Alberto Marchi
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View article: Genetic Profile of Pediatric-Onset Cardiac Channelopathies
Genetic Profile of Pediatric-Onset Cardiac Channelopathies Open
This study investigates the genetic background of pediatric-onset cardiac channelopathies, a rare group of genetic disorders causing arrhythmias and sometimes sudden death, whose genetic background remains partially unknown. The research a…
View article: Genetic Profile of Pediatric-Onset Cardiac Channelopathies
Genetic Profile of Pediatric-Onset Cardiac Channelopathies Open
This study investigates the genetic background of pediatric-onset cardiac channelopa-thies, a rare group of genetic disorders causing arrhythmias and sometimes sudden death whose genetic background remains partially unknown. The research a…
View article: Yield of Genetic Testing in Pediatric Cardiomyopathies: Implications for Novel Therapeutic Options
Yield of Genetic Testing in Pediatric Cardiomyopathies: Implications for Novel Therapeutic Options Open
Pediatric cardiomyopathies are rare, heterogeneous, and challenging conditions, often with a genetic etiology. We estimated the yield of genetic testing in a pediatric cohort with cardiomyopathies and evaluated the potential candidacy to c…
View article: Long-Term Effects of Mavacamten on Electromechanical Dispersion and Deformation in Obstructive Hypertrophic Cardiomyopathy
Long-Term Effects of Mavacamten on Electromechanical Dispersion and Deformation in Obstructive Hypertrophic Cardiomyopathy Open
Regional electromechanical abnormalities have long been known to characterize hypertrophic cardiomyopathy (HCM), particularly in the presence of left ventricular outflow tract obstruction, contributing considerably to left ventricular (LV)…
View article: Real‐world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population
Real‐world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population Open
Aims In the EXPLORER‐HCM trial, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved functional capacity of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients. We sought to define the potentia…
View article: Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions
Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions Open
Restrictive cardiomyopathy (RCM) is characterized by restrictive ventricular pathophysiology determined by increased myocardial stiffness. While suspicion of RCM is initially raised by clinical evaluation and supported by electrocardiograp…
View article: Impact of pregnancy on the natural history of women with hypertrophic cardiomyopathy
Impact of pregnancy on the natural history of women with hypertrophic cardiomyopathy Open
Aims Whether pregnancy is a modifier of the long-term course and outcome of women with hypertrophic cardiomyopathy (HCM) is unknown. We assessed the association of pregnancy with long-term outcomes in HCM women. Methods and results Retrosp…
View article: Stage-specific therapy for hypertrophic cardiomyopathy
Stage-specific therapy for hypertrophic cardiomyopathy Open
Hypertrophic cardiomyopathy (HCM) is the most common inherited myocardial disease and is defined by otherwise unexplained left ventricular hypertrophy. The main complications include heart failure and arrhythmias such as atrial fibrillatio…
View article: Genetic Testing and Counselling in Hypertrophic Cardiomyopathy: Frequently Asked Questions
Genetic Testing and Counselling in Hypertrophic Cardiomyopathy: Frequently Asked Questions Open
Genetic counselling and genetic testing in hypertrophic cardiomyopathy (HCM) represent an integral part of the diagnostic algorithm to confirm the diagnosis, distinguish it from phenocopies, and suggest tailored therapeutic intervention st…
View article: Clinical, functional and prognostic correlates of excess left ventricular force in hypertrophic cardiomyopathy
Clinical, functional and prognostic correlates of excess left ventricular force in hypertrophic cardiomyopathy Open
Background Excess force generation during myocardial contraction represents a cardinal feature of hypertrophic cardiomyopathy (HCM). Purpose To evaluate the anatomical, functional and prognostic correlates of left ventricular (LV) force in…
View article: 772 Incidence of stroke in patients with hypertrophic cardiomyopathy implanted with a cardiac implantable electronic device
772 Incidence of stroke in patients with hypertrophic cardiomyopathy implanted with a cardiac implantable electronic device Open
Aims Incidence of stroke in patients with an advanced stage hypertrophic cardiomyopathy (HCM) is associated with adverse outcome, impaired quality of life and loss of productivity. Still today, however, the real burden of stroke in both pa…
View article: Stress Echo 2030: The Novel ABCDE-(FGLPR) Protocol to Define the Future of Imaging
Stress Echo 2030: The Novel ABCDE-(FGLPR) Protocol to Define the Future of Imaging Open
With stress echo (SE) 2020 study, a new standard of practice in stress imaging was developed and disseminated: the ABCDE protocol for functional testing within and beyond CAD. ABCDE protocol was the fruit of SE 2020, and is the seed of SE …
View article: Emerging Medical Treatment for Hypertrophic Cardiomyopathy
Emerging Medical Treatment for Hypertrophic Cardiomyopathy Open
Hypertrophic cardiomyopathy (HCM) is a common myocardial disease characterized by otherwise unexplained left ventricular hypertrophy. The main cause of disabling symptoms in patients with HCM is left ventricular outflow tract (LVOT) obstru…
View article: Eosinophilic granulomatosis with polyangiitis, a new recurrent feature in an extremely rare disease
Eosinophilic granulomatosis with polyangiitis, a new recurrent feature in an extremely rare disease Open
Introduction Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare necrotizing vasculitis affecting small- and medium-sized vessels. EGPA may affect the heart leading to myocardial inflammation and necrosis. Although, o…
View article: Antiarrhythmic efficacy of anakinra in a young patient with autoimmune lymphocytic myocarditis
Antiarrhythmic efficacy of anakinra in a young patient with autoimmune lymphocytic myocarditis Open
IL-1 inhibition with anakinra suppressed ventricular arrhythmias refractory to conventional therapies in severe myocarditis.
View article: Sex-related differences in exercise performance and outcome of patients with hypertrophic cardiomyopathy
Sex-related differences in exercise performance and outcome of patients with hypertrophic cardiomyopathy Open
Aims Exercise performance is known to predict outcome in hypertrophic cardiomyopathy (HCM), but whether sex-related differences exist is unresolved. We explored whether functional impairment, assessed by exercise echocardiography, has comp…