Abstract | BACKGROUND: METHODS AND RESULTS: Twenty patients (15+/-14 years) with PHT and associated CHD (9 atrial septal defect, 7 ventricular septal defect, 4 transposition of the great vessels, 3 patient ductus arteriosus, 3 partial anomalous pulmonary venous return, and 1 aortopulmonary window) who failed conventional therapy (including digitalis; diuretics; oxygen; warfarin; calcium channel blockade, if indicated; and surgery, if operable) were treated with chronic PGI2. Eleven patients had previous cardiac surgery at a median age of 3 years (range, 5 days to 47 years). Eleven of 20 patients had residual systemic to pulmonary shunts. Hemodynamics, NYHA functional class, and exercise capacity were measured at baseline and after 1 year of PGI2 therapy. None of the patients acutely responded to PGI2 administration. Despite lack of an acute response, mean pulmonary artery pressure decreased 21% on chronic PGI2: 77+/-20 to 61+/-15 mm Hg (P<0.01, n=16). Cardiac index and pulmonary vascular resistance also improved on long-term PGI2: 3. 5+/-2.0 to 5.9+/-2.7 L. min-1. m-2 (P<0.01, n=16), and 25+/-13 to 12+/-7 U.m2 (P<0.01, n=16), respectively. NYHA functional class improved from 3.2+/-0.7 to 2.0+/-0.9 (P<0.0001, n=19). Exercise capacity increased from 408+/-149 to 460+/-99 m (P=0.13, n=14) on long-term PGI2. CONCLUSIONS: Chronic PGI2 improves hemodynamics and quality of life in patients with PHT and associated CHD who fail conventional therapy. As previously demonstrated in patients with primary pulmonary hypertension, long-term PGI2 may have an important role in the treatment of patients with PHT and associated CHD.
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Authors | E B Rosenzweig, D Kerstein, R J Barst |
Journal | Circulation
(Circulation)
Vol. 99
Issue 14
Pg. 1858-65
(Apr 13 1999)
ISSN: 1524-4539 [Electronic] United States |
PMID | 10199883
(Publication Type: Clinical Trial, Journal Article, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- Antihypertensive Agents
- Epoprostenol
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Topics |
- Adult
- Antihypertensive Agents
(administration & dosage, adverse effects, therapeutic use)
- Child
- Child, Preschool
- Epoprostenol
(administration & dosage, adverse effects, therapeutic use)
- Female
- Heart Defects, Congenital
(complications, surgery)
- Hemodynamics
(drug effects)
- Humans
- Hypertension, Pulmonary
(complications, drug therapy, physiopathology)
- Infant
- Male
- Physical Endurance
- Severity of Illness Index
- Time Factors
- Treatment Outcome
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