Publications by authors named "Camillo Autore"

96 Publications

The natural history of hypertrophic cardiomyopathy.

Eur Heart J Suppl 2020 Nov 18;22(Suppl L):L11-L14. Epub 2020 Nov 18.

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

In the early years of the disease recognition, hypertrophic cardiomyopathy (HCM) was viewed as an ominous disease with unfavourable prognosis and with an annual mortality between 4% and 6%. At that time, 73% of the patients reported in the literature came from only two referral centres. With the introduction of echocardiography, our understanding of HCM has improved and non-selected patient populations were assembled in several centres. A more benign prognostic profile was documented with an annual mortality rate of 1.5% or less. In the 2000s, important therapeutic interventions further improved the prognosis of patients with HCM: implantable-cardioverter defibrillator for prevention of sudden death, heart transplantation for treatment of severe refractory heart failure, and an extensive treatment with myectomy for relief of left ventricular outflow tract gradient. The natural history of HCM has changed substantially with contemporary treatment achieving an annual mortality rate less than 1% with extended longevity and a greatly improved quality of life.
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http://dx.doi.org/10.1093/eurheartj/suaa125DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7904071PMC
November 2020

Reply to the letter "Takotsubo syndrome: Any more covariates of its recurrence?"

Int J Cardiol 2021 Feb 25. Epub 2021 Feb 25.

Cardiology, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy.

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http://dx.doi.org/10.1016/j.ijcard.2021.02.063DOI Listing
February 2021

Clinical characteristics of patients with takotsubo syndrome recurrence: An observational study with long-term follow-up.

Int J Cardiol 2020 Dec 24. Epub 2020 Dec 24.

Cardiology Unit, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy.

Background: Research investigating takotsubo syndrome (TTS) recurrence yielded conflicting results. Aim of the present study is to describe clinical characteristics of patients with TTS recurrence in a cohort with available long-term follow-up.

Methods: The study population included 234 TTS patients enrolled in a prospective multicenter registry, median follow-up of 1328 (407, 2526) days. To investigate factors associated with TTS recurrence, we analyzed patients with recurrence (Group A) in comparison with a subgroup of TTS patients within the whole population (group B) who had similar age, sex and median follow-up length (Group A 2280 days vs Group B 2361 days).

Results: We observed 9 TTS recurrences affecting 8 patients, all women, with a rate of 0.9% patients/year. Median time to first recurrence was 1593 days (interquartile range: 950, 2516). We detected no significant differences between patients with and without recurrences regarding cardiovascular risk factors, symptoms, ECG and echocardiographic findings at presentation, discharge therapy. Physical trigger and chronic obstructive pulmonary disease (COPD) were more prevalent in patients who experienced a recurrence (75% vs 27% and 50% vs 14% with p = 0.01 and p = 0.022 respectively). Univariable Cox regression analysis identified physical trigger and history of COPD to be both associated with TTS recurrence [hazard ratio (HR) 11.4, 95% confidence interval (CI) 2.29-56.8, p = 0.003 and HR 4.94, 95% CI 1.16-20.99 p = 0.031 respectively].

Conclusion: TTS recurrence is relatively uncommon. Association with physical trigger and COPD would suggest a closer follow-up in this subgroup of patients.
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http://dx.doi.org/10.1016/j.ijcard.2020.12.047DOI Listing
December 2020

[ANMCO/SIC Consensus document on the management of myocarditis].

G Ital Cardiol (Rome) 2020 Dec;21(12):969-989

S.C. Cardiologia, Dipartimento Cardiotoracovascolare, Azienda Sanitaria Universitaria Giuliano Isontina-ASUGI, Università di Trieste.

Myocarditis is an inflammatory heart disease that can occur acutely, as in acute myocarditis, or persistently, as in chronic myocarditis or chronic inflammatory cardiomyopathy. Different agents can induce myocarditis, with viruses being the most common triggers. Generally, acute myocarditis affects relatively young people and men more than women. Myocarditis has a broad spectrum of clinical presentations and evolution trajectories, although most cases resolve spontaneously. Patients with reduced left ventricular ejection fraction, heart failure symptoms, advanced atrioventricular block, sustained ventricular arrhythmias or cardiogenic shock (the latter known as fulminant myocarditis) are at increased risk for death and heart transplantation. The presentation of chronic inflammatory cardiomyopathy may be more subtle, with progressive symptoms of heart failure or appearance of rhythm disturbance, not rarely preceded by an infective episode. Autoimmune disorder or systemic inflammatory conditions can be another significant predisposing substrate of myocarditis, especially in women. Emerging causes of myocarditis are drug-related like the new anticancer therapies, the immune checkpoint inhibitors. In this Italian Association of Hospital Cardiologists (ANMCO) and Italian Society of Cardiology (SIC) expert consensus document on myocarditis, we propose diagnostic strategies for identifying possible causes of the disease and factors associated with increased risk. Finally, we propose potential treatments and when referring patients to tertiary centers, especially for high-risk patients. Even if endomyocardial biopsy is the invasive diagnostic tool for making definitive diagnosis and differentiation of histological subtypes (i.e., lymphocytic vs eosinophilic vs giant cell myocarditis), it is not always readily available in all centers. Thus, we propose when this exam is mandatory or when it can be postponed or substituted by cardiac magnetic resonance imaging. This document reflects the Italian perspective on managing patients with myocarditis and their follow-up, considering also current US and European scientific position statements.
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http://dx.doi.org/10.1714/3472.34551DOI Listing
December 2020

Accuracy of the "International Criteria" for ECG screening in athletes in comparison with previous published criteria: rationale and design of a diagnostic meta-analysis.

Minerva Cardioangiol 2020 Sep 30. Epub 2020 Sep 30.

Mediterranea Cardiocentro, Naples, Italy.

Introduction: The impact of the 'International Criteria' for ECG interpretation in athletes has further improved the diagnostic accuracy of the 12-lead ECG use for pre-participation screening (PPS) and these criteria have been evaluated in different populations of athletes and settings proving good results.

Evidence Acquisition: We aim to perform a comprehensive review of the use of the 'International Criteria' for ECG interpretation in athletes, stemming from a systematic review to diagnostic meta-analysis, limiting our inclusion only to observational studies to determine the diagnostic accuracy of ECG for detecting cardiac anomalies related to sudden cardiac death in athletes.

Evidence Synthesis: This meta-analysis is expected to include several important studies related to PPS on different populations of athletes comparing different ECG criteria and detail important data on the diagnostic accuracy of ECG in PPS. Furthermore, we intend to highlight the advantage of using ECG in PPS.

Conclusions: The present diagnostic meta-analysis results will aid sports medicine physicians and cardiologist in adhering to the most accurate criteria for ECG evaluation in athletes and it may help to solve controversies aroused regarding the excess cost of ECG in PPS related to the amount of false positive cases.
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http://dx.doi.org/10.23736/S0026-4725.20.05347-5DOI Listing
September 2020

Arterial thrombo-embolic events in cardiac amyloidosis: a look beyond atrial fibrillation.

Amyloid 2020 Sep 28:1-7. Epub 2020 Sep 28.

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

Background: Intracardiac thrombosis is reported to occur frequently in cardiac amyloidosis (CA). However, data regarding arterial thrombo-embolic events (AEs) in CA are limited. We aimed at assessing prevalence, clinical characteristics and predictors of AEs in a large multicentric CA cohort.

Methods And Results: Four-hundred-six consecutive CA patients (134 AL, 73 ATTRm and 199 ATTRwt) from 5 Italian referral centres were retrospectively evaluated and followed-up for a median time of 19 months. Thirty-one patients (7.6%) suffered from an AE, of whom 10 (32.2%) were in sinus rhythm and had no history of AF. There were no significant differences in terms of age, gender and type of CA between patients with or without AEs. Fourteen (7.6%) of 185 patients on anticoagulation had an AE despite therapy. Anticoagulation therapy did not appear to fully protect from the risk of events (HR 1.23, 95%CI 0.52-2.92,  = .64). The only predictor of AEs, in particular among CA patients in sinus rhythm, was a CHA2DS2-VASC score ≥ 3 (HR 2.84, 95%CI 1.02-7.92,  = .05 in overall population; HR 10.13, 95%CI 1.12-91.19,  = .04 in patients in sinus rhythm).

Conclusions: In our large, multicentric, real-world cohort, prevalence and incidence rate of AEs was high. A consistent proportion of events occurred despite anticoagulation therapy or in patients in sinus rhythm. A higher CHA2DS2-VASc score might identify patients at risk of AEs also among those in sinus rhythm.
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http://dx.doi.org/10.1080/13506129.2020.1798922DOI Listing
September 2020

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

Transcatheter aortic valve replacement for aortic regurgitation after septal myectomy in patients with obstructive hypertrophic cardiomyopathy.

Cardiovasc Revasc Med 2020 Jun 15. Epub 2020 Jun 15.

Cardiology Unit, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy.

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http://dx.doi.org/10.1016/j.carrev.2020.06.010DOI Listing
June 2020

Multimodality Imaging in the Diagnostic Work-Up of Endocarditis and Cardiac Implantable Electronic Device (CIED) Infection.

J Clin Med 2020 Jul 14;9(7). Epub 2020 Jul 14.

Department of Nuclear Medicine, Department of Translational Research and New Technology in Medicine, University of Pisa, 56126 Pisa, Italy.

Infective endocarditis (IE) is a serious cardiac condition, which includes a wide range of clinical presentations, with varying degrees of severity. The diagnosis is multifactorial and a proper characterization of disease requires the identification of the primary site of infection (usually the cardiac valve) and the search of secondary systemic complications. Early depiction of local complications or distant embolization has a great impact on patient management and prognosis, as it may induce to aggressive antibiotic treatment or, in more advanced cases, cardiac surgery. In this setting, the multimodality imaging has assumed a pivotal role in the clinical decision making and it requires the physician to be aware of the advantages and disadvantages of each imaging technique. Echocardiography is the first imaging test, but it has several limitations. Therefore, the integration with other imaging modalities (computed tomography, magnetic resonance imaging, nuclear imaging) becomes often necessary. Different strategies should be applied depending on whether the infection is suspected or already ascertained, whether located in native or prosthetic valves, in the left or right chambers, or if it involves an implanted cardiac device. In addition, detection of extracardiac IE-related lesions is crucial for a correct management and treatment. The aim of this review is to illustrate strengths and weaknesses of the various methods in the most common clinical scenarios.
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http://dx.doi.org/10.3390/jcm9072237DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7408824PMC
July 2020

A systematic review on focal takotsubo syndrome: a not-so-small matter.

Heart Fail Rev 2020 Jun 13. Epub 2020 Jun 13.

Cardiology Unit, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Via Di Grottarossa 1035/1039, 00189, Rome, Italy.

A focal contraction pattern in takotsubo syndrome (TTS) is considered rare. Due to its peculiar presentation, which includes segmental left ventricular (LV) regional wall motion abnormalities (RWMA), the focal TTS pattern may be hardly differentiable from other entities, such as myocarditis or myocardial infarction. We performed a comprehensive systematic literature review researching for works in English published in Journals indexed in Embase, available online for consultation, using the following keywords (in Title and/or Abstract): ("takotsubo" OR "broken heart" OR "apical ballooning" OR "stress cardiomyopathy") AND ("focal" OR "atypical" OR "variant" OR "segments"). Thirty-three papers were retrieved: 17 case reports, 6 case series, and 10 population studies-with a total of 166 focal TTS patients. Prevalence of focal TTS ranged between 0.1% and 14% (pooled mean: 2.8%). Mean age of onset (58 years), gender distribution (80% of females), and type of triggers appeared similar to those reported in typical TTS. RWMA more frequently involved the interventricular septum and the anterolateral LV segments, with often preserved LV ejection fraction. In the majority of focal TTS reports that included adequate ECG information (n = 13), abnormalities were localized and not diffuse, always matching RWMA, and in 3 cases, reciprocal changes were observed. No in-hospital nor long-term deaths were reported. The focal TTS contraction pattern may be more prevalent than currently reported. Though possibly presenting with similar demographic background compared with typical TTS, the focal variant might be characterized by peculiar ECG modifications and better prognosis.
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http://dx.doi.org/10.1007/s10741-020-09988-yDOI Listing
June 2020

Clinical outcomes in adult athletes with hypertrophic cardiomyopathy: a 7-year follow-up study.

Br J Sports Med 2020 Aug 12;54(16):1008-1012. Epub 2020 Jun 12.

Departement of Medicine, Azienda Ospedaliera Sant Andrea, Roma, Lazio, Italy.

Objective: Current guidelines recommend precautionary disqualification from competitive sports in patients with hypertrophic cardiomyopathy (HCM). We assessed the incidence of cardiovascular events in a cohort of patients with HCM engaged in long-term exercise programmes and competitive sport.

Methods: We reviewed data on 88 consecutive athletes diagnosed with HCM, from 1997 to 2017; 92% male, 98% Caucasian, median age 31 (IQR: 19-44) years. All participated in regular exercise programmes and competitive sport at study entry.We performed follow-up evaluation after 7±5 (1-21) years. 61 (69%) of the athletes had substantially reduced or stopped exercise and sport (ie, HCM-detrained), and 27 had continued with regular training and sport competitions (HCM-trained). At baseline evaluation, both groups were similar for age, gender balance, symptoms, ECG abnormalities, extent of left ventricular hypertrophy, arrhythmias and risk profile for sudden cardiac death/arrest.

Results: During the follow-up period, two participants suffered sudden cardiac arrest or death (0.3% per year) both outside of sport participation. In addition, 19 (22%) reported symptoms (syncope in 3, palpitations in 10, chest pain in 4 and dyspnoea in 2). The Kaplan-Meier analyses of freedom from combined sudden cardiac arrest/death and symptoms (log-rank test p=0.264) showed no differences between HCM-trained and detrained patients.

Conclusion: In this adult cohort of low-risk HCM athletes, voluntary decision to pursue in participation in competitive sport events was not associated with increased risk for major cardiac events or clinical worsening compared with decision to reduce or withdraw from exercise programmes and sport. Similar results may not be seen in younger or racially diverse athlete populations, or in patients with more severe HCM phenotypes.
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http://dx.doi.org/10.1136/bjsports-2019-100890DOI Listing
August 2020

Risk Stratification in Hypertrophic Cardiomyopathy. Insights from Genetic Analysis and Cardiopulmonary Exercise Testing.

J Clin Med 2020 May 28;9(6). Epub 2020 May 28.

Department of Clinical and Molecular Medicine, Sapienza University, 00100 Rome, Italy.

The role of genetic testing over the clinical and functional variables, including data from the cardiopulmonary exercise test (CPET), in the hypertrophic cardiomyopathy (HCM) risk stratification remains unclear. A retrospective genotype-phenotype correlation was performed to analyze possible differences between patients with and without likely pathogenic/pathogenic (LP/P) variants. A total of 371 HCM patients were screened at least for the main sarcomeric genes (myosin binding protein C), (β-myosin heavy chain), (cardiac troponin I) and (cardiac troponin T): 203 patients had at least an LP/P variant, 23 patients had a unique variant of uncertain significance (VUS) and 145 did not show any LP/P variant or VUS. During a median 5.4 years follow-up, 51 and 14 patients developed heart failure (HF) and sudden cardiac death (SCD) or SCD-equivalents events, respectively. The LP/P variant was associated with a more aggressive HCM phenotype. However, left atrial diameter (LAd), circulatory power (peak oxygen uptake*peak systolic blood pressure, CP%) and ventilatory efficiency (C-index = 0.839) were the only independent predictors of HF whereas only LAd and CP% were predictors of the SCD end-point (C-index = 0.738). The present study reaffirms the pivotal role of the clinical variables and, particularly of those CPET-derived, in the HCM risk stratification.
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http://dx.doi.org/10.3390/jcm9061636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7356142PMC
May 2020

Prognostic implications of nonsustained ventricular tachycardia morphology in high-risk patients with hypertrophic cardiomyopathy.

J Cardiovasc Electrophysiol 2020 08 9;31(8):2093-2098. Epub 2020 Jun 9.

Division of Cardiology, Department of Clinical and Molecular Medicine, St. Andrea Hospital, Sapienza University, Rome, Italy.

Introduction: The prognostic impact of nonsustained ventricular tachycardia (NSVT) morphology has never been explored in hypertrophic cardiomyopathy (HCM). In a single-center cohort of consecutive HCM patients implanted with an implanted cardioverter-defibrillator (ICD), we assessed NSVT morphology patterns and their prognostic implications.

Methods: A cohort of consecutive HCM patients implanted with an ICD was followed from ICD implantation to last follow-up visit. Patients were assessed for NSVT as stored events in ICD memory. Ventricular tachycardias (VTs) were classified as monomorphic (MM) or polymorphic according to intracardiac electrogram morphology.

Results: One hundred nine consecutive HCM patients (68 males; mean age: 45 ± 17 years) composed the study population. During follow-up (71 ± 48 months), 7 polymorphic NSVT in 4 patients and 370 MM NSVT in 42 patients were retrieved from ICD memory. Among patients with only MM NSVT, 19 (45%) had one morphology, 17 (41%) had two morphologies, 3 (7%) had three morphologies, and 3 (7%) had four morphologies. Patients with polymorphic NSVT had the highest risk of ICD interventions (HR, 5.04; 95% CI, 1.26-20.19; P = .02). A stepwise increase of the risk of ICD interventions in patients with two, three, and four NSVT morphologies was observed. Out of 16 patients with both NSVT and ICD-treated VTs, 13 (81%) had at least one ICD-treated VT with the same morphology of a previous long-lasting NSVT.

Conclusions: In high-risk HCM patients, the occurrence of polymorphic NSVT and of NSVT with multiple morphologies carries a high risk for ICD interventions. Sustained VTs tend to recur with the same morphology of previous long-lasting NSVTs.
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http://dx.doi.org/10.1111/jce.14571DOI Listing
August 2020

Non-alcoholic fatty liver disease and heart valve disease: a neglected link.

Minerva Cardioangiol 2020 12 26;68(6):542-544. Epub 2020 Feb 26.

Department of Medical-Surgical Sciences and Biotechnologies, Sapienza University, Rome, Italy -

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http://dx.doi.org/10.23736/S0026-4725.20.05161-0DOI Listing
December 2020

Low Sensitivity of Bone Scintigraphy in Detecting Phe64Leu Mutation-Related Transthyretin Cardiac Amyloidosis.

JACC Cardiovasc Imaging 2020 06 18;13(6):1314-1321. Epub 2019 Dec 18.

Cardiology, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater University of Bologna, Bologna, Italy.

Objectives: The aim of this study was to assess the diagnostic accuracy of bone scintigraphy in a large multicenter cohort of patients with cardiac amyloidotic involvement and Phe64Leu transthyretin (TTR) mutation.

Background: Diagnostic accuracy of bone scintigraphy for transthyretin-related cardiac amyloidosis (TTR-CA) is considered extremely high, enabling this technique to be the noninvasive diagnostic standard for TTR-CA. Nevertheless, this approach has not been systematically validated across the entire spectrum of TTR mutations.

Methods: A total of 55 patients with Phe64Leu TTR mutation were retrospectively analyzed and evaluated between 1993 and 2018 at 7 specialized Italian tertiary centers. Cardiac involvement was defined as presence of an end-diastolic interventricular septum thickness ≥12 mm, without other possible causes of left ventricular hypertrophy (i.e., arterial hypertension or valvulopathies). A technetium-99m (99mTc)-diphosphonate (DPD) or 99mTc-hydroxyl-methylene-diphosphonate (HMDP) bone scintigraphy was reviewed, and visual scoring was evaluated according to Perugini's method.

Results: Among 26 patients with definite cardiac involvement, 19 underwent 99mTc-DPD or 99mTc-HMDP bone scintigraphy. Of them, 17 (89.5%) patients had low or absent myocardial bone tracer uptake, whereas only 2 (10.5%) showed high-grade myocardial uptake. The sensitivity and the accuracy of bone scintigraphy in detecting TTR-CA were 10.5% and 37%, respectively. Patients with cardiac involvement and low or absent bone tracer uptake were similar to those with high-grade myocardial uptake in terms of age, sex, and electrocardiographic and echocardiographic findings.

Conclusions: The sensitivity of bone scintigraphy (DPD and HMDP) in detecting TTR-CA is extremely low in patients with Phe64Leu TTR mutation, suggesting the need to assess diagnostic accuracy of bone scintigraphy to identify cardiac involvement across a wider spectrum of TTR mutations.
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http://dx.doi.org/10.1016/j.jcmg.2019.10.015DOI Listing
June 2020

Prognostic relevance of GRACE risk score in Takotsubo syndrome.

Eur Heart J Acute Cardiovasc Care 2020 Oct 23;9(7):721-728. Epub 2019 Oct 23.

Clinical and Interventional Cardiology, Sassari University Hospital, Italy.

Background: Takotsubo syndrome is an increasingly recognised cardiac condition that clinically mimics an acute coronary syndrome, but data regarding its prognosis remain controversial. It is currently unknown whether acute coronary syndrome risk scores could effectively be applied to Takotsubo syndrome patients. This study aims to assess whether the Global Registry of Acute Coronary Events (GRACE) score can predict clinical outcome in Takotsubo syndrome and to compare the prognosis with matched acute coronary syndrome patients.

Methods: A total of 561 Takotsubo syndrome patients was included in this prospective registry. According to the GRACE score, the population was divided into quartiles. The primary endpoint was all-cause mortality and the secondary endpoints were cardiocerebrovascular events (a composite of all-cause mortality, cardiovascular death, recurrence of Takotsubo syndrome and stroke).

Results: The median GRACE risk score was 139±27. Takotsubo syndrome patients with a higher GRACE risk score mostly have a higher rate of physical triggers and lower left ventricular ejection fraction on admission. During long-term follow-up, all-cause mortality rates were 5%, 11%, 12% and 22%, respectively, in the first, second, third and fourth quartile (<0.001). After multivariate analysis, the GRACE risk score was found to be a strong predictor of all-cause mortality (odds ratio (OR) 1.68, 95% confidence interval (CI) 1.28-2.20; =0.001) and cardiocerebrovascular events (OR 1.63, 95% CI 1.26-2.11; =0.001). Moreover, all-cause mortality in Takotsubo syndrome patients was comparable with the matched acute coronary syndrome cohort.

Conclusion: In Takotsubo syndrome, the GRACE risk score allows us to predict all-cause mortality and cardiocerebrovascular events at long-term follow-up.
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http://dx.doi.org/10.1177/2048872619882363DOI Listing
October 2020

Electrocardiographic changes in focal takotsubo syndrome: a case report.

J Cardiovasc Med (Hagerstown) 2019 Nov;20(11):783-786

Cardiology Unit, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome.

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http://dx.doi.org/10.2459/JCM.0000000000000856DOI Listing
November 2019

Real-world versus trial patients with transthyretin amyloid cardiomyopathy.

Eur J Heart Fail 2019 Nov 24;21(11):1479-1481. Epub 2019 Jul 24.

Emergency Medicine, Department of Internal Medicine, Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.

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http://dx.doi.org/10.1002/ejhf.1563DOI Listing
November 2019

Safety and efficacy of anti-tachycardia pacing in patients with hypertrophic cardiomyopathy implanted with an ICD.

Pacing Clin Electrophysiol 2019 06 5;42(6):610-616. Epub 2019 Apr 5.

Division of Cardiology, Department of Clinical and Molecular Medicine, St. Andrea Hospital, Sapienza University, Rome, Italy.

Background: In hypertrophic cardiomyopathy (HCM) patients implanted with an implantable cardioverter defibrillator (ICD), clinical outcomes of antitachycardia pacing (ATP) have been poorly explored. In a retrospective analysis of a cohort of consecutive HCM patients implanted with an ICD, we aimed to assess the efficacy, safety, and clinical value of ATP.

Methods: The cohort of HCM patients implanted with a transvenous ICD and followed in our center was assessed for device intervention from implantation to last clinical follow-up.

Results: Overall 77 patients (45 males; mean age: 46 ± 16 years) were analyzed. After 67 ± 41 months, 24 patients had 49 ventricular tachycardia/fibrillation (VT/VF) appropriately treated (5.8% per year). Among 39 monomorphic VTs, ATP was effective in 27 (success rate: 69%). Mean time from VT onset to ATP delivery was 9.1 ± 4.9 s. The only clinical variable improving ATP success was use of beta-blockers (81% vs 50%; P = .04). Out of 12 ineffectively treated VTs, one was immediately shocked, four self-terminated after 18 ± 12 s, and seven (18%) were accelerated to a new VT. ATP was also delivered for 27 of 42 inappropriately detected episodes and induced two de novo VTs (7%). In the per patient analysis, 14 out 77 (18%) patients had one or more appropriate and effective ATP (3.4% per year), and only six (8%; 1.4% per year) received more than one ATP.

Conclusion: ATP is moderately effective for the treatment of monomorphic VTs in HCM patients. However, the rate of appropriate ATP therapies is low, ATP is often prematurely delivered, and ATP-induced arrhythmia degeneration is of concern.
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http://dx.doi.org/10.1111/pace.13665DOI Listing
June 2019

Mutations in the GLA Gene and LysoGb3: Is It Really Anderson-Fabry Disease?

Int J Mol Sci 2018 Nov 23;19(12). Epub 2018 Nov 23.

Institute of Biomedicine and Molecular Immunology "A. Monroy", National Research Council, 90146 Palermo, Italy.

Anderson-Fabry disease (FD) is a rare, progressive, multisystem storage disorder caused by the partial or total deficit of the lysosomal enzyme α-galactosidase A (α-Gal A). It is an X-linked, lysosomal enzymopathy due to mutations in the galactosidase alpha gene (GLA), encoding the α-Gal A. To date, more than 900 mutations in this gene have been described. In our laboratories, the study of genetic and enzymatic alterations related to FD was performed in about 17,000 subjects with a symptomatology referable to this disorder. The accumulation of globotriaosylsphingosine (LysoGb3) was determined in blood of positives. Exonic mutations in the GLA gene were detected in 471 patients (207 Probands and 264 relatives): 71.6% of mutations were associated with the classic phenotype, 19.8% were associated with the late-onset phenotype, and 8.6% of genetic variants were of unknown significance (GVUS). The accumulation of LysoGb3 was found in all male patients with a mutation responsible for classic or late-onset FD. LysoGb3 levels were consistent with the type of mutations and the symptomatology of patients. α-Gal A activity in these patients is absent or dramatically reduced. In recent years, confusion about the pathogenicity of some mutations led to an association between non-causative mutations and FD. Our study shows that the identification of FD patients is possible by associating clinical history, GLA gene analysis, α-Gal A assay, and blood accumulation of LysoGB3. In our experience, LysoGB3 can be considered a reliable marker, which is very useful to confirm the diagnosis of Fabry disease.
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http://dx.doi.org/10.3390/ijms19123726DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320967PMC
November 2018

Response to letter from Madias regarding our article "Admission heart rate and in-hospital course of patients with Takotsubo syndrome".

Int J Cardiol 2019 01;274:64

Cardiology, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy.

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http://dx.doi.org/10.1016/j.ijcard.2018.09.077DOI Listing
January 2019

Sex-related differences in cardiomyopathies.

Int J Cardiol 2019 07 30;286:239-243. Epub 2018 Oct 30.

Institute of Cardiovascular Science, University College London and St. Bartholomew's Hospital, London, United Kingdom.

Cardiomyopathies (CMPs) are a heterogeneous group of heart muscle diseases with several different phenotypes defined as myocardial disorders in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular heart disease and congenital heart disease sufficient to explain the observed myocardial abnormality. CMPs can be classified into one of the following, i.e. hypertrophic CMP (HCM), dilated CMP (DCM), arrhythmogenic right ventricular CMP (ARVC), restrictive CMP (RCM), and unclassified CMPs. Although an increasing number of CMPs are now recognized to have a genetic basis, single mutations are associated with phenotypic variability and may cause not only a specific CMP, but also several different CMPs. Recently, it has become evident that, along with environmental interactions, age and sex may affect the penetrance of disease genes thus determining the phenotypic expression of CMPs. Noteworthy, an increasing body of data indicates that sex plays an important role in various forms of CMPs. The mode of inheritance may affect the sex-related occurrence of CMPs. Also, sex is a relevant determinant of the clinical manifestation of CMPs, and sex-related characteristics can be found in all forms. Sex-specific aspects of clinical disease expression as well as potential modes of inheritance should be therefore taken into proper consideration in order to improve the diagnostic work-up and treatment strategy of CMPs in both sexes.
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http://dx.doi.org/10.1016/j.ijcard.2018.10.091DOI Listing
July 2019

Effectiveness of the 2014 European Society of Cardiology guideline on sudden cardiac death in hypertrophic cardiomyopathy: a systematic review and meta-analysis.

Heart 2019 04 26;105(8):623-631. Epub 2018 Oct 26.

The Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, UK.

Objective: In 2014, the European Society of Cardiology (ESC) recommended the use of a novel risk prediction model (HCM Risk-SCD) to guide use of implantable cardioverter defibrillators (ICD) for the primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). We sought to determine the performance of HCM Risk-SCD by conducting a systematic review and meta-analysis of articles reporting on the prevalence of SCD within 5 years of evaluation in low, intermediate and high-risk patients as defined by the 2014 guidelines (predicted risk <4%, 4%-<6% and ≥6%, respectively).

Methods: The protocol was registered with PROSPERO (registration number: CRD42017064203). MEDLINE and manual searches for papers published from October 2014 to December 2017 were performed. Longitudinal, observational cohorts of unselected adult patients, without history of cardiac arrest were considered. The original HCM Risk-SCD development study was included a priori. Data were pooled using a random effects model.

Results: Six (0.9%) out of 653 independent publications identified by the initial search were included. The calculated 5-year risk of SCD was reported in 7291 individuals (70% low, 15% intermediate; 15% high risk) with 184 (2.5%) SCD endpoints within 5 years of baseline evaluation. Most SCD endpoints (68%) occurred in patients with an estimated 5-year risk of ≥4% who formed 30% of the total study cohort. Using the random effects method, the pooled prevalence of SCD endpoints was 1.01% (95% CI 0.52 to 1.61) in low-risk patients, 2.43% (95% CI 1.23 to 3.92) in intermediate and 8.4% (95% CI 6.68 to 10.25) in high-risk patients.

Conclusions: This meta-analysis demonstrates that HCM Risk-SCD provides accurate risk estimations that can be used to guide ICD therapy in accordance with the 2014 ESC guidelines.

Registration Number: PROSPERO CRD42017064203;Pre-results.
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http://dx.doi.org/10.1136/heartjnl-2018-313700DOI Listing
April 2019

Long-Term Left Ventricular Remodeling of Patients With Hypertrophic Cardiomyopathy.

Am J Cardiol 2018 12 8;122(11):1924-1931. Epub 2018 Sep 8.

Cardiology Unit, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy. Electronic address:

In hypertrophic cardiomyopathy (HC), a process of left ventricular (LV) remodeling carrying an adverse prognosis has been described. Conversely, a gradual and benign LV wall thinning has been suggested but never investigated. Therefore, we studied a HC cohort over a long period of time to evaluate the occurrence of a LV remodeling with a benign clinical course. Data of HC patients aged 18 to 65 years and without any condition known to influence LV remodeling were analyzed over a mean follow-up of 7.6 ± 5.7 years. Of 231 HC patients (65% males, mean age 46 ± 12 years), 47 (20%) developed LV remodeling, of whom 23 (10%) had a thinning ≥15% of LV maximal wall thickness from baseline without systolic dysfunction (MWT thinning); 13 (6%) progressed to a LV ejection fraction <50% (end-stage HC) and 11 (5%) developed an apical aneurysm. Follow-up length (odds ratio 1.07, 95% confidence interval 1.00 to 1.15, p = 0.06) and maximal LV wall thickness at baseline (odds ratio 1.14, 95% confidence interval 1.04 to 1.25, p = 0.004) were the main predictors of MWT thinning. Compared with patients with end-stage HC and apical aneurysm, those with MWT thinning showed lower HC-related morbidity (92% and 36% vs 22%, p = 0.003) and mortality (31% and 27% vs 4%, p = 0.02). Furthermore, they showed a combined HC-related morbidity and mortality similar to patients without LV remodeling (incidence 29/1000 vs 26/1000 patient-year, p = 0.77). In conclusion, a process of LV wall thinning with a benign outcome can occur over the long term in patients with HC. The prognostic importance of LV remodeling varies in relation to the different changes in LV morphology and function.
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http://dx.doi.org/10.1016/j.amjcard.2018.08.041DOI Listing
December 2018

Subcutaneous implantable cardioverter defibrillator in cardiomyopathies and channelopathies.

J Cardiovasc Med (Hagerstown) 2018 Nov;19(11):633-642

Department of Cardiac, Thoracic and Vascular Sciences, University of Padova.

: Cardiomyopathies and channelopathies are heterogeneous disorders that increase the risk of sudden cardiac death (SCD). Implantable cardioverter-defibrillator (ICD) therapy is safe and effective for preventing SCD in patients at risk for malignant ventricular arrhythmias. Because of the poor positive predictive value of current risk stratification tools, the majority of patients implanted with an ICD will never receive a life-saving therapy but will be exposed to the risk of complications such as device infection, lead failure and inappropriate therapy. Subcutaneous ICD (S-ICD) now constitutes a valuable alternative to conventional transvenous ICD in patients with cardiomyopathies and channelopathies as it provides protection from SCD while avoiding the risks of intravascular lead infection or failure. This may be particularly advantageous for young patients with a very long life expectancy. On the other hand, S-ICD cannot deliver antitachycardia pacing or antibradycardia pacing. The purpose of this article is to review the available evidence and the future perspectives of S-ICD therapy in patients with cardiomyopathies or channelopathies.
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http://dx.doi.org/10.2459/JCM.0000000000000712DOI Listing
November 2018

Admission heart rate and in-hospital course of patients with Takotsubo syndrome.

Int J Cardiol 2018 Dec 2;273:15-21. Epub 2018 Aug 2.

Cardiology, Clinical and Molecular Medicine Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy.

Background: In-hospital course of patients with Takotsubo syndrome (TS) is quite heterogeneous and life-threatening complications are not uncommon in the acute phase. The role of heart rate (HR) as a predictor of prognosis has not been sufficiently investigated in this setting. The study aims to assess the impact of HR at presentation on in-hospital course of patients with TS.

Methods: The study population included 221 patients with TS enrolled in a multicentric registry. HR at admission was evaluated on the first electrocardiogram. According to tertile distribution of HR at presentation, 3 groups were identified: Group A (HR ≤ 76 beats per minute (bpm), n = 76), Group B (HR 77-95 bpm, n = 74) and Group C (HR > 95 bpm, n = 71). Acute in-hospital complications were defined as occurrence of severe pump failure and major arrhythmias.

Results: 32 (14.4%) patients experienced complicated in-hospital course. HR on admission was significantly higher (108 bpm vs. 85 bpm; p < 0.001) and ejection fraction (EF) lower (35% vs. 40%; p = 0.009) in patients with complications than in those without. Patients in Group C experienced a 5-fold higher rate of complications compared to group A and B. After multivariate analysis, higher HR (odds ratio 1.34 per 10 bpm increase, 95% confidence interval (CI) 1.12-1.59; p = 0.001) and lower EF (odds ratio 1.24 per 5% decrease, 95% CI 1.01-1.54; p = 0.049) remained independently associated with a worse outcome.

Conclusion: In a large population with TS, high HR on admission independently predicted complicated in-hospital course.
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http://dx.doi.org/10.1016/j.ijcard.2018.07.145DOI Listing
December 2018

Clinical and prognostic impact of chronotropic incompetence in patients with hypertrophic cardiomyopathy.

Int J Cardiol 2018 Nov 4;271:125-131. Epub 2018 Aug 4.

Dpt Clinical and Molecular Medicine, Sapienza University, Rome, Italy.

Background: A blunted heart rate (HR) response is associated with an impaired peak oxygen uptake (pVO), a powerful outcome predictor in hypertrophic cardiomyopathy (HCM). The present multicenter study sought to determine the prognostic role for exercise-induced HR response in HCM.

Methods: A total of 681 consecutive HCM outpatients on optimized treatment were recruited. The heart failure (HF) end-point was death due to HF, cardiac transplantation, NYHA III-IV class progression, HF worsening leading to hospitalization and severe functional deterioration leading to septal reduction. The sudden cardiac death (SCD) end-point included SCD, aborted SCD and appropriate implantable cardioverter defibrillator discharges.

Results: During a median follow-up of 4.2 years (25-75th centile: 3.9-5.2), 81 patients reached the HF and 23 the SCD end-point. Covariates with independent effects on the HF end-point were left atrial diameter, left ventricular ejection fraction, maximal left ventricular outflow tract gradient and exercise cardiac power (ECP = pVO∗systolic blood pressure) (C-Index = 0.807) whereas the HCM Risk-SCD score and the ECP remained associated with the SCD end-point (C-Index = 0.674). When the VO-derived variables were not pursued, peak HR (pHR) re-entered in the multivariate HF model (C-Index = 0.777) and, marginally, in the SCD model (C-index = 0.656). A pHR = 70% of the maximum predicted resulted as the best cut-off value in predicting the HF-related events.

Conclusions: The cardiopulmonary exercise test is pivotal in the HCM management, however the pHR remains a meaningful alternative parameter. A pHR < 70% identified a HCM population at high risk of HF-related events, thus calling for a reappraisal of the chronotropic incompetence threshold in HCM.
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http://dx.doi.org/10.1016/j.ijcard.2018.04.019DOI Listing
November 2018

[A unique case of secondary takotsubo syndrome].

G Ital Cardiol (Rome) 2018 Apr;19(4):239-241

Cattedra di Cardiologia, Dipartimento di Medicina Clinica e Molecolare, Facoltà di Medicina e Psicologia, Sapienza Università di Roma.

Takotsubo syndrome (TTS) is an acute cardiac syndrome characterized by transient systolic left ventricular dysfunction frequently preceded by stressful events. It typically affects postmenopausal women without angiographic evidence of obstructive coronary artery disease. We report here an uncommon occurrence of secondary TTS in a male with coronary artery disease after exogenous catecholamine administration and pacemaker implantation. This unexpected case suggests that, in such clinical scenario, a TTS diagnosis might be considered even in unsuspected individuals.
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http://dx.doi.org/10.1714/2898.29218DOI Listing
April 2018

Prevalence and Clinical Outcome of Athletes With Mitral Valve Prolapse.

Circulation 2018 05;137(19):2080-2082

Institute of Sports Medicine and Science, Rome, Italy (S.C., F.M., J.C., A.P.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.033395DOI Listing
May 2018