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1.
Eur J Radiol ; 176: 111500, 2024 May 08.
Article in English | MEDLINE | ID: mdl-38772161

ABSTRACT

BACKGROUND: Antiphospholipid syndrome (APS) can occur primarily (PAPS) or secondary to another autoimmune disease (SAPS), most commonly systemic lupus erythematosus (SLE). Recently, we reported that subclinical brain involvement was highly prevalent in patients with autoimmune diseases, including SLE. We aimed to investigate whether patients with SLE, PAPS or SAPS and cardiac symptoms showed differences in cardiac/brain involvement based on combined brain-heart magnetic resonance imaging (MRI). METHODS: We prospectively recruited 15 patients with SAPS (86 % with SLE) and 3 patients with PAPS and compared their MRI findings to those of 13 patients with SLE from our previous publication. All patients underwent routine cardiovascular/neurological examination and standard echocardiography. RESULTS: No patients had abnormalities in routine clinical workup/echocardiography. The vast majority had white matter hyperintensities (WMHs) and all had evidence of myocardial fibrosis and/or inflammation. Patients with SAPS had a lower median WMH number [1.00 (1.00, 2.00)] than those with PAPS [3.00 (2.50, 3.00)] or SLE [2.00 (2.00, 3.00)] (p = 0.010). Subcortical and deep WM were highly prevalent. Periventricular WMHs were more frequent in patients with SLE [6 (46.2 %)] or PAPS [2 (66.7 %)] (p = 0.023). Higher lesion burdens (1 WMH vs. 2 WMHs vs. ≥ WMHs) were associated with the presence of cardiac fibrosis [3 (33.3 %) vs. 10 (83.3) vs. 7 (77.8), p = 0.039] and affected the deep and periventricular WM (p < 0.001 for both). CONCLUSION: In patients with PAPS, SAPS or SLE, cardiac symptoms and normal routine workup, combined brain-heart MRI identified abnormalities in both organs in the majority of patients. Combined brain-heart MRI offers excellent diagnostic value, but its incorporation into routine clinical practice should be further investigated. Clinical relevance statement Combined brain-heart magnetic resonance imaging in antiphospholipid syndrome may help to assess the presence of abnormalities in both organs.

2.
J Clin Med ; 13(10)2024 May 19.
Article in English | MEDLINE | ID: mdl-38792532

ABSTRACT

Takotsubo syndrome (TTS) is a type of cardiomyopathy usually precipitated by either emotional or physical stress and potentially leading to reversible heart failure. There is emerging evidence indicating an interaction between the brain and the heart in patients with TTS. Nevertheless, these new insights are not reflected in the current clinical approach to TTS. The application of novel and existing imaging modalities for the evaluation of brain-heart interactions is an interesting approach that could potentially augment diagnostic and prognostic yield, as well as improve our pathophysiologic understanding in the context of TTS. In this opinion piece, we discuss the evidence supporting a brain-heart interaction in patients with TTS and discuss how a combined evaluation of brain-heart interactions could potentially be implemented.

3.
Diagnostics (Basel) ; 14(7)2024 Mar 26.
Article in English | MEDLINE | ID: mdl-38611604

ABSTRACT

Cardiac involvement in sickle beta thalassemia (Sß-thal) patients has been poorly investigated. We aimed to evaluate cardiac function and myocardial iron overload by cardiovascular magnetic resonance (CMR) in patients with Sß-thal. One-hundred and eleven Sß-thal patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network were studied and compared with 46 sickle cell anemia (SCA) patients and with 111 gender- and age- matched healthy volunteers. Cine images were acquired to quantify biventricular function. Myocardial iron overload (MIO) was assessed by the T2* technique, while macroscopic myocardial fibrosis was evaluated by the late gadolinium enhancement (LGE) technique. In Sß-thal and SCA patients, the morphological and functional CMR parameters were not significantly different, except for the left atrial area and left ventricular (LV) stroke volume, indexed by body surface area (p = 0.023 and p = 0.048, respectively), which were significantly higher in SCA patients. No significant differences between the two groups were found in terms of myocardial iron overload and macroscopic myocardial fibrosis. When compared to healthy subjects, Sß-thal patients showed significantly higher bi-atrial and biventricular parameters, except for LV ejection fraction, which was significantly lower. The CMR analysis confirmed that Sß-thal and SCA patients are phenotypically similar. Since Sß-thal patients showed markedly different morphological and functional indices from healthy subjects, it would be useful to identify Sß-thal/SCA-specific bi-atrial and biventricular reference values.

4.
Article in English | MEDLINE | ID: mdl-38676849

ABSTRACT

PURPOSE: The difference between rest and peak stress end-systolic pressure-volume relation (ΔESPVR) is an afterload-independent index of left ventricular (LV) contractility. We assessed the independent prognostic value of ΔESPVR index by dipyridamole stress-cardiovascular magnetic resonance (CMR) in patients with known/suspected coronary artery disease (CAD). METHODS: We considered 196 consecutive patients (62.74 ± 10.66 years, 49 females). Wall motion and perfusion abnormalities at rest and peak stress were analysed. Replacement myocardial fibrosis was detected by late gadolinium enhancement (LGE) technique. The ESPVR was evaluated at rest and peak stress from raw measurement of systolic arterial pressure and end-systolic volume by biplane Simpson's method. RESULTS: A reduced ΔESPVR index (≤ 0.02 mmHg/mL/m2) was found in 88 (44.9%) patients and it was associated with a lower LV ejection fraction (EF) and with a higher frequency of abnormal stress CMR and myocardial fibrosis. During a mean follow-up of 53.17 ± 28.21 months, 50 (25.5%) cardiac events were recorded: 5 cardiac deaths, 17 revascularizations, one myocardial infarction, 23 hospitalisations for heart failure or unstable angina, and 4 ventricular arrhythmias. According to Cox regression analysis, diabetes, family history, LVEF, abnormal stress CMR, myocardial fibrosis, and reduced ΔESPVR were significant univariate prognosticators. In the multivariate analysis the independent predictors were ΔESPVR index ≤ 0.02 mmHg/mL/m2 (hazard ratio-HR = 2.58, P = 0.007), myocardial fibrosis (HR = 2.13, P = 0.036), and diabetes (HR = 2.33, P = 0.012). CONCLUSION: ΔESPVR index by stress-CMR was independently associated with cardiac outcomes in patients with known/suspected CAD, in addition to replacement myocardial fibrosis and diabetes. Thus, the assessment of ΔESPVR index may be included into the standard stress-CMR exam to further stratify the patients.

6.
Curr Opin Rheumatol ; 36(1): 76-93, 2024 01 01.
Article in English | MEDLINE | ID: mdl-37962165

ABSTRACT

PURPOSE OF REVIEW: In systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc. RECENT FINDINGS: SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI. SUMMARY: our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.


Subject(s)
Myocarditis , Scleroderma, Systemic , Humans , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/therapy , Heart , Myocarditis/drug therapy , Myocarditis/etiology , Myocarditis/pathology , Magnetic Resonance Imaging , Risk Assessment
7.
Endocrine ; 83(3): 548-558, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38129722

ABSTRACT

BACKGROUND: Cushing's Syndrome (CS) is associated with increased cardiovascular morbidity and mortality. In endogenous CS, cardiovascular mortality remains increased for up to 15 years post remission of hypercortisolism. Similarly, patients with exogenous CS have 4-fold increased incidence of cardiovascular events, regardless of pre-existing cardiovascular disease (CVD). OBJECTIVE: To present the pathophysiology, prognosis, clinical and imaging phenotype of cardiac disease in CS. METHODS: A Pubmed search for cardiac disease in CS over the last 20 years was conducted using combinations of relevant terms. Preclinical and clinical studies, as well as review papers reporting on subclinical heart failure (HF), cardiomyopathy, coronary heart disease (CHD), and cardiovascular imaging were selected. RESULTS: Cardiac disease in CS is associated with direct mineralocorticoid and glucocorticoid receptor activation, increased responsiveness to angiotensin II, ectopic epicardial adiposity, arterial stiffness and endothelial dysfunction, as well as with diabetes mellitus, hypertension, hyperlipidemia, obesity and prothrombotic diathesis. Subclinical HF and cardiomyopathy are principally related to direct glucocorticoid (GC) effects and markedly improve or regress post hypercortisolism remission. In contrast, CHD is related to both direct GC effects and CS comorbidities and persists post cure. In patients without clinical evidence of CVD, echocardiography and cardiac magnetic resonance (CMR) imaging reveal left ventricular hypertrophy, fibrosis, diastolic and systolic dysfunction, with the latter being underestimated by echocardiography. Finally, coronary microvascular disease is encountered in one third of cases. CONCLUSION: Cardiovascular imaging is crucial in evaluation of cardiac involvement in CS. CMR superiority in terms of reproducibility, operator independency, unrestricted field of view and capability of tissue characterisation makes this modality ideal for future studies.


Subject(s)
Cardiomyopathies , Cushing Syndrome , Heart Diseases , Humans , Cushing Syndrome/complications , Cushing Syndrome/diagnostic imaging , Cushing Syndrome/pathology , Reproducibility of Results , Heart Diseases/etiology , Magnetic Resonance Imaging , Glucocorticoids
8.
J Am Coll Cardiol ; 82(22): 2128-2151, 2023 11 28.
Article in English | MEDLINE | ID: mdl-37993205

ABSTRACT

Derangements in the innate and adaptive immune responses observed in systemic inflammatory syndromes contributes to unique elevated atherosclerotic risk and incident cardiovascular disease. Novel multimodality imaging techniques may improve diagnostic precision for the screening and monitoring of disease activity. The integrated application of these technologies lead to earlier diagnosis and noninvasive monitoring of cardiac involvement in systemic inflammatory diseases that will aid in preclinical studies, enhance patient selection, and provide surrogate endpoints in clinical trials, thereby improving clinical outcomes. We review the common cardiovascular manifestations of immune-mediated systemic inflammatory diseases and address the clinical and investigational role of advanced multimodality cardiac imaging.


Subject(s)
Cardiovascular Diseases , Heart , Humans , Cardiovascular Diseases/diagnostic imaging , Cardiovascular Diseases/etiology , Cardiac Imaging Techniques , Multimodal Imaging/methods
9.
Tomography ; 9(5): 1711-1722, 2023 09 11.
Article in English | MEDLINE | ID: mdl-37736989

ABSTRACT

BACKGROUND: The E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project is an Italian Network assuring high-quality quantification of tissue iron overload by magnetic resonance imaging (MRI). We evaluated the impact of the COVID-19 pandemic on E-MIOT services. METHODS: The activity of the E-MIOT Network MRI centers in the year 2020 was compared with that of 2019. A survey evaluated whether the availability of MRI slots for patients with hemoglobinopathies was reduced and why. RESULTS: The total number of MRI scans was 656 in 2019 and 350 in 2020, with an overall decline of 46.4% (first MRI: 71.7%, follow-up MRI: 36.9%), a marked decline (86.9%) in the period March-June 2020, and a reduction in the gap between the two years in the period July-September. A new drop (41.4%) was recorded in the period October-December for two centers, due to the general reduction in the total amount of MRIs/day for sanitization procedures. In some centers, patients refused MRI scans for fear of getting COVID. Drops in the MRI services >80% were found for patients coming from a region without an active MRI site. CONCLUSIONS: The COVID-19 pandemic had a strong negative impact on MRI multi-organ iron quantification, with a worsening in the management of patients with hemoglobinopathies.


Subject(s)
COVID-19 , Hemoglobinopathies , Iron Overload , Humans , COVID-19/diagnostic imaging , Pandemics , Hemoglobinopathies/complications , Hemoglobinopathies/diagnostic imaging , Iron Overload/diagnostic imaging , Magnetic Resonance Imaging
10.
J Scleroderma Relat Disord ; 8(3): 169-182, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37744047

ABSTRACT

Introduction: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients. Methods: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters. Results: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10. Conclusion: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice.

11.
Rheumatol Int ; 43(11): 1965-1982, 2023 11.
Article in English | MEDLINE | ID: mdl-37648884

ABSTRACT

The challenges associated with diagnosing and treating cardiovascular disease (CVD)/Stroke in Rheumatoid arthritis (RA) arise from the delayed onset of symptoms. Existing clinical risk scores are inadequate in predicting cardiac events, and conventional risk factors alone do not accurately classify many individuals at risk. Several CVD biomarkers consider the multiple pathways involved in the development of atherosclerosis, which is the primary cause of CVD/Stroke in RA. To enhance the accuracy of CVD/Stroke risk assessment in the RA framework, a proposed approach involves combining genomic-based biomarkers (GBBM) derived from plasma and/or serum samples with innovative non-invasive radiomic-based biomarkers (RBBM), such as measurements of synovial fluid, plaque area, and plaque burden. This review presents two hypotheses: (i) RBBM and GBBM biomarkers exhibit a significant correlation and can precisely detect the severity of CVD/Stroke in RA patients. (ii) Artificial Intelligence (AI)-based preventive, precision, and personalized (aiP3) CVD/Stroke risk AtheroEdge™ model (AtheroPoint™, CA, USA) that utilizes deep learning (DL) to accurately classify the risk of CVD/stroke in RA framework. The authors conducted a comprehensive search using the PRISMA technique, identifying 153 studies that assessed the features/biomarkers of RBBM and GBBM for CVD/Stroke. The study demonstrates how DL models can be integrated into the AtheroEdge™-aiP3 framework to determine the risk of CVD/Stroke in RA patients. The findings of this review suggest that the combination of RBBM with GBBM introduces a new dimension to the assessment of CVD/Stroke risk in the RA framework. Synovial fluid levels that are higher than normal lead to an increase in the plaque burden. Additionally, the review provides recommendations for novel, unbiased, and pruned DL algorithms that can predict CVD/Stroke risk within a RA framework that is preventive, precise, and personalized.


Subject(s)
Arthritis, Rheumatoid , Cardiovascular Diseases , Myocardial Infarction , Stroke , Humans , Artificial Intelligence , Cardiovascular Diseases/diagnosis , Cardiovascular Diseases/etiology , Cardiovascular Diseases/prevention & control , Precision Medicine , Arthritis, Rheumatoid/complications , Stroke/etiology , Stroke/prevention & control , Risk Assessment
12.
Curr Opin Rheumatol ; 35(6): 324-333, 2023 11 01.
Article in English | MEDLINE | ID: mdl-37582056

ABSTRACT

PURPOSE OF REVIEW: To describe the clinical significance of and the diagnostic approach to Raynaud phenomenon (RP) in the peripheral extremities and the heart. RECENT FINDINGS: Nailfold capillaroscopy has recently been standardized in an expert consensus paper. Abnormal capillaroscopy in combination with specific autoantibody profiles and clinical signs are highly predictive of progression of RP to systemic sclerosis (SSc). Magnetic resonance imaging (MRI) can also perform tissue characterization of both the extremities and the heart. Microvascular wall abnormalities detected using nailfold capillaroscopy in patients with SSc may lead to deposition of erythrocyte-derived iron, due to microhemorrhages, which may predispose to fibrosis. MRI can assess the presence of iron using T2∗ measurements. SUMMARY: RP is a hallmark of the microvasculopathy in SSc and can affect both the peripheral extremities and the heart. Nailfold capillaroscopy is the current gold standard for the evaluation of the peripheral microvasculature. Other imaging modalities include thermography, laser Doppler-derived methods, 99m Tc-pertechnetate hand perfusion scintigraphy, power Doppler ultrasonography, dynamic optical coherence tomography, MRI, and photoacoustic imaging, but these are currently not widely used. Cardiac RP can be investigated with positron emission tomography or cardiovascular magnetic resonance, with the latter offering the additional possibility of tissue characterization and iron content quantification secondary to microhemorrhages.


Subject(s)
Raynaud Disease , Scleroderma, Systemic , Humans , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnostic imaging , Raynaud Disease/diagnostic imaging , Raynaud Disease/etiology , Ultrasonography , Heart , Multimodal Imaging , Microscopic Angioscopy/methods
14.
J Pers Med ; 13(5)2023 Apr 25.
Article in English | MEDLINE | ID: mdl-37240894

ABSTRACT

Diabetes mellitus (DM) is a new epidemic which has presented an immense increase in recent decades, due to the rapid increase in obesity. Cardiovascular disease (CVD) significantly reduces life expectancy and is the main cause of death in type 2 diabetes mellitus (T2DM). Strict glycemic control is a well-established method to combat microvascular CVD of type 1 diabetes mellitus (T1DM); its role against CVD of the T2DM risk has not been well documented. Therefore, the most efficient prevention is multifactorial risk factor reduction. Recently, the European Society of Cardiology published its 2019 recommendations on CVD in DM. Although all clinical points were discussed in this document, only a few comments were presented about when and how we should recommend cardiovascular (CV) imaging. Currently, CV imaging is the "must" in CV noninvasive evaluation. Alterations in CV imaging parameters can lead to early recognition of various types of CVD. In this paper, we briefly discuss the role of noninvasive imaging modalities, emphasizing the benefits of including cardiovascular magnetic resonance (CMR) in the evaluation of DM. CMR, in the same examination, can provide an assessment of tissue characterization, perfusion and function, with excellent reproducibility and without radiation or limitations, due to the body habitus. Therefore, it can play a dominant role in the prevention and risk stratification of DM. The suggested protocol for DM evaluation should include routine annual echocardiographic evaluation of all DM patients and CMR assessment of those with poorly controlled DM, microalbuminuria, heart failure, arrhythmia and recent alterations in clinical or echocardiographic evaluation.

15.
Children (Basel) ; 10(5)2023 May 17.
Article in English | MEDLINE | ID: mdl-37238442

ABSTRACT

In this case series, we describe the diagnosis of post-COVID-19 myocarditis in asymptomatic patients with Duchenne Muscular Dystrophy (DMD) and a mild COVID-19 disease course. These patients were referred for CMR due to electrocardiographic and echocardiographic alterations, which did not exist before COVID-19 infection. CMR identified the presence of severe myocardial inflammation in all patients based on abnormally elevated myocardial T2 ratio, late gadolinium enhancement, native T1 mapping, T2 mapping, and extracellular volume fraction. This was paired with concurrent impairment of left ventricular function. Appropriate treatment was initiated in all cases. Two of the four patients developed episodes of ventricular tachycardia during the following 6 months, and a defibrillator was implanted. Despite the mild clinical presentation, this case series demonstrates the diagnostic strength of CMR in the diagnosis and evaluation of post-COVID-19 myocarditis and serves to increase awareness of this potential complication amongst treating physicians.

16.
Life (Basel) ; 13(4)2023 Mar 30.
Article in English | MEDLINE | ID: mdl-37109438

ABSTRACT

Cardiovascular morbidity and mortality are more prevalent in inflammatory arthritis (IA) compared to the general population. Recognizing the importance of addressing this issue, the European League Against Rheumatism (EULAR) published guidelines on cardiovascular disease (CVD) risk management in IA in 2016, with plans to update going forward based on the latest emerging evidence. Herein we review the latest evidence on cardiovascular disease in IA, taking a focus on rheumatoid arthritis, psoriatic arthritis, and axial spondylarthritis, reflecting on the scale of the problem and imaging modalities to identify disease. Evidence demonstrates that both traditional CVD factors and inflammation contribute to the higher CVD burden. Whereas CVD has decreased with the newer anti-rheumatic treatments currently available, CVD continues to remain an important comorbidity in IA patients calling for prompt screening and management of CVD and related risk factors. Non-invasive cardiovascular imaging has been attracting much attention in view of the possibility of detecting cardiovascular lesions in IA accurately and promptly, even at the pre-clinical stage. We reflect on imaging modalities to screen for CVD in IA and on the important role of rheumatologists and cardiologists working closely together.

17.
Int J Cardiol ; 376: 139-146, 2023 04 01.
Article in English | MEDLINE | ID: mdl-36731634

ABSTRACT

BACKGROUND: We evaluated the prevalence of myocardial involvement by native T1 and T2 mapping, the diagnostic performance of mapping in addition to conventional Lake Louise Criteria (LLC), as well as correlations between mapping findings and clinical or conventional cardiovascular magnetic resonance (CMR) parameters in systemic sclerosis (SSc) patients. METHODS: Fifty-five SSc patients (52.31 ± 13.24 years, 81.8% female) and 55 age- and sex-matched healthy subjects underwent clinical, bio-humoral assessment, and CMR. The imaging protocol included: T2-weighted, early post-contrast cine sequences, native T1 and T2 mapping by a segmental approach, and late gadolinium enhancement (LGE) technique. RESULTS: Global myocardial T1 and T2 values were significantly higher in SSc patients than in healthy subjects. An increase in native T1 and/or T2 was present in the 62.1% of patients with normal conventional CMR techniques (negative LGE and T2-weighted images). Respectively, 13.5% and 59.6% of patients fulfilled original and updated LLC (overall agreement = 53.9%). Compared with patients with normal native T1, patients with increased T1 (40.0%) featured significantly higher left ventricular end-diastolic volume index and cardiac index, biventricular stroke volume indexes, and global heart T2 values, and more frequently had a history of digital ulcers. Biochemical and functional CMR parameters were comparable between patients with normal and increased T2 (61.8%). CONCLUSION: T1 and T2 mapping are sensitive parameters that should be included in the routine clinical assessment of SSc patients for detecting early/subclinical myocardial involvement.


Subject(s)
Contrast Media , Scleroderma, Systemic , Humans , Female , Male , Magnetic Resonance Imaging, Cine , Case-Control Studies , Gadolinium , Myocardium/pathology , Predictive Value of Tests , Ventricular Function, Left
18.
J Clin Med ; 12(4)2023 Feb 16.
Article in English | MEDLINE | ID: mdl-36836108

ABSTRACT

INTRODUCTION: Myopathies are heterogeneous neuromuscular diseases of genetic and/or inflammatory etiology that affect both cardiac and skeletal muscle. We investigated the prevalence of cardiac inflammation in patients with myopathies, cardiovascular symptoms, and normal echocardiography using cardiovascular magnetic resonance (CMR). METHODS: We prospectively evaluated 51 patients with various genetic (n = 23) and inflammatory (n = 28) myopathies (median age, IQR: 12 (11-15) years, 22% girls; 61 (55-65) years, 46% women, respectively) and compared their CMR findings to corresponding age- and sex-matched controls (n = 21 and 20, respectively) and to each other. RESULTS: Patients with genetic myopathy had similar biventricular morphology and function to healthy controls but showed higher late gadolinium enhancement (LGE), native T1 mapping, extracellular volume fraction (ECV), and T2 mapping values. Collectively, 22 (95.7%) patients with genetic myopathy had a positive T1-criterion and 3 (13.0%) had a positive T2-criterion according to the updated Lake Louise criteria. Compared with healthy controls, patients with inflammatory myopathy showed preserved left ventricular (LV) function and reduced LV mass, while all CMR-derived tissue characterization indices were significantly higher (p < 0.001 for all). All patients had a positive T1-criterion, and 27 (96.4%) had a positive T2-criterion. A positive T2-criterion or T2-mapping > 50 ms could discriminate between patients with genetic and inflammatory myopathies with a sensitivity of 96.4% and a specificity of 91.3% (AUC = 0.9557). CONCLUSIONS: The vast majority of symptomatic patients with inflammatory myopathies and normal echocardiography show evidence of acute myocardial inflammation. In contrast, acute inflammation is rare in patients with genetic myopathies, who show evidence of chronic low-grade inflammation.

19.
Hellenic J Cardiol ; 70: 53-64, 2023.
Article in English | MEDLINE | ID: mdl-36706867

ABSTRACT

Cardiovascular disease (CVD) is the most common cause of morbidity/mortality worldwide. Early diagnosis is the key to improve CVD prognosis, and cardiovascular imaging plays a crucial role in this direction. Echocardiography is the most commonly used imaging modality. However, the need for early diagnosis/treatment favors the development of modalities providing information about tissue characterization beyond echocardiography. In this context, the rapid evolution of cardiovascular magnetic resonance (CMR) led to the coexistence of cardiologists and radiologists in the CMR field. Our aim was to provide an overview of indications, sequences, and reporting of CMR findings in various CVDs. The indications/limitations of CMR as well as the pathophysiological significance of various sequences in adult/pediatric CVDs are presented and discussed in detail. The role of CMR indices in the evaluation of the most common clinical scenarios in cardiology and their impact on CVD diagnosis/prognosis were analyzed in detail. Additionally, the comparison of CMR versus other imaging modalities is also discussed. Finally, future research directions are presented. CMR can provide cardiac tissue characterization and biventricular/biatrial functional assessment in the same examination, allowing for early and accurate identification of important subclinical abnormalities, before clinically overt CVD takes place.


Subject(s)
Cardiovascular Diseases , Adult , Humans , Child , Cardiovascular Diseases/diagnostic imaging , Magnetic Resonance Imaging/methods , Heart , Echocardiography/methods , Magnetic Resonance Spectroscopy
20.
Semin Arthritis Rheum ; 58: 152126, 2023 02.
Article in English | MEDLINE | ID: mdl-36434895

ABSTRACT

Systemic sclerosis (SSc) presents high morbidity/mortality, due to internal organ fibrosis, including the heart. Cardiac magnetic resonance (CMR) can perform myocardial function and tissue characterization in the same examination. The Lake Louise criteria (LLC) can identify recent myocardial inflammation using CMR. Abnormal values include: (a) myocardial over skeletal muscle ratio in STIRT2-W images >2, (b) early gadolinium enhancement values >4, (c) epicardial/intramyocardial late gadolinium enhancement (LGE). The diagnosis of myocarditis using LLC is considered if 2/3 criteria are positive. Parametric imaging including T2, native T1 mapping and extracellular volume fraction (ECV) has been recently used to diagnose inflammatory cardiomyopathy. According to expert recommendations, myocarditis should be considered if at least 2 indices, one T2 and one T1 parameter are positive, whereas native T1 mapping and ECV assess diffuse fibrosis or oedema, even in the absence of LGE. Moreover, transmural/subendocardial fibrosis following the distribution of coronary arteries and diffuse subendocardial fibrosis not related with epicardial coronary arteries are indicative of epicardial and micro-vascular coronary artery disease, respectively. To conclude, CMR can identify acute/active myocardial inflammation and myocardial infarction using classic and parametric indices in parallel with ventricular function evaluation.


Subject(s)
Myocarditis , Scleroderma, Systemic , Humans , Myocarditis/diagnostic imaging , Contrast Media , Gadolinium , Magnetic Resonance Imaging/methods , Fibrosis , Myocardium/pathology , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnostic imaging , Scleroderma, Systemic/pathology , Magnetic Resonance Spectroscopy , Inflammation/diagnostic imaging , Predictive Value of Tests
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