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[Left ventricular involvement in mucoviscidosis after 2 years of age]
P Ambrosi1, J P Chazalettes, L Viard
1Service de Cardiologie Isotopique, Hôpital de la Timone, Marseille.
Insights
Cystic fibrosis can affect the heart, leading to reduced left ventricular ejection fraction and perfusion abnormalities. These findings suggest myocardial fibrosis in advanced cystic fibrosis stages.
Area of Science:
- Cardiology
- Pediatrics
- Pulmonology
Background:
- Cystic fibrosis (CF) is associated with cardiac complications, including cardiomyopathy in infants and altered left ventricular function in children.
- While overt left ventricular insufficiency is rare in young CF patients, subclinical cardiac dysfunction may be present.
Purpose of the Study:
- To investigate cardiac involvement in young patients with cystic fibrosis (CF).
- To assess left ventricular (LV) function and myocardial perfusion in CF patients with low Shwachman scores.
Main Methods:
- Echocardiography, thallium-201 tomoscintigraphy, and 99mTc ventriculography were used to evaluate 67 pediatric CF patients (6-34 months old) with Shwachman scores < 70.
- Cardiac function and perfusion were assessed, with particular attention to right and left ventricular parameters.
Main Results:
- Thirty-two of 58 patients showed right ventricular dilation on echocardiography.
- Reduced left ventricular ejection fraction (<45%) was observed in 17 patients, with 6 exhibiting thallium hypofixation.
- Thallium hypofixation was more common in patients with lower left ventricular ejection fraction.
Conclusions:
- Resting myocardial perfusion abnormalities are more prevalent in cystic fibrosis patients with diminished left ventricular ejection fraction.
- These perfusion deficits indicate potential myocardial fibrosis in advanced stages of cystic fibrosis, highlighting the need for cardiac monitoring.
Background:
The cardiac involvement in cystic fibrosis includes the rare cardiomyopathy seen in infants and changes in left ventricular performance in older children.
Population And Methods:
67 patients, 6 to 34 months-old (mean: 16.7), 37 male and 30 female, with cystic fibrosis, were studied. Their Shwachman score was < 70. None showed any clinical manifestations of left ventricular insufficiency, but 6 patients had right ventricular insufficiency. Echocardiography was performed on 58 patients; it showed dilation of the right ventricle in 32 of them. Left ventricular perfusion was studied with thallium 201 tomoscintigraphy and left ventricular ejection fraction with 99mTc ventriculography.
Results:
The left ventricular ejection fraction was < 45% in 17 patients and scintigraphy showed hypofixation in 6 of them. In contrast, only 4 of the 50 patients with left ventricular ejection fraction > 45% had thallium hypofixation.
Conclusions:
Resting perfusion abnormalities are more frequent in patients with a low left ventricular ejection fraction. These perfusion abnormalities suggest that myocardial fibrosis complicates the advanced stages of cystic fibrosis.