Publications by authors named "Martina Perazzolo Marra"

121 Publications

CarDiac magnEtic Resonance for prophylactic Implantable-cardioVerter defibrillAtor ThErapy in Non-Ischaemic dilated CardioMyopathy: an international Registry.

Europace 2021 Apr 1. Epub 2021 Apr 1.

Department of Cardiovascular, Centro Cardiologico Monzino, IRCCS, Via C. Parea 4, 20138 Milan, Italy.

Aims: The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients.

Methods And Results: In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117-1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211-3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231-3.690, P = 0.007; HR: 3.161, 95% CI: 1.750-5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084-2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort.

Conclusion: In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation.
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http://dx.doi.org/10.1093/europace/euaa401DOI Listing
April 2021

Chronic thromboembolic pulmonary hypertension risk score evaluation and validation (CTEPH Solution): proposal of a study protocol aimed to realize a validated risk score for early diagnosis.

Minerva Cardiol Angiol 2021 Mar 11. Epub 2021 Mar 11.

Department of Cardiovascular and Respiratory Sciences, Sapienza University of Rome, Rome, Italy -

Background: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is the most serious long-term complication of acute pulmonary embolism (PE) though it is the only potentially reversible form of Pulmonary Hypertension (PH). Its incidence is mainly limited to the first 2 years following the embolic event, however it is often underdiagnosed or misdiagnosed.

Methods: This is a multicenter observational cross-sectional and prospective study. Patients with a prior diagnosis of PE will be enrolled and undergo baseline evaluation for prevalent PH detection through a clinical examination and an echocardiogram as first screening exam. All cases of intermediate-high echocardiographic probability of PH will be confirmed by right heart catheterization and then identified as CTEPH through appropriate imaging and functional examinations in order to exclude other causes of PH. A CTEPH Risk Score will be created using retrospective data from this prevalent cohort of patients and will be then validated on an incident cohort of patients with acute PE.

Results: 1000 retrospective and 218 prospective patients are expected to be enrolled and the study is expected to be completed by the end of 2021. Up to now 841 patients (620 retrospective and 221 prospective) have been enrolled.

Conclusions: This study is the first large prospective study for the prediction of CTEPH development in patients with PE. It aims to create a comprehensive scoring tool that includes echocardiographic data which may allow early detection of CTEPH and the application of targeted follow up screening programs in patients with PE.
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http://dx.doi.org/10.23736/S2724-5683.21.05575-7DOI Listing
March 2021

Small Vessel Disease: Another Component of the Hypertrophic Cardiomyopathy Phenotype Not Necessarily Associated with Fibrosis.

J Clin Med 2021 Feb 4;10(4). Epub 2021 Feb 4.

Cardiovascular Pathology Unit and Cardiology Unit, Azienda Ospedaliera, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua Medical School, Via A. Gabelli, 61, 35121 Padova, Italy.

Background: Hypertrophic cardiomyopathy (HCM) is characterized by myocardial disarray, small vessel disease (SVD), and fibrosis. The relationship between SVD and replacement-type fibrosis is still unclear.

Methods: Histopathologic assessment of replacement-type fibrosis and SVD in HCM patients with either end-stage heart failure (HF) or sudden cardiac death (SCD). Chronic ischemic heart disease (IHD) patients served as controls.

Results: Forty HCM hearts, 10 HF and 30 SCD, were studied. Replacement-type fibrosis was detected in all HF and in 57% of SCD cases. In SCD, replacement-type fibrosis was associated with older age, greater septal thickness, SVD prevalence, and score (all < 0.05). Prevalence of SVD did not show significant differences among SCD, HF, and IHD (73%, 100% and 95%, respectively), while SVD score was higher in HF than IHD and SCD (2.4, 1.95, and 1.18, respectively) and in areas with replacement-type fibrosis vs. those without in HF (3.4 vs. 1.4) and SCD (1.4 vs. 0.8) (all < 0.05).

Conclusions: SVD is a frequent feature in HCM independent of the clinical presentation. A higher SVD score is observed in HCM-HF and in areas with replacement-type fibrosis. Although SVD is part of the HCM phenotype, further remodeling of the microcirculation might occur secondarily to fibrosis.
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http://dx.doi.org/10.3390/jcm10040575DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7913811PMC
February 2021

The alcohol-induced cardiomyopathy: A cardiovascular magnetic resonance characterization.

Int J Cardiol 2021 May 2;331:131-137. Epub 2021 Feb 2.

Department of Cardiology, Guys and St Thomas' NHS Trust, London, United Kingdom; Department of Cardiovascular Sciences, - Faculty of Life Sciences & Medicine, King's College London, United Kingdom.

Background: Alcoholic cardiomyopathy(ACM) is part of the non-ischaemic dilated cardiomyopathy(NI-DCM) spectrum. Little is known about cardiovascular magnetic resonance(CMR) features in ACM patients. The aim of this study is to describe CMR findings and their prognostic impact in ACM patients.

Methods: Consecutive ACM patients evaluated in five referral CMR centres from January 2005 to December 2018 were enrolled. CMR findings and their prognostic value were compared to idiopathic NI-DCM(iNI-DCM) patients. The main outcome was a composite of death/heart transplantation/life-threatening arrhythmias.

Results: Overall 114 patients (52 with ACM and 62 with iNI-DCM) were included. ACM patients were more often males compared to iNI-DCM (90% vs 64%, respectively, p ≤ 0.001) and were characterized by a more pronounced biventricular adverse remodelling than iNI-DCM, i.e. lower LVEF (31 ± 12% vs 38 ± 11% respectively, p = 0.001) and larger left ventricular end-diastolic volume (116 ± 40 ml/m vs 67 ± 20 ml/m respectively, p < 0.001). Similarly to iNI-DCM, late gadolinium enhancement (LGE), mainly midwall, was present in more than 40% of ACM patients but, conversely, it was not associated with adverse outcome(p = 0.15). LGE localization was prevalently septal (87%) in ACM vs lateral in iNI-DCM(p < 0.05). Over a median follow-up of 42 months [Interquartile Range 24-68], adverse outcomes were similar in both groups(p = 0.67).

Conclusions: ACM represents a specific phenotype of NI-DCM, with severe morpho-functional features at the onset, but similar long-term outcomes compared to iNI-DCM. Despite the presence and pattern of distribution of LGE was comparable, ACM and iNI-DCM showed a different LGE localization, mostly septal in ACM and lateral in iNI-DCM, with different prognostic impact.
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http://dx.doi.org/10.1016/j.ijcard.2021.01.067DOI Listing
May 2021

Papillary Muscles Abnormalities in Athletes With Otherwise Unexplained T-Wave Inversion in the ECG Lateral Leads.

J Am Heart Assoc 2021 Feb 26;10(3):e019239. Epub 2021 Jan 26.

Department of Cardiac, Thoracic and Vascular Sciences and Public Health University of Padova Italy.

Background Papillary muscles (PMs) abnormalities may be associated with ECG repolarization abnormalities. We aimed to evaluate the relation between lateral T-wave inversion (TWI) and PMs characteristics in a cohort of athletes with no clinically demonstrable cardiac disease. Methods and Results We included 53 athletes (median age, 20 years; 87% men) with lateral TWI and no evidence of heart disease on clinical and cardiac magnetic resonance evaluation. A group of healthy athletes with normal ECG served as controls. We evaluated the PMs dimensions, such as diameters, area, volume, mass, and ratio between PMs and left ventricular mass, and the prevalence of PMs apical displacement. Compared with controls, athletes with TWI showed PMs hypertrophy with significantly increased PMs diameters, area, volume, and mass. The ratio between PMs and left ventricular mass was 4.4% in athletes with TWI and 3.0% in controls (<0.001). A PMs/left ventricular mass ratio >3.5% showed 85% sensitivity and 76% specificity for differentiating between athletes with TWI and controls. Apical displacement of PMs was found in 25 (47%) athletes with TWI versus 9 (17%) controls (=0.001). At multivariable analysis, PMs/left ventricular mass ratio and apical displacement remained independent predictors of TWI. Clinical outcome of the athletes with TWI and PMs abnormalities was uneventful despite continuation of their sports activity. Conclusions PMs hypertrophy and apical displacement may underlie otherwise unexplained lateral TWI in the athlete. Lateral TWI associated with PMs abnormalities appears as a distinct anatomo-clinical condition characterized by a favorable outcome.
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http://dx.doi.org/10.1161/JAHA.120.019239DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955426PMC
February 2021

The missing pieces in the puzzle of arrhythmic mitral valve prolapse: Papillary muscles, mitral annulus dysjunction, and myocardial scarring.

Heart Rhythm 2021 Apr 8;18(4):577-578. Epub 2021 Jan 8.

Cardiovascular Pathology, Azienda Ospedaliera, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua Medical School, Padova, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.hrthm.2021.01.004DOI Listing
April 2021

Predictors of Left Ventricular Scar Using Cardiac Magnetic Resonance in Athletes With Apparently Idiopathic Ventricular Arrhythmias.

J Am Heart Assoc 2021 Jan 31;10(1):e018206. Epub 2020 Dec 31.

Department of Cardiac, Thoracic and Vascular Sciences and Public Health University of Padova Italy.

Background In athletes with ventricular arrhythmias (VA) and otherwise unremarkable clinical findings, cardiac magnetic resonance (CMR) may reveal concealed pathological substrates. The aim of this multicenter study was to evaluate which VA characteristics predicted CMR abnormalities. Methods and Results We enrolled 251 consecutive competitive athletes (74% males, median age 25 [17-39] years) who underwent CMR for evaluation of VA. We included athletes with >100 premature ventricular beats/24 h or ≥1 repetitive VA (couplets, triplets, or nonsustained ventricular tachycardia) on 12-lead 24-hour ambulatory ECG monitoring and negative family history, ECG, and echocardiogram. Features of VA that were evaluated included number, morphology, repetitivity, and response to exercise testing. Left-ventricular late gadolinium-enhancement was documented by CMR in 28 (11%) athletes, mostly (n=25) with a subepicardial/midmyocardial stria pattern. On 24-hour ECG monitoring, premature ventricular beats with multiple morphologies or with right-bundle-branch-block and intermediate/superior axis configuration were documented in 25 (89%) athletes with versus 58 (26%) without late gadolinium-enhancement (<0.001). More than 3300 premature ventricular beats were recorded in 4 (14%) athletes with versus 117 (53%) without positive CMR (<0.001). At exercise testing, nonsustained ventricular tachycardia occurred at peak of exercise in 8 (29%) athletes with late gadolinium-enhancement (polymorphic in 6/8, 75%) versus 17 athletes (8%) without late gadolinium-enhancement (=0.002), (<0.0001). At multivariable analysis, all 3 parameters independently correlated with CMR abnormalities. Conclusions In athletes with apparently idiopathic VA, simple characteristics such as number and morphology of premature ventricular beats on 12-lead 24-hour ambulatory ECG monitoring and response to exercise testing predicted the presence of concealed myocardial abnormalities on CMR. These findings may help cost-effective CMR prescription.
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http://dx.doi.org/10.1161/JAHA.120.018206DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955495PMC
January 2021

'Hot phase' clinical presentation in arrhythmogenic cardiomyopathy.

Europace 2020 Dec 13. Epub 2020 Dec 13.

Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Via Giustiniani, 2, 35121 Padua, Italy.

Aims: The aim of this study is to evaluate the clinical features of patients affected by arrhythmogenic cardiomyopathy (AC), presenting with chest pain and myocardial enzyme release in the setting of normal coronary arteries ('hot phase').

Methods And Results: We collected detailed anamnestic, clinical, instrumental, genetic, and histopathological findings as well as follow-up data in a series of AC patients who experienced a hot phase. A total of 23 subjects (12 males, mean age at the first episode 27 ± 16 years) were identified among 560 AC probands and family members (5%). At first episode, 10 patients (43%) already fulfilled AC diagnostic criteria. Twelve-lead electrocardiogram recorded during symptoms showed ST-segment elevation in 11 patients (48%). Endomyocardial biopsy was performed in 11 patients, 8 of them during the acute phase showing histologic evidence of virus-negative myocarditis in 88%. Cardiac magnetic resonance was performed in 21 patients, 12 of them during the acute phase; oedema and/or hyperaemia were detected in 7 (58%) and late gadolinium enhancement in 11 (92%). At the end of follow-up (mean 17 years, range 1-32), 12 additional patients achieved an AC diagnosis. Genetic testing was positive in 77% of cases and pathogenic mutations in desmoplakin gene were the most frequent. No patient complained of sustained ventricular arrhythmias or died suddenly during the 'hot phase'.

Conclusion: 'Hot phase' represents an uncommon clinical presentation of AC, which often occurs in paediatric patients and carriers of desmoplakin gene mutations. Tissue characterization, family history, and genetic test represent fundamental diagnostic tools for differential diagnosis.
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http://dx.doi.org/10.1093/europace/euaa343DOI 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

Arrhythmogenic Cardiomyopathy.

Eur Heart J 2020 12;41(47):4457-4462

Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua Medical School, Padua, Italy.

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http://dx.doi.org/10.1093/eurheartj/ehaa719DOI Listing
December 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

Differential diagnosis of arrhythmogenic cardiomyopathy: phenocopies versus disease variants.

Minerva Med 2021 Apr 22;112(2):269-280. Epub 2020 Jul 22.

Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Padua, Italy -

Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease caused by mutations of desmosomal genes in about 50% of patients. Affected patients may have defective non-desmosomal genes. The ACM phenotype may occur in other genetic cardiomyopathies, cardio-cutaneous syndromes or neuromuscular disorders. A sizeable proportion of patients have non-genetic diseases with clinical features resembling ACM (phenocopies). The identification of biventricular and left-dominant phenotypic variants has made differential diagnosis more difficult because of the broader spectrum of phenocopies which requires a detailed clinical study with appropriate evaluation of most prominent and discriminatory disease features. Conditions that enter into differential diagnosis of ACM include heart muscle diseases affecting the right ventricle, the left ventricle, or both. To confirm a conclusive diagnosis of ACM, these differential possibilities need to be reasonably excluded by an accurate and targeted clinical evaluation. This article reviews the clinical and imaging features of major phenocopies of ACM and provides indications for differential diagnosis. The recent etiologic classification of Arrhythmogenic Cardiomyopathies, whose common denominator is the distinctive phenotype characterized by a hypokinetic and non-dilated ventricle with a large amount of myocardial fibrosis underlying its propensity to generate ventricular arrhythmias is also addressed.
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http://dx.doi.org/10.23736/S0026-4806.20.06782-8DOI Listing
April 2021

Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria.

Int J Cardiol 2020 11 16;319:106-114. Epub 2020 Jun 16.

Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, Italy.

The original designation of "Arrhythmogenic right ventricular (dysplasia/) cardiomyopathy"(ARVC) was used by the scientists who first discovered the disease, in the pre-genetic and pre-cardiac magnetic resonance era, to describe a new heart muscle disease predominantly affecting the right ventricle, whose cardinal clinical manifestation was the occurrence of malignant ventricular arrhythmias. Subsequently, autopsy investigations, genotype-phenotype correlations studies and the increasing use of contrast-enhancement cardiac magnetic resonance showed that the fibro-fatty replacement of the myocardium represents the distinctive phenotypic feature of the disease that affects the myocardium of both ventricles, with left ventricular involvement which may parallel or exceed the severity of right ventricular involvement. This has led to the new designation of "Arrhythmogenic Cardiomyopathy" (ACM), that represents the evolution of the original term of ARVC. The present International Expert Consensus document proposes an upgrade of the criteria for diagnosis of the entire spectrum of the phenotypic variants of ACM. The proposed "Padua criteria" derive from the diagnostic approach to ACM, which has been developed over 30 years by the multidisciplinary team of basic researchers and clinical cardiologists of the Medical School of the University of Padua. The Padua criteria are a working framework to improve the diagnosis of ACM by introducing new diagnostic criteria regarding tissue characterization findings by contrast-enhanced cardiac magnetic resonance, depolarization/repolarization ECG abnormalities and ventricular arrhythmia features for diagnosis of the left ventricular phenotype. The proposed diagnostic criteria need to be further validated by future clinical studies in large cohorts of patients.
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http://dx.doi.org/10.1016/j.ijcard.2020.06.005DOI Listing
November 2020

Congenital Pericardial Agenesis in Asymptomatic Individuals: Tips for the Diagnosis.

Circ Cardiovasc Imaging 2020 05 6;13(5):e010169. Epub 2020 May 6.

Sant'Antonio Civil Hospital, Padua, Italy (A.B).

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http://dx.doi.org/10.1161/CIRCIMAGING.119.010169DOI Listing
May 2020

Arrhythmogenic Right Ventricular Cardiomyopathy: Characterization of Left Ventricular Phenotype and Differential Diagnosis With Dilated Cardiomyopathy.

J Am Heart Assoc 2020 03 2;9(5):e014628. Epub 2020 Mar 2.

Department of Cardio-Thoraco-Vascular Sciences and Public Health University of Padua Italy.

Background This study assessed the prevalence of left ventricular (LV) involvement and characterized the clinical, electrocardiographic, and imaging features of LV phenotype in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Differential diagnosis between ARVC-LV phenotype and dilated cardiomyopathy (DCM) was evaluated. Methods and Results The study population included 87 ARVC patients (median age 34 years) and 153 DCM patients (median age 51 years). All underwent cardiac magnetic resonance with quantitative tissue characterization. Fifty-eight ARVC patients (67%) had LV involvement, with both LV systolic dysfunction and LV late gadolinium enhancement (LGE) in 41/58 (71%) and LV-LGE in isolation in 17 (29%). Compared with DCM, the ARVC-LV phenotype was statistically significantly more often characterized by low QRS voltages in limb leads, T-wave inversion in the inferolateral leads and major ventricular arrhythmias. LV-LGE was found in all ARVC patients with LV systolic dysfunction and in 69/153 (45%) of DCM patients. Patients with ARVC and LV systolic dysfunction had a greater amount of LV-LGE (25% versus 13% of LV mass; <0.01), mostly localized in the subepicardial LV wall layers. An LV-LGE ≥20% had a 100% specificity for diagnosis of ARVC-LV phenotype. An inverse correlation between LV ejection fraction and LV-LGE extent was found in the ARVC-LV phenotype (=-0.63; <0.01), but not in DCM (=-0.01; =0.94). Conclusions LV involvement in ARVC is common and characterized by clinical and cardiac magnetic resonance features which differ from those seen in DCM. The most distinctive feature of ARVC-LV phenotype is the large amount of LV-LGE/fibrosis, which impacts directly and negatively on the LV systolic function.
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http://dx.doi.org/10.1161/JAHA.119.014628DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335583PMC
March 2020

CMR for myocardial iron overload quantification: calibration curve from the MIOT Network.

Eur Radiol 2020 Jun 12;30(6):3217-3225. Epub 2020 Feb 12.

MRI Unit, Fondazione G. Monasterio CNR-Regione Toscana, Area della Ricerca S. Cataldo, Via Moruzzi, 1, 56124, Pisa, Italy.

Objectives: R2* cardiac magnetic resonance (CMR) allows the non-invasive measurement of myocardial iron. We calibrated cardiac R2* values against myocardial tissue-measured iron concentration by using a segmental approach and we assessed the iron distribution.

Methods: Five hearts of thalassemia patients were donated after death/transplantation to the CoreLab of the Myocardial Iron Overload in Thalassemia Network. A multislice multiecho R2* approach was adopted. After CMR, used as guidance, the heart was cut in three short-axis slices and each slice was cut into different equiangular segments according to AHA segmentation and differentiated into endocardial and epicardial layers. Tissue iron concentration was measured by atomic absorption spectrometer technique.

Results: Fifty-five samples were used since only for two hearts all the 16 samples were analyzed. Mean iron concentration was 4.71 ± 4.67 mg/g dw. Segmental iron levels ranged from 0.24 to 13.78 mg/g dw. The coefficient of variability of iron for myocardial segments ranged from 8.08 to 24.54% (mean 13.49 ± 6.93%). Iron concentration was significantly higher in the epicardial than in the endocardial layer (5.99 ± 6.01 vs 4.84 ± 4.87 mg/g dw; p = 0.042). Four different circumferential regions (anterior, septal, inferior, and lateral) were defined. A circumferential heterogeneity was noted, with more iron in the anterior region, followed by the inferior region. The direct nonlinear fitting of R2* and [Fe] data led to the calibration curve: [Fe] = 0.0022 ∙ (R2*-ROI) (R-square = 0.956).

Conclusions: Our data further validate R2* CMR using a segmental approach as a sensitive and early technique for quantifying iron distribution in the current clinical practice.

Key Points: • Calibration in humans for cardiovascular magnetic resonance R2* against myocardial iron concentration was provided. • A circumferential heterogeneity in cardiac iron distribution was detected: more iron was observed in the anterior region, followed by the inferior region. This finding corroborates the use of a segmental T2* CMR approach in the clinical practice to detect a heterogeneous iron distribution. • The comparison between the cardiac T2* values obtained with the region-based and the pixel-wise approaches showed a significant correlation and no significant difference but, in presence of significant iron load, the region-based approach resulted in significantly higher T2* values.
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http://dx.doi.org/10.1007/s00330-020-06668-1DOI Listing
June 2020

Right ventricular dilatation in arrhythmogenic right ventricular cardiomyopathy: need for a revision of the 2010 International Task Force criteria.

Eur Heart J 2020 04;41(14):1452-1453

Cardiac Magnetic Resonance Unit, Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padova, Via N. Giustiniani 2, 35128 Padova, Italy.

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http://dx.doi.org/10.1093/eurheartj/ehaa003DOI Listing
April 2020

Screening young athletes for diseases at risk of sudden cardiac death: role of stress testing for ventricular arrhythmias.

Eur J Prev Cardiol 2020 02 2;27(3):311-320. Epub 2019 Dec 2.

Center for Sports Medicine, ULSS2 Marca Trevigiana, Treviso, Italy.

Aims: The athletic preparticipation evaluation (PPE) protocol proposed by he European Society of Cardiology includes history, physical examination and resting electrocardiogram (ECG). The aim of this study was to assess the results of adding constant-load ECG stress testing (EST) to the protocol for the evaluation of ventricular arrhythmias (VA) inducibility.

Methods: We evaluated a consecutive cohort of young athletes with history, physical examination, resting ECG and EST. Athletes with VA induced by EST underwent 24-hour 12-lead Holter monitoring and echocardiography. Cardiac magnetic resonance (CMR) was reserved for those with frequent, repetitive or exercise-worsened VA, and for athletes with echocardiographic abnormalities.

Results: Of 10,985 athletes (median age 15 years, 66% males), 451 (4.1%) had an abnormal history, physical examination or resting ECG and 31 (0.28%) were diagnosed with a cardiac disease and were at risk of sudden cardiac death. Among the remaining 10,534 athletes, VA at EST occurred in 524 (5.0%) and a previously missed at-risk condition was identified in 23 (0.22%); the most common ( = 10) was an echocardiographically silent non-ischaemic left-ventricular fibrosis evidenced by CMR. The addition of EST increased the diagnostic yield of PPE by 75% (from 0.28% to 0.49%) and decreased the positive predictive value by 20% (from 6.9% to 5.5%). During a 32 ± 21 months follow-up, no cardiac arrests occurred among either eligible athletes or non-eligible athletes with cardiovascular disease.

Conclusions: The addition of exercise testing for the evaluation of VA inducibility to history, physical examination and ECG resulted in an increase of the diagnostic yield of PPE at the expense of an increase in false-positive findings.
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http://dx.doi.org/10.1177/2047487319890973DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7008549PMC
February 2020

Multiple mycotic aneurysms of the aortic root after aortic valve replacement.

Cardiovasc Pathol 2020 Jan - Feb;44:107152. Epub 2019 Oct 24.

Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padova, Italy.

A 74-year-old woman with severe aortic valve stenosis underwent aortic valve replacement with a pericardial bioprosthesis. Histological analysis of the excised valve showed dystrophic calcification associated with signs of healed infective endocarditis. Two months later, the patient died due to congestive heart failure. Autopsy revealed a bowel infarction and the presence of multiple mycotic aneurysms of the aortic root with mural thrombosis, which were unnoticed during the patient hospital stay and surgical window.
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http://dx.doi.org/10.1016/j.carpath.2019.107152DOI Listing
March 2020

Prognostic Value of Repeating Cardiac Magnetic Resonance in Patients With Acute Myocarditis.

J Am Coll Cardiol 2019 11;74(20):2439-2448

Clinical and Experimental Department of Medicine, University of Messina, Messina, Italy.

Background: Cardiac magnetic resonance (CMR) is widely used to confirm the diagnosis of acute myocarditis (AM) in the acute setting. CMR is often repeated after 6 months to assess the evolution of myocardial involvement. However, the clinical and prognostic role of 6-month CMR is unknown.

Objectives: This multicenter study aimed to evaluate the clinical and prognostic role of 6-month repetition of CMR in patients with AM.

Methods: In a subgroup of 187 patients from the ITAMY (ITAlian study in MYocarditis) registry, CMR was performed within the first week after symptom onset (CMR-I) and repeated after 6 months (CMR-II).

Results: Myocardial edema was detected in all the patients at CMR-I and persisted in 31 (16%) at CMR-II. LGE was detected in 182 (96%) patients at CMR-I and in 164 (86%) at CMR-II. At CMR-II, 20 (11%) patients presented a complete recovery from edema and LGE, 30 (16%) patients had edema with LGE, and 137 (73%) presented LGE without edema. LGE disappeared completely in 18 (10%) patients, the number of LGE segments decreased in 87 (46%), unchanged in 58 (31%), and increased in 26 (14%). During a median clinical follow-up of 7 years (25th to 75th percentile: 6 to 8 years) cardiac events occurred in 22 patients. At Kaplan-Meier curves, patients with LGE and without edema had worse prognosis than others (p < 0.0001). Patients with increased extent of LGE (p = 0.02) had a worse prognosis than those with decreased/unchanged LGE. At multivariate Cox regression analysis, the midwall septal pattern of LGE and the presence of LGE without edema at CMR-II were independent predictors of a cardiac event.

Conclusions: In the acute setting, LGE does not mean definite fibrosis, and it may disappear at 6 months. The presence of LGE without edema at 6-month CMR is associated with worse prognosis, particularly when distributed with a midwall septal pattern. LGE without edema could represent definite fibrosis whereas the presence of edema suggests a residual chance of recovery.
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http://dx.doi.org/10.1016/j.jacc.2019.08.1061DOI Listing
November 2019

Mitral Valve Prolapse, Ventricular Arrhythmias, and Sudden Death.

Circulation 2019 09 9;140(11):952-964. Epub 2019 Sep 9.

Clinical Cardiology Unit (S.I., M.P.M.), Azienda Ospedaliera; and Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua Medical School, Padova, Italy.

Despite a 2% to 3% prevalence of echocardiographically defined mitral valve prolapse (MVP) in the general population, the actual burden, risk stratification, and treatment of the so-called arrhythmic MVP are unknown. The clinical profile is characterized by a patient, usually female, with mostly bileaflet myxomatous disease, mid-systolic click, repolarization abnormalities in the inferior leads, and complex ventricular arrhythmias with polymorphic/right bundle branch block morphology, without significant regurgitation. Among the various pathophysiologic mechanisms of electrical instability, left ventricular fibrosis in the papillary muscles and inferobasal wall, mitral annulus disjunction, and systolic curling have been recently described by pathological and cardiac magnetic resonance studies in sudden death victims and patients with arrhythmic MVP. In addition, premature ventricular beats arising from the Purkinje tissue as ventricular fibrillation triggers have been documented by electrophysiologic studies in MVP patients with aborted sudden death. The genesis of malignant ventricular arrhythmias in MVP probably recognizes the combination of the substrate (regional myocardial hypertrophy and fibrosis, Purkinje fibers) and the trigger (mechanical stretch) eliciting premature ventricular beats because of a primary morphofunctional abnormality of the mitral valve annulus. The main clinical challenge is how to identify patients with arrhythmic MVP (which imaging technique and in which patient) and how to treat them to prevent sudden death. Thus, there is a necessity for prospective multicenter studies focusing on the prognostic role of cardiac magnetic resonance and electrophysiologic studies and on the therapeutic efficacy of targeted catheter ablation and mitral valve surgery in reducing the risk of life-threatening arrhythmias, as well as the role of implantable cardioverter defibrillators for primary prevention.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.034075DOI Listing
September 2019

Coronary flow reserve is related to the extension and transmurality of myocardial necrosis and predicts functional recovery after acute myocardial infarction.

Echocardiography 2019 05 19;36(5):844-853. Epub 2019 Apr 19.

Clinical Cardiology, Department of Medical Science and Public Health, University of Cagliari, Cagliari, Italy.

Background: Few studies have examined the effect of transmurality of myocardial necrosis on coronary microcirculation. The aim of this study was to examine the influence of cardiac magnetic resonance-derived (GE-MRI) structural determinants of coronary flow reserve (CFR) after anterior myocardial infarction (STEMI), and their predictive value on regional functional recovery.

Methods: Noninvasive CFR and GE-MRI were studied in 37 anterior STEMI patients after primary coronary angioplasty. The wall motion score index in the left descending anterior coronary artery territory (A-WMSI) was calculated at admission and follow-up (FU). Recovery of regional left ventricular (LV) function was defined as the difference in A-WMSI at admission and FU. The necrosis score index (NSI) and transmurality score index (TSI) by GE-MRI were calculated in the risk area. Baseline (BMR) and hyperemic (HMR) microvascular resistance, arteriolar resistance index (ARI), and coronary resistance reserve (CRR) were calculated at the Doppler echocardiography.

Results: Bivariate analysis indicated that the CPK and troponin I peak, heart rate, NSI, TSI, BMR, the ARI, and CRR were related to CFR. Multivariable analysis revealed that TSI was the only independent determinant of CFR. The CFR value of >2.27, identified as optimal by ROC analysis, was 77% specific and 73% sensitive with accuracy of 76% in identifying patients with functional recovery.

Conclusions: Preservation of microvascular function after AMI is related to the extent of transmurality of myocardial necrosis, is an important factor influencing regional LV recovery, and can be monitored by noninvasive CFR.
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http://dx.doi.org/10.1111/echo.14337DOI Listing
May 2019

Mechanical dispersion and arrhythmic mitral valve prolapse: substrate and trigger in electrical instability.

Heart 2019 07 10;105(14):1053-1054. Epub 2019 Apr 10.

Cardiovascular Pathology Unit, Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua Medical School, Padova, Italy.

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http://dx.doi.org/10.1136/heartjnl-2019-314788DOI Listing
July 2019

How to improve therapy in myocarditis: role of cardiovascular magnetic resonance and of endomyocardial biopsy.

Eur Heart J Suppl 2019 Mar 29;21(Suppl B):B19-B22. Epub 2019 Mar 29.

Cardiology, Department of Cardiology Thoracic and Vascular Sciences and Public Health, University of Padua, Italy.

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http://dx.doi.org/10.1093/eurheartj/suz014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439898PMC
March 2019