title: Left ventricular perfusion deficit in patients with cystic fibrosis.
abstract: Left ventricular failure is not considered an important feature in cystic fibrosis (CF), but abnormalities of left ventricular function have been reported. Except for a few cases of heart failure in neonates with CF, there is no evidence of a primary disorder of the myocardium in patients with CF. Since left ventricular perfusion disturbances can cause left ventricular dysfunction, we decided to investigate left ventricular perfusion during exercise using sestamibi-Tc-99m-labeled macroaggregates. Eighteen CF patients with varying degrees of disease severity participated in the study. They underwent a thorough clinical evaluation, lung perfusion scan, pulmonary function testing, echocardiography, transcutaneous measurement of oxygen saturation at rest and during exercise, and an exercise test with injection of sestamibi-Tc-99m-labeled macroaggregates at peak exercise. Six patients (33%) showed abnormalities of the myocardial distribution of sestamibi-Tc-99m-labeled macroaggregates during exercise. Scanning abnormalities correlated with the clinical score, mean maximum expiratory flow at 50% of vital capacity (MEF50), and arterial oxygen desaturation during exercise. We conclude that deficits in left ventricular uptake of sestamibi-Tc-99m-labeled macroaggregates during exercise seem common in patients with severe CF lung disease. The cause of these deficits is not fully understood, but the occurrence seems to be associated with a poor prognosis.
authors.affiliation: Department of Pediatrics, AZ VUB, Vrije Universiteit Brussels, Belgium.
authors.forename: D
authors.initials: D
authors.lastname: De Wolf
authors.forename: P
authors.initials: P
authors.lastname: Franken
authors.forename: A
authors.initials: A
authors.lastname: Piepsz
authors.forename: I
authors.initials: I
authors.lastname: Dab
mesh_terms: D000328:Adult; D003326:Coronary Circulation; D003550:Cystic Fibrosis / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D005080:Exercise Test; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging; D008297:Male; D008448:Maximal Expiratory Flow Rate; D010100:Oxygen / Q000097:blood; D017256:Technetium Tc 99m Sestamibi; D015899:Tomography, Emission-Computed, Single-Photon; D018487:Ventricular Dysfunction, Left / Q000150:complications; D014797:Vital Capacity
chemical_list: D017256:Technetium Tc 99m Sestamibi; D010100:Oxygen