Cardiac involvement in cystic fibrosis evaluated using cardiopulmonary magnetic resonance

Cystic fibrosis (CF) transmembrane conductance regulator is expressed in myocardium, but cardiac involvement in CF remains poorly understood. The recent development of a combined cardiopulmonary magnetic resonance imaging technology allows for a simultaneous interrogation of cardiac and pulmonary structure and function. The aim of this study was to investigate myocardial manifestations in adults with CF, both in a stable state and during an acute respiratory exacerbation, and to investigate the relationship between cardiac and pulmonary disease. Healthy adult volunteers (n = 12) and adults with CF (n = 10) were studied using a multiparametric cardiopulmonary magnetic resonance protocol. CF patients were scanned during an acute respiratory exacerbation and re-scanned when stable. Stable CF was associated with left ventricular dilatation and hypertrophy (LVH; left ventricular mass: CF 59 ± 9 g/m2 vs. control 50 ± 8 g/m2; p = 0.028). LVH was predominantly driven by extracellular myocardial matrix expansion (extracellular matrix mass: CF 27.5 ± 3.4 g vs. control 23.6 ± 5.2 g; p = 0.006; extracellular volume [ECV]: CF 27.6 [24.7–29.8]% vs. control 24.8 [22.9–26.0]%; p = 0.030). Acute CF was associated with an acute reduction in left ventricular function (ejection fraction: acute 57 ± 3% vs. stable 61 ± 5%; p = 0.025) and there was a suggestion of myocardial oedema. Myocardial oedema severity was strongly associated with the severity of airflow limitation (r = − 0.726, p = 0.017). Multiparametric cardiopulmonary magnetic resonance technology allows for a simultaneous interrogation of cardiac and pulmonary structure and function. Stable CF is associated with adverse myocardial remodelling, including left ventricular systolic dilatation and hypertrophy, driven by myocardial fibrosis. CF exacerbation is associated with acute myocardial contractile dysfunction. There is also a suggestion of myocardial oedema in the acute period which is related to pulmonary disease severity.

Early Myocardial Changes in Patients with Rheumatoid Arthritis without Known Cardiovascular Diseases—A Comprehensive Cardiac Magnetic Resonance Study

Clinically silent cardiac disease is frequently observed in rheumatoid arthritis (RA), and cardiovascular complications are the leading cause of mortality in RA. We sought to evaluate the myocardium of young RA patients without known cardiac disease using cardiac magnetic resonance (CMR), including T1/T2 mapping sequences. Eighteen RA patients (median age 41 years, 83% females) mainly with low disease activity or in remission and without any known cardiovascular disease were prospectively included to undergo CMR. A control group consisted of 10 sex- and age-matched patients without RA or any known structural cardiovascular disease. Heart chambers size and left/right ventricular systolic function were similar in patients with RA and controls. Signs of myocardial oedema were present in up to 39% of RA patients, including T2 time above cut-off value in 7 patients (39%) in comparison to none of the controls (p = 0.003) and T2 signal intensity ratio above the cut-off value in 6 patients (33%) and in none of the controls (p = 0.06). Extracellular volume was similar in both groups signifying a lack of diffuse fibrosis in studied group of RA patients. There were also no signs of late gadolinium enhancement (LGE) in either group except for one patient with RA who was found to have prior silent myocardial infarction. No correlation was found between markers of disease severity and markers of oedema observed on CMR in patients with RA. Nevertheless, patients with increased T2 time (≥50 ms) were more likely to have X-ray erosions (p = 0.02) and a longer duration between symptom onset and diagnosis (p = 0.02). Finally, there were no significant arrhythmias on 24-h ECG Holter monitoring in RA patients. CMR features of myocardial oedema without signs of myocardial fibrosis were found in 39% of young RA patients without known heart disease or cardiac symptoms. Presence of myocardial oedema was associated with X-ray erosions and a longer duration between symptom onset and diagnosis. The clinical significance of the observed early myocardial changes accompanying RA requires additional studies.

646 Acute peri-myocarditis following COVID-19 Pfizer-Biontech vaccine second dose delivery in a male teenager: the good prognosis and unusual ECG

Aims Myocarditis due to COVID-19 mRNA vaccine is an uncommon side effect and the cases seem to have occurred predominantly in young adults under 30 years old. The estimated incidence is 12.6 cases per one million second dose m-RNA vaccine delivery. Methods and results A 17-years-old male was admitted at our department after 18 days COVID-19 Pfizer-BioNtech vaccine second dose delivery with persistent chest pain without respiratory symptoms and, ST-elevation and PR-depression in V3–V6 at the ECG on 3 August 2021. He had no history of heart disease. Physical examination didn’t show anything relevant except for mildly tachycardic heart sounds. In addition blood test showed increase in C-reactive protein, cardiac troponin and N-terminal-pro-B-type natriuretic peptide. An echocardiography showed widespread hypokinesia with reduced left ventricular ejection fraction and highly echogenic pericardium. During the first day cardiac magnetic resonance (CMR) was performed, which showed mild diffuse myocardial oedema on T2-weighted images and T2 mapping and two thin areas of delayed enhancement with non-ischaemic pattern in the lateral wall with involvement of the pericardial sheets confirming peri-myocarditis diagnosis. After 24 h, the ECG showed spread and deep T-waves with QTc prolongation. We performed multiple ECG during the days after to assess morphology changes and QTc. The patient has been asymptomatic for all the hospitalization and on day 7 was performed an echocardiography which describe a full recovery in terms of kinesia and left ventricular ejection fraction. He was discharged asymptomatic with ‘better’ but still negative T-waves and QTc normalization. Two months after discharge CMR was repeated and showed normal left ventricular function without myocardial oedema and pericardial involvement, but with persistent the areas of delayed enhancement with non-ischaemic pattern in the lateral wall. Conclusions In this case report we describe an uncommon COVID-19 m-RNA Vaccine side effect. The first issue is the timing of presentation. On 19 July 2021, AIFA stated that myocarditis is a very uncommon side effect and it usually presents within 14 days after 2nd dose delivery. Our patient was admitted at our department after that time period, probably because we reported the ending part of the myocarditis presented with symptoms of pericarditis; indeed we didn’t report the cardiac troponin plateau but only the descending cardiac troponin wave and we attend a very quick recovery. The second issue in the unique ECG with a very quick evolution (Tako-Tsubo morphology like) which could be characteristic of this kind of Myocarditis. Third, the good progress of the inflammation and quick recovery. Surely is a serious side effect but it’s still less frequent and with better prognosis than COVID-19 Myocarditis. European Medicines Agency (EMA) and Centers for Disease Control and Prevention (CDC) recently stated authorized COVID-19 vaccines advantages are still above risks in all age groups beyond 12 y/o. Why is myocarditis a side effect, Why are adolescent males affected the most and Why is the onset after second dose of m-RNA vaccine are questions still unanswered.

Left ventricular systolic dysfunction with concomitant bradyarrhythmia in a patient with POEMS syndrome: a case report

Background POEMS syndrome (PS) is a paraneoplastic disorder from plasma cell dyscrasia, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Vascular endothelial growth factors (VEGFs)-driven fluid extracellular matrix expansion plays a key role in this condition. Associated cardiac involvement has been sparsely reported thus far. Case summary A 55-year-old woman with PS presented with a pleural effusion and respiratory failure requiring mechanical ventilation. Transthoracic echocardiogram revealed left ventricular (LV) systolic dysfunction with a moderate pericardial effusion. She developed intermittent complete heart block and ventricular standstill, requiring temporary transcutaneous pacing. Further evaluation revealed no significant coronary stenosis on coronary angiogram and cardiac magnetic resonance (CMR) showed elevated T1 and extracellular volume suggestive of myocardial oedema with possible early cardiac infiltration. She had a dual-chamber permanent pacemaker implanted in view of recurrent high-grade heart block. She was initiated on a daratumumab-based chemotherapy regimen prior to discharge. She recovered well subsequently with a promising clinical response to chemotherapy. Discussion We describe the first case of LV systolic dysfunction with concomitant significant bradyarrhythmia in a patient with PS. CMR revealed evidence suggestive of LV myocardial oedema and/or possible early infiltration. VEGF overexpression could explain oedema-related LV dysfunction which reversed with adequate diuresis, as well as damage to the conduction system. Early cardiac amyloidosis, which can be associated with PS, is an important differential diagnosis. Pacemaker implantation, adequate diuresis, and definitive chemotherapy are key to the management of concomitant ventricular myocardial and electrical dysfunction in such rare case.

Outcome of clinical and subclinical myocardial injury in systemic lupus erythematosus – A prospective cohort study

Objectives To determine the outcome of subclinical lupus myocarditis (LM) over twelve months with regards to: mortality; incidence of clinical LM and change in imaging parameters (echocardiography and cardiac magnetic resonance [CMR]). To evaluate the impact of immunosuppression on CMR evidence of myocardial tissue injury. Methods SLE patients with and without CMR evidence of myocardial injury (as per 2009 Lake Louise criteria [LLC]) were included. Analysis at baseline and follow-up included: clinical evaluation, laboratory and imaging analyses (echocardiography and CMR). Clinical LM was defined as clinical features of LM supported by echocardiographic and/or biochemical evidence of myocardial dysfunction. Subclinical LM was defined as CMR myocardial injury without clinical LM. Results Forty-nine SLE patients were included with follow-up analyses (after 12 months) available in 36 patients. Twenty-five patients (51%) received intensified immunosuppressive therapy during follow-up for indications related to SLE. Disease activity (SLEDAI-2K) improved (p < 0.001) from 13 (median;IQR:9–20) to 7 (3–11). One patient without initial CMR evidence of myocardial injury developed clinical LM. Mortality (n = 10) and SLE clinical features were similar between patients with and without initial CMR myocardial injury. Echocardiographic left ventricular ejection fraction (LVEF) (p = 0.014), right ventricular function (p = 0.001) and wall motion abnormalities (p = 0.056) improved significantly but not strain analyses nor the left LV internal diameter index. CMR mass index (p = 0.011) and LVEF (p < 0.001) improved with follow-up but not parameters identifying myocardial tissue injury (LLC). A trend towards a reduction in the presence of CMR criteria was counterbalanced by persistence (n = 7) /development of new criteria (n = 11) in patients. Change in CMR mass index correlated with change in T2-weighted signal (myocardial oedema) (r = 386;p = 0.024). Intensified immunosuppressive therapy had no significant effect on CMR parameters. Conclusion CMR evidence of subclinical LM persisted despite improved SLEDAI-2K, serological markers, cardiac function and CMR mass index. Subclinical LM did not progress to clinical LM and had no significant prognostic implications over 12 months. Immunosuppressive therapy did not have any significant effect on the presence of CMR evidence of myocardial tissue injury. Improvement in CMR mass index correlated with reduction in myocardial oedema and may be used to monitor SLE myocardial injury.

No superiority of ticagrelor over prasugrel in remote myocardial inflammation in patients with acute myocardial infarction with ST elevation: a CMR T1 and T2 mapping study

Background A number of studies suggest that acute myocardial ischaemia triggers a non-specific systemic inflammatory response of remote myocardium through the increase of plasma concentrations of acute-phase proteins, which causes myocardial oedema. As ticagrelor has been shown to significantly decrease the circulating levels of several pro-inflammatory cytokines in patients after acute myocardial infarction with ST elevation (STEMI), we sought to investigate a potential suppressive effect of ticagrelor over prasugrel on cardiac magnetic resonance (CMR) T1 and T2 values in remote myocardium. Methods Ninety patients presenting with acute STEMI were prospectively included and randomised to receive either ticagrelor or prasugrel maintenance treatment after successful primary percutaneous coronary intervention (PPCI). The patients underwent CMR 2–7 days after the PPCI. Studies were done on a 1.5 T clinical scanner, the protocol included long and short axis cine imaging, T1 mapping through the infarct core using a single breath-hold Shortened Modified Look-Locker Inversion Recovery (ShMOLLI), T2 mapping and late gadolinium enhancement imaging. Results After excluding 30 patients due to either missing images or insufficient quality of T1 or T2 maps, 60 patients were included in our analysis. Of those, 29 patients have been randomised to the ticagrelor arm and 31 patients to the prasugrel arm of the study. The mean age at inclusion was 61±10 years, 81.7% of included patients were men, the distribution was even between the two groups. There were no statistically significant differences between groups regarding past medical history and medication prior to the inclusion in the study. CMR scans were performed 5.03±1.96 days after successful PPCI in the ticagrelor group, and 5.10±0.87 days in the prasugrel group. Remote myocardium T1: The mean T1 value of the remote myocardium was 937±27 ms in the ticagrelor group and 936±23 ms in the prasugrel group, showing no statistical difference (p=0.85) between the groups receiving different P2Y12 inhibitor after PPCI. Remote myocardium T2: The mean T2 value of the remote myocardium was 53.8±4.6 ms in the ticagrelor group and 53.6±4.7 ms in the prasugrel group, showing no statistical difference (p=0.86) between compared groups. Both T1 and T2 values of the remote myocardium were above normal values published in literature. Conclusion In patients with STEMI after PPCI, ticagrelor maintenance therapy did not show superiority to prasugrel in preventing early remote myocardial inflammation as assessed by T1 and T2 mapping. Additionally, findings support the premise of remote myocardial oedema following STEMI. Funding Acknowledgement Type of funding source: Other. Main funding source(s): Presented abstract is from a sub-study of the REDUCE-MVI study, which was conducted with financial support from Astra Zeneca through an unrestricted research grant. In addition, the study was financed by the Ministry of Economic Affairs of the Netherlands by means of a PPP Allowance made available by the Top Sector Life Sciences & Health to stimulate public-private partnerships. The first author was awarded the ESC training grant in 2019; this research was conducted during the training for which the grant was awarded.

Multimodality cardiac evaluation in children and young adults with multisystem inflammation associated with COVID-19

Aims Following the peak of the UK COVID-19 epidemic, a new multisystem inflammatory condition with significant cardiovascular effects emerged in young people. We utilized multimodality imaging to provide a detailed sequential description of the cardiac involvement. Methods and Results Twenty consecutive patients (mean age 10.6 ± 3.8 years) presenting to our institution underwent serial echocardiographic evaluation on admission (median day 5 of illness), the day coinciding with worst cardiac function (median day 7), and the day of discharge (median day 15). We performed cardiac computed tomography (CT) to assess coronary anatomy (median day 15) and cardiac magnetic resonance imaging (CMR) to assess dysfunction (median day 20). On admission, almost all patients displayed abnormal strain and tissue Doppler indices. Three-dimensional (3D) echocardiographic ejection fraction (EF) was &lt;55% in half of the patients. Valvular regurgitation (75%) and small pericardial effusions (10%) were detected. Serial echocardiography demonstrated that the mean 3D EF deteriorated (54.7 ± 8.3% vs. 46.4 ± 8.6%, P = 0.017) before improving at discharge (P = 0.008). Left main coronary artery (LMCA) dimensions were significantly larger at discharge than at admission (Z score –0.11 ± 0.87 vs. 0.78 ± 1.23, P = 0.007). CT showed uniform coronary artery dilatation commonly affecting the LMCA (9/12). CMR detected abnormal strain in all patients with global dysfunction (EF &lt;55%) in 35%, myocardial oedema in 50%, and subendocardial infarct in 5% (1/20) patients. Conclusions Pancarditis with cardiac dysfunction is common and associated with myocardial oedema. Patients require close monitoring due to coronary artery dilatation and the risk of thrombotic myocardial infarction.