Publications by authors named "Andrea Barison"

133 Publications

Myocardial T1 Values at 1.5 T: Normal Values for General Electric Scanners and Sex-Related Differences.

J Magn Reson Imaging 2021 Apr 13. Epub 2021 Apr 13.

Magnetic Resonance Imaging Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.

Background: No data are available about normal ranges for native T1 in human myocardium using General Electric (GE) scanners.

Purpose: To establish normal ranges for myocardial T1 values and evaluate regional variability and the influence of physiological factors.

Study Type: Prospective.

Subjects: One hundred healthy volunteers with normal electrocardiogram, no cardiovascular/systemic diseases, or risk factors (age range: 20-70 years; 50 females).

Field Strength/sequence: 1.5 T/Steady-state free precession cine and a modified Look-Locker inversion recovery sequence in diastole (also in systole for 61 volunteers).

Assessment: Image analysis was performed by operators with >10 years experience in cardiac MR using commercially available software. T1 values were calculated for 16 myocardial segments, and the global value was the mean. Segments were grouped according to circumferential region (anterior, septal, inferior, and lateral) and to level (basal, medial, apical). Twenty images were analyzed twice by the same operator and by a different operator to assess reproducibility.

Statistical Tests: Independent-samples t-test or Mann-Whitney test; paired sample t-test or Wilcoxon signed-rank test; one-way repeated measures ANOVA or Friedman tests; Pearson's or Spearman's correlation. Reproducibility evaluated using coefficient of variability (CoV).

Results: Due to artifacts and/or partial-volume effects, 45/1600 (2.8%) segments were excluded. A good intra- and inter-operator reproducibility was detected (CoV < 5%). There were significant differences in segmental T1 values (P < 0.05). A significant circumferential variability was present (P < 0.05): the mean native T1 value over the lateral region was significantly lower than in the other three regions. An increasing gradient from basal to apical slices was detected (P < 0.05). Segmental and global T1 values were not associated with age (range P = 0.052-0.911) but were significantly lower in males than in females (global: 993 ± 32 vs. 1037 ± 27 ms; P < 0.05) and significantly correlated with heart rate (range R for segmental values = 0.247-0.920; P < 0.05). Almost all segmental T1 values were inversely correlated with wall thickness (R from -0.233 to -0.514; P < 0.05). Systolic T1 values were significantly lower than diastolic values in basal anteroseptal segment, in all medial segments except the inferior one, and in all apical segments (P < 0.05).

Data Conclusion: Myocardial T1 values differ among myocardial regions, are influenced by sex, heart rate, and wall thickness and vary according to the cardiac cycle in healthy adults.

Level Of Evidence: 2 TECHNICAL EFFICACY: Stage 2.
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http://dx.doi.org/10.1002/jmri.27639DOI Listing
April 2021

The prognostic role of CMR using global planimetric criteria in patients with excessive left ventricular trabeculation.

Eur Radiol 2021 Apr 5. Epub 2021 Apr 5.

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

Objectives: Although cardiovascular magnetic resonance (CMR) is widely used in the assessment of left ventricular non-compaction (LVNC), there are no universally accepted diagnostic criteria and limited data regarding their prognostic value. We assessed the long-term prognostic role of the planimetric global Grothoff's criteria and of the CMR findings in predicting adverse cardiovascular events (CE).

Methods: We prospectively enrolled 78 patients (46.7 ± 18.7 years, 33.3% females) with documented positive Jenni's echocardiographic criteria for LVNC. Cine images were used to quantify function parameters and to assess for the presence of all four quantitative Grothoff's criteria (global Grothoff's criteria). Late gadolinium enhancement (LGE) images were acquired to detect the presence of replacement myocardial fibrosis.

Results: Petersen's CMR criterion for LVNC (NC/C ratio > 2.3 in at least one myocardial segment) was fulfilled in the whole population. Twenty-six patients fulfilled the global Grothoff's criteria (four out of four). The mean duration of the follow-up was 44.2 ± 27.4 months and 28 CE were registered: 10 ventricular tachycardias, 12 episodes of heart failure (HF), four strokes, and two cardiac deaths. In the multivariate analysis, the independent predictive factors for CE were positive global Grothoff's criteria (hazard ratio, HR = 3.33, 95% CI = 1.52-7.29; p = 0.003) and myocardial fibrosis (HR = 2.41, 95% CI = 1.08-5.36; p = 0.032).

Conclusions: Positive global Grothoff's criteria and myocardial fibrosis were powerful predictors of CE in patients with a diagnosis of LVNC by CMR Petersen's criterion. Thus, we strongly suggest a step approach confirming the diagnosis of LVNC by using the global planimetric Grothoff's criteria, which showed a prognostic impact.

Key Points: • Positive global Grothoff's criteria and replacement myocardial fibrosis were powerful predictors of cardiovascular events in patients with a diagnosis of LVNC by CMR Petersen's criterion. • Positive global Grothoff's criteria were associated with a higher frequency of ventricular arrhythmias in patients with a diagnosis of LVNC by CMR Petersen's criterion.
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http://dx.doi.org/10.1007/s00330-021-07875-0DOI Listing
April 2021

Partial Lipodystrophy and LMNA p.R545H Variant.

J Clin Med 2021 Mar 9;10(5). Epub 2021 Mar 9.

Obesity and Lipodystrophy Center, Endocrinology Unit, University Hospital of Pisa, 56124 Pisa, Italy.

Laminopathies are disorders caused by gene mutations, which selectively affect different tissues and organ systems, and present with heterogeneous clinical and pathological traits. The molecular mechanisms behind these clinical differences and tissue specificity have not been fully clarified. We herein examine the case of a patient carrying a heterozygous c.1634G>A (p.R545H) variant with a mild, transient myopathy, who was referred to our center for the suspicion of lipodystrophy. At physical examination, an abnormal distribution of subcutaneous fat was noticed, with fat accumulation in the anterior regions of the neck, resembling the fat distribution pattern of familial partial lipodystrophy type 2 (FPLD2). The R545H missense variant has been found at very low allelic frequency in public databases, and in silico analysis showed that this amino acid substitution is predicted to have a damaging role. Other patients carrying the heterozygous p.R545H allele have shown a marked clinical heterogeneity in terms of phenotypic body fat distribution and severity of organ system involvement. These findings indicate that the p.R545H heterozygous variant exhibits incomplete penetrance and highly variable expressivity. We hypothesized that additional genetic factors, epigenetic mechanisms, or environmental triggers might explain the variable expressivity of phenotypes among various patients.
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http://dx.doi.org/10.3390/jcm10051142DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7963176PMC
March 2021

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 Cardiology, ASST Monza, P.O. Desio, 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

Cocaine and methamphetamine use and hospitalization for acute heart failure: Epidemiological evidence from a nationwide dataset.

Int J Cardiol 2021 Mar 3. Epub 2021 Mar 3.

Fondazione Toscana G. Monasterio, Pisa, Italy; Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.

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http://dx.doi.org/10.1016/j.ijcard.2021.02.076DOI Listing
March 2021

Indications of beta-adrenoceptor blockers in Takotsubo syndrome and theoretical reasons to prefer agents with vasodilating activity.

Int J Cardiol 2021 Mar 2. Epub 2021 Mar 2.

San Raffaele Hospital and Vita Salute University, Milan, Italy.

Takotsubo syndrome (TTS) is estimated to account for 1-3% of all patients presenting with suspected ST-segment elevation myocardial infarction. A sudden surge in sympathetic nervous system is considered the cause of TTS. Nonetheless, no specific recommendations have been provided regarding β-blocking therapy. Apart from specific contra-indications (severe LV dysfunction, hypotension, bradycardia and corrected QT interval >500 ms), treatment with a β-blocker seems reasonable until full recovery of LV ejection fraction, though evidence is limited to a few animal studies, case reports or observational studies. In this review, we will reappraise the rationale for β-blocker therapy in TTS and speculate on the pathophysiologic basis for preferring non-selective agents with vasodilating activity over β-selective drugs.
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http://dx.doi.org/10.1016/j.ijcard.2021.02.074DOI Listing
March 2021

Morphologies and prognostic significance of left ventricular volume/time curves with cardiac magnetic resonance in patients with non-ischaemic heart failure and left bundle branch block.

Int J Cardiovasc Imaging 2021 Feb 26. Epub 2021 Feb 26.

Fondazione Toscana Gabriele Monasterio, Piazza Martiri della Libertà 33, 56124, Pisa, Italy.

Patients with non-ischaemic systolic heart failure (HF) and left bundle branch block (LBBB) can display a wide or narrow pattern (WP/NP) of the systolic phase of the left ventricular (LV) volume/time (V/t) curve in cardiac magnetic resonance (CMR). The clinical and prognostic significance of these patterns is unknown. Consecutive patients with non-ischaemic HF, LV ejection fraction < 50% and LBBB underwent 1.5 T CMR. Maximal dyssynchrony time (time between the earliest and latest end-systolic peaks), systolic dyssynchrony index (standard deviation of times to peak volume change), and contractility index (maximum rate of change of pressure-normalized stress) were calculated. The endpoint was a composite of cardiovascular death, HF hospitalization, and appropriate defibrillator shock. NP was found in 29 and WP in 72 patients. WP patients had higher volumes and NT-proBNP, and lower LVEF. WP patients had a longer maximal dyssynchrony time (absolute duration: 192 ± 80 vs. 143 ± 65 ms, p < 0.001; % of RR interval: 25 ± 11% vs. 8 ± 4%, p < 0.001), a higher systolic dyssynchrony index (13 ± 4 vs. 7 ± 3%, p < 0.001), and a lower contractility index (2.6 ± 1.2 vs 3.2 ± 1.7, p < 0.05). WP patients had a shorter survival free from the composite endpoint regardless of age, NT-proBNP or LVEF. Nonetheless, WP patients responded more often to cardiac resynchronization therapy (CRT) than those with NP (24/28 [86%] vs. 1/11 [9%] responders, respectively; p < 0.001). In patients with non-ischaemic systolic HF and LBBB, the WP of V/t curves identifies a subgroup of patients with greater LV dyssynchrony and worse outcome, but better response to CRT.
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http://dx.doi.org/10.1007/s10554-021-02194-3DOI Listing
February 2021

Appropriate use criteria for cardiovascular magnetic resonance imaging (CMR): SIC-SIRM position paper part 1 (ischemic and congenital heart diseases, cardio-oncology, cardiac masses and heart transplant).

Radiol Med 2021 Mar 24;126(3):365-379. Epub 2021 Feb 24.

Department of Radiological, Oncological and Pathological Sciences, Sapienza University of Rome, Rome, Italy.

Cardiac magnetic resonance (CMR) has emerged as new mainstream technique for the evaluation of patients with cardiac diseases, providing unique information to support clinical decision-making. This document has been developed by a joined group of experts of the Italian Society of Cardiology and Italian society of Radiology and aims to produce an updated consensus statement about the current state of technology and clinical applications of CMR. The writing committee consisted of members and experts of both societies who worked jointly to develop a more integrated approach in the field of cardiac radiology. Part 1 of the document will cover ischemic heart disease, congenital heart disease, cardio-oncology, cardiac masses and heart transplant.
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http://dx.doi.org/10.1007/s11547-020-01332-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7937599PMC
March 2021

Cardiac magnetic resonance in patients with muscular dystrophies.

Eur J Prev Cardiol 2020 May 16. Epub 2020 May 16.

Fondazione Toscana Gabriele Monasterio, Italy.

Muscular dystrophies are inherited disorders sharing similar clinical features and dystrophic changes on muscle biopsy. Duchenne muscular dystrophy is the most common inherited muscle disease of childhood, and Becker muscular dystrophy is a milder allelic variant with a slightly lower prevalence. Myotonic dystrophy is the most frequent form in adults. Cardiac magnetic resonance is the gold standard technique for the quantification of cardiac chamber volumes and function, and also enables a characterisation of myocardial tissue. Most cardiac magnetic resonance studies in the setting of muscular dystrophy were carried out at single centres, evaluated small numbers of patients and used widely heterogeneous protocols. Even more importantly, those studies analysed more or less extensively the patterns of cardiac involvement, but usually did not try to establish the added value of cardiac magnetic resonance to standard echocardiography, the evolution of cardiac disease over time and the prognostic significance of cardiac magnetic resonance findings. As a result, the large and heterogeneous amount of information on cardiac involvement in muscular dystrophies cannot easily be translated into recommendations on the optimal use of cardiac magnetic resonance. In this review, whose targets are cardiologists and neurologists who manage patients with muscular dystrophy, we try to summarise cardiac magnetic resonance findings in patients with muscular dystrophy, and the results of studies evaluating the role of cardiac magnetic resonance as a tool for diagnosis, risk stratification and follow-up. Finally, we provide some practical recommendations about the need and timing of cardiac magnetic resonance examination for the management of patients with muscular dystrophy.
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http://dx.doi.org/10.1177/2047487320923052DOI Listing
May 2020

Cardiovascular magnetic resonance: What clinicians should know about safety and contraindications.

Int J Cardiol 2021 May 9;331:322-328. Epub 2021 Feb 9.

Division of Cardiology, Clinica Villa dei Fiori Acerra, Naples, Italy.

Cardiovascular magnetic resonance (MR) is a multiparametric, non-ionizing, non-invasive imaging technique, which represents the imaging gold standard to study cardiac anatomy, function and tissue characterization. Faced with a wide range of clinical application, in this review we aim to provide a comprehensive guide for clinicians about MR safety, contraindications and image quality. Starting from the physical interactions of the static magnetic fields, gradients and radiofrequencies with the human body, we will describe the most common metal and electronic devices which are allowed (MR-safe), allowed under limited conditions (MR-conditional) or contraindicated (MR-unsafe). Moreover, some conditions potentially affecting image quality and patient comfort will be mentioned, including arrhythmias, claustrophobia, and poor breath-hold capacity. Finally, we will discuss the pharmacodynamics and pharmacokinetics of current gadolinium-based contrast agents, their contraindications and their potential acute and chronic adverse effects, as well as the safety issue concerning the use of vasodilating/inotropic agents in stress cardiac MR.
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http://dx.doi.org/10.1016/j.ijcard.2021.02.003DOI Listing
May 2021

A novel echocardiographic method for estimation of pulmonary artery wedge pressure and pulmonary vascular resistance.

ESC Heart Fail 2021 Apr 10;8(2):1216-1229. Epub 2021 Feb 10.

Cardiology and Cardiovascular Medicine Department, Fondazione Toscana G. Monasterio, Pisa, Italy.

Aims: This study aimed to evaluate a novel echocardiographic algorithm for quantitative estimation of pulmonary artery wedge pressure (PAWP) and pulmonary vascular resistance (PVR) in patients with heart failure and pulmonary hypertension (PH) scheduled to right heart catheterization (RHC).

Methods And Results: In this monocentric study, 795 consecutive patients (427 men; age 68.4 ± 12.1 years) undergoing echocardiography and RHC were evaluated. Multiple regression analysis was performed to identify echocardiographic predictors of PAWP and PVR measured by RHC in the derivation group (the first 200 patients). The diagnostic accuracy of the model was then tested in the validation group (the remaining 595 patients). PH was confirmed by RHC in 507 (63.8%) patients, with 192 (24.2%) cases of precapillary PH, 248 (31.2%) of postcapillary PH, and 67 (8.4%) of combined PH. At regression analysis, tricuspid regurgitation maximal velocity, mitral E/e' ratio, left ventricular ejection fraction, right ventricular fractional area change, inferior vena cava diameter, and left atrial volume index were included in the model (R = 0.8, P < 0.001). The model showed a high diagnostic accuracy in estimating elevated PAWP (area under the receiver operating characteristic curve = 0.97, 92% sensitivity, and 93% specificity, P < 0.001) and PVR (area under the receiver operating characteristic curve = 0.96, 89% sensitivity, and 92% specificity, P < 0.001), outperforming 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging recommendations (P < 0.001) and Abbas' equation (P < 0.001). Bland-Altman analysis showed satisfactory limits of agreement between echocardiography and RHC for PAWP (bias 0.7, 95% confidence interval -7.3 to 8.7) and PVR (bias -0.1, 95% confidence interval -2.2 to 1.9 Wood units), without indeterminate cases.

Conclusions: A novel quantitative echocardiographic approach for the estimation of PAWP and PVR has high diagnostic accuracy in patients with heart failure and PH.
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http://dx.doi.org/10.1002/ehf2.13183DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8006655PMC
April 2021

Cardiac CT angiography: normal and pathological anatomical features-a narrative review.

Cardiovasc Diagn Ther 2020 Dec;10(6):1918-1945

Department of Radiology, CNR (National Council of Research)/Tuscany Region "Gabriele Monasterio" Foundation (FTGM), Massa, Italy.

The normal and pathological anatomy of the heart and coronary arteries are nowadays widely developed topics and constitute a fundamental part of the cultural background of the radiologist. The introduction of cardiac ECG-gated synchronized CT scanners with an ever-increasing number of detectors and with increasingly high structural characteristics (increase in temporal resolution, increase in contrast resolution with dual-source, dual energy scanners) allows the virtual measurement of anatomical structures complying with heart rate with submillimetric precision permitting to clearly depict the normal anatomy and follow the pathologic temporal evolution. Accordingly to these considerations, cardiac computed tomography angiography (CCTA) asserts itself as a gold standard method for the anatomical evaluation of the heart and permits to evaluate, verify, measure and characterize structural pathological alterations of both congenital and acquired degenerative diseases. Accordingly, CCTA is increasingly used as a prognostic model capable of modifying the outcome of diseased patients in planning interventions and in the post-surgical/interventional follow-up. The profound knowledge of cardiac anatomy and function through highly detailed CCTA analysis is required to perform an efficient and optimal use in real-world clinical practice.
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http://dx.doi.org/10.21037/cdt-20-530DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7758747PMC
December 2020

Evolution of acute myocarditis in a pediatric population: An MRI based study.

Int J Cardiol 2021 Apr 25;329:226-233. Epub 2020 Dec 25.

Unité médico-chirurgicale de cardiologie congénitale et pédiatrique, centre de référence des maladies cardiaques congénitales complexes - M3C, Hôpital universitaire Necker-Enfants Malades, Université de Paris, France and Laboratory of Embriology and Genetic Malformation, INSERM UMR 1163, Imagine Institute, F-75015 Paris, France.

Background: Cardiac Magnetic Resonance (CMR) data regarding myocarditis presentation and disease course is still lacking in pediatric patients. We evaluate baseline CMR and evolution of functional and tissue abnormalities in children with acute myocarditis.

Methods: CMR was performed in 125 patients with clinical diagnosis of acute myocarditis. Clinical follow-up was performed for a median of 498 (214-923) days.

Results: LVEF was depressed (<55%) in 56 cases (45%) upon baseline CMR. LGE was found in 93 patients (77%) of cases. LGE was exclusively subepicardial in 29 patients (23%), while other LGE patterns (midwall/mixed) were present in 64 (51%). CMR was repeated in 92 (74%) patients. 67% presented recover of function at a median of 170 (70-746) days after onset of symptoms. Midwall/mixed LGE pattern had a statistically significant correlation with absent recover of function (OR 0.20 p 0.036). Thirteen patients (16%) had recovery from LV dysfunction but with persistence of LGE. Sub-epicardial pattern of LGE (OR 3.33, 95% CI 1.08-10.2, p = 0.036) and the presence of fever at admission (OR 4.67, 95% CI 1.16-18.7, p = 0.03) were associated with a significantly higher likelihood of complete normalization while midwall/mixed LGE pattern was associated with non-recovery.

Conclusions: In pediatric myocarditis, midwall/mixed LGE pattern is associated with absent recover of function. Patients with recover of function may still have persistence of LGE, while a complete recovery from functional and tissue abnormalities is found only in a third of patients. Midwall/mixed pattern of LGE at first MRI was associated to worse outcome.
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http://dx.doi.org/10.1016/j.ijcard.2020.12.052DOI Listing
April 2021

18-FDG PET for large vessel vasculitis diagnosis and follow-up.

Clin Exp Rheumatol 2020 Dec 9. Epub 2020 Dec 9.

Clinical Immunology Unit, Department of Clinical and Experimental Medicine, University of Pisa and Azienda Ospedaliera Universitaria Pisana, Pisa, Italy.

Objectives: Large vessel vasculitis (LVV) are chronic inflammatory diseases that affect arteries. While a mere clinical-serological approach does not seem sensitive either in the initial evaluation nor in long-term monitoring, 18-FDG positron emission tomography (18-FDG PET) is currently considered a useful assessment tool in LVV. We aimed at exploring the utility of 18-FDG, compared with traditional assessments, in the short- and long-term follow-up of patients with LVV. In addition, we compared patterns of vascular involvement in patients with Takayasu's arteritis (TAK) and giant cell arteritis (GCA).

Methods: We retrospectively analysed 47 patients affected by LVV, evaluating clinics, blood chemistry and 18-FDG PET results, at two time points, short-term (average 8 months after diagnosis) and long-term (average 29 months).

Results: 18-FDG PET uptake, expressed as mean value of SUV max, decreased significantly during follow-up in all the patients. A low concordance between 18-FDG PET and acute phase reactants levels was observed, but also a good sensitivity in detecting the response to treatment.

Conclusions: The results confirm the role of 18-FDG PET as a powerful tool in the evaluation of LVV, both at the time of diagnosis and during monitoring. Furthermore, the data confirm that GCA and TAK are part of the same disease spectrum.
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December 2020

Deep learning to diagnose cardiac amyloidosis from cardiovascular magnetic resonance.

J Cardiovasc Magn Reson 2020 12 7;22(1):84. Epub 2020 Dec 7.

Deep Health Unit, Fondazione Toscana Gabriele Monasterio, Pisa-Massa, Italy.

Background: Cardiovascular magnetic resonance (CMR) is part of the diagnostic work-up for cardiac amyloidosis (CA). Deep learning (DL) is an application of artificial intelligence that may allow to automatically analyze CMR findings and establish the likelihood of CA.

Methods: 1.5 T CMR was performed in 206 subjects with suspected CA (n = 100, 49% with unexplained left ventricular (LV) hypertrophy; n = 106, 51% with blood dyscrasia and suspected light-chain amyloidosis). Patients were randomly assigned to the training (n = 134, 65%), validation (n = 30, 15%), and testing subgroups (n = 42, 20%). Short axis, 2-chamber, 4-chamber late gadolinium enhancement (LGE) images were evaluated by 3 networks (DL algorithms). The tags "amyloidosis present" or "absent" were attributed when the average probability of CA from the 3 networks was ≥ 50% or < 50%, respectively. The DL strategy was compared to a machine learning (ML) algorithm considering all manually extracted features (LV volumes, mass and function, LGE pattern, early blood-pool darkening, pericardial and pleural effusion, etc.), to reproduce exam reading by an experienced operator.

Results: The DL strategy displayed good diagnostic accuracy (88%), with an area under the curve (AUC) of 0.982. The precision (positive predictive value), recall score (sensitivity), and F1 score (a measure of test accuracy) were 83%, 95%, and 89% respectively. A ML algorithm considering all CMR features had a similar diagnostic yield to DL strategy (AUC 0.952 vs. 0.982; p = 0.39).

Conclusions: A DL approach evaluating LGE acquisitions displayed a similar diagnostic performance for CA to a ML-based approach, which simulates CMR reading by experienced operators.
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http://dx.doi.org/10.1186/s12968-020-00690-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7720569PMC
December 2020

A simple echocardiographic score to rule out cardiac amyloidosis.

Eur J Clin Invest 2021 May 23;51(5):e13449. Epub 2020 Nov 23.

Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.

Background: Early diagnosis of cardiac amyloidosis (CA) is warranted to initiate specific treatment and improve outcome. The amyloid light chain (AL) and inferior wall thickness (IWT) scores have been proposed to assess patients referred by haematologists or with unexplained left ventricular (LV) hypertrophy, respectively. These scores are composed of 4 or 5 variables, respectively, including strain data.

Methods: Based on 2 variables common to the AL and IWT scores, we defined a simple score named AMYLoidosis Index (AMYLI) as the product of relative wall thickness (RWT) and E/e' ratio, and assessed its diagnostic performance.

Results: In the original cohort (n = 251), CA was ultimately diagnosed in 111 patients (44%). The 2.22 value was selected as rule-out cut-off (negative likelihood ratio [LR-] 0.0). In the haematology subset, AL CA was diagnosed in 32 patients (48%), with 2.36 as rule-out cut-off (LR- 0.0). In the hypertrophy subset, ATTR CA was diagnosed in 79 patients (43%), with 2.22 as the best rule-out cut-off (LR- 0.0). In the validation cohort (n = 691), the same cut-offs proved effective: indeed, there were no patients with CA in the whole population or in the haematology or hypertrophy subsets scoring < 2.22, <2.36 or < 2.22, respectively.

Conclusions: The AMYLI score (RWT*E/e') may have a role as an initial screening tool for CA. A < 2.22 value excludes the diagnosis in patients undergoing a diagnostic screening for CA, while a < 2.36 and a < 2.22 value may be better considered in the subsets with suspected cardiac AL amyloidosis or unexplained hypertrophy, respectively.
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http://dx.doi.org/10.1111/eci.13449DOI Listing
May 2021

Prevalence and prognostic impact of nonischemic late gadolinium enhancement in stress cardiac magnetic resonance.

J Cardiovasc Med (Hagerstown) 2020 Dec;21(12):980-985

Fondazione Gabriele Monasterio CNR/Regione Toscana.

Aim: To assess the prevalence and prognostic significance of NI-LGE in patients undergoing stress-CMR.

Methods: Stress-CMR with either dipyridamole or adenosine was performed in 283 patients (228 men, 81%) including perfusion imaging, wall motion evaluation and LGE. Follow-up was completed in all enrolled patients (median time: 1850 days; interquartile range: 1225-2705 days). Composite endpoint included cardiac death, ventricular tachycardia, myocardial infarction, stroke, hospitalization for cardiac cause and coronary revascularization performed beyond 90 days from stress-CMR scans.

Results: One hundred and twelve patients (40%) had negative LGE (no-LGE), 140 patients (49%) I-LGE and 31 patients (11%) NI-LGE. Twenty-five events occurred in the no-LGE group, 68 in I-LGE and 11 in the NI-LGE group. On survival curves, patients with NI-LGE had worse prognosis than patients with no-LGE regardless of the presence of inducible perfusion defects. No significant prognostic differences were found between I-LGE and NI-LGE.

Conclusion: NI-LGE can be detected in 11% of patients during stress-CMR providing a diagnosis of nonischemic cardiac disease. Patients with NI-LGE have worse prognosis than those with no-LGE.
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http://dx.doi.org/10.2459/JCM.0000000000001016DOI Listing
December 2020

Imaging predictors of incident heart failure: a systematic review and meta-analysis.

J Cardiovasc Med (Hagerstown) 2021 May;22(5):378-387

School of Biomedical Engineering and Imaging Sciences, King's College London, London, UK.

Background: Preventing the evolution of subclinical cardiac disease into overt heart failure is of paramount importance. Imaging techniques, particularly transthoracic echocardiography (TTE), are well suited to identify abnormalities in cardiac structure and function that precede the development of heart failure.

Methods: This meta-analysis provides a comprehensive evaluation of 32 studies from 11 individual cohorts, which assessed cardiac indices from TTE (63%), cardiovascular magnetic resonance (CMR; 34%) or cardiac computed tomography (CCT; 16%). Eligible studies focused on measures of left ventricular geometry and function and were highly heterogeneous.

Results: Among the variables that could be assessed through a meta-analytic approach, left ventricular systolic dysfunction, defined as left ventricular ejection fraction (LVEF) lower than 50%, and left ventricular dilation were associated with a five-fold [hazard ratio (HR) 4.76, 95% confidence interval (95% CI) 1.85-12.26] and three-fold (HR 3.14, 95% CI 1.37 -7.19) increased risk of heart failure development, respectively. Any degree of diastolic dysfunction conveyed an independent, albeit weaker, association with heart failure (HR 1.48, 95% CI 1.11-1.96), although there was only a trend for left ventricular hypertrophy in predicting incident heart failure (hazard ratio 2.85, 95% CI 0.82-9.85).

Conclusion: LVEF less than 50%, left ventricular dilation and diastolic dysfunction are independent predictors of incident heart failure among asymptomatic individuals, while left ventricular hypertrophy seems less predictive. These findings may serve as a framework for implementing imaging-based screening strategies in patients at risk of heart failure and inform future studies testing preventive or therapeutic approaches aiming at thwarting or halting the progression from asymptomatic (preclinical) to overt heart failure.
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http://dx.doi.org/10.2459/JCM.0000000000001133DOI Listing
May 2021

Colchicine for the Treatment of Coronary Artery Disease.

Trends Cardiovasc Med 2020 Oct 20. Epub 2020 Oct 20.

Hospital Universitari Germans Trias i Pujol, Badalona (Barcelona), Spain; CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain.

Inflammation plays an important role in atherosclerosis. Acute coronary syndromes (ACS), and particularly myocardial infarction (MI), are associated with a systemic inflammatory response that may accelerate coronary atherosclerotic processes, leading to plaque destabilization and increased risk of further cardiovascular events. These considerations provide a conceptual framework for the use of anti-inflammatory therapies in patients with chronic coronary syndrome or ACS. Following the diverging results of trials on canakinumab and methotrexate, the Colchicine Cardiovascular Outcomes Trial (COLCOT) and the Low-Dose Colchicine trial-2 (LoDoCo2) have sparked new interest in the perspective of an anti-inflammatory therapy for CAD by showing that colchicine confers a prognostic benefit in patients with a recent MI or CCS, respectively. Colchicine blocks multiple steps of the inflammatory cascade and modulates also platelet function and endothelial activation. It has a better safety profile than canakinumab and is a very inexpensive drug throughout the world. We deemed it useful to reappraise the available literature on colchicine and coronary artery disease to assess the likelihood that it might become part of the therapeutic armamentarium of this condition.
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http://dx.doi.org/10.1016/j.tcm.2020.10.007DOI Listing
October 2020

Late gadolinium enhancement role in arrhythmic risk stratification of patients with LMNA cardiomyopathy: results from a long-term follow-up multicentre study.

Europace 2020 12;22(12):1864-1872

Department of Arrhythmology and Cardiac Electrophysiology, IRCCS San Raffaele Hospital, Milan, Italy.

Aims: We aimed at addressing the role of late gadolinium enhancement (LGE) in arrhythmic risk stratification of LMNA-associated cardiomyopathy (CMP).

Methods And Results: We present data from a multicentre national cohort of patients with LMNA mutations. Of 164 screened cases, we finally enrolled patients with baseline cardiac magnetic resonance (CMR) including LGE sequences [n = 41, age 35 ± 17 years, 51% males, mean left ventricular ejection fraction (LVEF) by echocardiogram 56%]. The primary endpoint of the study was follow-up (FU) occurrence of malignant ventricular arrhythmias [MVA, including sustained ventricular tachycardia (VT), ventricular fibrillation, and appropriate implantable cardioverter-defibrillator (ICD) therapy]. At baseline CMR, 25 subjects (61%) had LGE, with non-ischaemic pattern in all of the cases. Overall, 23 patients (56%) underwent ICD implant. By 10 ± 3 years FU, eight patients (20%) experienced MVA, consisting of appropriate ICD shocks in all of the cases. In particular, the occurrence of MVA in LGE+ vs. LGE- groups was 8/25 vs. 0/16 (P = 0.014). Of note, no significant differences between LGE+ and LGE- patients were found in currently recognized risk factors for sudden cardiac death (male gender, non-missense mutations, baseline LVEF <45% and non-sustained VT), all P-value >0.05.

Conclusions: In LMNA-CMP patients, LGE at baseline CMR is significantly associated with MVA. In particular, as suggested by this preliminary experience, the absence of LGE allowed to rule-out MVA at 10 years mean FU.
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http://dx.doi.org/10.1093/europace/euaa171DOI Listing
December 2020

Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy.

ESC Heart Fail 2020 Sep 23. Epub 2020 Sep 23.

Cardio-thoraco-vascular Department, University of Trieste, Trieste, Italy.

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high risk of sudden cardiac death. Three different prediction models for the indication of implanted cardioverter defibrillator (ICD) are now available: the 5 year ARVC risk score, the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. We compared these three prediction models in a validation cohort of patients with definite ARVC.

Methods And Results: In a cohort of 140 patients with definite ARVC, the 5 year ARVC risk score and the ITFC and HRS criteria were compared for the prediction of a major combined endpoint of sudden cardiac death, appropriate ICD intervention, resuscitated cardiac arrest, and sustained ventricular tachycardia. During the follow-up, 65 major events occurred. The 5 year ARVC risk score with a threshold >10%, derived from the maximally selected rank statistic, predicted 62 (95%) events [odds ratio (OR) 9.1, 95% confidence interval (CI) 2.6-32, P = 0.0006], the ITFC criteria 53 (81%, OR 4.8, 95% CI 2.2-10.3, P = 0.0001), and the HRS criteria 29 (45%, OR 4.2, 95% CI 1.9-9.3, P = 0.0003). At the analysis of decision curve for ICD implantation, a 5 year ARVC risk score >10% showed a greater net benefit than the ITFC and HRS criteria over a wide range of threshold probability of events. Finally, at multivariate analysis, the 5 year ARVC risk score >10% was the only independent predictor of major events.

Conclusions: The 5 year score with a threshold of >10% was more effective for predicting events than the ITFC and HRS criteria.
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http://dx.doi.org/10.1002/ehf2.13019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7755004PMC
September 2020

Echocardiography versus computed tomography and cardiac magnetic resonance for the detection of left heart thrombosis: a systematic review and meta-analysis.

Clin Res Cardiol 2020 Sep 13. Epub 2020 Sep 13.

School of Biomedical Engineering and Imaging Sciences, King's College London, St Thomas' Hospital Campus, London, UK.

Background: Accurate and reproducible diagnostic techniques are essential to detect left-sided cardiac thrombi [either in the left ventricle (LV) or in the left atrial appendage (LAA)] and to guide the onset and duration of antithrombotic treatment while minimizing the risk for thromboembolic and hemorrhagic events.

Methods: We conducted a systematic review and meta-analysis aiming to compare the diagnostic performance of transthoracic echocardiography (TTE) vs. cardiac magnetic resonance (CMR) for the detection of LV thrombi, and transesophageal echocardiography (TEE) vs. computed tomography (CT) for the detection of LAA thrombi.

Results: Six studies were included in the first meta-analysis (TTE vs. CMR for LV thrombosis). Pooled sensitivity and specificity values were 62% [95% confidence interval (CI), 37-81%] and 97% (95% CI, 94-99%). The shape of the hierarchical summary receiver operating characteristic (HSROC) curve and the area under the curve (AUC) of 0.96 suggested a high accuracy. Ten studies were included in the second meta-analysis (CT versus TEE for LAA thrombosis). The pooled values of sensitivity and specificity were 97% (95% CI, 77-100%) and 94% (95% CI, 87-98%). The pooled diagnostic odds ratio (DOR) was 500 (95% CI, 52-4810), and the pooled likelihood ratios (LR + and LR-) were 17% (95% CI, 7-40%) and 3% (95% CI, 0-28%). The shape of the HSROC curve and 0.99 AUC suggested a high accuracy of CT vs. TEE.

Conclusions: TTE is a fair alternative to DE-CMR for the identification of LV thrombi, while CT has a good accuracy compared to TEE for the detection of LAA thrombosis.

Prospero Registration: CRD42020185842.
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http://dx.doi.org/10.1007/s00392-020-01741-7DOI Listing
September 2020

Atypical Progeroid Syndrome and Partial Lipodystrophy Due to Gene p.R349W Mutation.

J Endocr Soc 2020 Oct 1;4(10):bvaa108. Epub 2020 Aug 1.

Obesity and Lipodystrophy Center, Endocrinology Unit, University Hospital of Pisa, Pisa, Italy.

Atypical progeroid syndrome (APS) comprises heterogeneous disorders characterized by variable degrees of fat loss, metabolic alterations, and comorbidities that affect skeleton, muscles, and/or the heart. We describe 3 patients that were referred to our center for the suspicion of lipodystrophy. They had precocious aging traits such as short stature, mandibular hypoplasia, beaked nose, and partial alopecia manifesting around 10 to 15 years of age recurrently associated with: (1) partial lipodystrophy; (2) proteinuric nephropathy; (3) heart disease (rhythm disorders, valvular abnormalities, and cardiomyopathy); and (4) sensorineural hearing impairment. In all patients, genetic testing revealed a missense heterozygous lamin A/C gene () mutation c.1045 C > T (p.Arg349Trp). Ten patients with p.R349W mutation have been reported so far, all presenting with similar features, which represent the key pathological hallmarks of this subtype of APS. The associated kidney and cardiac complications occurring in the natural history of the disease may reduce life expectancy. Therefore, in these patients a careful and periodic cardiac and kidney function evaluation is required.
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http://dx.doi.org/10.1210/jendso/bvaa108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7474543PMC
October 2020

Cardiovascular magnetic resonance for the diagnosis and management of heart failure with preserved ejection fraction.

Heart Fail Rev 2020 Jun 22. Epub 2020 Jun 22.

Fondazione Toscana Gabriele Monasterio, Via Moruzzi, 1, 56124, Pisa, Italy.

Heart failure with preserved ejection fraction (HFpEF) is characterized by an impaired ventricular filling resulting in the development of dyspnea and other HF symptoms. Even though echocardiography is the cornerstone to demonstrate structural and/or functional alterations of the heart as the underlying cause for the clinical presentation, cardiovascular magnetic resonance (CMR) represents the noninvasive gold standard to assess cardiac morphology, function, and tissue changes. Indeed, CMR allows quantification of biventricular volumes, mass, wall thickness, systolic function, and intra- and extracardiac flows; diastolic functional indices include transmitral and pulmonary venous velocities, left ventricular and left atrial filling velocities from volumetric changes, strain analysis from myocardial tagging, tissue phase contrast, and feature tracking. Moreover, CMR allows superior tissue characterization of the myocardium and the pericardium, which are crucial for a noninvasive etiological and histopathological assessment of HFpEF: conventional T1-weighted, T2-weighted, and post-contrast sequences are now complemented by quantitative mapping sequences, including T1 and T2 mapping as well as extracellular volume quantification. Further experimental sequences comprise diffusion tensor analysis, blood oxygenation-dependent sequences, hyperpolarized contrast agents, spectroscopy, and elastography. Finally, artificial intelligence is beginning to help clinicians deal with an increasing amount of information from CMR exams.
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http://dx.doi.org/10.1007/s10741-020-09998-wDOI Listing
June 2020

The Role of MRI in Prognostic Stratification of Cardiomyopathies.

Curr Cardiol Rep 2020 06 19;22(8):61. Epub 2020 Jun 19.

Fondazione Gabriele Monasterio/CNR Regione Toscana, via Moruzzi 1, 56100, Pisa, Italy.

Purpose Of The Review: The aim of this review was to discuss the role of cardiac magnetic resonance (CMR) for the prognostic stratification of cardiomyopathies, highlighting strengths and limitations.

Recent Findings: CMR is considered as a diagnostic pillar in the management of non-ischemic cardiomyopathies. Over the last years, attention has shifted from CMR's diagnostic capability towards prognostication in the various settings of cardiomyopathies. CMR is considered the gold standard imaging technique for the evaluation of ventricular volumes and systolic function as well as providing non-invasive virtual-histology by means of specific myocardial tissue characterization pulse sequences. CMR is an additive tool to risk stratifying patients and to identify those that require strict monitoring and more aggressive treatment.
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http://dx.doi.org/10.1007/s11886-020-01311-3DOI Listing
June 2020

Cardiac magnetic resonance in cocaine-induced myocardial damage: cocaine, heart, and magnetic resonance.

Heart Fail Rev 2020 Jun 2. Epub 2020 Jun 2.

Fondazione Toscana Gabriele Monasterio, Via Giuseppe Moruzzi, 156124, Pisa, Italy.

The use of cocaine constitutes a major health problem. Cocaine use is associated with acute and chronic complications that might involve any system, the most common being the cardiovascular system. The precise incidence of cocaine-induced cardiomyopathy remains mysterious and probably underreported. Cocaine use should be considered in young patients presenting with chest pain or heart failure without other underlying risk factors. Cocaine-related cardiovascular complications can be acute or chronic and include ischemic and non-ischemic events. Frequent cocaine users have a seven-fold higher risk of myocardial infarction. In addition to its ischemic effects, other cardiovascular complications of cocaine use and abuse are hypertensive crises, aortic dissection or aortic rupture, cerebral hemorrhage, arrhythmias and sudden cardiac death, myocarditis, dilated cardiomyopathy, heart failure, and endocarditis. The mechanism of cocaine's cardiovascular toxicity relates to its sympathomimetic effect, to the block of voltage-dependent K and Na channels, and a hypersensitivity reaction to drug or contaminants, such as amphetamine, sugars, or talc. Cardiac magnetic resonance (CMR) can provide a valuable assessment of cocaine-induced myocardial damage both in acute and chronic cardiac complications: it gives prognostic information in clinically relevant settings, and it identifies silent myocardial damage in asymptomatic patients. Indeed, CMR study should be considered in symptomatic cocaine users to assess the extent and evolution of myocardial injury. Furthermore, it was suggested to repeat CMR after 4-8 months of appropriate management to evaluate myocardial response to abstinence and medical therapy.
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http://dx.doi.org/10.1007/s10741-020-09983-3DOI Listing
June 2020

Familial LCAT deficiency and cardiovascular disease: the game is not over. A case of dramatic multivessel atherosclerosis.

Minerva Med 2020 May 29. Epub 2020 May 29.

Lipoapheresis Unit, Reference Center for Diagnosis and Treatment of Inherited Dyslipidemias, Fondazione Toscana "Gabriele Monasterio", Pisa, Italy.

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http://dx.doi.org/10.23736/S0026-4806.20.06633-1DOI Listing
May 2020

Cardiac magnetic resonance in patients with muscular dystrophies.

Eur J Prev Cardiol 2020 May 16:2047487320923052. Epub 2020 May 16.

Fondazione Toscana Gabriele Monasterio, Italy.

Muscular dystrophies are inherited disorders sharing similar clinical features and dystrophic changes on muscle biopsy. Duchenne muscular dystrophy is the most common inherited muscle disease of childhood, and Becker muscular dystrophy is a milder allelic variant with a slightly lower prevalence. Myotonic dystrophy is the most frequent form in adults. Cardiac magnetic resonance is the gold standard technique for the quantification of cardiac chamber volumes and function, and also enables a characterisation of myocardial tissue. Most cardiac magnetic resonance studies in the setting of muscular dystrophy were carried out at single centres, evaluated small numbers of patients and used widely heterogeneous protocols. Even more importantly, those studies analysed more or less extensively the patterns of cardiac involvement, but usually did not try to establish the added value of cardiac magnetic resonance to standard echocardiography, the evolution of cardiac disease over time and the prognostic significance of cardiac magnetic resonance findings. As a result, the large and heterogeneous amount of information on cardiac involvement in muscular dystrophies cannot easily be translated into recommendations on the optimal use of cardiac magnetic resonance. In this review, whose targets are cardiologists and neurologists who manage patients with muscular dystrophy, we try to summarise cardiac magnetic resonance findings in patients with muscular dystrophy, and the results of studies evaluating the role of cardiac magnetic resonance as a tool for diagnosis, risk stratification and follow-up. Finally, we provide some practical recommendations about the need and timing of cardiac magnetic resonance examination for the management of patients with muscular dystrophy.
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http://dx.doi.org/10.1177/2047487320923052DOI Listing
May 2020