The cardiomyopathy of cystic fibrosis: a modern form of Keshan disease.

Introduction: We conducted a study to determine the prevalence of structural heart disease in patients with CF, the characteristics of a cardiomyopathy not previously described in this population, and its possible relationship with nutritional deficiencies in CF. Methods: We studied 3 CMP CF patients referred for heart-lung transplantation and a prospective series of 120 adult CF patients. All patients underwent a clinical examination, blood tests including levels of vitamins and trace elements, and echocardiography with evaluation of myocardial strain. Cardiac magnetic resonance imaging (CMR) was performed in patients with CMP and in a control group. Histopathological study was performed on hearts obtained in transplant or necropsy. Results: We found a prevalence of 10% (CI 4.6%-15.4%) of left ventricular (LV) dysfunction in the prospective cohort. Myocardial strain parameters were already altered in CF patients with otherwise normal hearts. Histopathological examination of 4 hearts from CF CMP patients showed a unique histological pattern of multifocal myocardial fibrosis similar to Keshan disease. Four of the five CF CMP patients undergoing CMR showed late gadolinium uptake, with a characteristic patchy pattern in 3 cases (p < 0.001 vs. CF controls). Selenium deficiency (Se < 60 µg/L) was associated with more severe LV dysfunction, higher prevalence of CF CMP, higher NTproBNP levels, and more severe pulmonary and digestive involvement. Conclusion: 10% of adults with CF showed significant cardiac involvement, with histological and imaging features resembling Keshan disease. Selenium deficiency was associated with the presence and severity of LV dysfunction in these patients.

The diagnostic performance of non-contrast 3-Tesla magnetic resonance imaging (3-T MRI) versus 1.5-Tesla magnetic resonance arthrography (1.5-T MRA) in femoro-acetabular impingement.

OBJECTIVE: The aim of this study was to evaluate the diagnostic accuracy of 3-T non-contrast MRI versus 1.5-T MRA for assessing labrum and articular cartilage lesions in patients with clinical suspicion of femoro-acetabular impingement (FAI). SUBJECTS AND METHODS: Fifty patients (thirty men and twenty women, mean age 42.5 years) underwent 1.5-T MRA, 3-T MRI and arthroscopy on the same hip. An optimized high-resolution proton density spin echo pulse sequence was included in the 3-T non-contrast MRI protocol. RESULTS: The 3-T non-contrast MRI identified forty-two of the forty-three arthroscopically proven tears at the labral-chondral transitional zone (sensitivity, 97.7%; specificity, 100%; positive predictive value (PPV), 100%; negative predictive value (NPV), 87.5%; accuracy 98%). With 1.5-T MRA, forty-four tears were diagnosed. However, there was one false positive (sensitivity, 100%; specificity, 85.7%; PPV, 97.7%; NPV, 100%; accuracy 98%). Agreement between arthroscopy and MRI, whether 3-T non-contrast MRI or 1.5-T MRA, as to the degree of chondral lesion in the acetabulum was reached in half of the patients and in the femur in 76% of patients. CONCLUSION: Non-invasive assessment of the hip is possible with 3-T MR magnet. 3-T non-contrast MRI could replace MRA as the workhorse technique for assessing hip internal damage. MRA would then be reserved for young adults with a strong clinical suspicion of FAI but normal findings on 3-T non-contrast MRI. When compared with 1.5-T MRA, optimized sequences with 3-T non-contrast MRI help in detecting normal variants and in diagnosing articular cartilage lesions.