Publications by authors named "Camillo Autore"

121 Publications

Myocardial infarction with non-obstructive coronary arteries in hypertrophic cardiomyopathy vs Fabry disease.

Int J Cardiol 2022 Aug 2. Epub 2022 Aug 2.

Department of Cardiovascular Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

Background: Little is known about prevalence and predictors of myocardial infarction with non-obstructive coronary arteries (MINOCA) in Fabry disease (FD) and hypertrophic cardiomyopathy (HCM). We assessed and compared the prevalence and predictors of MINOCA in a large cohort of HCM and FD patients.

Methods: In this multicenter, retrospective study we enrolled 2870 adult patients with HCM and 267 with FD. The only exclusion criterion was documented obstructive coronary artery disease. MINOCA was defined according to guidelines. For each patient we collected clinical, ECG and echocardiographic data recorded at initial evaluation.

Results: Overall, 36 patients had MINOCA during a follow-up period of 4.5 ± 11.2 years, MINOCA occurred in 16 patients with HCM (0.5%) and 20 patients with FD (7.5%; p < 0.001). The difference between the 2 groups was highly significant, also after adjustment for the main clinical, ECG and echocardiographic variables (OR 6.12;95%CI:2.80-13.3;p < 0.001). In the FD population MINOCA occurred in 17 out of 96 patients with left ventricle hypertrophy (LVH, 17.7%) and in 3 out of 171 patients without LVH (1.7%; OR 12.0;95%CI 3.43-42.3;p < 0.001). At multivariable analysis, voltage criteria for LVH at ECG (OR 7.3;95%CI 1.93-27.7;p = 0.003) and maximal LV wall thickness at echocardiography (OR 1.15; 95%CI 1.05-1.27;p = 0.002) maintained an independent association with MINOCA. No major significant differences were found in clinical, ECG and echocardiographic findings between HCM patients with or without MINOCA.

Conclusions: MINOCA was rare in HCM patients, and 6-fold more frequent in FD patients. MINOCA may be considered a red flag for FD and aid in the differential diagnosis from HCM.
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http://dx.doi.org/10.1016/j.ijcard.2022.07.046DOI Listing
August 2022

Relation of delayed intrinsicoid deflection of the QRS complex to sudden cardiac death in patients with hypertrophic cardiomyopathy.

Int J Cardiol 2022 Jun 30. Epub 2022 Jun 30.

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

Aims: Predictors of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) do not include ECG variables. Intrinsicoid deflection (ID) represents the early ventricular depolarization on surface ECG. Delayed ID (DID) has been associated with sudden cardiac arrest (SCA) in the community. In a cohort of consecutive patients with HCM, we assessed whether DID predicts SCA or its surrogates.

Methods: We reviewed ECG, clinical and follow-up data of 344 consecutive HCM patients. DID (ID ≥50 ms) was classified as lateral (leads I or aVL), inferior (leads II, III or aVF), and precordial (leads V5 or V6). The endpoint was a combination of SCD, resuscitated SCA or appropriate ICD intervention.

Results: The SCA group was composed by 2 secondary prevention ICD recipients and 23 patients that reached the endpoint during follow-up (108 ± 73 months). SCA patients had more frequently massive LV hypertrophy (LVH) or end-stage HCM. ECG indexes of LVH were comparable between SCA and controls. SCA patients were more likely to have DID on ECG lateral leads I/aVL (72% vs 44%; p = 0.008). A non significant trend was observed for inferior and V5/V6 leads. DID I/aVL was associated with SCA in multivariate analysis after correction for massive LVH and end-stage disease (HR: 2.86; 95%CI: 1.14-7.13; p = 0.02).

Conclusions: In HCM patients DID is associated with increased risk of SCA. Its prognostic value extends beyond that of LVH. If confirmed in prospective studies, the prognostic power of this ECG marker could be used to refine risk prediction.
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http://dx.doi.org/10.1016/j.ijcard.2022.06.066DOI Listing
June 2022

Impact of a Variant on the Occurrence of Acute Coronary Syndromes.

Front Cardiovasc Med 2022 31;9:921244. Epub 2022 May 31.

Cardiology Unit, Department of Clinical and Molecular Medicine, School of Medicine and Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy.

Background: Among several potential mechanisms, mitochondrial dysfunction has been proposed to be involved in the pathogenesis of coronary artery disease (CAD). A mitochondrial complex I deficiency severely impairs cardiovascular health and contributes to CAD development. Previous evidence highlighted a key role of , a subunit of complex I, deficiency in the increased occurrence of renal and cerebrovascular damage in an animal model of hypertension, and of juvenile ischemic stroke occurrence in humans. Furthermore, a significant decrease of mRNA was detected in peripheral blood mononuclear cells from patients experiencing acute coronary syndrome (ACS). The T allele at /rs23117379 variant is known to associate with reduced gene expression and mitochondrial dysfunction.

Objective: In the present study we tested the impact of the T/C /rs23117379 variant on occurrence of ACS in a prospective cohort of CAD patients ( = 260).

Results: Hypertension, smoking habit, diabetes and hypercholesterolemia were present in a large proportion of patients. Non-ST-elevation myocardial infarction (NSTEMI) represented the most frequent type of ACS (44%, = 115), followed by ST-elevation myocardial infarction (STEMI) (34%, = 88) and unstable angina (22%, = 57). The alleles/genotypes distribution for T/C at /rs23117379 revealed that the TT genotype was associated with a trend toward the development of ACS at an earlier age (TT 61 ± 12, CT 65 ± 12 and CC 66 ± 11 years; = 0.051 after adjustment for gender, hypertension, smoking habit, diabetes and hypercholesterolemia) and with a significant predictive role for ACS recurrence (hazard ratio [HR]1.671; 95% confidence interval [CI], 1.138-2.472; = 0.009).

Conclusions: Our findings are consistent with a deleterious effect of deficiency on acute coronary events predisposition and further support a role of the /rs23117379 variant as a genetic cardiovascular risk factor.
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http://dx.doi.org/10.3389/fcvm.2022.921244DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9197441PMC
May 2022

Clinical application of CMR in cardiomyopathies: evolving concepts and techniques : A position paper of myocardial and pericardial diseases and cardiac magnetic resonance working groups of Italian society of cardiology.

Heart Fail Rev 2022 May 10. Epub 2022 May 10.

Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

Cardiac magnetic resonance (CMR) has become an essential tool for the evaluation of patients affected or at risk of developing cardiomyopathies (CMPs). In fact, CMR not only provides precise data on cardiac volumes, wall thickness, mass and systolic function but it also a non-invasive characterization of myocardial tissue, thus helping the early diagnosis and the precise phenotyping of the different CMPs, which is essential for early and individualized treatment of patients. Furthermore, several CMR characteristics, such as the presence of extensive LGE or abnormal mapping values, are emerging as prognostic markers, therefore helping to define patients' risk. Lastly new experimental CMR techniques are under investigation and might contribute to widen our knowledge in the field of CMPs. In this perspective, CMR appears an essential tool to be systematically applied in the diagnostic and prognostic work-up of CMPs in clinical practice. This review provides a deep overview of clinical applicability of standard and emerging CMR techniques in the management of CMPs.
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http://dx.doi.org/10.1007/s10741-022-10235-9DOI Listing
May 2022

Incidence and risk factors for pacemaker implantation in light-chain and transthyretin cardiac amyloidosis.

Eur J Heart Fail 2022 Jul 16;24(7):1227-1236. Epub 2022 May 16.

Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Aims: The incidence and risk factors of pacemaker (PM) implantation in patients with cardiac amyloidosis (CA) are largely unexplored. We sought to characterize the trends in the incidence of permanent PM and to identify baseline predictors of future PM implantation in light-chain (AL) and transthyretin (ATTR) CA.

Methods And Results: Consecutive patients with AL and ATTR-CA diagnosed at participating centres (2017-2020) were included. Clinical data recorded within ±1 month from diagnosis were collected from electronic medical records. The primary study outcome was the need for clinically-indicated PM implantation. Patients with PM (n = 41) and/or permanent defibrillator in situ (n = 13) at CA diagnosis were excluded. The study population consisted of 405 patients: 29.4% AL, 14.6% variant ATTR and 56% wild-type ATTR; 82.5% were male, median age 76 years. During a median follow-up of 33 months (interquartile range 21-46), 36 (8.9%) patients experienced the primary outcome: 10 AL-CA, 2 variant ATTR-CA and 24 wild-type ATTR-CA (p = 0.08 at time-to-event analysis). At multivariable analysis, history of atrial fibrillation (hazard ratio [HR] 3.80, p = 0.002), PR interval (HR 1.013, p = 0.002) and QRS >120 ms (HR 4.7, p = 0.001) on baseline electrocardiogram were independently associated with PM implantation. The absence of these three factors had a negative predictive value of 92% with an area under the curve of 91.8% at 6 months.

Conclusion: In a large cohort of AL and ATTR-CA patients, 8.9% received a PM within 3 years after diagnosis. History of atrial fibrillation, PR >200 ms and QRS >120 ms predicted future PM implantation.
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http://dx.doi.org/10.1002/ejhf.2533DOI Listing
July 2022

Unmasking the prevalence of amyloid cardiomyopathy in the real world: results from Phase 2 of the AC-TIVE study, an Italian nationwide survey.

Eur J Heart Fail 2022 Apr 13. Epub 2022 Apr 13.

University Cardiology, A.O.U. Città della Salute e della Scienza di Torino, Turin, Italy.

Aim: To investigate the prevalence of amyloid cardiomyopathy (AC) and the diagnostic accuracy of echocardiographic red flags of AC among consecutive adult patients undergoing transthoracic echocardiogram for reason other than AC in 13 Italian institutions.

Methods And Results: This is an Italian prospective multicentre study, involving a clinical and instrumental work-up to assess AC prevalence among patients ≥55 years old with an echocardiogram suggestive of AC (i.e. at least one echocardiographic red flag of AC in hypertrophic, non-dilated left ventricles with preserved ejection fraction). The study was registered at ClinicalTrials.gov (NCT04738266). Overall, 381 patients with an echocardiogram suggestive of AC were identified among a cohort of 5315 screened subjects, and 217 patients completed the investigations. A final diagnosis of AC was made in 62 patients with an estimated prevalence of 29% (95% confidence interval 23%-35%). Transthyretin-related AC (ATTR-AC) was diagnosed in 51 and light chain-related AC (AL-AC) in 11 patients. Either apical sparing or a combination of ≥2 other echocardiographic red flags, excluding interatrial septum thickness, provided a diagnostic accuracy >70%.

Conclusion: In a cohort of consecutive adults with echocardiographic findings suggestive of AC and preserved left ventricular ejection fraction, the prevalence of AC (either ATTR or AL) was 29%. Easily available echocardiographic red flags, when combined together, demonstrated good diagnostic accuracy.
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http://dx.doi.org/10.1002/ejhf.2504DOI Listing
April 2022

Diagnosis and Management of Rare Cardiomyopathies in Adult and Paediatric Patients. A Position Paper of the Italian Society of Cardiology (SIC) and Italian Society of Paediatric Cardiology (SICP).

Int J Cardiol 2022 06 30;357:55-71. Epub 2022 Mar 30.

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Division of Cardiology, Sant'Andrea Hospital, Via di Grottarossa 1035-1039, 00189 Rome, Italy.

Cardiomyopathies (CMPs) are myocardial diseases in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality. Thought for a long time to be rare diseases, it is now clear that most of the CMPs can be easily observed in clinical practice. However, there is a group of specific heart muscle diseases that are rare in nature whose clinical/echocardiographic phenotypes resemble those of the four classical morphological subgroups of hypertrophic, dilated, restrictive, arrhythmogenic CMPs. These rare CMPs, often but not solely diagnosed in infants and paediatric patients, should be more properly labelled as specific CMPs. Emerging consensus exists that these conditions require tailored investigation and management. Indeed, an appropriate understanding of these conditions is mandatory for early treatment and counselling. At present, however, the multisystemic and heterogeneous presentation of these entities is a challenge for clinicians, and time delay in diagnosis is a significant concern. The aim of this paper is to define practical recommendations for diagnosis and management of the rare CMPs in paediatric or adult age. A modified Delphi method was adopted to grade the recommendations proposed by each member of the writing committee.
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http://dx.doi.org/10.1016/j.ijcard.2022.03.050DOI Listing
June 2022

Exercise oxygen pulse kinetics in patients with hypertrophic cardiomyopathy.

Heart 2022 Mar 10. Epub 2022 Mar 10.

Heart Failure Unit, Centro Cardiologico Monzino Istituto di Ricovero e Cura a Carattere Scientifico, Milano, Lombardia, Italy

Objectives: Reduced cardiac output (CO) has been considered crucial in symptoms' genesis in hypertrophic cardiomyopathy (HCM). Absolute value and temporal behaviour of O-pulse (oxygen uptake/heart rate (VO/HR)), and the VO/work relationship during exercise reflect closely stroke volume (SV) and CO changes, respectively. We hypothesise that adding O-pulse absolute value and kinetics, and VO/work relationship to standard cardiopulmonary exercise testing (CPET) could help identify more exercise-limited patients with HCM.

Methods: CPETs were performed in 3 HCM dedicated clinical units. We retrospectively enrolled non-end-stage consecutive patients with HCM, grouped according to left ventricle outflow tract obstruction (LVOTO) at rest or during Valsalva manoeuvre (72% of patients with LVOTO <30; 10% between 30 and 49 and 18% ≥50 mm Hg). We evaluated the CPET response in HCM focusing on parameters strongly associated with SV and CO, such as O-pulse and VO, respectively, considering their absolute values and temporal behaviour during exercise.

Results: We included 312 patients (70% males, age 49±18 years). Peak VO (percentage of predicted), O-pulse and ventilation to carbon dioxide production (VE/VCO) slope did not change across LVOTO groups. Ninety-six (31%) patients with HCM presented an abnormal O-pulse temporal behaviour, irrespective of LVOTO values. These patients showed lower peak systolic pressure, workload (106±45 vs 130±49 W), VO (21.3±6.6 vs 24.1±7.7 mL/min/kg; 74%±17% vs 80%±20%) and O-pulse (12 (9-14) vs 14 (11-17) mL/beat), with higher VE/VCO slope (28 (25-31) vs 27 (24-31)) (p<0.005 for all). Only 2 patients had an abnormal VO/work slope.

Conclusion: None of the frequently used CPET parameters, either as absolute values or dynamic relationships, were associated with LVOTO. Differently, an abnormal temporal behaviour of O-pulse during exercise, which is strongly related to inadequate SV increase, correlates with reduced functional capacity (peak and anaerobic threshold VO and workload) and increased VE/VCO slope, identifying more advanced disease irrespectively of LVOTO.
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http://dx.doi.org/10.1136/heartjnl-2021-320569DOI Listing
March 2022

Post-infarction ventricular septal rupture with a contained right ventricular pseudoaneurysm formation.

BJR Case Rep 2022 Jan 17;8(1):20210129. Epub 2021 Oct 17.

Cardiac Surgery Unit, Department of Clinical and Molecular Medicine, Sapienza University of Rome - Sant'Andrea University Hospital, Rome, Italy.

Mechanical complication of acute myocardial infarction, such as left ventricular free-wall or septal rupture, pseudo-aneurysm or true aneurysm, are uncommon but potentially fatal conditions, that require an early diagnosis and management. We describe a case of post-infarction ventricular septal rupture with pseudoaneurysm formation included in the right ventricle.
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http://dx.doi.org/10.1259/bjrcr.20210129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8803230PMC
January 2022

Patients with hypertrophic cardiomyopathy are getting older.

Int J Cardiol 2022 04 22;353:73-74. Epub 2022 Jan 22.

Centro per la Cardiomiopatia Ipertrofica, Policlinico di Monza, Monza, Italy.

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http://dx.doi.org/10.1016/j.ijcard.2022.01.031DOI Listing
April 2022

Implantable cardioverter-defibrillators for hypertrophic cardiomyopathy: The Times They Are a-Changin'.

Europace 2021 Dec 30. Epub 2021 Dec 30.

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

The implantable cardioverter-defibrillator (ICD) is a life-saving therapy in patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden cardiac death. The heterogeneity of clinical scenarios in HCM and the availability of ICDs with distinct characteristics emphasizes the need for selecting the right device for the right patient. There is growing awareness that unnecessarily complex devices can lead to short- and long-term complications without adding significant clinical benefits. Young patients have the greatest potential years of life gained from the ICD but are also most exposed to device-related complications. This increases the complexity of decision-making of ICD prescription in these often otherwise well patients in whom device selection should be tailored to preserve survival benefit without introducing morbidity. In the light of the multiple clinical phenotypes characterizing HCM, the present article offers evidence-based perspectives helpful in predicting the individual impact of the ICD and choosing the most appropriate device.
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http://dx.doi.org/10.1093/europace/euab309DOI Listing
December 2021

Prognostic implications of different clinical profiles in hypertrophic cardiomyopathy.

Minerva Cardiol Angiol 2022 Apr 29;70(2):189-206. Epub 2021 Oct 29.

Unit of Cardiology, Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology, Sant'Andrea Hospital, Sapienza University, Rome, Italy -

Hypertrophic cardiomyopathy (HCM) is a myocardial genetic disease relatively common in the general population with heterogenous clinical presentation, natural history and prognosis. About 60% of HCM patients have a stable clinical course, while others may experience a variety of HCM-related complications which follows relatively independent pathways, and that can be distinguished in different subgroups. These subgroups are represented by patients with left ventricular outflow tract obstruction; patients with end-stage disease and reduced or preserved systolic function; patients with apical hypertrophy; patients with apical aneurysm; patients with atrial fibrillation, patients at high risk of sudden death and patients with preclinical HCM. The purpose of this review was to describe each of these clinical profiles with its prognostic implications.
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http://dx.doi.org/10.23736/S2724-5683.21.05752-5DOI Listing
April 2022

Long-term management of Takotsubo syndrome: a not-so-benign condition.

Rev Cardiovasc Med 2021 Sep;22(3):597-611

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

Takotsubo syndrome (TTS) is an intriguing clinical entity, characterized by usually transient and reversible abnormalities of the left ventricular systolic function, mimicking the myocardial infarction with non-obstructive coronary arteries. TTS was initially regarded as a benign condition, however recent studies have unveiled adverse outcomes in the short- and long-term, with rates of morbidity and mortality comparable to those experienced after an acute myocardial infarction. Given the usual transient nature of TTS, this is an unexpected finding. Moreover, long-term mortality seems to be mainly driven by non-cardiovascular causes. The uncertain long-term prognosis of TTS warrants a comprehensive outpatient follow-up after the acute event, although there are currently no robust data indicating its modality and timing. The aim of the present review is to summarize recent available evidence regarding long-term prognosis in TTS. Moreover methods, timing and findings of the long-term management of TTS will be discussed.
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http://dx.doi.org/10.31083/j.rcm2203071DOI Listing
September 2021

Novel Imaging and Genetic Risk Markers in Takotsubo Syndrome.

Front Cardiovasc Med 2021 17;8:703418. Epub 2021 Aug 17.

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

Takotsubo syndrome (TTS) is an increasingly recognized condition burdened by significant acute and long-term adverse events. The availability of novel techniques expanded the knowledge on TTS and allowed a more accurate risk-stratification, potentially guiding clinical management. The present review aims to summarize the recent advances in TTS prognostic evaluation with a specific focus on novel imaging and genetic markers. Parametric deformation analysis by speckle-tracking echocardiography, as well as tissue characterization by cardiac magnetic resonance imaging T1 and T2 mapping techniques, currently appear the most clinically valuable applications. Notwithstanding, computed tomography and nuclear imaging studies provided limited but promising data. A genetic predisposition to TTS has been hypothesized, though available evidence is still not sufficient. Although a genetic predisposition appears likely, further studies are needed to fully characterize the genetic background of TTS, in order to identify genetic markers that could assist in predicting disease recurrences and help in familial screening.
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http://dx.doi.org/10.3389/fcvm.2021.703418DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8415918PMC
August 2021

Takotsubo syndrome: hyperthyroidism, pheochromocytoma, or both? A case report.

Eur Heart J Case Rep 2021 Aug 16;5(8):ytab270. Epub 2021 Aug 16.

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

Background: Takotsubo syndrome (TTS) is a transient left ventricular dysfunction usually with apical akinesia (classical pattern). Other less frequent variants have been described: the mid-ventricular pattern is characterized by hypokinesia of the mid-left ventricle and hypercontractile apical and basal segments; the inverted or basal pattern is characterized by basal and mid-ventricular segment hypokinesia or akinesia with preserved contractility or hypercontractility of apical segments and finally the focal pattern. There are also biventricular variants and forms with exclusive involvement of the right ventricle. There is a correlation between endocrine disorders and TTS, the one most frequently described is with pheochromocytoma. Catecholamine-mediated myocarditis, focal and diffuse myocardial fibrosis, and myocardial dysfunction are described in pheochromocytoma.

Case Summary: We describe a case of a 69-year-old patient with a recent diagnosis of hypertension and Graves' disease, hospitalized for persistent chest pain, hypertensive crisis, tachycardia, dyspnoea, and diaphoresis. Thyroid hormones, antibodies to TSH receptors, and hs-troponin I were increased. Electrocardiogram showed sinus tachycardia at 130 b.p.m., first-degree atrioventricular block, signs of left ventricular hypertrophy with inverted T wave in V4-V6. Echocardiogram demonstrated left ventricular apical and para-apical akinesia. Coronary angiography ruled out an obstructive coronary artery disease. Computed tomography angiogram aortic dissection ruled out aortic dissection but incidentally revealed a left adrenal mass compatible with a pheochromocytoma. Plasma and urinary metanephrines were increased. A TTS secondary to pheochromocytoma and hyperthyroidism was diagnosed. Pharmacological treatment included nitrates, urapidil and esmolol IV and methimazole at high doses. Type 2 multiple endocrine neoplasia has been excluded. After a complete haemodynamic stability on 20th day of hospitalization, the patient underwent an adrenalectomy.

Discussion: High levels of catecholamines in pheochromocytoma can lead to myocardial dysfunction. Similarly, an excess of thyroid hormones with up-regulation of adrenergic system can lead to myocardial dysfunction. These two conditions, if both present, define a high haemodynamic risk profile. How do catecholamines interact with the thyroid gland? The clinical case is of interest as a relationship has been hypothesized between the incretion of plasma catecholamines and Graves' disease. We suppose an imbalance of the immune system with a predominance of the T helper-type 2 (Th2)-mediated response. Predominance of Th2-mediated immune response may induce humoral immunity causing Graves' disease. In addition Th2 cytokines are strong inducers of M2 macrophages (alternatively activated) that are involved in autoimmune diseases, myocarditis, and myocardial fibrosis. Knowing the interaction between the cardiovascular system, immune response, and endocrine glands can help define the patient's risk class, possible complications, and follow-up.
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http://dx.doi.org/10.1093/ehjcr/ytab270DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8374971PMC
August 2021

Yield of bone scintigraphy screening for transthyretin-related cardiac amyloidosis in different conditions: Methodological issues and clinical implications.

Eur J Clin Invest 2021 Dec 22;51(12):e13665. Epub 2021 Aug 22.

Cardiology Unit, Department of Internal Medicine, University of Genoa, Genoa, Italy.

Background: Transthyretin-related cardiac amyloidosis (TTR-CA) is thought to be particularly common in specific at-risk conditions, including aortic stenosis (AS), heart failure with preserved ejection fraction (HFpEF), carpal tunnel syndrome (CTS) and left ventricular hypertrophy or hypertrophic cardiomyopathy (LVH/HCM).

Methods: We performed a systematic revision of the literature, including only prospective studies performing TTR-CA screening with bone scintigraphy in the above-mentioned conditions. Assessment of other forms of CA was also evaluated. For selected items, pooled estimates of proportions or means were obtained using a meta-analytic approach.

Results: Nine studies (3 AS, 2 HFpEF, 2 CTS and 2 LVH/HCM) accounting for 1375 screened patients were included. One hundred fifty-six (11.3%) TTR-CA patients were identified (11.4% in AS, 14.8% in HFpEF, 2.6% in CTS and 12.9% in LVH/HCM). Exclusion of other forms of CA and use of genetic testing was overall puzzled. Age at TTR-CA recognition was significantly older than that of the overall screened population in AS (86 vs. 83 years, p = .04), LVH/HCM (75 vs. 63, p < .01) and CTS (82 vs. 71), but not in HFpEF (83 vs. 79, p = .35). In terms of comorbidities, hypertension, diabetes and atrial fibrillation were highly prevalent in TTR-CA-diagnosed patients, as well as in those with an implanted pacemaker.

Conclusions: Screening with bone scintigraphy found an 11-15% TTR-CA prevalence in patients with AS, HFpEF and LVH/HCM. AS and HFpEF patients were typically older than 80 years at TTR-CA diagnosis and frequently accompanied by comorbidities. Several studies showed limitations in the application of recommended TTR-CA diagnostic algorithm, which should be addressed in future prospective studies.
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http://dx.doi.org/10.1111/eci.13665DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9286629PMC
December 2021

Current patterns of beta-blocker prescription in cardiac amyloidosis: an Italian nationwide survey.

ESC Heart Fail 2021 08 14;8(4):3369-3374. Epub 2021 May 14.

Department of Internal Medicine, University of Genova, Viale Benedetto XV, 10, Genova, 16132, Italy.

Aims: The use of beta-blocker therapy in cardiac amyloidosis (CA) is debated. We aimed at describing patterns of beta-blocker prescription through a nationwide survey.

Methods And Results: From 11 referral centres, we retrospectively collected data of CA patients with a first evaluation after 2016 (n = 642). Clinical characteristics at first and last evaluation were collected, with a focus on medical therapy. For patients in whom beta-blocker therapy was started, stopped, or continued between first and last evaluation, the main reason for beta-blocker management was requested. Median age of study population was 77 years; 81% were men. Arterial hypertension was found in 58% of patients, atrial fibrillation (AF) in 57%, and coronary artery disease in 16%. Left ventricular ejection fraction was preserved in 62% of cases, and 74% of patients had advanced diastolic dysfunction. Out of the 250 CA patients on beta-blockers at last evaluation, 215 (33%) were already taking this therapy at first evaluation, while 35 (5%) were started it, in both cases primarily because of high-rate AF. One-hundred-nineteen patients (19%) who were on beta-blocker at first evaluation had this therapy withdrawn, mainly because of intolerance in the presence of heart failure with advanced diastolic dysfunction. The remaining 273 patients (43%) had never received beta-blocker therapy. Beta-blockers usage was similar between CA aetiologies. Patients taking vs. not taking beta-blockers differed only for a greater prevalence of arterial hypertension, coronary artery disease, AF, and non-restrictive filling pattern (P < 0.01 for all) in the former group.

Conclusions: Beta-blockers prescription is not infrequent in CA. Such therapy may be tolerated in the presence of co-morbidities for which beta-blockers are routinely used and in the absence of advanced diastolic dysfunction.
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http://dx.doi.org/10.1002/ehf2.13411DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8318433PMC
August 2021

Left Ventricular Remodeling in Hypertrophic Cardiomyopathy: An Overview of Current Knowledge.

J Clin Med 2021 Apr 7;10(8). Epub 2021 Apr 7.

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

While most patients with hypertrophic cardiomyopathy (HCM) show a relatively stable morphologic and clinical phenotype, in some others, progressive changes in the left ventricular (LV) wall thickness, cavity size, and function, defined, overall, as "LV remodeling", may occur. The interplay of multiple pathophysiologic mechanisms, from genetic background to myocardial ischemia and fibrosis, is implicated in this process. Different patterns of LV remodeling have been recognized and are associated with a specific impact on the clinical course and management of the disease. These findings underline the need for and the importance of serial multimodal clinical and instrumental evaluations to identify and further characterize the LV remodeling phenomenon. A more complete definition of the stages of the disease may present a chance to improve the management of HCM patients.
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http://dx.doi.org/10.3390/jcm10081547DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8067545PMC
April 2021

The Many Faces of Arterial Hypertension in Hypertrophic Cardiomyopathy and Its Phenocopies: Bystander, Consequence, Modifier.

High Blood Press Cardiovasc Prev 2021 Jul 27;28(4):327-329. Epub 2021 Apr 27.

Division of Cardiology, Sapienza University of Rome, Sant'Andrea Hospital, Via di Grottarossa, 1035-1039, 00189, Rome, Italy.

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http://dx.doi.org/10.1007/s40292-021-00458-6DOI Listing
July 2021

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

Biomolecules 2021 03 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

TNNI3 and KCNQ1 co-inherited variants in a family with hypertrophic cardiomyopathy and long QT phenotypes: A case report.

Mol Genet Metab Rep 2021 Jun 18;27:100743. Epub 2021 Mar 18.

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

QTc prolongation is reported in patients with hypertrophic cardiomyopathy (HCM). However, the causes of the QTc interval increase remain unclear. The main contribution to QTc prolongation in HCM is attributed to the myocardial hypertrophy and related structural damage. In a 24-year-old male proband, affected by HCM and long QTc, we identified by Next Generation Sequencing a pathogenic variant in gene co-inherited with a damaging variant in gene. This evidence suggests the possibility that QTc interval prolongation and its dispersion in HCM could be associated not only to the severity of left ventricular hypertrophy but also to the co-inheritance of pathogenic variants related to both long QT Syndrome (LQTS) and HCM. Although the simultaneous presence of pathogenic variants in genes related to different heart diseases is extremely rare, counseling and genetic testing appear crucial for the clinical diagnosis. Screening of LQTS genes should be considered in HCM patients to clarify the origin of long QTc, to provide more information about the clinical presentation and to evaluate the incidence of the co-existence of LQTS/HCM gene variants that could occur more frequently than so far reported.
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http://dx.doi.org/10.1016/j.ymgmr.2021.100743DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7985525PMC
June 2021

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 06 26;333:54. Epub 2021 Feb 26.

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
June 2021

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

Int J Cardiol 2021 04 24;329:23-27. 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
April 2021

[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 Cardiol Angiol 2022 08 30;70(4):484-490. 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 preparticipation screening (PPS); moreover, these criteria have been evaluated in different populations of athletes and settings proving good results.

Evidence Acquisition: We aimed 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/S2724-5683.20.05347-5DOI Listing
August 2022
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