Publications by authors named "Giuseppe Limongelli"

246 Publications

Drug-utilisation profiles and COVID-19.

Sci Rep 2021 04 26;11(1):8913. Epub 2021 Apr 26.

CIRFF, Center of Drug Utilisation and Pharmacoeconomics, University of Naples Federico II, 80131, Naples, Italy.

Coronavirus disease 2019 (COVID-19) has substantially challenged healthcare systems worldwide. By investigating population characteristics and prescribing profiles, it is possible to generate hypotheses about the associations between specific drug-utilisation profiles and susceptibility to COVID-19 infection. A retrospective drug-utilisation study was carried out using routinely collected information from a healthcare database in Campania (Southern Italy). We aimed to discover the prevalence of drug utilisation (monotherapy and polytherapy) in COVID-19 versus non-COVID-19 patients in Campania (~ 6 million inhabitants). The study cohort comprised 1532 individuals who tested positive for COVID-19. Drugs were grouped according to the Anatomical Therapeutic Chemical (ATC) classification system. We noted higher prevalence rates of the use of drugs in the ATC categories C01, B01 and M04, which was probably linked to related comorbidities (i.e., cardiovascular and metabolic). Nevertheless, the prevalence of the use of drugs acting on the renin-angiotensin system, such as antihypertensive drugs, was not higher in COVID-19 patients than in non-COVID-19 patients after adjustments for age and sex. These results highlight the need for further case-control studies to define the effects of medications and comorbidities on susceptibility to and associated mortality from COVID-19.
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http://dx.doi.org/10.1038/s41598-021-88398-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8076316PMC
April 2021

Feasibility of semi-recumbent bicycle exercise Doppler echocardiography for the evaluation of the right heart and pulmonary circulation unit in different clinical conditions: the RIGHT heart international NETwork (RIGHT-NET).

Int J Cardiovasc Imaging 2021 Apr 18. Epub 2021 Apr 18.

Cardiology Division, A Cardarelli Hospital, Naples, Italy.

Exercise Doppler echocardiography (EDE) is a well-validated tool in ischemic and valvular heart diseases. However, its use in the assessment of the right heart and pulmonary circulation unit (RH-PCU) is limited. The aim of this study is to assess the semi-recumbent bicycle EDE feasibility for the evaluation of RH-PCU in a large multi-center population, from healthy individuals and elite athletes to patients with overt or at risk of developing pulmonary hypertension (PH). From January 2019 to July 2019, 954 subjects [mean age 54.2 ± 16.4 years, range 16-96, 430 women] underwent standardized semi-recumbent bicycle EDE with an incremental workload of 25 watts every 2 min, were prospectively enrolled among 7 centers participating to the RIGHT Heart International NETwork (RIGHT-NET). EDE parameters of right heart structure, function and pressures were obtained according to current recommendations. Right ventricular (RV) function at peak exercise was feasible in 903/940 (96%) by tricuspid annular plane systolic excursion (TAPSE), 667/751 (89%) by tissue Doppler-derived tricuspid lateral annular systolic velocity (S') and 445/672 (66.2%) by right ventricular fractional area change (RVFAC). RV-right atrial pressure gradient [RV-RA gradient = 4 × tricuspid regurgitation velocity (TRV)] was feasible in 894/954 patients (93.7%) at rest and in 816/954 (85.5%) at peak exercise. The feasibility rate in estimating pulmonary artery pressure improved to more than 95%, if both TRV and/or right ventricular outflow tract acceleration time (RVOT AcT) were considered. In high specialized echocardiography laboratories semi-recumbent bicycle EDE is a feasible tool for the assessment of the RH-PCU pressure and function.
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http://dx.doi.org/10.1007/s10554-021-02243-xDOI Listing
April 2021

Impact of Regular Physical Activity on Aortic Diameter Progression in Paediatric Patients with Bicuspid Aortic Valve.

Pediatr Cardiol 2021 Apr 16. Epub 2021 Apr 16.

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Naples, Italy.

Patients with bicuspid aortic valve (BAV) have an increased risk of aortic dilation and aortic dissection or rupture. The impact of physical training on the natural course of aortopathy in BAV patients remains unclear. The aim of this study was to evaluate the impact of regular physical activity on aortic diameters in a consecutive cohort of paediatric patients with BAV. Consecutive paediatric BAV patients were evaluated and categorized into two groups: physically active and sedentary subjects. Only the subjects with a complete 2-year follow-up were included in the study. To evaluate the potential impact of physical activity on aortic size, aortic diameters were measured at the sinus of Valsalva and mid-ascending aorta using echocardiography. We defined aortic diameter progression the increase of aortic diameter ≥ 10% from baseline. Among 90 BAV patients (11.5 ± 3.4 years of age, 77% males), 53 (59%) were physically active subjects. Compared to sedentary, physically active subjects were not significantly more likely to have > 10% increase in sinus of Valsalva (13% vs. 8%, p-value = 0.45) or mid-ascending aorta diameter (9% vs. 13%, p-value = 0.55) at 2 years follow-up, both in subjects with sinus of Valsalva diameter progression (3.7 ± 1.0 mm vs. 3.5 ± 0.8 mm, p-value = 0.67) and in those with ascending aorta diameter progression (3.0 ± 0.8 mm vs. 3.2 ± 1.3 mm, p-value = 0.83). In our paediatric cohort of BAV patients, the prevalence and the degree of aortic diameter progression was not significantly different between physically active and sedentary subjects, suggesting that aortic dilation is unrelated to regular physical activity over a 2-year period.
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http://dx.doi.org/10.1007/s00246-021-02591-4DOI Listing
April 2021

[Alcohol septal ablation for hypertrophic obstructive cardiomyopathy: the emerging role of the Cardiomyopathy Team].

G Ital Cardiol (Rome) 2021 03;22(3 Suppl 1):25S-31S

U.O. Cardiologia e Angiologia, Dipartimento di Scienze Cardiovascolari, Sapienza Università di Roma, Roma.

Alcohol septal ablation is a minimally invasive, safe, and effective procedure for the treatment of left ventricular outflow tract (LVOT) obstruction in patients with hypertrophic obstructive cardiomyopathy (HOCM) who remain symptomatic despite maximal medical therapy. Originally performed by Ulrich Sigwart in 1994, the procedure causes a iatrogenic infarction - through the injection of absolute alcohol - of the basal portion of the interventricular septum and aims at reducing LVOT obstruction in order to improve patient's hemodynamics and symptoms. Numerous studies have demonstrated the effectiveness and safety of the procedure, making it a valid alternative to surgical myectomy. The success of alcohol septal ablation depends upon the selection of the patient and the experience of both the operators and the center where the procedure is performed. In this review, we summarize current evidence on alcohol septal ablation, describe its procedural aspects and propose a multidisciplinary approach that involves a team of clinical cardiologists, interventionists, and cardiac surgeons, the Cardiomyopathy Team, with high experience in the clinical management of these patients.
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http://dx.doi.org/10.1714/3578.35631DOI Listing
March 2021

Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy.

Biomolecules 2021 Mar 2;11(3). Epub 2021 Mar 2.

Department of Clinical and Molecular Medicine, Sapienza University, 00189 Rome, Italy.

The usefulness of cardiopulmonary exercise test (CPET) in adult hypertrophic cardiomyopathy (HCM) patients is well-known, whereas its role in pediatric HCM patients has not yet been explored. The present study investigates possible insights from a CPET assessment in a cohort of pediatric HCM outpatients in terms of functional and prognostic assessment. Sixty consecutive pediatric HCM outpatients aged <18 years old were enrolled, each of them undergoing a full clinical assessment including a CPET; a group of 60 healthy subjects served as controls. A unique composite end-point of heart failure (HF) related and sudden cardiac death (SCD) or SCD-equivalent events was also explored. During a median follow-up of 53 months (25th-75th: 13-84 months), a total of 13 HF- and 7 SCD-related first events were collected. Compared to controls, HCM patients showed an impaired functional capacity with most of them showing peak oxygen uptake (pVO) values of <80% of the predicted, clearly discrepant with functional New York Heart Association class assessment. The composite end-point occurred more frequently in patients with the worst CPETs' profiles. At the univariate analysis, pVO% was the variable with the strongest association with adverse events at follow-up (C-index = 0.72, = 0.025) and a cut-off value equal to 60% was the most accurate in identifying those patients at the highest risk. In a pediatric HCM subset, the CPET assessment allows a true functional capacity estimation and it might be helpful in identifying early those patients at high risk of events.
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http://dx.doi.org/10.3390/biom11030376DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7999553PMC
March 2021

Nuclear factor-kappa B predicts long-term clinical outcome in patients with hypertrophic cardiomyopathy: 10-year follow-up study.

Eur J Prev Cardiol 2021 Mar 24. Epub 2021 Mar 24.

Department of Cardiovascular Sciences, Sapienza University, Via del Policlinico 155, 00161 Rome, Italy.

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http://dx.doi.org/10.1093/eurjpc/zwab047DOI Listing
March 2021

Hypertrophic Cardiomyopathy in Children: Pathophysiology, Diagnosis, and Treatment of Non-sarcomeric Causes.

Front Pediatr 2021 25;9:632293. Epub 2021 Feb 25.

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Naples, Italy.

Hypertrophic cardiomyopathy (HCM) is a myocardial disease characterized by left ventricular hypertrophy not solely explained by abnormal loading conditions. Despite its rare prevalence in pediatric age, HCM carries a relevant risk of mortality and morbidity in both infants and children. Pediatric HCM is a large heterogeneous group of disorders. Other than mutations in sarcomeric genes, which represent the most important cause of HCM in adults, childhood HCM includes a high prevalence of non-sarcomeric causes, including inherited errors of metabolism (i.e., glycogen storage diseases, lysosomal storage diseases, and fatty acid oxidation disorders), malformation syndromes, neuromuscular diseases, and mitochondrial disease, which globally represent up to 35% of children with HCM. The age of presentation and the underlying etiology significantly impact the prognosis of children with HCM. Moreover, in recent years, different targeted approaches for non-sarcomeric etiologies of HCM have emerged. Therefore, the etiological diagnosis is a fundamental step in designing specific management and therapy in these subjects. The present review aims to provide an overview of the non-sarcomeric causes of HCM in children, focusing on the pathophysiology, clinical features, diagnosis, and treatment of these rare disorders.
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http://dx.doi.org/10.3389/fped.2021.632293DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7947260PMC
February 2021

Multiple hormonal and metabolic deficiency syndrome predicts outcome in heart failure: the T.O.S.CA. Registry.

Eur J Prev Cardiol 2021 Mar 7. Epub 2021 Mar 7.

Clinical Medicine and Surgery Department, Federico II University, Naples, Italy.

Aims: Recent evidence supports the occurrence of multiple hormonal and metabolic deficiency syndrome (MHDS) in chronic heart failure (CHF). However, no large observational study has unequivocally demonstrated its impact on CHF progression and outcome. The T.O.S.CA. (Trattamento Ormonale nello Scompenso CArdiaco; Hormone Treatment in Heart Failure) Registry has been specifically designed to test the hypothesis that MHDS affects morbidity and mortality in CHF patients.

Methods And Results: The T.O.S.CA. Registry is a prospective, multicentre, observational study involving 19 Italian centres. Thyroid hormones, insulin-like growth factor-1, total testosterone, dehydropianoandrosterone sulfate, insulin resistance, and the presence of diabetes were evaluated. A MHDS was defined as the presence of ≥2 hormone deficiencies (HDs). Primary endpoint was a composite of all-cause mortality and cardiovascular hospitalizations. Four hundred and eighty heart failure patients with ejection fraction ≤45% were enrolled. MHDS or diabetes was diagnosed in 372 patients (77.5%). A total of 271 events (97 deaths and 174 cardiovascular hospitalizations) were recorded, 41% in NO-MHDS and 62% in MHDS (P < 0.001). Median follow-up was of 36 months. MHDS was independently associated with the occurrence of the primary endpoint [hazard ratio 95% (confidence interval), 1.93 (1.37-2.73), P < 0.001] and identified a group of patients with a higher mortality [2.2 (1.28-3.83), P = 0.01], with a graded relation between HDs and cumulative events (P < 0.01).

Conclusion: MHDS is common in CHF and independently associated with increased all-cause mortality and cardiovascular hospitalization, representing a promising therapeutic target.

Trial Registration: ClinicalTrials.gov identifier: NCT023358017.
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http://dx.doi.org/10.1093/eurjpc/zwab020DOI Listing
March 2021

Combined Effect of Mediterranean Diet and Aerobic Exercise on Weight Loss and Clinical Status in Obese Symptomatic Patients with Hypertrophic Cardiomyopathy.

Heart Fail Clin 2021 Apr 10;17(2):303-313. Epub 2021 Feb 10.

Cardiomyopathy Unit and Genetic Unit, Careggi University Hospital, Largo Brambrilla, 50134 Florence, Italy.

We evaluated the impact of weight loss (WL) using a Mediterranean diet and mild-to-moderate-intensity aerobic exercise program, on clinical status of obese, symptomatic patients with hypertrophic cardiomyopathy (HCM). Compared with nonresponders, responders showed a significant reduction of left atrial diameter, left atrial volume index (LAVI), E/E'average, pulmonary artery systolic pressure (PASP), and a significant increase in Vo (%) and peak workload. Body mass index changes correlated with reduction in left atrial diameter, LAVI, E/E'average, PASP, and increase of Vo (mL/Kg/min), Vo (%), peak workload. Mediterranean diet and aerobic exercise is associated with clinical-hemodynamic improvement in obese symptomatic HCM patients.
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http://dx.doi.org/10.1016/j.hfc.2021.01.003DOI Listing
April 2021

Rare case of Kawasaki disease with cardiac tamponade and giant coronary artery aneurysms.

Cardiol Young 2021 Feb 11:1-2. Epub 2021 Feb 11.

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Naples, Italy.

Kawasaki disease is an acute systemic vascular disease, generally self-limited and typically affecting children <5 years old, which leads to coronary artery aneurysms in about 25% of untreated cases. Cardiovascular involvement is characterised by transient pancarditis, in acute phase, while coronary illness, ranging from mild dilation to giant CAAs occurs late, rarely before the 10th day since fever onset. Here, we describe a peculiar case of KD, which occurred in a 4-month-old infant and presented with exudate cardiac tamponade and early giant aneurism of both the proximal right coronary artery) and the left circumflex coronary artery, in acute phase of the disease.
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http://dx.doi.org/10.1017/S1047951120004989DOI Listing
February 2021

Exercise, Immune System, Nutrition, Respiratory and Cardiovascular Diseases during COVID-19: A Complex Combination.

Int J Environ Res Public Health 2021 01 21;18(3). Epub 2021 Jan 21.

Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy.

Coronaviruses (CoVs) represent a large family of RNA viruses that can infect different living species, posing a global threat to human health. CoVs can evade the immune response, replicate within the host, and cause a rapid immune compromise culminating in severe acute respiratory syndrome. In humans, the immune system functions are influenced by physical activity, nutrition, and the absence of respiratory or cardiovascular diseases. This review provides an in-depth study between the interactions of the immune system and coronaviruses in the host to defend against CoVs disease.
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http://dx.doi.org/10.3390/ijerph18030904DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7908487PMC
January 2021

A multicentric quality-control study of exercise Doppler echocardiography of the right heart and the pulmonary circulation. The RIGHT Heart International NETwork (RIGHT-NET).

Cardiovasc Ultrasound 2021 Jan 20;19(1). Epub 2021 Jan 20.

IRCCS SDN, Naples, Italy.

Purpose: This study was a quality-control study of resting and exercise Doppler echocardiography (EDE) variables measured by 19 echocardiography laboratories with proven experience participating in the RIGHT Heart International NETwork.

Methods: All participating investigators reported the requested variables from ten randomly selected exercise stress tests. Intraclass correlation coefficients (ICC) were calculated to evaluate the inter-observer agreement with the core laboratory. Inter-observer variability of resting and peak exercise tricuspid regurgitation velocity (TRV), right ventricular outflow tract acceleration time (RVOT Act), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler tricuspid lateral annular systolic velocity (S'), right ventricular fractional area change (RV FAC), left ventricular outflow tract velocity time integral (LVOT VTI), mitral inflow pulsed wave Doppler velocity (E), diastolic mitral annular velocity by TDI (e') and left ventricular ejection fraction (LVEF) were measured.

Results: The accuracy of 19 investigators for all variables ranged from 99.7 to 100%. ICC was > 0.90 for all observers. Inter-observer variability for resting and exercise variables was for TRV = 3.8 to 2.4%, E = 5.7 to 8.3%, e' = 6 to 6.5%, RVOT Act = 9.7 to 12, LVOT VTI = 7.4 to 9.6%, S' = 2.9 to 2.9% and TAPSE = 5.3 to 8%. Moderate inter-observer variability was found for resting and peak exercise RV FAC (15 to 16%). LVEF revealed lower resting and peak exercise variability of 7.6 and 9%.

Conclusions: When performed in expert centers EDE is a reproducible tool for the assessment of the right heart and the pulmonary circulation.
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http://dx.doi.org/10.1186/s12947-021-00238-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7819251PMC
January 2021

Current use of cardiac magnetic resonance in tertiary referral centres for the diagnosis of cardiomyopathy: the ESC EORP Cardiomyopathy/Myocarditis Registry.

Eur Heart J Cardiovasc Imaging 2021 Jan 8. Epub 2021 Jan 8.

Members of the European Reference Network on Heart Diseases (ERN GUARD-HEART), Coordinating Centre: Academic Medical Center, Amsterdam, the Netherlands.

Aims : Cardiac magnetic resonance (CMR) is recommended in the diagnosis of cardiomyopathies, but it is time-consuming, expensive, and limited in availability in some European regions. The aim of this study was to determine the use of CMR in cardiomyopathy patients enrolled into the European Society of Cardiology (ESC) cardiomyopathy registry [part of the EURObservational Research Programme (EORP)].

Methods And Results : Three thousand, two hundred, and eight consecutive adult patients (34.6% female; median age: 53.0 ± 15 years) with cardiomyopathy were studied: 1260 with dilated (DCM), 1739 with hypertrophic (HCM), 66 with restrictive (RCM), and 143 with arrhythmogenic right ventricular cardiomyopathy (ARVC). CMR scans were performed at baseline in only 29.4% of patients. CMR utilization was variable according to cardiomyopathy subtypes: from 51.1% in ARVC to 36.4% in RCM, 33.8% in HCM, and 20.6% in DCM (P < 0.001). CMR use in tertiary referral centres located in different European countries varied from 1% to 63.2%. Patients undergoing CMR were younger, less symptomatic, less frequently had implantable cardioverter-defibrillator (ICD)/pacemaker implanted, had fewer cardiovascular risk factors and comorbidities (P < 0.001). In 28.6% of patients, CMR was used along with transthoracic echocardiography (TTE); 67.6% patients underwent TTE alone, and 0.9% only CMR.

Conclusion : Less than one-third of patients enrolled in the registry underwent CMR and the use varied greatly between cardiomyopathy subtypes, clinical profiles of patients, and European tertiary referral centres. This gap with current guidelines needs to be considered carefully by scientific societies to promote wider availability and use of CMR in patients with cardiomyopathies.
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http://dx.doi.org/10.1093/ehjci/jeaa329DOI Listing
January 2021

Novel autophagic vacuolar myopathies: Phenotype and genotype features.

Neuropathol Appl Neurobiol 2021 Jan 4. Epub 2021 Jan 4.

Department of Advanced Medical and Surgical Sciences, 2nd Division of Neurology, Center for Rare Diseases and Inter University Center for Research in Neurosciences, University of Campania "Luigi Vanvitelli", Naples, Italy.

Background: Autophagic vacuolar myopathies (AVMs) are an emerging group of heterogeneous myopathies sharing histopathological features on muscle pathology, in which autophagic vacuoles are the pathognomonic morphologic hallmarks. Glycogen storage disease type II (GSDII) caused by lysosomal acid α-glucosidase (GAA) deficiency is the best-characterised AVM.

Aims: This study aimed to investigate the mutational profiling of seven neuromuscular outpatients sharing clinical, myopathological and biochemical findings with AVMs.

Methods: We applied a diagnostic protocol, recently published by our research group for suspected late-onset GSDII (LO-GSDII), including counting PAS-positive lymphocytes on blood smears, dried blood spot (DBS)-GAA, muscle biopsy histological and immunofluorescence studies, GAA activity assay and expression studies on muscle homogenate, GAA sequencing, GAA multiplex ligation-dependent probe amplification (MLPA) and whole exome sequencing (WES).

Results: The patients had a limb girdle-like muscular pattern with persistent hyperCKaemia; vacuolated PAS-positive lymphocytes, glycogen accumulation and impaired autophagy at muscle biopsy. Decreased GAA activity was also measured. While GAA sequencing identified no pathogenic mutations, WES approach allowed us to identify for each patient an unexpected mutational pattern in genes cooperating in lysosomal-autophagic machinery, some of which have never been linked to human diseases.

Conclusions: Our data suggest that reduced GAA activity may occur in any condition of impaired autophagy and that WES approach is advisable in all genetically undefined cases of autophagic myopathy. Therefore, deficiency of GAA activity and PAS-positive lymphocytes should be considered as AVM markers together with LC3/p62-positive autophagic vacuoles.
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http://dx.doi.org/10.1111/nan.12690DOI Listing
January 2021

A pilot clinical trial with losartan in Myhre syndrome.

Am J Med Genet A 2021 03 24;185(3):702-709. Epub 2020 Dec 24.

Department of Translational Medicine, Section of Pediatrics, Federico II University, Naples, Italy.

Introduction: Myhre syndrome (MS) is an ultra-rare disorder due to pathogenic variants in the SMAD4 gene that encodes a protein regulating the TGF-β pathway and extra-cellular matrix (ECM) homeostasis. Main clinical features of MS include thickening of skin and joint stiffness. Previous studies showed that losartan improved ECM deposition in MS fibroblasts.

Materials And Methods: Four molecularly confirmed MS subjects (mean age 23.8 ± 17 years) were evaluated for: (a) skin thickness by Rodnan score, (b) joint range of motion (ROM) by goniometry, and (c) speckle-tracking echocardiogram. Following baseline evaluations, three MS individuals received losartan for 12 months and pre-defined endpoints were monitored after 6 and 12 months of treatment.

Results: At baseline, Rodnan scores were increased, joint ROM was reduced, and speckle-tracking echocardiogram revealed reduced myocardial strain. In three MS subjects, improvements in skin thickness, joint ROM and to a lesser extent of myocardial strain, were observed after 6 and 12 months of losartan treatment.

Conclusions: Although further long-term controlled clinical trials with a larger number of affected individuals are needed, the present study suggests that losartan might improve skin, joint and heart abnormalities of MS.
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http://dx.doi.org/10.1002/ajmg.a.62019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7898344PMC
March 2021

Dietary Thiols: A Potential Supporting Strategy against Oxidative Stress in Heart Failure and Muscular Damage during Sports Activity.

Int J Environ Res Public Health 2020 12 16;17(24). Epub 2020 Dec 16.

Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Via S. Pansini 5, 80131 Naples, Italy.

Moderate exercise combined with proper nutrition are considered protective factors against cardiovascular disease and musculoskeletal disorders. However, physical activity is known not only to have positive effects. In fact, the achievement of a good performance requires a very high oxygen consumption, which leads to the formation of oxygen free radicals, responsible for premature cell aging and diseases such as heart failure and muscle injury. In this scenario, a primary role is played by antioxidants, in particular by natural antioxidants that can be taken through the diet. Natural antioxidants are molecules capable of counteracting oxygen free radicals without causing cellular cytotoxicity. In recent years, therefore, research has conducted numerous studies on the identification of natural micronutrients, in order to prevent or mitigate oxidative stress induced by physical activity by helping to support conventional drug therapies against heart failure and muscle damage. The aim of this review is to have an overview of how controlled physical activity and a diet rich in antioxidants can represent a "natural cure" to prevent imbalances caused by free oxygen radicals in diseases such as heart failure and muscle damage. In particular, we will focus on sulfur-containing compounds that have the ability to protect the body from oxidative stress. We will mainly focus on six natural antioxidants: glutathione, taurine, lipoic acid, sulforaphane, garlic and methylsulfonylmethane.
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http://dx.doi.org/10.3390/ijerph17249424DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7765667PMC
December 2020

Beta-blockers in heart failure prognosis: Lessons learned by MECKI Score Group papers.

Eur J Prev Cardiol 2020 12;27(2_suppl):65-71

Department of Cardiology, University of Foggia, Italy.

Heart failure is a complex syndrome affecting several organs including kidney, lungs, liver, brain muscles and sympathetic system. Each of these organs might contribute to its severity and prognosis. The prognosis assessment is critical for a correct heart failure management. It has already been demonstrated that a single parameter is weaker for prognosis than different parameters combined. The Metabolic Exercise test data combined with Cardiac and Kidney Indexes (MECKI) score has been built and validated for heart failure with reduced ejection fraction (HFrEF) patients by considering cardiopulmonary exercise test data combined with clinical, laboratory and echocardiographic measurements. The betablockers treatment is a milestone in the HFrEF management. In the MECKI score database, the association of betablockers treatment with outcome has been investigated in different settings.
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http://dx.doi.org/10.1177/2047487320951109DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7691627PMC
December 2020

Clinical Profile of Cardiac Involvement in Danon Disease: A Multicenter European Registry.

Circ Genom Precis Med 2020 12 5;13(6):e003117. Epub 2020 Nov 5.

Leviev Heart Center, Sheba Medical Center and Sackler School of Medicine, Tel Aviv University, Israel (D.L., Y.W., M.A.).

Background: The X-linked Danon disease manifests by severe cardiomyopathy, myopathy, and neuropsychiatric problems. We designed this registry to generate a comprehensive picture of clinical presentations and outcome of patients with Danon disease in cardiomyopathy centers throughout Europe.

Methods: Clinical and genetic data were collected in 16 cardiology centers from 8 European countries.

Results: The cohort comprised 30 male and 27 female patients. The age at diagnosis was birth to 42 years in men and 2 to 65 in women. Cardiac involvement was observed in 96%. Extracardiac manifestations were prominent in men but not in women. Left ventricular (LV) hypertrophy was reported in 73% of male and 74% of female patients. LV systolic dysfunction was reported in 40% of men (who had LV ejection fraction, 34±11%) and 59% of women (LV ejection fraction, 28±13%). The risk of arrhythmia and heart failure was comparable among sexes. The age of first heart failure hospitalization was lower in men (18±6 versus 28±17 years; <0.003). Heart failure was the leading cause of death (10 of 17; 59%), and LV systolic dysfunction predicted an adverse outcome. Eight men and 8 women (28%) underwent heart transplantation or received an LV assist device. Our cohort suggests better prognosis of female compared with male heart transplant recipients.

Conclusions: Danon disease presents earlier in men than in women and runs a malignant course in both sexes, due to cardiac complications. Cardiomyopathy features, heart failure and arrhythmia, are similar among the sexes. Clinical diagnosis and management is extremely challenging in women due to phenotypic diversity and the absence of extracardiac manifestations.
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http://dx.doi.org/10.1161/CIRCGEN.120.003117DOI Listing
December 2020

Potential role of imaging markers in predicting future disease expression of arrhythmogenic cardiomyopathy.

Future Cardiol 2020 Oct 21. Epub 2020 Oct 21.

Department of Translational Medical Sciences, Inherited & Rare Cardiovascular Diseases, University of Campania 'Luigi Vanvitelli', Monaldi Hospital, Naples, Italy.

To evaluate the predictive accuracy of trabecular hypertrophy/derangement and late gadolinium enhancement in predicting diagnosis of arrhythmogenic cardiomyopathy (AC). Fifty-nine suspected AC patients were evaluated. To evaluate the ability of these markers to detect patients fulfilling definite diagnosis at 4-year follow-up, sensitivity (Se), specificity (Sp), positive and negative predictive value (PPV and NPV) and predictive accuracy (PA) were calculated. Presence of trabecular hypertrophy/derangement showed high NPV, while late gadolinium enhancement at cardiac MRI high Sp, NPV and PA. Their combination with symptoms and/or revised Task Force Criteria showed a high Sp, NPV and PA for definitive diagnosis during follow-up. In suspected AC patients, the absence of these markers allows to identify those with lower risk of disease progression.
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http://dx.doi.org/10.2217/fca-2020-0107DOI Listing
October 2020

Feasibility and functional correlates of left atrial volume changes during stress echocardiography in chronic coronary syndromes.

Int J Cardiovasc Imaging 2021 Mar 15;37(3):953-964. Epub 2020 Oct 15.

Cardiology Department, Saint Petersburg State University Clinic, Saint Petersburg State University, St Petersburg, Russian Federation.

An enlarged left atrial volume index (LAVI) at rest mirrors increased LA pressure and/or impairment of LA function. A cardiovascular stress may acutely modify left atrial volume (LAV) within minutes. Aim of this study was to assess the feasibility and functional correlates of LAV-stress echocardiography (SE) Out of 514 subjects referred to 10 quality-controlled labs, LAV-SE was completed in 490 (359 male, age 67 ± 12 years) with suspected or known chronic coronary syndromes (n = 462) or asymptomatic controls (n = 28). The utilized stress was exercise in 177, vasodilator in 167, dobutamine in 146. LAV was measured with the biplane disk summation method. SE was performed with the ABCDE protocol. The intra-observer and inter-observer LAV variability were 5% and 8%, respectively. ∆-LAVI changes (stress-rest) were negatively correlated with resting LAVI (r = - 0.271, p < 0.001) and heart rate reserve (r = -.239, p < 0.001). LAV-dilators were defined as those with stress-rest increase ≥ 6.8 ml/m, a cutoff derived from a calculated reference change value above the biological, analytical and observer variability of LAVI. LAV dilation occurred in 56 patients (11%), more frequently with exercise (16%) and dipyridamole (13%) compared to dobutamine (4%, p < 0.01). At multivariable logistic regression analysis, B-lines ≥ 2 (OR: 2.586, 95% CI = 1.1293-5.169, p = 0.007) and abnormal contractile reserve (OR: 2.207, 95% CI = 1.111-4.386, p = 0.024) were associated with LAV dilation. In conclusion, LAV-SE is feasible with high success rate and low variability in patients with chronic coronary syndromes. LAV dilation is more likely with reduced left ventricular contractile reserve and pulmonary congestion.
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http://dx.doi.org/10.1007/s10554-020-02071-5DOI Listing
March 2021

Molecular Basis of Inflammation in the Pathogenesis of Cardiomyopathies.

Int J Mol Sci 2020 Sep 4;21(18). Epub 2020 Sep 4.

Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", 80131 Naples, Italy.

Cardiomyopathies (CMPs) represent a diverse group of heart muscle diseases, grouped into specific morphological and functional phenotypes. CMPs are associated with mutations in sarcomeric and non-sarcomeric genes, with several suspected epigenetic and environmental mechanisms involved in determining penetrance and expressivity. The understanding of the underlying molecular mechanisms of myocardial diseases is fundamental to achieving a proper management and treatment of these disorders. Among these, inflammation seems to play an important role in the pathogenesis of CMPs. The aim of the present study is to review the current knowledge on the role of inflammation and the immune system activation in the pathogenesis of CMPs and to identify potential molecular targets for a tailored anti-inflammatory treatment.
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http://dx.doi.org/10.3390/ijms21186462DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7554875PMC
September 2020

Lipoprotein(a): a genetic marker for cardiovascular disease and target for emerging therapies.

J Cardiovasc Med (Hagerstown) 2021 Mar;22(3):151-161

Department of Translational Medical Sciences, University of Campania 'Luigi Vanvitelli', Naples.

Lipoprotein(a) [Lp(a)] is an established cardiovascular risk factor, and growing evidence indicates its causal association with atherosclerotic disease because of the proatherogenic low-density lipoprotein (LDL)-like properties and the prothrombotic plasminogen-like activity of apolipoprotein(a) [apo(a)]. As genetics significantly influences its plasma concentration, Lp(a) is considered an inherited risk factor of atherosclerotic cardiovascular disease (ASCVD), especially in young individuals. Moreover, it has been suggested that elevated Lp(a) may significantly contribute to residual cardiovascular risk in patients with coronary artery disease and optimal LDL-C levels. Nonetheless, the fascinating hypothesis that lowering Lp(a) could reduce the risk of cardiovascular events - in primary or secondary prevention - still needs to be demonstrated by randomized clinical trials. To date, no specific Lp(a)-lowering agent has been approved for reducing the lipoprotein levels, and current lipid-lowering drugs have limited effects. In the future, emerging therapies targeting Lp(a) may offer the possibility to further investigate the relation between Lp(a) levels and cardiovascular outcomes in randomized controlled trials, ultimately leading to a new era in cardiovascular prevention. In this review, we aim to provide an updated overview of current evidence on Lp(a) as well as currently investigated therapeutic strategies that specifically address the reduction of the lipoprotein.
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http://dx.doi.org/10.2459/JCM.0000000000001077DOI Listing
March 2021

Combined PTPN11 and MYBPC3 Gene Mutations in an Adult Patient with Noonan Syndrome and Hypertrophic Cardiomyopathy.

Genes (Basel) 2020 08 17;11(8). Epub 2020 Aug 17.

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

In this report, an atypical case of Noonan syndrome (NS) associated with sarcomeric hypertrophic cardiomyopathy (HCM) in a 33-year-old patient was described. Genetic testing revealed two different disease-causing mutations: a mutation in the PTPN11 gene, explaining NS, and a mutation in the MYBPC3 gene, known to be associated with HCM. This case exemplifies the challenge in achieving a definite etiological diagnosis in patients with HCM and the need to exclude other diseases mimicking this condition (genocopies or phenocopies). Compound heterozygous mutations are rare but possible in HCM patients. In conclusion, this study highlights the important role of genetic testing as a necessary diagnostic tool for performing a definitive etiological diagnosis of HCM.
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http://dx.doi.org/10.3390/genes11080947DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7463848PMC
August 2020

The hospitalizations in hypertrophic cardiomyopathy: "The dark side of the moon".

Int J Cardiol 2020 11 15;318:101-102. Epub 2020 Aug 15.

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, Naples, Italy; Institute of Cardiovascular Sciences, University College of London and St. Bartholomew's Hospital, London, UK. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2020.08.054DOI Listing
November 2020

Resilience and response of the congenital cardiac network in Italy during the COVID-19 pandemic.

J Cardiovasc Med (Hagerstown) 2021 01;22(1):9-13

Pediatric Hospital, 'Giovanni XXIII' Pediatric Hospital, Bari.

: The worldwide response to the current COVID-19 pandemic has been focused on how to prevent the disease and to protect the high-risk patient from a potentially lethal infection. Several consensus and guidelines articles have been published dealing with the cardiac patient with systemic hypertension, heart transplant or heart failure. Very little is known about the patients, both in the pediatric as well as in the adult age, with congenital heart disease. The peculiar physiology of the heart with a native, repaired or palliated congenital heart defect deserves a specialized care. Hereby we describe the early recommendations issued by the Italian Society of Pediatric Cardiology and Congenital Heart Disease and how the network of the congenital cardiac institutions in Italy reacted to the threat of potential wide spread of the infection among this fragile kind of patient.
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http://dx.doi.org/10.2459/JCM.0000000000001063DOI Listing
January 2021

Management of nonischemic-dilated cardiomyopathies in clinical practice: a position paper of the working group on myocardial and pericardial diseases of Italian Society of Cardiology.

J Cardiovasc Med (Hagerstown) 2020 Dec;21(12):927-943

Cardiothoracovascular Department , Center for Diagnosis and Management of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), University of Trieste, Trieste.

: Nonischemic-dilated cardiomyopathy (NIDCM) is an entity that gathers extremely heterogeneous diseases. This awareness, although leading to continuous improvement in survival, has increased the complexity of NIDCM patients' management. Even though the endorsed 'red-flags' approach helps clinicians in pursuing an accurate etiological definition in clinical practice, it is not clear when and how peripheral centers should interact with referral centers with specific expertise in challenging scenarios (e.g. postmyocarditis and genetically determined dilated cardiomyopathy) and with easier access to second-line diagnostic tools and therapies. This position paper will summarize each step in NIDCM management, highlighting the multiple interactions between peripheral and referral centers, from first-line diagnostic workup and therapy to advanced heart failure management and long-term follow-up.
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http://dx.doi.org/10.2459/JCM.0000000000001050DOI Listing
December 2020

Use of sacubitril/valsartan as 'bridge to transplant' in patients with end-stage hypertrophic cardiomyopathy.

Future Cardiol 2021 Jan 29;17(1):89-94. Epub 2020 Jul 29.

Heart Failure & Cardiac Rehabilitation Unit, Department of Cardiology, AORN dei Colli, Monaldi Hospital, Naples 80100, Italy.

The end stage or burned-out phase is an uncommon but challenging clinical evolution of hypertrophic cardiomyopathy (HCM). The management of end-stage HCM is empirically based on the use of drugs approved for heart failure with reduced ejection fraction; however, cardiac transplantation often represents the best option to improve survival. In our case, we describe the use of sacubitril/valsartan as a 'bridge to transplant' in a patient with end-stage HCM. After introducing the drug, enhancements in functional capacity, a reduction in natriuretic peptides and an increase in left ventricular ejection fraction occurred. Given their improved volume of oxygen consumption (VO) peak and hemodynamic parameters, our patient was left off the waiting list for cardiac transplant and continues to be regularly followed-up with every 3 months.
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http://dx.doi.org/10.2217/fca-2020-0048DOI Listing
January 2021

An expert consensus document on the management of cardiovascular manifestations of Fabry disease.

Eur J Heart Fail 2020 07 14;22(7):1076-1096. Epub 2020 Aug 14.

Institute of Cardiovascular Science, University College London and Barts Heart Centre, London, UK.

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by pathogenic variants in the α-galactosidase A (GLA) gene that leads to reduced or undetectable α-galactosidase A enzyme activity and progressive accumulation of globotriaosylceramide and its deacylated form globotriaosylsphingosine in cells throughout the body. FD can be multisystemic with neurological, renal, cutaneous and cardiac involvement or be limited to the heart. Cardiac involvement is characterized by progressive cardiac hypertrophy, fibrosis, arrhythmias, heart failure and sudden cardiac death. The cardiac management of FD requires specific measures including enzyme replacement therapy or small pharmacological chaperones in patients carrying amenable pathogenic GLA gene variants and more general management of cardiac symptoms and complications. In this paper, we summarize current knowledge of FD-related heart disease and expert consensus recommendations for its management.
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http://dx.doi.org/10.1002/ejhf.1960DOI Listing
July 2020