A5019117678- Cardiomyopathy and Myosin Studies
- Cardiovascular Effects of Exercise
- Cardiac Arrhythmias and Treatments
- Cardiac pacing and defibrillation studies
- Cardiovascular Function and Risk Factors
- Cardiac Valve Diseases and Treatments
- Cardiac Imaging and Diagnostics
- Coronary Artery Anomalies
- Congenital Heart Disease Studies
- Cardiac electrophysiology and arrhythmias
- Aortic Disease and Treatment Approaches
- Muscle Physiology and Disorders
- Cardiovascular Disease and Adiposity
- Heart Failure Treatment and Management
- Cardiac Arrest and Resuscitation
- Cardiac Structural Anomalies and Repair
- Infective Endocarditis Diagnosis and Management
- Neurogenetic and Muscular Disorders Research
- Congenital heart defects research
- Genomic variations and chromosomal abnormalities
- Kawasaki Disease and Coronary Complications
- Genetic Neurodegenerative Diseases
- Coronary Interventions and Diagnostics
- Mechanical Circulatory Support Devices
- Pericarditis and Cardiac Tamponade
Bambino Gesù Children's Hospital
2019-2025
Inova Fairfax Hospital
2021
Centre Hospitalier Universitaire de Liège
2019-2020
University of Liège
2019-2020
Sapienza University of Rome
2016-2017
The present study sought to evaluate the correlation between indices of non-invasive myocardial work (MW) and left ventricle (LV) size, traditional advanced parameters LV systolic diastolic function by 2D echocardiography (2DE).A total 226 (85 men, mean age: 45 ± 13 years) healthy subjects were enrolled at 22 collaborating institutions Normal Reference Ranges for Echocardiography (NORRE) study. Global index (GWI), global constructive (GCW), waste (GWW), efficiency (GWE) estimated from...
Since the spread of COVID-19, pediatric patients were initially considered less affected by SARS-COV-2, but current literature reported subsets children with multisystem inflammatory syndrome (MIS-C). This study aims to describe cardiac manifestation SARS-COV-2 infection in a large cohort admitted two Italian referral centers. Between March 2020 and 2021, we performed evaluation 294 (mean age 9 ± 5.9 years, male 60%) active or previous infection. Twenty-six showed ECG abnormalities: 63...
The present study sought to assess the impact of aortic stenosis (AS) on myocardial function as assessed by layer-specific longitudinal strain (LS) and its relationship with symptoms outcome.We compared 211 patients (56% males, mean age 73 ± 12 years) severe AS left ventricular ejection fraction (LVEF) ≥50% (114 symptomatic, 97 asymptomatic) 50 controls matched for sex. LS was from endocardium, mid-myocardium, epicardium 2D speckle-tracking echocardiography. Despite similar LVEF, multilayer...
MIS-C is a multisystem inflammatory syndrome that characterized by multi-organ failure and cardiac involvement. The aim of this study was to describe the long-term cardiovascular outcome in cohort pediatric patients, who were admitted two Italian Pediatric Referral Centers. Sixty-seven patients (mean age 8.7 ± 4.7 years, male 60%) included; 65 (97%) them showed All completed one month follow-up, 47% 1 year it. ECG abnormalities present 65% them, arrhythmias 9% during an acute phase it...
There is growing interest in the aortic valve (AV) neocuspidalization technique for treatment of disease (AVD). We report our medium-term results with this procedure performed a paediatric patient population.Between July 2016 and May 2020, 22 patients both congenital acquired isolated AVD were treated neocuspidalization. The primary outcome was progression preoperatively assessed immediate postoperative course at follow-up. Secondary freedom from reintervention by material used. Potential...
Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, characterized by age related penetrance. Diagnosis management MFS in adult population well-described literature. Few studies focused on pediatric...
Lamin A/C (LMNA) encodes for two nuclear intermediate filament proteins. Mutations in LMNA cause a highly heterogeneous group of diseases predominantly affecting striated and cardiac muscles, adipose tissue peripheral nerves. Laminopathies lead to muscular or disease, lipodystrophy syndromes, neuropathy, accelerated aging disorders. Cardiac involvement includes progressive arrhythmias (brady/tachyarrhythmias, SCD). Furthermore, cardiomyocyte damage often progresses into dilated...
Friedreich ataxia (FRDA) is the most common form of in late childhood. Neurological manifestations often precede cardiac involvement, presenting mainly as hypertrophic cardiomyopathy. We describe a toddler with apparently isolated severe heart failure, successfully managed transplant (HT). Although well described adolescents and adults, onset FRDA very uncommon toddlers neurological ataxic features are predominant. The symptom cardiomyopathy rare. Similar history rarely reported literature,...
Pediatric patients with cardiomyopathies are at risk of malignant arrhythmias and sudden cardiac death (SCD). An ICD may prevent SCD. The aim this study was to evaluate implantation outcomes, compare transvenous subcutaneous ICDs (S-ICDs) implanted in pediatric cardiomyopathies.The is single center retrospective, includes who required (2010-2021). Outcomes were recorded for appropriate/inappropriate therapy surgical complications. Transvenous S-ICD compared. Data presented as median values...
Filamin C is a protein specifically expressed in myocytes and cardiomyocytes involved several biological functions, including sarcomere contractile activity, signaling, cellular adhesion, repair. FLNC variants are associated with different disorders ranging from striated muscle (myofibrillar distal or proximal) myopathy to cardiomyopathies (CMPs) (restrictive, hypertrophic, dilated), both. The outcome depends on functional consequences of the detected variants, which result either...
Deletions involving the distal portion of short arm chromosome 8(8p23.1) show a high phenotypic variability. Congenital heart diseases (CHD) are often described. GATA4 when mutated or deleted is reported to be involved in cardiac morphogenesis. Only twice, left ventricular non compaction (LVNC) was literature association with 8p23.1 deletion. The present cohort includes five new patients deletions including GATA4. spectrum CHD variable. Moreover, four patients, LV hypertrabeculation detected...
Remote monitoring-enabled insertable cardiac monitors (ICMs) are useful tools for arrhythmias and symptom management. This study sought to evaluate the outcome of ICM implantation in a large, heterogeneous cohort pediatric young adult patients.Single centre, retrospective analysis patients who underwent 2010-2019. Patients were analysed according age, symptoms, underlying heart disease.A total 200 consecutive (58% male), aged 11.5 ± 5.8 years at implantation, included. Follow-up was 31 18...
Abstract Background In asymptomatic patients with aortic stenosis (AS), the optimal timing for intervention is still challenging. Previous studies demonstrated that advanced stages of cardiac damage are associated excess mortality. The role myocardial work to identify dysfunction in AS and predict prognosis has not been investigated. Purpose We aimed evaluate modification indices related their prognostic value. Methods This study analysed clinical, echocardiographic outcome data 170 (aortic...
The presence of T wave inversion on screening electrocardiogram may represent an early sign cardiomyopathies in athletes. This finding even very young athletes can generate some suspicion and determine a contraindication to practice competitive sport. aim this study is evaluate the prevalence population whether they be associated with occurrence absence other pathological features.
Left ventricular hypertrabeculation (LVHT) is a myocardial disorder with different clinical manifestations, from total absence of symptoms to heart failure, arrhythmias, sudden cardiac death (SCD), and thromboembolic events. It challenging distinguish between the benign pathological forms LVHT. The aim this study was describe arrhythmic manifestations LVHT in large group pediatric patients correlate them genetic results or other markers.