CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Cardiac involvement in cystic fibrosis: early noninvasive detection and vasodilator therapy.

Study design (if review, criteria of inclusion for studies)

part 1) prospective study. Part 2) inpatient double-blinded randomization

Participants

part 1) 23 ambulatory CF patients with mild CF (clinical score m = 79 +/- 8, range 61 to 90). Part 2) All 13 with RVD

Interventions

part 1) Echocardiography (echo) and radionuclide angiography (RA) were performed at rest and with exercise stress testing (EST) in all patients to select those with early RVD. Part 2) placebo or hydralazine orally without changing other standard therapy

Outcome measures

echo and RA score

Main results

part 1) 13 of twenty-three had RVD on echo or RA evidenced by increased RV dimensions (P less than 0.001) on echo and decreased RV ejection fraction (EF) on RA (P less than 0.01) but 30% of these patients were abnormal only with EST. part 2) With hydralazine no change from placebo was seen on any echo or RA measurement at rest or with EST other than left ventricular STI which fell (P less than 0.05).

Authors' conclusions

These data indicate: 1) EST with echo and RA detects RVD in CF earlier than resting studies, 2) patients with mild CF (clinical score less than 85) frequently have RVD on EST, and 3) hydralazine does not improve RVD in CF even very early in its development.

Keywords: Adolescent; Adult; Child; pharmacological_intervention; Oral; exercise;