Abstract 12212: A Challenging Search for Heart Dysfunction in Cystic Fibrosis
The two main affectations that characterize cystic fibrosis (CF) disease are an obstructive neumopathy and a pancreatic exocrine deficiency; the latter being responsible for the frequently associated malapsortion syndrome. A rare form of cardiomyopathy (CMP) has been described in children with CF since the 1950’s, with a histology similar to those seen in malnourishment cardiomyopathies, such as Keshan’s disease. Our aim was to describe the prevalence and features of CMP in a population of CF patients.
Methods: We studied a series of unselected adult CF patients without known heart disease. After obtaining clinical and genetic information, we performed a blood test, a proBNP, and an echocardiographic study. We defined systolic dysfunction as left ventricular ejection fraction<55% (Simpson's method). Diastolic dysfunction was defined by pathological patterns in mitral flow as classically obtained by doppler ultrasound. Patients with data of CMP were also studied with magnetic resonance (MR).
Results: Studied population included 120 adult CF patients recruited from 4 specialized outpatient clinics. Mean age was 31± 9 years, and 50 % were male. Pancreatic disease was present in 81% of them, and low levels of vitamins and trace elements were common in spite of receiving dietary supplements. Left heart disease of any kind was present in 12 patients, with a prevalence of 10%; systolic dysfunction was evident in 5 patients (4%), and diastolic dysfunction in 7 patients (6%).Systolic dysfunction was significantly associated with a patchy myocardial enhancement pattern on MR (P<0.05), as well as with a higher frequency of Pseudomonas aeuriginosa airway colonization (p=0.03) and a significantly lower body mass index (19±3 kg/m2 vs 24±2 kg/m2; p=0.02). Median value for proBNP in systolic dysfunction was 1600 pg/ml (range 31 - 8000 pg/ml).
Conclusions: The prevalence of CMP in a group of adults with CF was10%. The patients with left ventricular systolic dysfunction had patchy myocardial fibrosis, similar to the one in malabsorption cardimyopathies. This kind of heart disease can now be included in the clinical spectrum of CF and should be ruled out, especially in those with severe malabsorption or under evaluation for pulmonary transplant.
- © 2012 by American Heart Association, Inc.