COMPARATIVE STUDY
JOURNAL ARTICLE

Effectiveness of transition from intravenous epoprostenol to oral/inhaled targeted pulmonary arterial hypertension therapy in pediatric idiopathic and familial pulmonary arterial hypertension

Laura Melnick, Robyn J Barst, Cherise A Rowan, Diane Kerstein, Erika B Rosenzweig
American Journal of Cardiology 2010 May 15, 105 (10): 1485-9
20451700
Before 2001, intravenous epoprostenol was the only approved drug for patients with idiopathic pulmonary arterial hypertension (IPAH) or familial pulmonary arterial hypertension (FPAH) who were nonresponsive to high-dose calcium channel blockade. The investigators report transitioning select pediatric patients with IPAH or FPAH from intravenous epoprostenol to oral and/or inhaled agents for pulmonary arterial hypertension. A retrospective review was performed on all pediatric patients with IPAH or FPAH treated at Columbia University (1987 to 2008) who transitioned off epoprostenol to oral or inhaled drugs. Criteria for transition included functional class I or II, mean pulmonary arterial pressure <35 mm Hg, normal cardiac index, and age >6 years. Hemodynamic and clinical data were obtained at baseline (before epoprostenol), at peak epoprostenol dose, and after epoprostenol discontinuation. Fourteen of 104 pediatric patients with IPAH or FPAH transitioned off epoprostenol to oral or inhaled drugs from April 2003 to July 2008. Of the 14 subjects, 13 transitioned off epoprostenol successfully to oral or inhaled drugs. No significant changes in functional class, hemodynamics, or exercise data were seen after epoprostenol (mean follow-up duration 7.0 +/- 5.8 months) compared to peak epoprostenol, but further improvement was reported in World Health Organization functional class (p <0.005) after epoprostenol discontinuation. After successful epoprostenol discontinuation, 77% of patients were treated with endothelin receptor antagonists, 69% with phosphodiesterase-5 inhibitors, 38% with calcium channel blockers, and 8% inhaled iloprost. At the cut-off date (May 2009), there was 100% survival and 93% transition success. In conclusion, in carefully selected children with IPAH or FPAH initiated on intravenous epoprostenol before the availability of nonparenteral therapy, transition to oral or inhaled therapy for pulmonary arterial hypertension appears safe, with efficacy maintained when performed with close follow-up at a pulmonary hypertension specialty center.

Full Text Links

Find Full Text Links for this Article

Discussion

You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read
20451700
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"