Right ventricular dysfunction in adolescents with mild cystic fibrosis

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• 作者：Antonio Ba&ntilde ; o-Rodrigo^a ; ^c ; ^{antonio_bano@hotmail.com} ; Antonio Salcedo-Posadas^b ; Jose R. Villa-Asensi^b ; ^c ; Amalia Tamariz-Martel^a ; Alejandro Lopez-Neyra^b ; Elena Blanco-Iglesias^a
• 关键词：ACT ; right ventricular acceleration time ; AT-wave ; tissue Doppler late diastolic tricuspid annular velocity ; A-wave ; tricuspid peak late diastolic filling velocity ; BSA ; body surface area ; CF ; cystic fibrosis ; CFTR ; cystic fibrosis transmembrane conductan
• 刊名：Journal of Cystic Fibrosis
• 出版年：2012
• 期刊代码：298_15691993
• 类别：med
• 出版时间：July, 2012
• 卷：11
• 期：4
• 页码：274-280
• 文件大小：152 K

摘要

Background

In cystic fibrosis (CF) patients the right ventricle (RV) suffers a progressive deterioration, but it is not clear when these changes begin. The aim of this study was to analyze the RV function in CF patients with mild respiratory disease.

Methods

Color-Doppler-Echocardiographic studies were prospectively performed in CF adolescent patients and an age-matched control group. Findings were correlated with pulmonary function tests (PFT), genotype, chronic bacterial colonization, pancreatic status and clinical scores. Only patients with mild CF were selected.

Results

Thirty seven CF patients and 40 healthy controls were recruited. In CF patients all echocardiographic parameters were abnormal compared to controls. Doppler analysis showed slightly elevated pulmonary artery pressure values, and abnormal relaxation and systolic function for all indexes. No correlation was found with any of the features studied.

Conclusions

In CF patients, abnormalities in the structure and function of the RV may be present at early stages of the disease. These abnormalities are subclinical and do not correlate with clinical scores, PFT, genotype, chronic bacterial colonization or pancreatic insufficiency.