BackgroundLeft atrial (LA) late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) imaging is indicative of fibrosis, and has been correlated with reduced LA function, increased LA volume, and poor procedural outcomes in cohorts with atrial fibrillation (AF). However, the role of LGE as a prognostic biomarker for arrhythmia in cardiac disease has not been examined.MethodsIn this study, we assessed LA LGE using a 3D LGE CMR sequence to examine its relationships with new onset atrial arrhythmia, and LA and left ventricular (LV) mechanical function.ResultsLA LGE images were acquired in 111 patients undergoing CMR imaging, including 66 patients with no prior history of an atrial arrhythmia. During the median follow-up of 2.7 years (interquartile range (IQR) 1.8–3.7 years), 15/66 (23%) of patients developed a new atrial arrhythmia. LA LGE ≥10% of LA myocardial volume was significantly associated with an increased rate of new-onset atrial arrhythmia, with a hazard ratio of 3.16 (95% CI 1.14–8.72), p = 0.026. There were significant relationships between LA LGE and both LA ejection fraction (r = − 0.39, p < 0.0005) and echocardiographic LV septal e’ (r = − 0.24, p = 0.04) and septal E/e’ (r = 0.31, p = 0.007).ConclusionsElevated LA LGE is associated with reduced LA function and reduced LV diastolic function. LA LGE is associated with new onset atrial arrhythmia during follow-up.Electronic supplementary materialThe online version of this article (10.1186/s12968-018-0514-3) contains supplementary material, which is available to authorized users.
X-linked hypophosphatemia is an inheritable disorder of renal phosphate wasting that clinically manifests with rachitic bone pathology. X-linked hypophosphatemia is frequently misdiagnosed and mismanaged. Optimized medical therapy is the cornerstone of treatment. Even with ideal medical management, progressive bony deformity may develop in some children and adults. Medical treatment is paramount to the success of orthopaedic surgical procedures in both children and adults with X-linked hypophosphatemia. Successful correction of complex, multiapical bone deformities found in patients with X-linked hypophosphatemia is possible with careful surgical planning and exacting surgical technique. Multiple methods of deformity correction are used, including acute and gradual correction. Treatment of some pediatric bony deformity with guided growth techniques may be possible.
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