Increased temporal dispersion of myocardial repolarization in myotonic dystrophy type 1: beyond the cardiac conduction system

Objectives

To assess ventricular dysfunction and ventricular interaction after repair of Tetralogy of Fallot (ToF) employing echocardiography speckle-tracking and cardiac magnetic resonance imaging (CMR).

Background

Severe pulmonary regurgitation and right ventricular (RV) dysfunction are common after repair of ToF and may also affect the shape and function of the left ventricle (LV). Recent studies suggest that LV dysfunction may be of particular prognostic value.

Methods and results

Twenty-one consecutive adults with repaired ToF (15 male, mean age 38 ± 11 years, 7 with severe PR) underwent a comprehensive echocardiographic exam including speckle-tracking analysis, CMR and cardiopulmonary exercise testing. Twenty-one subjects without relevant heart disease served as controls. Echocardiographically measured RV diameters correlated with RV volumes obtained from CMR (r = 0.63; p = 0.006). In addition, a close correlation was found between RV and LV function on CMR (r = 0.74, p = 0.002), speckle-tracking LV and RV peak longitudinal 2D strain (r = 0.66, p = 0.003) and mitral and tricuspid annular plain systolic excursion (r = 0.71, p = 0.0003). While LV ejection fraction was normal in the majority of patients and not different from controls, LV longitudinal strain was significantly reduced in ToF patients (− 16.5 ± 3.3 vs. -20.5 ± 2.7%, p = 0.0001).

Conclusion

Left and right ventricular function both by CMR and speckle-tracking is interrelated in adults with repaired ToF. Despite normal LV ejection fraction, 2D longitudinal strain is significantly reduced in ToF patients, suggesting subclinical LV myocardial damage. Considering the potential prognostic value of LV dysfunction in ToF, this measurement may gain importance and should be included in future outcome studies.     

Metastatic Crohn's disease. Response to adalimumab dose intensification

Inflammatory bowel disease is accompanied by extraintestinal manifestations in a high percentage of patients. Cutaneous lesions are the second most prevalent of these manifestations, and within these, metastatic Crohn's disease is one of the least common, being the least frequent specific cutaneous manifestation of Crohn's disease. This entity includes cutaneous and subcutaneous lesions with a non-caseating granulomatous appearance on histological analysis identical to that of Crohn's disease. These lesions are not found adjacent to the digestive tract. Due to the low prevalence of these manifestations, conclusive trials on the treatment of choice have not been performed and there is no well-defined therapeutic strategy. Distinct therapies with varying results have been reported. We report the case of a female patient with longstanding and complex Crohn's disease who developed metastatic cutaneous manifestations while receiving adalimumab. The cutaneous manifestations responded well to dose intensification of this drug. A review of the literature is provided.