BACKGROUND:Pathogenic heterozygous desmoplakin (DSP) variants cause arrhythmogenic cardiomyopathy, predisposing to sudden cardiac death, and occasionally are found in dermatology patients with palmoplantar epidermal differentiation disorders (pEDDs) and curly/woolly hair. DSP variants of uncertain significance (VUS) in patients with pEDD complicate cardiac risk assessment. OBJECTIVES:To characterize cardiocutaneous phenotypes associated with heterozygous DSP variants. METHODS:We enrolled 45 heterozygous DSP carriers [aged 2-80 years; 18 probands followed for cardiomyopathy (n = 16) or pEDD (n = 2) and 27 family members] and 10 family members who were noncarriers from 18 families at Helsinki University Hospital, Finland. Genetic, cardiac and dermatological evaluations included next-generation sequencing panels, whole exome or Sanger sequencing, laboratory tests, electrocardiography, echocardiography, cardiac magnetic resonance imaging, skin histology, immunohistochemistry and hair microscopy. RESULTS:Seventeen DSP variants (10 pathogenic/likely pathogenic, 7 VUS) were identified. Fifteen were newly associated with pEDD, including six also new for cardiomyopathy. Characteristic focal hyperkeratosis around heel rims and outer edges of soles (DSP-pEDD) was observed in 86% (36/42) of carriers, accompanied by palmar hyperkeratosis (36%, 15/42), pEDD-related pain (59%, 16/27), aquagenic whitening (58%, 19/33) and curly/wavy hair (57%, 24/42). Cardiac abnormalities occurred in 72% (31/43), with 60% (26/43) meeting cardiomyopathy criteria, 44% (19/43) exhibiting arrhythmias, and 16% (7/43) requiring resuscitation. VUS-associated phenotypes were similar to pathogenic/likely pathogenic variants. Onset of pEDD (median 13 years, range 1.5-70 years) preceded cardiomyopathy by three decades. Cardiac abnormalities affected 10% (3/29) of adults with pEDD by age 30, 52% (15/29) by age 50 and 83% (24/29) by age 70 years. CONCLUSIONS:Heterozygous DSP variants cause childhood-onset focal pEDD preceding midlife-onset arrhythmogenic cardiomyopathy. Genetic testing is essential in pEDD and cardiac evaluation in patients with DSP variants.

The AAMS open-label clinical study demonstrated the safety and feasibility of epicardial transplantation of autologous right atrial appendage micrografts (AAMs) during coronary artery bypass grafting (CABG) surgery. The study also provided the first indications of therapeutic efficacy of the AAMs, as delivered within an extracellular matrix patch, to reduce ischemic scar and increase viable ventricular wall thickness. To further evaluate the initial beneficial effects observed in the AAMS study, we designed the randomized, double-blinded, and placebo-controlled AAMS2 trial. Focusing on patients with ischemic heart disease (IHD) and myocardial scar, the AAMS2 trial aims to generate state-of-the-art structural and functional imaging data of the myocardium treated with an AAMs-patch during CABG. The AAMS2 trial recruits IHD patients who are set to undergo non-urgent CABG and present with an ischemic myocardial scar in preoperative cardiac magnetic resonance imaging (CMRI) with late gadolinium enhancement. Patients are randomized (1:1) to receive a collagen-based matrix patch (Hemopatch®), with or without AAMs, epicardially onto the scar border. The primary endpoint, assessed by CMRI preoperatively and at 6 months post-operative follow-up, focuses on the left ventricle scar mass. The secondary endpoints center on the change in scar mass by the AAMs-patch site and evaluation of therapy safety and feasibility as well as its effects on myocardial structure and function by echocardiography. Change in blood N-terminal-pro-BNP levels in the timeframe is the co-primary endpoint. Data from the AAMS2 trial provides the first randomized, blinded, and placebo-controlled evaluation of efficacy on epicardial AAMs transplantation for ischemic myocardial scar. This data will pave the road towards rational design of larger AAMs therapeutic efficacy-addressing trial(s). ClinicalTrials.gov, NCT05632432, registered 30 November 2022, https://clinicaltrials.gov/study/NCT05632432.

This study investigated whether systolic phase imaging improves cardiac magnetic resonance (CMR) mapping by reducing artifacts compared with diastolic phase analyses. We evaluated T1 and T2 relaxation times and extracellular volume (ECV) in healthy individuals, early rheumatoid arthritis (ERA), and aortic stenosis (AS) patients. A total of 114 participants (54 healthy adults, 30 ERA and 30 AS patients) underwent 1.5 T CMR with T1, T2, and ECV mapping in both diastolic and systolic phases. Imaging artifacts were categorized as minor or major, and inter-observer agreement was assessed for image quality and reproducibility. Systolic phase mapping significantly reduced minor imaging artifacts across all cohorts (14

Aims:The aim of this study was to determine the impact of obstructive coronary artery disease (CAD) and diffuse atherosclerosis on left ventricular systolic function, measured as global longitudinal strain (GLS) by cardiac magnetic resonance (CMR) feature tracking, in patients with severe aortic stenosis (AS). Methods and results:In this single-centre prospective cohort study, patients with severe AS and transcatheter aortic valve implantation (TAVI) between October 2018 and February 2022 were referred for transthoracic echocardiography, CMR, and TAVI computed tomography. Invasive coronary angiography was performed before TAVI. Ninety-four patients (80 ± 7 years) were included. The prevalence of obstructive CAD was 20 (21%) patients. The mean GLS in patients with severe AS was -24.0 ± 6.1%, and the median coronary calcium score was 802 (interquartile range 302-2130). GLS was not reduced in patients with obstructive CAD compared to other patients (23.7 ± 5.9% vs. 24.1 ± 6.2%, P = 0.83). A high coronary calcium score was not associated with GLS. Indexed left ventricular mass was independently related to GLS. Patients with an ischaemic scar on CMR had lower GLS (20.3 ± 5.1% vs. 24.6 ± 6.1%, P = 0.02). Conclusion:GLS correlated with left ventricular mass and the presence of ischaemic scar, suggesting that intrinsic myocardial disease, rather than concomitant CAD alone, accounts for reduced GLS in this population.

Background. Invasive coronary angiography (ICA) is the gold standard in evaluating stent patency after percutaneous coronary intervention (PCI), but it carries a risk of potentially life-threatening complications. Third-generation coronary computed tomography angiography (CCTA) offers a non-invasive, safer follow-up method, but real-world data are lacking. This study evaluated the ability of CCTA to rule out in-stent restenosis (ISR) in long stents at long-term follow-up. Methods. This prospective, single-centre study (NCT06543641) included consecutive patients treated with PCI for coronary chronic total occlusion with long stents (left anterior descending coronary artery and right coronary artery ≥38 mm, left circumflex coronary artery ≥30 mm) in 2014-2019. All patients underwent third-generation dual-source CCTA. Patients with CCTA showing significant ISR, inconclusive results, or symptomatic native artery lesions underwent ICA. Results. The study included 45 patients (median age 67 (IQR 62-73) years, 87% males) with 47 stents (median length 51 mm, range 36-132 mm). CCTA ruled out significant ISR in 87% (n = 39) of the patients. CCTA indicated five ISRs and one inconclusive result in six (13%) patients, all of whom underwent ICA. Additionally, ICA was conducted for five patients due to a native artery lesion(s) on CCTA and angina. ICA showed significant stenosis in all six patients (100%) with ISR or inconclusive CCTA finding in the long stent. Conclusions. Third-generation CCTA could rule out significant ISR in a vast majority of cases (87%, n = 39) and without a risk of complications associated with ICA. CCTA provides a non-invasive, lower risk method for long-term revascularization follow-up.

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a rare disease with variable clinical manifestations. MELAS is most often caused by the human mitochondrial DNA (mtDNA) m.3243A>G variant. We describe cardiac magnetic resonance (CMR) imaging findings and clinical features of 22 subjects with the m.3243A>G mutation and endeavored to discover the role of CMR in MELAS cardiomyopathy diagnostics. The clinical symptoms, ECG findings, and laboratory tests were retrospectively collected from the electronic medical record. Ten subjects (46%) had cardiac symptoms, and eighteen subjects (82%) had some clinical symptoms or signs of MELAS. Seventeen subjects (77%) showed cardiac findings compatible with MELAS. An ECG showed a short PR interval in six subjects (27%). Two patients had a first-degree atrioventricular block. Repolarization changes in the ECG were observed in thirteen subjects (59%), whereas left ventricular hypertrophy voltage criteria were only observed in one subject. Patients with ECG abnormalities had a strong link between proBNP value and cardiac tissue composition (T1 relaxation, p < 0.02) and showed decreased CMR-based strain (p < 0.025). The CMR findings are heterogeneous in subjects with m.3243A>G. Cardiac MELAS may include left ventricular hypertrophy, which mimics sarcomericcardiomyopathy but maypredispose individuals to severe heart failure episodes triggered by acute critical situations. CMR may be used to clarify ECG findings. This study indicates that the genetic testing of MELAS should be considered in new cases of HCM or sudden heart failure phenotypes of unknown etiology.

Background: Laminopathies are a heterogenous group of heritable diseases caused by variants in the Lamin A/C gene (LMNA). They manifest as cardiac and muscular myopathies, lipodystrophies, neuropathies, and progeria. Cardiac manifestations include dilated cardiomyopathy and arrhythmias. Case presentation: A Finnish woman in her 40s who was found to carry two heterozygous likely pathogenic (LP) variants in LMNA, c.1003C>T p.Arg335Trp and c.1303C>T p.Arg435Cys. She was diagnosed with dilated cardiomyopathy and received cardiac resynchronization therapy with a defibrillator. Conclusions: Double heterozygous LMNA variants are exceedingly rare. Even though the patient presented with two LP variants, the age of onset was typical, and the phenotype was not markedly more severe than in those with only one LP variant.

Bone marrow mononuclear cells (BMMCs) have been evaluated for their ability to improve cardiac repair and benefit patients with severe ischemic heart disease and heart failure. In our single-center trial in 2006–2011 we demonstrated the safety and efficacy of BMMCs injected intramyocardially in conjunction with coronary artery bypass surgery. The effect persisted in the follow-up study 5 years later. In this study, we investigated the efficacy of BMMC therapy beyond 10 years. A total of 18 patients (46%) died during over 10-years follow-up and 21 were contacted for participation. Late gadolinium enhancement cardiac magnetic resonance imaging (CMRI) and clinical evaluation were performed on 14 patients, seven from each group. CMRIs from the study baseline, 1-year and 5-years follow-ups were re-analyzed to enable comparison. The CMRI demonstrated a 2.1-fold larger reduction in the mass of late gadolinium enhancement values between the preoperative and the over 10-years follow-up, suggesting less scar or fibrosis after BMMC treatment (− 15.1%; 95% CI − 23 to − 6.7% vs. − 7.3%; 95% CI − 16 to 4.5%, p = 0.039), compared to placebo. No differences in mortality or morbidity were observed. Intramyocardially injected BMMCs may exert long-term benefits in patients with ischemic heart failure. This deserves further evaluation in patients who have received BMMCs in international clinical studies over two decades.

Objective We aimed to study whether myocardial changes are already detectable by cardiac magnetic resonance (CMR) imaging at the time of rheumatoid arthritis (RA) diagnosis. Methods This single-centre prospective study included 39 treatment-naive patients with early rheumatoid arthritis (ERA, symptom duration <1 year) without any history of heart disease, and 38 age- and sex-matched healthy volunteers. The disease severity was assessed with clinical evaluation (Disease Activity Score-28 for Rheumatoid Arthritis with CRP (DAS28-CRP) score) and serological testing (rheumatoid factor (RF) and anti-citrullinated protein antibodies (ACPA)). The ERA patients were classified into group A (DAS28-CRP score >= 3.2, positive RF and ACPA; n=17) and group B (not fulfilling the group A criteria). The ERA patients and healthy controls underwent 1.5T CMR. Results Group A patients had significantly higher myocardial global T1 relaxation times than the healthy controls, 987 [965, 1003] ms vs. 979 [960, 991] ms (median [IQR]; p=0.041). A significant difference in T1 was found in the basal, mid inferior and mid anterolateral segments. In a multivariate analysis, prolonged global T1 relaxation time was independently associated with female sex (95% CI [5.62, 51.31] ms, p=0.016), and group A status (95% CI [4.65, 39.01] ms p=0.014). Conclusion At the time of diagnosis, ERA patients with a higher disease activity (DAS28-CRP score >= 3.2) and both positive RF and ACPA showed prolonged T1 relaxation times in basal myocardial segments. These segments could be most susceptible to the development of myocardial fibrosis, and a segmental reporting style could be useful when estimating the first signs of myocardial fibrosis.

Background:Pathogenic variants in DSP associate with cardiac and cutaneous manifestations including arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, curly or wavy hair, and palmoplantar keratoderma (PPK). Episodes of myocardial inflammation associated with DSP cardiomyopathy might be confused in clinical work with myocarditis of other etiologies such as viral. Cardiac magnetic resonance imaging (CMR) may help in differential diagnosis.Methods and results:This study comprised 49 Finnish patients: 34 participants from families with suspected DSP cardiomyopathy (9 index patients and 25 family members) and 15 patients with myocarditis. All 34 participants underwent genetic testing and cardiac evaluation, and 29 of them also underwent CMR. Participants with the DSP variant, numbering 22, were dermatologically examined. The 15 patients with myocarditis underwent CMR and were evaluated during their hospitalization.A heterozygous truncating DSP c.6310delA p.(Thr2104Glnfs*12) variant was confirmed in 29 participants. Only participants with the DSP variant had pacemakers and life-threatening ventricular arrhythmias. Of the participants with the DSP variant, 24% fulfilled cardiomyopathy criteria, and the median age at diagnosis was 53. Upon CMR, myocardial edema was found to be more common in patients with myocarditis. Both groups had a substantial percentage of late gadolinium enhancement (LGE). A ring-like LGE and increased trabeculation were observed only in participants with the DSP variant. All the studied participants with the DSP variant had PPK and curly or wavy hair. Hyperkeratosis developed before the age of 20 in most patients.Conclusions:The DSP c.6310delA p.(Thr2104Glnfs*12) variant associates with curly hair, PPK, and arrhythmogenic cardiomyopathy with increased trabeculation. Cutaneous symptoms developing in childhood and adolescence might help recognize these patients at an earlier stage. CMR, together with dermatologic characteristics, may help in diagnosis.

BACKGROUND:Long-term symptoms are frequent after coronavirus disease 2019 (COVID-19). We studied the prevalence of post-acute myocardial scar on cardiac magnetic resonance imaging (CMR) in patients hospitalized due to COVID-19 and its association with long-term symptoms.MATERIALS AND METHODS:In this prospective observational single-center study, 95 formerly hospitalized COVID-19 patients underwent CMR imaging at the median of 9 months after acute COVID-19. In addition, 43 control subjects were imaged. Myocardial scar characteristic of myocardial infarction or myocarditis were noted from late gadolinium enhancement images (LGE). Patient symptoms were screened using a questionnaire. Data are presented as mean ± standard deviation or median (interquartile range).RESULTS:The presence of any LGE was higher in COVID-19 patients (66% vs. 37%, p<0.01) as was the presence of LGE suggestive of previous myocarditis (29% vs. 9%, p = 0.01). The prevalence of ischemic scar was comparable (8% vs. 2%, p = 0.13). Only two COVID-19 patients (7%) had myocarditis scar combined with left ventricular dysfunction (EF <50%). Myocardial edema was not detected in any participant. The need for intensive care unit (ICU) treatment during initial hospitalization was comparable in patients with and without myocarditis scar (47% vs. 67%, p = 0.44). Dyspnea, chest pain, and arrhythmias were prevalent in COVID-19 patients at follow-up (64%, 31%, and 41%, respectively) but not associated with myocarditis scar on CMR.CONCLUSIONS:Myocardial scar suggestive of possible previous myocarditis was detected in almost one-third of hospital-treated COVID-19 patients. It was not associated with the need for ICU treatment, greater symptomatic burden, or ventricular dysfunction at 9 months follow-up. Thus, post-acute myocarditis scar on COVID-19 patients seems to be a subclinical imaging finding and does not commonly require further clinical evaluation.

The European Society of Cardiology Guidelines on cardiac pacing from 2021 allow magnetic resonance imaging (MRI) in patients with cardiac implantable electronic devices (CIEDs) but do not recommend MRI in patients with epicardial pacing leads. The clinical dilemma remains whether performing an MRI in patients with CIED and epicardial leads is safe. We aimed to evaluate the safety of performing an MRI in patients with CIED and abandoned or functioning epicardial pacing leads. We included all adult patients who underwent clinically indicated MRIs with CIED and functioning or abandoned epicardial leads in a single tertiary hospital between November 2011 and October 2019. The data were retrospectively collected. Twenty-six MRIs were performed on 17 patients with functioning or abandoned epicardial pacing leads. Sixty-nine percent of the MRI scans (18/26) were conducted on patients with functioning epicardial pacing leads. A definite adverse event occurred in one MRI scan. This was a transient elevation of the pacing threshold in a patient with a functioning epicardial ventricular pacing lead implanted 29 years previously. An irreversible atrial pacing lead impedance elevation was detected 6 months after the MRI in another patient; the association with the previous MRI remained unclear. No adverse events were detected in MRIs performed on patients with modern (implanted in 2000 or later) functioning epicardial leads. MRIs in patients with CIED and modern functioning epicardial pacing leads were performed without detectable adverse events. Further large-scale studies are necessary to confirm MRI safety in patients with epicardial pacing leads. • Currently, MRI in patients with cardiac implantable electronic devices (CIEDs) and functioning or abandoned epicardial pacing leads is not recommended. • MRIs in patients with CIED and modern functioning epicardial leads (implanted in 2000 or later) were performed without detectable adverse events in our patient cohort. • Allowing MRI in patients with epicardial pacing leads may significantly improve the diagnostic work-up, especially in specific patient groups, such as patients with congenital heart disease.

Background Some myocardial diseases, such as cardiac sarcoidosis, predispose to complete atrioventricular block. The European Society of Cardiology Guidelines on cardiac pacing in 2021 recommend myocardial disease screening in patients with conduction disorder requiring pacemaker with multimodality imaging, including cardiac magnetic resonance (CMR) imaging. The ability of CMR imaging to detect myocardial disease in patients with a temporary pacing wire is not well documented. Methods and Results Our myocardial disease screening protocol is based on using an active fixation pacing lead connected to a reusable extracorporeal pacing generator (temporary permanent pacemaker) as a bridge to a permanent pacemaker. From 2011 to 2019, we identified 17 patients from our CMR database who underwent CMR imaging with a temporary permanent pacemaker for atrioventricular block. We analyzed their clinical presentations, CMR data, and pacemaker therapy. All CMRs were performed without adverse events. Pacing leads induced minor artifacts to the septal myocardial segments. The extent of late gadolinium enhancement in CMR imaging was used to screen patients for the presence of myocardial disease. Patients with evidence of late gadolinium enhancement underwent endomyocardial biopsy. If considered clinically indicated, also 18-F-fluorodeoxyglucose positron emission tomography and extracardiac tissue biopsy were performed if sarcoidosis was suspected. Eventually, 8 of 17 patients (47.1%) were diagnosed with histologically confirmed granulomatous inflammatory cardiac disease. Importantly, only 1 had a previously diagnosed extracardiac sarcoidosis at the time of presentation with high-degree atrioventricular block. Conclusions CMR imaging with temporary permanent pacemaker protocol is an effective and safe early screening tool for myocardial disease in patients presenting with atrioventricular block requiring immediate, continuous pacing for bradycardia.

Objectives Cardiac implantable electronic device (CIED)–induced metal artefacts possibly significantly diminish the diagnostic value of magnetic resonance imaging (MRI), particularly cardiac MR (CMR). Right-sided generator implantation, wideband late-gadolinium enhancement (LGE) technique and raising the ipsilateral arm to the generator during CMR scanning may reduce the CIED-induced image artefacts. We assessed the impact of generator location and the arm-raised imaging position on the CIED-induced artefacts in CMR. Methods We included all clinically indicated CMRs performed on patients with normal cardiac anatomy and a permanent CIED with endocardial pacing leads between November 2011 and October 2019 in our institution ( n = 171). We analysed cine and LGE sequences using the American Heart Association 17-segment model for the presence of artefacts. Results Right-sided generator implantation and arm-raised imaging associated with a significantly increased number of artefact-free segments. In patients with a right-sided pacemaker, the median percentage of artefact-free segments in short-axis balanced steady-state free precession LGE was 93.8% (IQR 9.4%, n = 53) compared with 78.1% (IQR 20.3%, n = 58) for left-sided pacemaker ( p < 0.001). In patients with a left-sided implantable cardioverter-defibrillator, the median percentage of artefact-free segments reached 87.5% (IQR 6.3%, n = 9) using arm-raised imaging, which fell to 62.5% (IQR 34.4%, n = 9) using arm-down imaging in spoiled gradient echo short-axis cine ( p = 0.02). Conclusions Arm-raised imaging represents a straightforward method to reduce CMR artefacts in patients with left-sided generators and can be used alongside other image quality improvement methods. Right-sided generator implantation could be considered in CIED patients requiring subsequent CMR imaging to ensure sufficient image quality. Key Points • Cardiac implantable electronic device (CIED)–induced metal artefacts may significantly diminish the diagnostic value of an MRI, particularly in cardiac MRIs. • Raising the ipsilateral arm relative to the CIED generator is a cost-free, straightforward method to significantly reduce CIED-induced artefacts on cardiac MRIs in patients with a left-sided generator. • Right-sided generator implantation reduces artefacts compared with left-sided implantation and could be considered in CIED patients requiring subsequent cardiac MRIs to ensure adequate image quality in the future.

Introduction Finnish gelsolin amyloidosis (AGel amyloidosis) is an inherited systemic amyloidosis with well-known ophthalmological, neurological and cutaneous symptoms. Additionally, cardiomyopathies, conduction disorders and need of cardiac pacemakers occur in some patients. This study focuses on electrocardiographic (ECG) findings in AGel amyloidosis and their relation to cardiac magnetic resonance (CMR) changes. We also assessed whether ECG abnormalities were associated with pacemaker implantation and mortality. Materials and methods In this cohort study, 51 genetically verified AGel amyloidosis patients (mean age 66 years) without cardiac pacemakers underwent 12-lead ECG and CMR imaging with contrast agent in 2017. Patients were followed-up for 3 years. Results Conduction disturbances were found in 22 patients (43%). Nine (18%) presented with first-degree atrioventricular block, six (12%) with left anterior hemiblock, seven (14%) with left or right bundle branch block and two (4%) with non-specific intraventricular conduction delay. Low QRS voltage was present in two (4%) patients. Late gadolinium enhancement (LGE) concentrating on the interventricular septum and inferior parts of the heart was present in 19 (86%) patients with conduction abnormalities. During the follow-up, only one patient received a pacemaker, and one patient died. Discussion Conduction disorders and septal LGE are common in AGel amyloidosis, whereas other ECG and CMR findings typically observed in most common cardiac amyloidosis types were rare. Septal pathology seen in CMR may interfere with the cardiac conduction system in AGel amyloidosis, explaining conduction disorders, although pacemaker therapy is rarely required.

Background: Familial dilated cardiomyopathy (DCM) is a monogenic disorder typically inherited in an autosomal dominant pattern. We have identified two Finnish families with familial cardiomyopathy that is not explained by a variant in any previously known cardiomyopathy gene. We describe the cardiac phenotype related to homozygous truncating GCOM1 variants. Methods and Results: This study included two probands and their relatives. All the participants are of Finnish ethnicity. Whole-exome sequencing was used to test the probands; bi-directional Sanger sequencing was used to identify the GCOM1 variants in probands' family members. Clinical evaluation was performed, medical records and death certificates were obtained. Immunohistochemical analysis of myocardial samples was conducted. A homozygous GCOM1 variant was identified altogether in six individuals, all considered to be affected. None of the nine heterozygous family members fulfilled any cardiomyopathy criteria. Heart failure was the leading clinical feature, and the patients may have had a tendency for atrial arrhythmias. Conclusions: This study demonstrates the significance of GCOM1 variants as a cause of human cardiomyopathy and highlights the importance of searching for new candidate genes when targeted gene panels do not yield a positive outcome.

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): HUS Medical Imaging Center research grant Background The Heart and Rhythm Society’s consensus statement 2017 approves magnetic resonance imaging (MRI) with cardiac implantable electronic devices (CIED), but excludes patients with epicardial and abandoned leads. Potential safety hazards of an MRI with epicardial pacing leads include heating of the tip of the lead and induction of current in the pacing lead resulting in inappropriate cardiac stimulation. Only a few small studies of MRI safety with epicardial pacing leads have been published and adverse events have been rare. The clinical dilemma remains, whether performing an MRI on a patient with CIED and epicardial pacing leads is safe. We have performed MRIs on patients with CIED and epicardial pacing leads when benefits have been considered to outweigh the risks after careful case-by-case evaluation following our institutional MRI with CIED safety protocol. Purpose The aim of this study was to evaluate the safety of performing an MRI scan on patients with CIED and abandoned or functional epicardial pacing leads. Methods All the clinically indicated MRI examinations conducted on adult patients with CIED and functional or abandoned epicardial leads (n = 24) performed in our hospital between November 2011 and October 2019 were included in this observational retrospective study. The data were retrospectively collected from electronic medical records. Results Altogether 24 MRIs were performed to 16 patients with functional or abandoned epicardial pacing leads (Table). 93.8% (15/16) patients had congenital heart disease. Cardiac MRI was the most frequent examination (21/24, 91.7%). 66.7% of the MRI scans (16/24) were conducted on patients with functional epicardial pacing leads. In 5/24 (20.8%) MRIs, the patient was pacemaker-dependent. A clinically significant event occurred in one MRI scan. This was transient elevation of the pacing lead threshold in a patient with functional epicardial ventricular pacing lead, that was implanted 29 years prior to the MRI. In another patient with 30-year-old functional epicardial pacing leads, clinically significant irreversible elevation in atrial pacing lead impedance was detected 6 months after the MRI and unlikely related to previous MRI examination. None of the patients experienced sensations leading to cessation of the MRI scans. No clinically significant pacing lead parameter changes were detected after MRIs performed on patients with modern (implanted year 2000 or later) functional epicardial pacing leads or functional endocardial leads and abandoned epicardial leads. Conclusions MRI examinations in patients with CIED and modern functional epicardial pacing leads were performed without detectable adverse events. Performing an MRI with old functional epicardial pacing leads may involve more risks.

Type of funding sources: Public hospital(s). Main funding source(s): HUS diagnostic imaging center Subclinical myocardial disease is common in patients with rheumatoid arthritis (RA). Impaired cardiac function, myocardial fibrosis and inflammation have previously correlated with RA disease activity. Our aim was to study whether myocardial changes are detectable by cardiac magnetic resonance (CMR) at the time of RA diagnosis. Material and methods: We recruited 21 untreated early RA patients without history of heart disease in Helsinki University Hospital and Lohja Hospital (Finland) between 10/2018 and 2/2020, and nine healthy volunteers. The patients underwent a clinical examination, laboratory tests, and CMR including mapping of extracellular volume fraction (ECV), and T1 and T2 relaxation times. The healthy controls underwent non-contrast CMR.  The RA patients were older than the controls (median 58.1 years vs. 41.6 years, respectively, table 1.). T1 was slightly higher in RA patients compared with healthy controls in anteroseptal segments (1015 ms vs. 982 ms, P = 0.017) (table 2).  No difference in T2 was detected and the ECV values were considered normal. Segmental T1, T2 or ECV showed no significant correlations with age, duration of the symptoms or with RA disease activity (DAS28-CRP score).  The minor, but statistically significant, elevation of T1 relaxation time in the anteroseptal segments suggests that myocardial changes may occur already in the early phase of RA, the anteroseptal segments being most vulnerable. The elevation of T1 relaxation time can be caused by mild myocardial inflammation or fibrosis. Although no significant correlation with DAS28-CRP was observed, subclinical systemic inflammation may have contributed to the myocardial abnormalities. Table 1. Pre-contrast T1 relaxation time (ms) T2 relaxation time (ms) ECV (%) Mean RA patients Controls P-value RA patients Controls P-value RA patients Global myocardial mean 996 (978-1011) 982 (964-1000) 0.304 48.0 (44.6-49.7) 46.3 (44.1-48.7) 0.295 26.6 (25.7-28.5) Anterior segments 954 (919-1000) 951 (921-998) 0.929 47.7 (46.4-49.8) 47.9 (43.5-50.1) 0.871 26.7 (25.3-28.8) Anteroseptal segments 1015 (987-1041) 982 (947-996) 0.017 48.3 (45.5-49.9) 45.7 (43.8-48.4) 0.150 28.3 (26.8-29.2) Inferoseptal segments 1012 (992-1023) 998 (967-1003) 0.077 48.0 (43.5-49.4) 44.9 (43.5-46.2) 0.533 26.7 (26.0-27.9) Inferior segments 1016 (987-1064) 1003 (992-1025) 0.625 47.5 (45.1-50.3) 46.1 (44.2-48.4) 0.304 27.3 (25.6-29.0) Inferolateral segments 997 (974-1043) 992 (980-1016) >0.999 46.6 (42.6-49.1) 45.1 (42.4-49.1) 0.689 25.8 (25.3-28.2) Anterolateral segments 973 (945-973) 981 (954-1010) 0.563 47.0 (45.1-48.5) 46.6 (43.5-49.6) 0.625 26.4 (24.7-28.0) T1 and T2 relaxation time mapping and ECV results. Abstract Figure. ECV mapping

Abstract Funding Acknowledgements Type of funding sources: None. Background Global longitudinal strain (GLS) by echocardiography is a sensitive method for measuring left ventricular (LV) function, and of better prognostic value in valvular heart disease than ejection fraction (EF). Cardiac magnetic resonance imaging (CMR) is the most accurate method for measuring LV volume and EF, but GLS has not been possible to measure by CMR until recently. Purpose This study compares GLS obtained by CMR and echocardiography in patients with severe aortic valve stenosis. Normal values for GLS by CMR are reported as well. Methods GLS was measured in 32 patients with severe aortic valve stenosis with speckle tracking echocardiography, using GE Vivid E95 (n = 15) and Philips EPIQ (n = 17) ultrasound machines, as well as with CMR (Avanto 1.5T FIT, Siemens Medical Solutions). For normal values, GLS was measured by CMR in 9 healthy controls. Endo- and epicardial borders of two, three and four chamber cine images were traced for CMR GLS using dedicated software (Qstrain 2.0, Medis, NL). Both CMR and Vivid E95 measured midmyocardial strain, whereas the EPIQ AutoStrain method measures endomyocardial strain. Absolute values of GLS are reported. Pearson correlation coefficient was calculated and paired Student’s t-test was used for comparisons. Results A significant correlation (r = 0.45, p = 0.01) was found between echocardiographic and CMR GLS (Figure). GLS by Vivid E95 had a very good correlation with CMR GLS (r = 0.84, p = 0.0001), whereas GLS by Philips EPIQ did not correlate significantly (r = 0.14, p = 0.01). In patients with aortic stenosis and healthy controls, the average GLS by CMR was 18.3 ± 3% and 20.9 ± 2% respectively. The average GLS by CMR was comparable to that obtained by GE Vivid E95 (17.3 ± 4% vs. 17.2 ± 3%, p = 0.92), and higher than by Philips EPIQ (19.2 ± 2% vs. 15.4 ± 2%, p < 0.0001). Conclusion This study shows that GLS by CMR is feasible and correlates with GLS obtained by echocardiography, especially when quantifying midmyocardial strain. Echocardiographic GLS values based on endomyocardial strain were lower. Patient characteristics Age 75 ± 14 y NYHA 1 1 (3 %) NYHA 2 20 (67 %) NYHA 3 8 (27 %) NYHA 4 1 (3 %) CMR EF 66 ± 8 % AVA 0.7 ± 0.2 cm² NYHA = NYHA class of symptoms, EF = ejection fraction by CMR, AVA = aortic valve area by echocardiography Abstract Figure. GLS by CMR vs. Echocardiography

Background Aortic valve stenosis (AS) is the most prevalent valvular disease in the developed countries. Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) is an emerging imaging technique, which has been suggested to improve the evaluation of AS severity compared to two-dimensional (2D) flow and transthoracic echocardiography (TTE). We investigated the reliability of CMR 2D flow and 4D flow techniques in measuring aortic transvalvular peak systolic flow in patients with severe AS. Methods We prospectively recruited 90 patients referred for aortic valve replacement due to severe AS (73.3 ± 11.3 years, aortic valve area 0.7 ± 0.1 cm 2 , and 54/36 tricuspid/bicuspid), and 10 non-valvular disease controls. All the patients underwent echocardiography and 2D flow and 4D flow CMR. Peak flow velocity measurements were compared using Wilcoxon signed rank sum test and Bland–Altman analysis. Results 4D flow underestimated peak flow velocity in the AS group when compared with TTE (bias − 1.1 m/s, limits of agreement ± 1.4 m/s) and 2D flow (bias − 1.2 m/s, limits of agreement ± 1.6 m/s). The differences between values obtained by TTE (median 4.3 m/s, range 2.7–6.1 m/s) and 2D flow (median 4.5 m/s, range 2.9–6.5 m/s) compared to 4D flow (median 3.1 m/s, range 1.7–5.1 m/s) were significant (p < 0.001). The difference between 2D flow and TTE were insignificant (bias 0.07 m/s, limits of agreement ± 1.5 m/s). In non-valvular disease controls, peak flow velocity was measured higher by 4D flow than 2D flow (1.4 m/s, 1.1–1.7 m/s and 1.3 m/s, 1.1–1.5 m/s, respectively; bias 0.2 m/s, limits of agreement ± 0.16 m/s). Conclusions CMR 4D flow significantly underestimates systolic peak flow velocity in patients with severe AS. 2D flow, in turn, estimated the AS velocity accurately, with measured peak flow velocities comparable to TTE.