 2005; 45: 286-289. (Pubitemid 40411281)
23. Miwa K, Matsubara T, Uno Y, Yasuda T, Sakata K, Tsuda T, et al. Combination therapy with oral sildenafil and beraprost for pulmonary arterial hypertension associated with CREST syndrome. Int Heart J 2007; 48: 417-422. (Pubitemid 47181015)
24. Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: A randomized placebo- controlled study. Lancet 2001; 358: 1119-1123. (Pubitemid 32971843)
25. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903. (Pubitemid 34984638)