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Left ventricular perfusion deficit in patients with cystic fibrosis
D De Wolf1, P Franken, A Piepsz
1Department of Pediatrics, AZ VUB, Vrije Universiteit Brussels, Belgium.
Insights
Abnormalities in left ventricular perfusion are common in cystic fibrosis (CF) patients with severe lung disease. These perfusion deficits during exercise may indicate a poor prognosis for individuals with CF.
Area of Science:
- Cardiology
- Pulmonology
- Nuclear Medicine
Background:
- Left ventricular (LV) dysfunction is not a primary feature of cystic fibrosis (CF), but functional abnormalities have been reported.
- Myocardial perfusion disturbances can lead to LV dysfunction, prompting investigation in CF patients.
Purpose of the Study:
- To investigate left ventricular (LV) myocardial perfusion during exercise in cystic fibrosis (CF) patients using sestamibi-Tc-99m-labeled macroaggregates.
Main Methods:
- Eighteen CF patients underwent clinical evaluation, lung perfusion scans, pulmonary function tests, echocardiography, and exercise testing with myocardial perfusion imaging.
- Sestamibi-Tc-99m-labeled macroaggregates were injected at peak exercise to assess LV perfusion.
Main Results:
- Six out of eighteen (33%) CF patients exhibited abnormal myocardial distribution of sestamibi-Tc-99m-labeled macroaggregates during exercise.
- These perfusion abnormalities correlated with clinical severity scores, reduced MEF50, and exercise-induced arterial oxygen desaturation.
Conclusions:
- Deficits in LV uptake of sestamibi-Tc-99m-labeled macroaggregates during exercise are common in severe CF lung disease.
- The underlying cause remains unclear, but these perfusion deficits appear associated with a poorer prognosis in CF patients.
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.