Publications by authors named "Grazia Delle Donne"

Article Synopsis
  • - This study explored the long-term outcomes and factors that predict mortality in children under 18 with hypertrophic cardiomyopathy (HCM) associated with various RASopathy syndromes, including Noonan syndrome and others.
  • - Researchers analyzed data from 149 patients over an average follow-up of about 16 years, finding that 15.43% of these children died, with survival rates differing significantly based on the specific RASopathy syndrome.
  • - Key predictors of mortality and sudden cardiac death included the type of RASopathy, symptoms at diagnosis, heart failure presence, and certain heart function measurements, particularly highlighting a milder HCM type within Noonan-like syndrome that still had poorer survival rates.
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Article Synopsis
  • - RASopathies contribute to about 20% of childhood hypertrophic cardiomyopathy (HCM) cases and are linked to a risk of sudden cardiac death (SCD), but the specific risk factors for SCD in this group hadn’t been thoroughly researched until now.
  • - The study analyzed 169 children with RASopathy-related HCM across 15 cardiology centers and found that 6.5% experienced SCD or similar events, with many of those categorized as low risk by the existing HCM Risk-Kids model.
  • - Key predictors for SCD identified were unexplained syncope and non-sustained ventricular tachycardia, indicating that the current risk prediction model may not effectively identify risks in this
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Objective: Hypertrophic cardiomyopathy (HCM) is an important predictor of long-term outcomes in Friedreich's ataxia (FA), but the clinical spectrum and survival in childhood is poorly described. This study aimed to describe the clinical characteristics of children with FA-HCM.

Design And Setting: Retrospective, longitudinal cohort study of children with FA-HCM from the UK.

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Aims: Children presenting with hypertrophic cardiomyopathy (HCM) in infancy are reported to have a poor prognosis, but this heterogeneous group has not been systematically characterized. This study aimed to describe the aetiology, phenotype, and outcomes of infantile HCM in a well-characterized multicentre European cohort.

Methods And Results: Of 301 children diagnosed with infantile HCM between 1987 and 2019 presenting to 17 European centres [male n = 187 (62.

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Background: Cardiovascular magnetic resonance (CMR) derived fractal analysis of the left ventricle (LV) has been shown in adults to be a useful quantitative measure of trabeculation with high reproducibility and accuracy for the diagnosis of LV non-compaction (LVNC). The aim of this study was to investigate the utility and feasibility of fractal analysis in children.

Methods: Eighty-four subjects underwent CMR: (1) 28 patients with LVNC (as defined by the Petersen criteria with NC/C ratio [Formula: see text] 2.

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Background: Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF).

Aims: To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO).

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Objective: Ivabradine is a selective and specific inhibitor of the I(f) current in the sinoatrial and atrioventricular nodes. It decreases heart rate and myocardial oxygen consumption at rest and during exercise. It is used in adults for management of heart failure and angina, but promising results have been obtained in postural orthostatic tachycardia syndrome (POTS).

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Background: Although indexed left atrial volume (iLAV) is the most accurate measure of left atrial size, it has not been evaluated prospectively as predictor of recurrence of atrial fibrillation (AFib) after successful cardioversion (CV).

Methods: We prospectively selected 76 patients (mean age 66.1 ± 13.

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Aims: Atrial fibrillation (AFib) induces remodelling of the left atrium (LA). Indexed LA volume (iLAV) as more accurate measure of LA size has not been evaluated as predictor of recurrence of AFib after cardioversion.

Methods And Results: We identified 411 adults (mean age 64.

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The aim of the present study was to evaluate cardiac function and texture in patients with subclinical hypothyroidism (sHT) both by conventional and new ultrasonic intramyocardial tissue techniques. sHT was characterized by normal serum free tetraiodotironine and free triiodotironine levels and slightly increased serum TSH level. Twenty-four patients affected by sHT and 24 sex- and age-matched healthy volunteers were studied.

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Article Synopsis
  • The study aimed to evaluate how severe aortic valve stenosis affects myocardial reflectivity patterns using integrated backscatter (IBS) analysis among selected patients.
  • The research involved 35 patients with aortic stenosis divided into groups with depressed or normal left ventricular systolic function, and comparisons were made with 25 healthy subjects through echocardiography and IBS analysis.
  • Key findings indicated that patients with depressed function showed significantly higher myocardial echo intensity and lower IBS variation, and post-aortic valve replacement, a reduction in left ventricular mass and improvement in ejection fraction were noted, linked to decreased interstitial fibrosis.
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Background: Few data are available concerning the effects on clinical outcome and left ventricular function of abciximab administration in patients undergoing rescue percutaneous transluminal coronary angioplasty (PTCA) after failed thrombolysis for acute myocardial infarction. The aim of the study was to investigate such effects.

Methods: Eighty-nine consecutive patients referred to our laboratory from other hospitals for rescue PTCA within 24 hours from the onset of chest pain were prospectively randomized before the procedure to abciximab treatment (44 patients) or placebo (45 patients).

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