Comprehensive Cardiovascular Management of Myotonic Dystrophy Type 1 Patients: A Report from the Italian Neuro-Cardiology Network.

Myotonic dystrophy is a hereditary disorder with systemic involvement. The Italian Neuro-Cardiology Network-"Rete delle Neurocardiologie" (INCN-RNC) is a unique collaborative experience involving neurology units combined with cardio-arrhythmology units. The INCN facilitates the creation of integrated neuro-cardiac teams in Neuromuscular Disease Centers for the management of cardiovascular involvement in the treatment of myotonic dystrophy type 1 (MD1).

Effects of Exercise on Arrhythmia (and Viceversa): Lesson from the Greek Mythology.

Exercise represents an important lifestyle factor in all human ages when felt in harmony with other psycho-physical and environmental variables that affect individual life (e. g. quality of interest, affections, environment, diet and food). Consequently, in addition to the training level, the amount, intensity and modality of exercise (ana-/aerobic, isometric/isotonic), need to be personalized, considering the underlying diseases, which may benefit from it or worsening.Greek mythology gives us good examples of the exercise concept's evolution.From Discus-thrower to Spear-carrier the idea of physical activity is more effectively expressed. The Myron Discobolus displays the enduring pattern of athletic energy translated into the dynamic force given by the exercise. In Doryphoros instead, the physical activity is oriented to the achievement of the required psyco-physical harmony, who's the concept is aimed of being expressed by the sculpture.As outlined below, even in the field of arrhythmia, scientific evidence as well as clinical experience, supports the same concept: physical activity may be important while safely managed and personalized.

Passive diastolic modelling of human ventricles: Effects of base movement and geometrical heterogeneity.

Left-ventricular (LV) remodelling, associated with diastolic heart failure, is driven by an increase in myocardial stress. Therefore, normalisation of LV wall stress is the cornerstone of many therapeutic treatments. However, information regarding such regional stress-strain for human LV is still limited. Thus, the objectives of our study were to determine local diastolic stress-strain field in healthy LVs, and consequently, to identify the regional variations amongst them due to geometric heterogeneity. Effects of LV base movement on diastolic model predictions, which were ignored in the literature, were further explored. Personalised finite-element modelling of five normal human bi-ventricles was carried out using subject-specific myocardium properties. Model prediction was validated individually through comparison with end-diastolic volume and a new shape-volume based measurement of LV cavity, extracted from magnetic resonance imaging. Results indicated that incorporation of LV base movement improved the model predictions (shape-volume relevancy of LV cavity), and therefore, it should be considered in future studies. The LV endocardium always experienced higher fibre stress compared to the epicardium for all five subjects. The LV wall near base experienced higher stress compared to equatorial and apical locations. The lateral LV wall underwent greater stress distribution (fibre and sheet stress) compared to other three regions. In addition, normal ranges of different stress-strain components in different regions of LV wall were reported for five healthy ventricles. This information could be used as targets for future computational studies to optimise diastolic heart failure treatments or design new therapeutic interventions/devices.

Pathology and function of conduction tissue in Fabry disease cardiomyopathy.

BACKGROUND: Cardiac arrhythmias are common in Fabry disease (FD) and may occur in prehypertrophic cardiomyopathy suggesting an early compromise of conduction tissue (CT). Therefore, FD X-linked and CT may be variously involved in male and female patients with FD cardiomyopathy, affecting CT function. METHODS AND RESULTS: Among 74 patients with endomyocardial biopsy diagnosis of FD cardiomyopathy, 13 (6 men; 7 women; mean age, 50.1±13.5 years; maximal wall thickness, 16.7±3.7 mm) had CT included in histological specimens and 6 also at electron microscopy. CT glycolipid infiltration was defined as focal, moderate, extensive, or massive, if involved ≤30%, ≤50%, >50%, or 100% of cells; identified as loosely arranged small myocytes positive to HCN4 immunostaining, supplied by a centrally placed thick-walled arteriole. CT involvement was correlated with age, sex, and α-Gal gene mutation. CT function was evaluated by electrophysiological study and arrhythmias at Holter registration. CT infiltration was focal/moderate in 4 women with no arrhythmias and normal electrophysiological study, extensive in 3 women with atrial or ventricular arrhythmias and short HV interval, and massive in 6 men with atrial fibrillation or ventricular arrhythmias and short HV. Short PR/AH with increased refractoriness was additionally found in 3 patients with extensive/massive CT infiltration. A male patient with the shortest HV presented infra-Hissian block during decremental atrial stimulation. There was no correlation with age, maximal wall thickness, and type of gene mutation. CONCLUSIONS: CT infiltration in FD cardiomyopathy is constant in men and variable in women because of skewed X-chromosome inactivation; its extensive/massive involvement causes accelerated conduction with prolonged refractoriness and electric instability.

Improving comfort of shoe sole through experiments based on CAD-FEM modeling.

It was reported that next to style, comfort is the second key aspect in purchasing footwear. One of the most important components of footwear is the shoe sole, whose design is based on many factors such as foot shape/size, perceived comfort and materials. The present paper focuses on the parametric analysis of a shoe sole to improve the perceived comfort. The sensitivity of geometric and material design factors on comfort degree was investigated by combining real experimental tests and CAD-FEM simulations. The correlation between perceived comfort and physical responses, such as plantar pressures, was estimated by conducting real tests. Four different conditions were analyzed: subjects wearing three commercially available shoes and in a barefoot condition. For each condition, subjects expressed their perceived comfort score. By adopting plantar sensors, the plantar pressures were also monitored. Once given such a correlation, a parametric FEM model of the footwear was developed. In order to better simulate contact at the plantar surface, a detailed FEM model of the foot was also generated from CT scan images. Lastly, a fractional factorial design array was applied to study the sensitivity of different sets of design factors on comfort degree. The findings of this research showed that the sole thickness and its material highly influence perceived comfort. In particular, softer materials and thicker soles contribute to increasing the degree of comfort.

Effects of thread features in osseo-integrated titanium implants using a statistics-based finite element method.

OBJECTIVE: To investigate the influence of implant design factors in terms of bone integrity and implant stability. MATERIALS AND METHODS: A 3D parametric CAD model was developed. Then, once domain settings and boundary conditions were defined, a 3D FEM model was created. To simulate the physical interaction at the bone-implant interface, identity pairs were introduced. After generating different design scenarios with a DOE approach, the most significant design factors were obtained. RESULTS: This study showed that the geometry of the screw thread highly influenced the implant stability. In particular the degree of bone damage became minimal when adopting 0.40 mm for the thread width and 0.05 mm for the thickness. SIGNIFICANCE: Thread width and thickness play a crucial role to reduce induced stresses and damage in bone. Considering these preliminary results, future improvements should focus on investigating also two-factor and higher interactions to better understand the implant loading mechanism.

Serum levels of vitamin E are associated with early recurrence of atrial fibrillation after electric cardioversion.

BACKGROUND: Oxidative stress is suggested to play a role in favoring the occurrence of atrial fibrillation (AF). We analyzed whether vitamin E, a known antioxidant, or markers of oxidative stress are associated with AF recurrence in patients undergoing electric cardioversion. METHODS AND RESULTS: A total of 144 patients (83 men; mean age, 71.1±5.4 years) underwent successful biphasic electric cardioversion of nonvalvular persistent AF. At baseline, urinary 8-isoprostaglandin F2α and serum soluble NOX2-derived peptide (sNOX2-dp), high-sensitivity C-reactive protein (hs-CRP), and vitamin E levels were measured in each patient. All patients underwent 3 months of clinical follow-up, including an office visit with ECG every week or in cases of symptom recurrence. During the follow-up, 94 patients maintained sinus rhythm, whereas 50 experienced AF recurrence. In unadjusted analysis, left atrial diameter and levels of urinary isoprostanes and serum sNOX2-dp and hs-CRP were significantly higher and serum vitamin E lower in patients with AF recurrence. In multivariable Cox analysis, serum vitamin E (hazard ratio, 0.734; 95% CI, 0.605-0.891; P<0.001) and, to a lesser extent, hs-CRP (P=0.047) remained significantly associated with AF recurrence. Urinary isoprostanes and serum sNOX2-dp levels were inversely correlated with serum vitamin E level (r=-0.626, P<0.001, and r=-0.460, P<0.001, respectively). CONCLUSIONS: The study shows that low serum vitamin E levels are associated with AF recurrence in patients who underwent cardioversion. Because vitamin E inversely correlated with oxidative stress, the findings reinforce the hypothesis of an interplay between oxidative stress and AF.

Mechanical behavior of post-restored upper canine teeth: a 3D FE analysis.

OBJECTIVES: The aim was to evaluate the stress distribution, comparing an anterior sound tooth with post-endodontic restored teeth under mechanical loading. METHODS: A three-dimensional finite element analysis was performed based on micro-CT scan images of a maxillary canine. Twelve models with different crown properties and post-configurations were simulated. The model of the maxillary sound canine was also created and investigated. A load of 50N was applied at a 63° angle with respect to the longitudinal axis of the tooth on the palatal surface of the crown. Principal stresses were registered. Numerical FEA results were statistically analyzed to show the influence of post shape and crown materials. RESULTS: All analyzed models (M1-M12) exhibited a high stress gradient, due to different material stiffnesses present at the various interfaces. The most uniform mechanical behavior of the investigated models, very similar to sound tooth, was the combination of a composite crown and a cylindrical or conical fiber-glass post. SIGNIFICANCE: The results of this study facilitate informed clinical choice between possible material combinations in restorative procedures of endodontically treated anterior teeth.

Additive value of right ventricular dyssynchrony indexes in predicting the success of cardiac resynchronization therapy: a speckle-tracking imaging study.

BACKGROUND: The purpose of our study was to analyze the evolution of left and right ventricular (LV, RV) parameters before and after cardiac resynchronization therapy (CRT) using speckle-tracking imaging (STI). METHODS AND RESULTS: Eighty-one patients with dilated cardiomyopathy (New York Heart Association functional class III or IV), left bundle branch block (QRS ≥120 ms), and LV ejection fraction ≤35% were studied with STI echocardiography before and after CRT. LV longitudinal (LV-SD12-l), radial (LV-SD6-r), and circumferential (LV-SD6-c) intraventricular dyssynchrony and LV twist (LV-t) were determined. RV dyssynchrony (RV-SD6) was defined as the standard deviation of the 6 time to peak systolic strain values. At 6 months' follow-up after CRT, the degree of dyssynchrony correlated significantly with LV ejection fraction improvement and end-systolic volume reduction. In receiver operating characteristic curve analysis, the following variables predictive of successful CRT were obtained: LV-SD12-l (area under the curve [AUC] 0.69), LV-SD6-c (AUC 0.66), LV-SD6-r (AUC 0.79), LV-t (AUC 0.81), and RV-SD6+LV-SD6-r (AUC 0.83). By combining LV and RV intraventricular dyssynchrony (LV-SD12-l + LV-SD6-r + RV-SD6), the AUC was significantly improved to 0.89 (P < .005 compared with RV-SD6+LV-SD6-r; P < .001 compared with LV-t). CONCLUSIONS: Our data show that assessment of RV dyssynchrony parameters has an incremental value in the evaluation of candidates for CRT and may supplement LV dyssynchrony information.

Numerical fatigue 3D-FE modeling of indirect composite-restored posterior teeth.

OBJECTIVE: In restored teeth, stresses at the tooth-restoration interface during masticatory processes may fracture the teeth or the restoration and cracks may grow and propagate. The aim was to apply numerical methodologies to simulate the behavior of a restored tooth and to evaluate fatigue lifetimes before crack failure. MATERIALS AND METHODS: Using a CAD-FEM procedure and fatigue mechanic laws, the fatigue damage of a restored molar was numerically estimated. Tessellated surfaces of enamel and dentin were extracted by applying segmentation and classification algorithms, to sets of 2D image data. A user-friendly GUI, which enables selection and visualization of 3D tessellated surfaces, was developed in a MatLab(®) environment. The tooth-boundary surfaces of enamel and dentin were then created by sweeping operations through cross-sections. A class II MOD cavity preparation was then added into the 3D model and tetrahedral mesh elements were generated. Fatigue simulation was performed by combining a preliminary static FEA simulation with classical fatigue mechanical laws. RESULTS: Regions with the shortest fatigue-life were located around the fillets of the class II MOD cavity, where the static stress was highest. SIGNIFICANCE: The described method can be successfully adopted to generate detailed 3D-FE models of molar teeth, with different cavities and restorative materials. This method could be quickly implemented for other dental or biomechanical applications.

Tissue Doppler Imaging in the assessment of selection and response from cardiac resynchronization therapy.

Mechanistic studies, observational evaluations, and randomized trials have consistently demonstrated the beneficial effects of cardiac resynchronization therapy (CRT) in patients with moderate-to-severe chronic systolic heart failure and ventricular dyssynchrony who have failed optimal medical treatment. However, despite the promising results, in some patients undergoing CRT, the symptoms of heart failure do not improve or even worse. One of the most important reasons for this failure is probably the lack of distinct mechanical dyssynchrony before implantation. This review discusses the actual and potential role of Tissue Doppler Imaging in selection of patients and optimisation of CRT.

Echocardiographic assessment of ventricular asynchrony in dilated cardiomyopathy and congenital heart disease: tools and hopes.

Ventricular dyssynchrony is a relatively common problem in patients with heart failure, in particular those with wide QRS complex, and appears to have a deleterious effect on the natural history of heart failure, as it has been associated with increased mortality. Mechanistic studies, observational evaluations, and randomized trials have consistently demonstrated the beneficial effects of cardiac resynchronization therapy (CRT) in patients with moderate-to-severe chronic systolic heart failure and ventricular dyssynchrony who have failed optimal medical treatment. However, despite the promising results, it is estimated that in approximately 30% of patients undergoing CRT, the symptoms of heart failure do not improve or become even worse. One of the most important reasons for this failure is probably the lack of distinct mechanical dyssynchrony before implantation. A number of echocardiographic tools have been developed during the past 3 years for quantitative measurement of the severity of dyssynchrony before and after CRT. This review discusses the actual and potential role of different echocardiographic techniques in selection of patients and optimization of CRT and the value of some new clinical applications such as in congenital heart disease.

Implantable loop recorders: a novel method to judge patient perception of atrial fibrillation. Preliminary results from a pilot study.

INTRODUCTION: At the present time, several techniques are used or are under investigation for atrial fibrillation (AF) therapy. Nowadays, no well-defined target for such therapies has been yet completely identified. Furthermore, AF is an arrhythmia with high rates of recurrences, both symptomatic and asymptotic. Thus the measure of therapy success rates not only based on symptom perception remains a goal to be reached. AIMS OF THE STUDY: This study investigates the role of an implantable loop recorder (ILR) as an additional tool to identify initiating and perpetuating mechanisms of AF. The role of right atrial linear ablation (RALA) procedures is also investigated using the monitoring capabilities of the ILR. METHODS AND RESULTS: Nine patients (mean age 63.8 +/- 5.9) with paroxysmal AF were referred to our institution as candidates for AF ablation. All patients (pts) had in their medical history several years of AF episodes. Therefore pts were aware of AF related symptoms. Six of them were implanted with an ILR before ablation and were monitored one month before and six months after the procedure. The ILRs stored 54 patient activated events (PAE) and 124 automatically activated events (AAE). 68% of PAEs and 67% of AAEs were classified as appropriate. Most common reasons for inappropriate detections were premature atrial or ventricular contractions among PAEs and undersensing among AAEs. The arrhythmia onset was properly identified in 4 pts (44%). The average AF recurrence rate was 10.8 +/- 3.5 ep/month before ablation and 5.0 +/- 1.8 ep/month after the procedure ( p = 0.042). CONCLUSION: The ILR may be a helpful tool in monitoring pts undergoing ablation. Dedicated AF detection characteristics could give additional value to the device. RALA appears as a feasible, safe and relatively effective first approach in AF therapy.

The linear ablation of atrial fibrillation in the right atrium: can the isthmus ablation improve its efficacy?

OBJECTIVES: The aims of this study were to compare the efficacy of a 2- versus a 3 RF lesions ablation procedure in the RA in patients with paroxysmal AF, and to map right atrial activation during AF. BACKGROUND: RF catheter-mediated ablation lines mimicking the Maze operation have been proposed as a novel curative approach for AF. The relationship between this type of ablation and right atrial mapping has not been extensively studied. METHODS: Twenty-four patients with recurrent, drug-refractory, paroxysmal AF underwent an extensive mapping of the RA before attempting linear lesion RF ablation. Patients were divided into two groups: 15 patients received two linear lesions (Group 1), 9 patients three linear lesions (Group 2). RESULTS: One-month success rate AF did not recur in 40% of Group 1 patients (6/15) and in 66% (6/9) of Group 2 patients. 27% of patients in Group 1 and 11% of patients in Group 2 had recurrences controlled by drugs. No benefit in 33% of Group 1 patients and 22% of Group 2. Long-term follow-up In the absence of any drug treatment, no AF recurrences were observed in 26% of Group 1 patients (FU: 23 to 47 months) and 55% of Group 2 (FU: 14 to 23 months). No complications were reported. CONCLUSIONS: Right atrial linear ablation is safe and may be proposed for AF treatment in selected patients.