Becker-Kiener muscular dystrophy (BMD) represents an X-linked genetic disease associated with myocardial involvement potentially resulting in dilated cardiomyopathy (DCM). Early diagnosis of cardiac ...involvement may permit earlier institution of heart failure treatment and extend life span in these patients. Both echocardiography and nuclear imaging methods are capable of detecting later stages of cardiac involvement characterised by wall motion abnormalities. Cardiovascular magnetic resonance (CMR) has the potential to detect cardiac involvement by depicting early scar formation that may appear before onset of wall motion abnormalities.
In a prospective two-center-study, 15 male patients with BMD (median age 37 years; range 11 years to 56 years) underwent comprehensive neurological and cardiac evaluations including physical examination, echocardiography and CMR. A 16-segment model was applied for evaluation of regional wall motion abnormalities (rWMA). The CMR study included late gadolinium enhancement (LGE) imaging with quantification of myocardial damage.
Abnormal echocardiographic results were found in eight of 15 (53.3%) patients with all of them demonstrating reduced left ventricular ejection fraction (LVEF) and rWMA. CMR revealed abnormal findings in 12 of 15 (80.0%) patients (p = 0.04) with 10 (66.6%) having reduced LVEF (p = 0.16) and 9 (64.3%) demonstrating rWMA (p = 0.38). Myocardial damage as assessed by LGE-imaging was detected in 11 of 15 (73.3%) patients with a median myocardial damage extent of 13.0% (range 0 to 38.0%), an age-related increase and a typical subepicardial distribution pattern in the inferolateral wall. Ten patients (66.7%) were in need of medical heart failure therapy based on CMR results. However, only 4 patients (26.7%) were already taking medication based on clinical criteria (p = 0.009).
Cardiac involvement in patients with BMD is underdiagnosed by echocardiographic methods resulting in undertreatment of heart failure. The degree and severity of cardiac involvement in this population is best characterised when state-of-the-art CMR methods are applied. Further studies need to demonstrate whether earlier diagnosis and institution of heart failure therapy will extend the life span of these patients.
Abstract Background The diagnosis of cardiac involvement in rheumatic disorders is challenging due to its varying clinical presentation. Since clinical consequences range from immediate treatment ...changes to adverse long-term outcome, individual risk stratification is of great clinical interest. Primary aim was to evaluate the prevalence of cardiac involvement in patients with different rheumatic disorders using late gadolinium enhancement–cardiac magnetic resonance imaging (LGE-CMR). In addition, we sought to investigate if different rheumatic disorders would demonstrate different LGE patterns. Methods Two-hundred-ninety-seven patients with rheumatic disorders were included and underwent LGE-CMR for work-up of cardiac involvement, which was defined by the presence of LGE in the myocardium. Patients were divided into five subgroups: 1) ANCA-associated vasculitis, 2) non-ANCA-associated vasculitis, 3) connective tissue disorders, 4) arthritis, and 5) sarcoidosis. Results Mean ejection fraction in the overall population was 65%, with a mean age of 55 yrs. Prevalence of cardiac involvement in the five subgroups were as follows: 54% in the ANCA-associated vasculitis group, 22% in the non-ANCA-associated vasculitis group, 14% in the group with connective tissue disorders, 21% in the arthritis group, and 24% in sarcoid patients. Each of the five subgroups demonstrated a distinct pattern of LGE. Conclusion There is a wide range in the prevalence of cardiac involvement in different rheumatic disorders (54%–14%). Different groups of rheumatic disorders demonstrate different patterns of LGE. Condensed abstract Primary aim of the study was to evaluate the presence of cardiac involvement in patients with different rheumatic disorders using LGE-CMR. In addition, we sought to investigate if different rheumatic disorders would reveal different LGE patterns. In our 297 patients, the highest prevalence of cardiac involvement was found in patients with ANCA-associated vasculitis (54%), whereas the lowest prevalence was demonstrated in patients with connective tissue disorders (14%). Furthermore, different groups of rheumatic disorders demonstrate distinct patterns of LGE.
Aims
In the placebo-controlled, double-blind BOne marrOw transfer to enhance ST-elevation infarct regeneration (BOOST) 2 trial, intracoronary autologous bone marrow cell (BMC) transfer did not ...improve recovery of left ventricular ejection fraction (LVEF) at 6 months in patients with ST-elevation myocardial infarction (STEMI) and moderately reduced LVEF. Regional myocardial perfusion as determined by adenosine stress perfusion cardiac magnetic resonance imaging (S-CMR) may be more sensitive than global LVEF in detecting BMC treatment effects. Here, we sought to evaluate (i) the changes of myocardial perfusion in the infarct area over time (ii) the effects of BMC therapy on infarct perfusion, and (iii) the relation of infarct perfusion to LVEF recovery at 6 months.
Methods and results
In 51 patients from BOOST-2 (placebo,
n
= 10; BMC,
n
= 41), S-CMR was performed 5.1 ± 2.9 days after PCI (before placebo/BMC treatment) and after 6 months. Infarct perfusion improved from baseline to 6 months in the overall patient cohort as reflected by the semi-quantitative parameters, perfusion defect–infarct size ratio (change from 0.54 ± 0.20 to 0.43 ± 0.22;
P
= 0.006) and perfusion defect–upslope ratio (0.54 ± 0.23 to 0.68 ± 0.22; P < 0.001), irrespective of randomised treatment. Perfusion defect–upslope ratio at baseline correlated with LVEF recovery (
r
= 0.62;
P
< 0.001) after 6 months, with a threshold of 0.54 providing the best sensitivity (79%) and specificity (74%) (area under the curve, 0.79; 95% confidence interval, 0.67–0.92).
Conclusion
Infarct perfusion improves from baseline to 6 months and predicts LVEF recovery in STEMI patients undergoing early PCI. Intracoronary BMC therapy did not enhance infarct perfusion in the BOOST-2 trial.
Graphic abstract
Cardiovascular magnetic resonance (CMR) has favorable characteristics for diagnostic evaluation and risk stratification of patients with known or suspected CAD. CMR utilization in CAD detection is ...growing fast. However, data on its cost-effectiveness are scarce. The goal of this study is to compare the costs of two strategies for detection of significant coronary artery stenoses in patients with suspected coronary artery disease (CAD): 1) Performing CMR first to assess myocardial ischemia and/or infarct scar before referring positive patients (defined as presence of ischemia and/or infarct scar to coronary angiography (CXA) versus 2) a hypothetical CXA performed in all patients as a single test to detect CAD.
A subgroup of the European CMR pilot registry was used including 2,717 consecutive patients who underwent stress-CMR. From these patients, 21% were positive for CAD (ischemia and/or infarct scar), 73% negative, and 6% uncertain and underwent additional testing. The diagnostic costs were evaluated using invoicing costs of each test performed. Costs analysis was performed from a health care payer perspective in German, United Kingdom, Swiss, and United States health care settings.
In the public sectors of the German, United Kingdom, and Swiss health care systems, cost savings from the CMR-driven strategy were 50%, 25% and 23%, respectively, versus outpatient CXA. If CXA was carried out as an inpatient procedure, cost savings were 46%, 50% and 48%, respectively. In the United States context, cost savings were 51% when compared with inpatient CXA, but higher for CMR by 8% versus outpatient CXA.
This analysis suggests that from an economic perspective, the use of CMR should be encouraged as a management option for patients with suspected CAD.
Aims
Increased high-sensitive cardiac troponin I (hs-cTnI) levels are common in patients with acute ischemic stroke. However, only a minority demonstrates culprit lesions on coronary angiography, ...suggesting other mechanisms, e.g., inflammation, as underlying cause of myocardial damage. Late Gadolinium Enhancement (LGE)-cardiac magnetic resonance (CMR) with mapping techniques T1, T2, extracellular volume (ECV) allow the detection of both focal and diffuse myocardial abnormalities. We investigated the prevalence of culprit lesions by coronary angiography and myocardial tissue abnormalities by a comprehensive CMR protocol in troponin-positive stroke patients.
Methods and results
Patients with troponin-positive acute ischemic stroke and no history of coronary artery disease were prospectively enrolled. Coronary angiography and CMR (LGE, T1 + T2 mapping, ECV) were performed within the first days of the acute stroke. Twenty-five troponin-positive patients (mean age 62 years, 44% females) were included. 2 patients (8%) had culprit lesions on coronary angiography and underwent percutaneous coronary intervention. 13 patients (52%) demonstrated LGE: (i)
n
= 4 ischemic, (ii)
n
= 4 non-ischemic, and (iii)
n
= 5 ischemic AND non-ischemic. In the 12 LGE-negative patients, mapping revealed diffuse myocardial damage in additional 9 (75%) patients, with a high prevalence of increased T2 values.
Conclusions
Our data show a low prevalence of culprit lesions in troponin-positive stroke patients. However, > 50% of the patients demonstrated myocardial scars (ischemic + non-ischemic) by LGE-CMR. Mapping revealed additional myocardial abnormalities (mostly inflammatory) in the majority of LGE-negative patients. Therefore, a comprehensive CMR protocol gives important insights in the etiology of troponin which might have implications for the further work-up of troponin-positive stroke patients.
A coronary-pulmonary artery fistula with giant aneurysmal dilatation is an extremely rare clinical constellation. The natural course of this disease and the incidence of complications are unknown. ...Hence, optimal treatment, particularly in asymptomatic patients, is still a matter of
debate. Here we report a case of a 71-year-old asymptomatic woman with a diastolic murmur. Comprehensive cardiovascular assessments including cardiac computed tomography and invasive coronary angiography revealed a coronary-pulmonary artery fistula with giant aneurysmal dilatation. The patient
was managed conservatively and has now been followed up for 5 years without any events.
Prognosis in Myocarditis Mahrholdt, Heiko; Greulich, Simon
Journal of the American College of Cardiology,
10/2017, Letnik:
70, Številka:
16
Journal Article
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