Autopsy and Cardiac Magnetic Resonance Image Case of Bevacizumab-Related Cardiomyopathy.
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SUBMITTER: Hashimoto N
PROVIDER: S-EPMC9318594 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Takotsubo cardiomyopathy (TC) is a reversible condition in which there is transient left ventricular (LV) dysfunction characterised most commonly by basal hyperkinesis and mid-apical LV ballooning and hypokinesia. It is said to be triggered by stress and mimics, such as acute coronary syndrome (ACS) clinically. Diagnosis is usually suspected on echocardiography due to the characteristic contraction pattern in a patient with symptoms and signs of ACS but normal coronary arteries on catheter angiography. Cardiac magnetic resonance (CMR), with its latest advancements, is the diagnostic modality of choice for diagnosis, prognosis and follow-up of patients. The advances in CMR (including T1, T2, ECV mapping and threshold-based late gadolinium enhancement (LGE) measurements have revolutionised the role of CMR in tissue characterisation and prognostication in patients with TC. In this review, we highlight the current role of CMR in management of TC and enumerate the CMR findings in TC as well the current advances in the field of CMR, which could help in prognosticating these patients.
| S-EPMC8519651 | biostudies-literature
Project description:Myocardial fibrosis detected by cardiac magnetic resonance (CMR) has been reported in patients with desmin-related myopathy, although its characteristics remain unclear. Here, we describe a case of desmin-related restrictive cardiomyopathy wherein CMR imaging revealed myocardial oedema, ischaemia, and fibrosis in the left ventricle; the different types and processes of myocardial injury were detected by CMR. Middle wall left ventricular enhancement may be a feature of late gadolinium enhancement, and the lateral wall is often involved in cases of myocardial injury. CMR is useful for the early detection of cardiac involvement and the prediction of prognosis in patients diagnosed with desmin-related myopathy.
| S-EPMC8712804 | biostudies-literature
Project description:BackgroundHypertrophic cardiomyopathy (HCM) is a genetic cardiomyopathy with a prevalence of about 1:200. It is characterized by left ventricular hypertrophy, diastolic dysfunction and interstitial fibrosis; HCM might lead to sudden cardiac death (SCD) especially in the young. Due to low autopsy frequencies of sudden unexplained deaths (SUD) the true prevalence of SCD and especially of HCM among SUD remains unclear. Even in cases of proven SCD genetic testing is not a routine procedure precluding appropriate risk stratification and counseling of relatives.MethodsHere we report a case of SCD in a 19-year-old investigated by combined forensic and molecular autopsy.ResultsDuring autopsy of the index-patient HCM was detected. As no other possible cause of death could be uncovered by forensic autopsy the event was classified as SCD. Molecular autopsy identified two (probably) pathogenic genetic variants in FHL1 and MYBPC3. The MYBPC3 variant had an incomplete penetrance. The FHL1 variant was a de novo mutation. We detected reduced FHL1 mRNA levels and no FHL1 protein in muscle samples suggesting nonsense-mediated mRNA decay and/or degradation of the truncated protein in the SCD victim revealing a plausible disease mechanism.ConclusionThe identification of the genetic cause of the SCD contributed to the rational counseling of the relatives and risk assessment within the family. Furthermore our study revealed evidences for the pathomechanism of FHL1 mutations.
| S-EPMC6687666 | biostudies-literature
Project description:BackgroundTakotsubo cardiomyopathy (TCM) is a reversible acute cardiac disease that mimics acute myocardial infarction (AMI). In patients with a history of an old myocardial infarction who develop TCM, cardiac magnetic resonance imaging (CMRI) is the first-line non-invasive diagnostic tool for clearly discriminating between AMI and TCM.Case summaryA 65-year-old woman who suffered a myocardial infarction in 2012, was admitted to undergo sigmoid colon tumour surgery in January 2017. Soon after surgery, she developed angina associated with ST-segment elevation, as shown by electrocardiography and increased cardiac enzyme levels. Acute coronary syndrome was diagnosed. She had a good clinical response to treatment with intravenous nitroglycerine, opioids, clopidogrel, aspirin, bisoprolol, and atorvastatin. Transthoracic echocardiography (TTE) showed anteroseptal and apical left ventricular akinesia with balloon-like dilatation, akinesia of the inferior wall and inferior interventricular septum, and global systolic dysfunction. Coronary angiography did not identify coronary artery lesions, and a tentative diagnosis of TCM was made. Cardiac magnetic resonance imaging confirmed the segmental contraction abnormalities seen in the TTE and identified myocardial oedema without delayed hyperenhancement after gadolinium administration, which confirmed the diagnosis of TCM and ruled out acute ischaemic damage. She was discharged when asymptomatic. Cardiac magnetic resonance imaging at 2 months showed disappearance of the anteroseptal and apical contraction abnormality, regression of myocardial oedema and normalization of global left ventricular systolic function.DiscussionThis case confirms that CMRI is a non-invasive diagnostic method for accurately differentiating between AMI and TCM in patients with a prior myocardial infarction. cardiac magnetic resonance imaging should be incorporated as a diagnostic criterion for TCM.
| S-EPMC6439425 | biostudies-literature
Project description:Background Phenylketonuria is the most common inborn error of amino acid metabolism, where oxidative stress and collateral metabolic abnormalities are likely to cause cardiac structural and functional modifications. We aim herein to characterize the cardiac phenotype of adult subjects with phenylketonuria using advanced cardiac imaging. Methods and Results Thirty-nine adult patients with phenylketonuria (age, 30.5±8.7 years; 10-year mean phenylalanine concentration, 924±330 µmol/L) and 39 age- and sex-matched healthy controls were investigated. Participants underwent a comprehensive cardiac magnetic resonance and echocardiography examination. Ten-year mean plasma levels of phenylalanine and tyrosine were used to quantify disease activity and adherence to treatment. Patients with phenylketonuria had thinner left ventricular walls (septal end-diastolic thickness, 7.0±17 versus 8.8±1.7 mm [P<0.001]; lateral thickness, 6.1±1.4 versus 6.8±1.2 mm [P=0.004]), more dilated left ventricular cavity (end-diastolic volume, 87±14 versus 80±14 mL/m2 [P=0.0178]; end-systolic volume, 36±9 versus 29±8 mL/m2 [P<0.001]), lower ejection fraction (59±6% versus 64±6% [P<0.001]), reduced systolic deformation (global circumferential strain, -29.9±4.2 % versus -32.2±5.0 % [P=0.027]), and lower left ventricular mass (38.2±7.9 versus 47.8±11.0 g/m2 [P<0.001]). T1 native values were decreased (936±53 versus 996±26 ms [P<0.001]), with particular low values in patients with phenylalanine >1200 µmol/L (909±48 ms). Both mean phenylalanine (P=0.013) and tyrosine (P=0.035) levels were independently correlated with T1; and in a multiple regression model, higher phenylalanine levels and higher left ventricular mass associate with lower T1. Conclusions Cardiac phenotype of adult patients with phenylketonuria reveals some traits of an early-stage cardiomyopathy. Regular cardiology follow-up, tighter therapeutic control, and prophylaxis of cardiovascular risk factors, in particular dyslipidemia, are recommended.
| S-EPMC8649272 | biostudies-literature
Project description:Background:Takotsubo cardiomyopathy (TTS) is an extremely rare complication of fluorouracil containing chemotherapy regimes such as FOLFOX used for colorectal cancer, occurring in only five previous case reports. Due to its potentially fatal outcomes, yet infrequent presence in the literature, it is worthwhile reviewing the clinical features and outcomes of this phenomenon. Case summary:A 54-year-old lady was admitted with cardiogenic shock. A cardiac magnetic resonance imaging (CMR) showed mid-ventricle to apical hypokinesis and confirmed TTS. She was managed with inotropes and non-invasive ventilation after which she recovered fully both clinically and in her CMR features 6?weeks following discharge. Discussion:This is the first case showing the acute CMR features of this complication and highlights the need for awareness of this rarely occurring cardiotoxicity. It also shows the potentially fatal phenomenon can be fully reversible when diagnosed and managed promptly even in patients with metastatic cancer and critical illness.
| S-EPMC6939794 | biostudies-literature
Project description:The relationship between circulating fibrosis-related molecules and magnetic resonance-assessed cardiac fibrosis in dilated cardiomyopathy (DCM) is poorly understood. To compare circulating biomarkers between DCM patients with high and low fibrosis burdens, we performed a prospective, single-center, observational study. The study population was composed of 100 DCM patients (87 male, mean age 45.2 ± 11.8 years, mean ejection fraction 29.7% ± 10.1%). Replacement fibrosis was quantified by means of late gadolinium enhancement (LGE), whereas interstitial fibrosis was assessed via extracellular volume (ECV). Plasma concentrations of cardiotrophin-1, growth differentiation factor-15, platelet-derived growth factor, procollagen I C-terminal propeptide, procollagen III N-terminal propeptide, and C-terminal telopeptide of type I collagen were measured. There were 44% patients with LGE and the median ECV was 27.7%. None of analyzed fibrosis serum biomarkers were associated with the LGE or ECV, whereas NT-proBNP was independently associated with both LGE and ECV, and troponin T was associated with ECV. None of the circulating fibrosis markers differentiated between DCM patients with and without replacement fibrosis, or patients stratified according to median ECV. However, cardiac-specific markers, such as NT-proBNP and hs-TnT, were associated with fibrosis. Levels of circulating markers of fibrosis seem to have no utility in the diagnosis and monitoring of cardiac fibrosis in DCM.
| S-EPMC8224556 | biostudies-literature
Project description:The application of magnetic resonance image (MRI)-guided brachytherapy has demonstrated significant growth during the past 2 decades. Clinical improvements in cervix cancer outcomes have been linked to the application of repeated MRI for identification of residual tumor volumes during radiotherapy. This has changed clinical practice in the direction of individualized dose administration, and resulted in mounting evidence of improved clinical outcome regarding local control, overall survival as well as morbidity. MRI-guided prostate high-dose-rate and low-dose-rate brachytherapies have improved the accuracy of target and organs-at-risk delineation, and the potential exists for improved dose prescription and reporting for the prostate gland and organs at risk. Furthermore, MRI-guided prostate brachytherapy has significant potential to identify prostate subvolumes and dominant lesions to allow for dose administration reflecting the differential risk of recurrence. MRI-guided brachytherapy involves advanced imaging, target concepts, and dose planning. The key issue for safe dissemination and implementation of high-quality MRI-guided brachytherapy is establishment of qualified multidisciplinary teams and strategies for training and education.
| S-EPMC4147854 | biostudies-literature
Project description:AimsSudden cardiac death (SCD) is an appalling complication of hypertrophic cardiomyopathy (HCM). There is an ongoing discussion about the optimal SCD risk stratification strategy since established SCD risk models have suboptimal discriminative power. The aim of this study was to evaluate the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for SCD risk stratification compared to the European Society of Cardiology (ESC) SCD risk score and traditional risk factors in an >10-year follow-up.Methods and resultsTwo hundred and twenty consecutive patients with HCM and LGE-CMR were enrolled. Follow-up data were available in 203 patients (median age 58 years, 61% male) after a median follow-up period of 10.4 years. LGE was present in 70% of patients with a median LGE amount of 1.6%, the median ESC 5-year SCD risk score was 1.84. In the overall cohort, SCD rates were 2.3% at 5 years, 4.8% at 10 years, and 15.7% at 15 years, independent from established risk models. An LGE amount of >5% left ventricular (LV) mass portends the highest risk for SCD with SCD prevalences of 5.5% at 5 years, 13.0% at 10 years, and 33.3% at 15 years. Conversely, patients with no or ≤5% LGE of LV mass have favourable prognosis.ConclusionsLGE-CMR in HCM patients allows effective 10-year SCD risk stratification beyond established risk factors. LGE amount might be added to established risk models to improve its discriminatory power. Specifically, patients with >5% LGE should be carefully monitored and might be adequate candidates for primary prevention implantable cardioverter-defibrillator during the clinical long-term course.
| S-EPMC8219365 | biostudies-literature
Project description:ObjectivesCardiomyopathy is a key factor in accelerated cardiovascular mortality in haemodialysis (HD) patients. We aimed to phenotype cardiac and vascular dysfunction by tagged cardiovascular magnetic resonance (CMR) imaging in patients recently commencing HD.MethodsFifty-four HD patients and 29 age and sex-matched controls without kidney disease were studied. Left ventricular (LV) mass, volumes, ejection fraction (EF), concentric remodelling, peak-systolic circumferential strain (PSS), peak diastolic strain rate (PDSR), LV dyssynchrony, aortic distensibility and aortic pulse wave velocity were determined.ResultsGlobal systolic function was reduced (EF 51?±?10%, HD versus 59?±?5%, controls, p?<?0.001; PSS 15.9?±?3.7% versus 19.5?±?3.3%, p?<?0.001). Diastolic function was decreased (PDSR 1.07?±?0.33s(-1) versus 1.31?±?0.38s(-1), p?=?0.003). LV mass index was increased (63[54,79]g/m(2) versus 46[42,53]g/m(2), p?<?0.001). Anteroseptal reductions in PSS were apparent. These abnormalities remained prevalent in the subset of HD patients with preserved EF >50% (n?=?35) and the subset of HD patients without diabetes (n?=?40). LV dyssynchrony was inversely correlated to diastolic function, EF and aortic distensibility. Diastolic function was inversely correlated to LV dyssynchrony, concentric remodelling, age and aortic pulse wave velocity.ConclusionPatients new to HD have multiple cardiac and aortic abnormalities as characterised by tagged CMR. Cardio-protective interventions are required from initiation of therapy.Key points• First characterisation of cardiomyopathy by tagged CMR in haemodialysis patients. • Diastolic function was correlated to LV dyssynchrony, concentric remodelling and aortic PWV. • Reductions in strain localised to the septal and anterior wall. • Bioimpedance measures were unrelated to LV strain, suggesting volume-independent pathogenetic mechanisms. • Multiple abnormalities persisted in the HD patient subset with preserved EF or without diabetes.
| S-EPMC4927657 | biostudies-literature