Background and Objectives: Over the past decade, there has been increasing attention paid to advanced and innovative cardiovascular magnetic resonance (CMR) modalities, such as T1 and T2 mapping, ...which play a major role in diagnosing diffuse myocardial disease. There is little data summarizing the current evidence regarding the diagnostic accuracy of T1 and T2 mapping, and extracellular volume (ECV) in acute myocarditis. The aim of our study was to select, analyze, and systematically review the recent scientific literature on the diagnostic value of CMR T1 and T2 parametric mapping in clinically suspected acute myocarditis. Materials and Methods: The literature search was performed in the PubMed database. Articles published in the years 2014–2024 were included in the analysis. At the initial stage, 458 articles were reviewed, and 13 exploratory research studies were further analyzed and presented in this systematic literature review. Results: The analysis included 686 patients with clinically suspected myocarditis and 372 subjects in the control group. The average age of patients with suspected myocarditis was 40.25 years; 26% of them were women. Prolonged native myocardial T1 relaxation time provides diagnostic accuracy in the setting of suspected acute myocarditis ranging from 69 to 99%, with sensitivity from 64 to 98% and specificity from 87 to 100%. Diagnostic accuracy of prolonged T2 relaxation time ranges from 47 to 87%, with sensitivity being from 48% to 94% and specificity from 60% to 92%. ECV alone showed moderate diagnostic performance, with diagnostic accuracy ranging from 62% to 76%, sensitivity from 47% to 73%, and specificity from 76% to 90%. T1 and T2 mapping and ECV, combined with the late gadolinium enhancement (LGE) technique, increases the probability of detecting myocardial inflammatory changes at various stages of the disease, improving the diagnostic accuracy to 96%. Conclusions: New quantitative CMR techniques, i.e., T1 and T2 mapping, have an advantage over conventional CMR sequences in detecting inflammatory myocardial structural changes and play an important role in diagnosing acute myocarditis. Incorporating these sequences in daily clinical practice increases the diagnostic value of CMR in acute myocarditis and becomes an alternative to endomyocardial biopsy, which has been considered the gold standard until now.
Primary cardiac lymphoma is one of the rarest tumours of the heart. It belongs to the extra-nodal non-Hodgkin's lymphomas. The most common type of this tumour is diffuse large B cell lymphoma. ...Usually, right atrium and right ventricle are involved. This tumour is fatal unless diagnosed and treated in time. In this article two female patients who were diagnosed with primary cardiac lymphoma and treated at our clinic are described. The first patient went to remission after the treatment, while the second patient died. The goals of this article are to show the difficulties of diagnosing and treating this disease, the role of cardiac surgery in its treatment and to raise awareness of this disease.
In this article two female patients who were diagnosed with primary cardiac lymphoma and treated at our clinic are described. The first patient went to remission after the treatment, while the second patient died.
Primary cardiac lymphoma is a very rare disease. At the moment the most effective treatment is chemotherapy. Palliative surgery may be necessary to correct hemodynamics when venous blood flow to the lungs is disturbed.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
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Epicardial adipose tissue (EAT) is shown to be an important factor in the development of coronary artery disease, but numerous pathophysiological mechanisms of its action are still only partially ...understood. There is a lack of studies on its association with different grades of essential hypertension (EH). Therefore, we aimed to evaluate the association between size of EAT depots and the risk of EH taking into account its grade.
Non-obese adult patients with various cardiovascular diseases were investigated: 157 of them had essential hypertension and 101 did not. Hypertensive patients were assigned to three groups according to the grade of hypertension. EAT volume and thickness on ventricular free walls (6 locations) and grooves (5 locations) were measured using cardiac magnetic resonance imaging and compared between groups. A regression model for the prediction of EH was constructed.
In general, thickness (in all locations) and volume of EAT depots was greater among hypertensive patients than in normotensive (NORM) group. Mean EAT thickness in all 11 locations and EAT volume were lower in NORM than in grade 1 hypertension group; similarly, EAT volume was lower in grade 1 than in grade 2 hypertension group. EAT accumulation did not differ between grade 2 and severe hypertension groups. EAT volume, dyslipidaemia status, body mass index, and age were independent predictors for EH in regression model.
EAT accumulation is larger among hypertensive than normotensive individuals. Measurement of EAT depots could be beneficial for identification of hypertensive patients and prediction of hypertension severity.
The original version of this article, published on 12 August 2019, unfortunately contained a mistake. The funding note was incorrect; the correct funding note is given below.
H-bond clustering of water molecules confined in an argon matrix and in nano-structured calcium hydroxyapatites (Ca10(PO4)6(OH)2, CaHA) was studied by means of FTIR and 1H MAS NMR spectroscopy. FTIR ...spectra of water in matrix were measured from T=9K to 40K stepping by 2K with sequent 2D correlation analysis (2DCOR) carried out in the range of O–H stretching vibrations 3000–3800cm−1. The peaks of monomer, dimer and the H-bond clusters from trimer to hexamer as well as the surface (dangling) modes were resolved. Some peaks in 2DCOR spectra drop out from the water clustering scheme. They were related to the enhancement of vibration–rotation motion due to increasing temperature and volume of the cavities in argon matrix. Water clusters were revealed in nano-CaHAs in FTIR spectra for the first time. Beside the sharp peak at 3570cm−1 of the stretching vibration of O–H− ion in the bulk, the bands of water dimer, tetramer, hexamer and several surface O–H and P–O–H modes were resolved at 2900–3900cm−1. They were assigned comparing with the spectra of water in argon matrix and in hydrophobic solvent (CCl4). The cluster structure and the size depends on the hydration level. Hexamer- or even higher structures were observed only in the samples containing significant amount of adsorbed water. The amount of adsorbed water in the studied nano-CaHAs was probed using the intensity of the broad 1H signal at ca 5.1ppm normalized respect to OH– peak at 0ppm. Two narrow signals observed in 1H MAS NMR spectra at 0.8 and 1.3ppm are originated from the surface structured H2O molecules. The NMR data perfectly correlate with those obtained by FTIR spectroscopy.
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•2D correlation analysis of FTIR spectra of water in argon matrix was carried out.•Water clusters and surface modes were revealed in nano-structured hydroxyapatites.•Amount of adsorbed- and surface structured water was probed using 1H MAS NMR.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPUK, ZRSKP
Abstract Tatton-Brown–Rahman syndrome (TBRS) is a rare congenital genetic disorder caused by autosomal dominant pathogenic variants in the DNA methyltransferase DNMT3A gene. Typical TBRS clinical ...features are overgrowth, intellectual disability, and minor facial anomalies. However, since the syndrome was first described in 2014, a widening spectrum of abnormalities is being described. Cardiovascular abnormalities are less commonly reported but can be a major complication of the syndrome. This article describes a family of three individuals diagnosed with TBRS in adulthood and highlights the variable expression of cardiovascular features. A 34-year-old proband presented with progressive aortic dilatation, mitral valve (MV) regurgitation, left ventricular (LV) dilatation, and ventricular arrhythmias. The affected family members (mother and brother) were diagnosed with MV regurgitation, LV dilatation, and arrhythmias. Exome sequencing and computational protein analysis suggested that the novel familial DNMT3A mutation Ser775Tyr is located in the methyltransferase domain, however, distant from the active site or DNA-binding loops. Nevertheless, this bulky substitution may have a significant effect on DNMT3A protein structure, dynamics, and function. Analysis of peripheral blood cfDNA and transcriptome showed shortened mononucleosome fragments and altered gene expression in a number of genes related to cardiovascular health and of yet undescribed function, including several lncRNAs. This highlights the importance of epigenetic regulation by DNMT3A on cardiovascular system development and function. From the clinical perspective, we suggest that new patients diagnosed with congenital DNMT3A variants and TBRS require close examination and follow-up for aortic dilatation and valvular disease because these conditions can progress rapidly. Moreover, personalized treatments, based on the specific DNMT3A variants and the different pathways of their function loss, can be envisioned in the future.
Myocardial fibrosis in aortic stenosis is associated with worse survival following aortic valve replacement. We assessed myocardial fibrosis in severe AS patients, integrating echocardiographic, ...cardiovascular magnetic resonance (CMR) and histological data. A total of 83 severe AS patients (age 66.4 ± 8.3, 42% male) who were scheduled for surgical AVR underwent CMR with late gadolinium enhancement and T1 mapping and global longitudinal strain analysis. Collagen volume fraction was measured in myocardial biopsies (71) that were sampled at the time of AVR. Results. CVF correlated with imaging and serum biomarkers of LV systolic dysfunction and left side chamber enlargement and was higher in the sub-endocardium compared with midmyocardium (
p
<0.001). CVF median values were higher in LGE-positive versus LGE-negative patients 28.7% (19–33) vs 20.7% (15–30), respectively,
p
=0.040. GLS was associated with invasively (CVF; r=−0.303,
p
=0.013) and non-invasively (native T1; r=−0.321,
p
<0.05) measured myocardial fibrosis. GLS and native T1 correlated with parameters of adverse LV remodelling, systolic and diastolic dysfunction and serum biomarkers of heart failure and myocardial injury. Conclusion. Our data highlight the role of myocardial fibrosis in adverse cardiac remodelling in AS. GLS has potential as a surrogate marker of myocardial fibrosis, and high native T1 and low GLS values differentiated patients with more advanced cardiac remodelling.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Adverse cardiac remodeling with a myocardial fibrosis as a key pathophysiologic component may be associated to worse survival in aortic stenosis (AS) patients. Therefore, with the application of ...advanced cardiac imaging we aim to investigate left ventricular myocardial fibrosis in severe AS patients undergoing aortic valve replacement (AVR) and determine its impact with post-intervention clinical outcomes.
In a prospective, observational, cohort study patients with severe AS scheduled either for surgical or transcatheter AVR will be recruited from two tertiary heart centers in Denmark and Lithuania. All patients will receive standard of care in accordance with the current guidelines and will undergo additional imaging testing before and after AVR: echocardiography with deformation analysis and cardiovascular magnetic resonance (CMR) with T1 parametric mapping. Those undergoing surgical AVR will also have a myocardial biopsy sampled at the time of a surgery for histological validation. Patients will be recruited over a 2-year period and followed up to 2 years to ascertain clinical outcomes. Follow-up CMR will be performed 12 months following AVR, and echocardiography with deformation analysis will be performed 3, 12, and 24 months following AVR. The study primary outcome is a composite of all-cause mortality and major adverse cardiovascular events.
Despite continuous effort of research community there is still a lack of early predictors of left ventricular decompensation in AS, which could improve patient risk stratification and guide the optimal timing for aortic valve intervention, before irreversible left ventricular damage occurs. Advanced cardiac imaging and CMR derived markers of diffuse myocardial fibrosis could be utilized for this purpose. FIB-AS study is intended to invasively and non-invasively assess diffuse myocardial fibrosis in AS patients and investigate its prognostic significance in post-interventional outcomes. The results of the study will expand the current knowledge of cardiac remodeling in AS and will bring additional data on myocardial fibrosis and its clinical implications following AVR.
The study has full ethical approval and is actively recruiting patients. The results will be disseminated through scientific journals and conference presentations.
ClinicalTrials.govNCT03585933. Registered on 02 July 2018.
The optimal timing for aortic valve replacement (AVR) in aortic stenosis (AS) is still controversial and may be guided by markers of adverse left ventricular (LV) remodeling. We aim to assess ...electrocardiographic (ECG) strain in relation to LV remodeling and myocardial fibrosis. 83 severe AS patients underwent surgical AVR, with preoperative 12-lead ECG, cardiovascular magnetic resonance with T1 mapping and echocardiography with global longitudinal strain analysis. Collagen volume fraction (CVF) was measured in myocardial biopsies sampled during AVR. Patients with ECG strain had more severe AS, more advanced LV remodeling and evidence of heart failure. Patients with ECG strain had more diffuse fibrosis, as evident by higher mean native T1 values (974.8 ± 34 ms vs. 946.5 ± 28 ms, p < 0.001). ECG strain was the only predictor of increased LV mass index on multivariate regression analysis (OR = 7.10, 95% CI 1.46–34.48, p = 0.02). Patients with persistent ECG strain at 1 year following AVR had more advanced LV remodeling and more histological fibrosis (CVF 12.5% vs. 7.3%, p = 0.009) at baseline assessment. Therefore, ECG strain is a marker of adverse LV remodeling and interstitial myocardial fibrosis. Lack of improvement in ECG strain following AVR indicates more advanced baseline LV injury and higher levels of myocardial fibrosis.
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