Use of Implantable Cardioverter-Defibrillator in Inherited Arrhythmogenic Diseases: Data from Italian ICD Registry for the Years 2001–6

Background: Previous studies have demonstrated the lifesaving role of an implantable cardioverter-defibrillator (ICD) in high-risk patients with genetic arrhythmogenic diseases.
Aim: To evaluate the clinical data of patients with hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVD/C), idiopathic ventricular arrhythmia (IDIO-VA) including Brugada syndrome, short QT syndrome, and long QT syndrome (LQT) enrolled in the Italian ICD Registry in the years 2001–6.
Methods: The survey collected prospectively clinical data and technical characteristics of ICD implants on the basis of EURID form.
Results: The number of patients was 1,274 in the HCM group, 520 in the ARVD/C group, 460 in the IDIO-VA group, and 245 in the LQT group. Primary prevention involved 29.5% of patients in the HCM group, 15.9% in the ARVD/C group, 16.9% in the IDIO-VA group, and 16.6% in the LQT group, respectively. Ventricular tachycardia and ventricular fibrillation were reported in 49.0% and 24.8% in the HCM group, 72.9% and 20.0% in the ARVD/C group, 48.2% and 42.9% in the IDIO-VA group, and 21.2% and 61.6% in the LQT group, respectively. Single-, dual-, and triple-chamber ICDs were implanted in 41.5%, 47.6%, and 10.9% in the HCM group; 57.9%, 36.7%, and 5.4% in the ARVD/C group; 55.0%, 45.0%, and 0% in the IDIO-VA group; and 57.4%, 42.6%, and 0% in LQT group, respectively.
Conclusion: ICD therapies in the years 2001–6 for patients with inherited arrhythmia syndromes were utilized in Italy in a still-limited number of patients. Secondary prevention represented the major indication for ICD implant and the majority of patients were treated by single-chamber ICDs.     

Positive Association between Helicobacter pylori Infection and Food Allergy in Children

Background: In children Helicobacter pylori has been involved as a pathogenetic factor in gastritis and duodenal ulcer and as a cofactor in protein-losing enteropathy, chronic diarrhoea, short stature, and gastritis lymphoproliferative disease. A subset of an H. pylori strain possesses an antigen, CagA, as a virulence factor. In the present study we determined anti-H. pylori IgG and anti-CagA IgG titres in children with food allergy. Methods: Ninety paediatric patients were studied: 30 with food allergy, 30 with atopic asthma, and 30 with inflammatory bowel disease. Anti-H. pylori IgG and anti-CagA IgG were determined in all children by means of a commercial enzyme immunoassay (ELISA). Results: The anti-H. pylori IgG titre was significantly higher in allergic patients than in the other two groups. The anti-CagA IgG titre did not differ significantly between the patients. Conclusions: These findings show a positive association between H. pylori infection and food allergy in children. We hypothesize that virulence factors other than CagA may be involved in the pathogenesis of H. pylori infection in paediatric patients with food allergy.     

Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study

This study explored the burden in parents and healthy siblings of 4-17 year-old patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies, and whether the burden varied according to clinical aspects and social resources.Data on socio-demographic characteristics, patient''s clinical history, parent and healthy children burden, and on parent''s social resources were collected using self-reported questionnaires administered to 336 parents of patients with DMD (246) and BMD (90).Parents of patients with DMD reported higher burden than those of patients with BMD, especially concerning feeling of loss (84.3% DMD vs. 57.4% BMD), stigma (44.2% DMD vs. 5.5% BMD) and neglect of hobbies (69.0% DMD vs. 32.5% BMD). Despite the burden, 66% DMD and 62.4% BMD parents stated the caregiving experience had a positive impact on their lives. A minority of parents believed MD has a negative influence on the psychological well-being (31.0% DMD vs. 12.8% BMD), and social life of unaffected children (25.7% vs. 18.4%).In the DMD group, burden correlated with duration of illness and parent age, and burden was higher among parents with lower social contacts and support in emergencies. In DMD, difficulties among healthy children were reported as higher by parents who were older, had higher burden and lower social contacts. In both groups, burden increased in relation to patient disability.These findings underline that the psychological support to be provided to parents of patients with MD, should take into account clinical features of the disease.Key words: Duchenne muscular dystrophy, Becker muscular dystrophy, parents, healthy siblings, burden, social network     

Usefulness of Biplanar Transesophageal Echocardiography in Arrhythmogenic Right Ventricular Dysplasia

This study evaluated the clinical usefulness of biplanar transesophageal echocardiography (TEE) in suspected arrhythmogenic right ventricular dysplasia (ARVD). Seven symptomatic subjects (3 male and 4 female: ages 18–64 years, mean 45) with clinical features of ARVD (typical ventricular arrhythmias) underwent comprehensive noninvasive assessment including transthoracic echocardiography (TTE), nuclear magnetic resonance (NMR), and TEE. Other systemic and cardiac diseases were reasonably excluded. TEE identified a significant right ventricular regional alteration in one subject with negative TTE and NMR, and nonsignificant abnormalities in two subjects with negative or no TTE and positive NMR. TEE confirmed the pathological findings detected by TTE in the four remaining patients and visualized several more abnormalities that approximately corresponded to NMR results. On the basis of these initial results, biplanar TEE appears to be comparable to NMR in the assessment of ARVD and a promising technique in identifying ARVD morphological alterations.     

Caffeine Stimulates in vivo Platelet Reactivity

ABSTRACT The effect of coffee drinking on platelet reactivity was studied in 12 healthy subjects. Plasma beta-thromboglobulin concentration was determined before and one hour after administration of 100 mg of caffeine, corresponding to one cup of coffee. Mean values were 47.0±19.3 and 179.3±85.5 ng/ml before and after caffeine administration respectively. The increase, 298±150%, is highly significant (p<0.001).     

Myocardial bridges and ischemic heart disease

The clinical, electrocardiographs and angiocardiographic dataare reported of 37 patients with myocardial bridges, in theabsence of coronary atherosclerosis. An intramyocardial courseof the left anterior descending coronary artery was presentin all cases: myocardial bridges on the posterior descendingand first diagonal branch were associated in one patient. The clinical presentation was that of ischemic heart diseasein all cases, with stable angina in nine, unstable angina in19, the intermediate syndrome in five, and a history or evidenceof previous myocardial infarction in four. In 20 cases, left ventricular wall motion appeared to be abnormalon the antero-lateral or apical profile. One patient died suddenly the day following hospital discharge;of 27 patients followed over a 3–36 month interval, onlyfour (15%) were free of symptoms on medical treatment. The frequent association of myocardial bridges with a clinicalpicture of ischemic heart disease which is sometimes severe,is emphasized together with the disappointing results of medicaltreatment.