Cardiac Sarcoidosis-Diagnostic and Therapeutic Challenges.

Sarcoidosis is a multisystem disorder of unknown etiology. The leading hypothesis involves an antigen-triggered dysregulated T-cell-driven immunologic response leading to non-necrotic granulomas. In cardiac sarcoidosis (CS), the inflammatory response can lead to fibrosis, culminating in clinical manifestations such as atrioventricular block and ventricular arrhythmias. Cardiac manifestations frequently present as first and isolated signs or may appear in conjunction with extracardiac manifestations. The incidence of sudden cardiac death (SCD) is high. Diagnosis remains a challenge. For a definite diagnosis, endomyocardial biopsy (EMB) is suggested. In clinical practice, compatible findings in advanced imaging using cardiovascular magnetic resonance (CMR) and/or positron emission tomography (PET) in combination with extracardiac histological proof is considered sufficient. Management revolves around the control of myocardial inflammation by employing immunosuppression. However, data regarding efficacy are merely based on observational evidence. Prevention of SCD is of particular importance and several guidelines provide recommendations regarding device therapy. In patients with manifest CS, outcome data indicate a 5-year survival of around 90% and a 10-year survival in the range of 80%. Data for patients with silent CS are conflicting; some studies suggest an overall benign course of disease while others reported contrasting observations. Future research challenges involve better understanding of the immunologic pathogenesis of the disease for a targeted therapy, improving imaging to aid early diagnosis, assessing the need for screening of asymptomatic patients and randomized trials.

A case series of double-chambered left ventricle detected by cardiovascular magnetic resonance.

Background: Double-chambered left ventricle (DCLV) is a rare congenital condition, and few case reports are mentioned in literature. Entity, clinical course, and prognosis remain unclear. Cardiovascular magnetic resonance (CMR) is often used for characterization of various congenital heart diseases and can be particularly useful for imaging rare phenomena. Case summary: Three cases of DCLV were detected by CMR within 2 years in our CMR centre with and without associated congenital heart disease or hypertrabecularization. The patients did not suffer from cardiac symptoms despite the presence of premature ventricular complexes in one patient. Diagnosis of DCLV was made based on a first CMR study that was performed in adulthood, although some anatomical suspicion was already raised by previous echocardiography. Discussion: Double-chambered left ventricle, synonymous with the terminus 'cor triventriculare sinistrum', has been previously perceived as a rare phenomenon compared with double-chambered right ventricle. It has to be distinguished from ventricular aneurysm or cardiac diverticulum and is characterized by an additional contractile septum with normal wall structure that divides the LV cavum into two (rather) same-sized chambers. The prognosis seems to be benign, since there is no restriction in functionality and no increased thrombogenicity until adulthood. Consequently, there is (presumably) no need for a tailored therapy-at least in the cases present here. Accordingly, we recommend follow-up CMR examinations for progress monitoring and recognize CMR's significant role for diagnosis and follow-up of cardiac abnormalities in orphan diseases. Due to its broader availability, we expect further cases of DLVC in the future.

Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging.

OBJECTIVES: The purpose of this study was to carefully analyse the therapeutic benefit of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) and cardiomyopathy (ATTRwt-CM) after one year of therapy based on serial multi-parametric cardiovascular magnetic resonance (CMR) imaging. BACKGROUND: Non-sponsored data based on multi-parametric CMR regarding the effect of tafamidis on the cardiac phenotype of patients with ATTRwt-CM are not available so far. METHODS: The present study comprised N = 40 patients with ATTRwt-CM who underwent two serial multi-parametric CMR studies within a follow-up period of 12 ± 3 months. Baseline (BL) clinical parameters, serum biomarkers and CMR findings were compared to follow-up (FU) values in patients treated "with" tafamidis 61 mg daily (n = 20, group A) and those "without" tafamidis therapy (n = 20, group B). CMR studies were performed on a 1.5-T system and comprised cine-imaging, pre- and post-contrast T1-mapping and additional calculation of extracellular volume fraction (ECV) values. RESULTS: While left ventricular ejection fraction (LV-EF), left ventricular mass index (LVMi), left ventricular wall thickness (LVWT), native T1- and ECV values remained unchanged in the tafamidis group A, a slight reduction in LV-EF (p = 0.003) as well as a subtle increase in LVMi (p = 0.034), in LVWT (p = 0.001), in native T1- (p = 0.038) and ECV-values (p = 0.017) were observed in the untreated group B. Serum NT-proBNP levels showed an overall increase in both groups, however, with the untreated group B showing a relatively higher increase compared to the treated group A. Assessment of NYHA class did not result in significant intra-group differences when BL were compared with FU, but a trend to improvement in the treated group A compared to a worsening trend in the untreated group B (∆p = 0.005). CONCLUSION: As expected, tafamidis does not improve cardiac phenotype in patients with ATTRwt-CM after one year of therapy. However, tafamidis seems to slow down cardiac disease progression in patients with ATTRwt-CM compared to those without tafamidis therapy based on multi-parametric CMR data already after one year of therapy.

A compartment-based myocardial density approach helps to solve the native T1 vs. ECV paradox in cardiac amyloidosis.

Cardiovascular magnetic resonance (CMR) plays an important clinical role for diagnosis and therapy monitoring of cardiac amyloidosis (CA). Previous data suggested a lower native T1 value in spite of a higher LV mass and higher extracellular volume fraction (ECV) value in wild-type transthyretin amyloidosis (ATTRwt) compared to light-chain amyloidosis (AL)-resulting in the still unsolved "native T1 vs. ECV paradox" in CA. The purpose of this study was to address this paradox. The present study comprised N = 90 patients with ATTRwt and N = 30 patients with AL who underwent multi-parametric CMR studies prior to any specific treatment. The CMR protocol comprised cine- and late-gadolinium-enhancement (LGE)-imaging as well as T2-mapping and pre-/post-contrast T1-mapping allowing to measure myocardial ECV. Left ventricular ejection fraction (LV-EF), left ventricular mass index (LVMi) and left ventricular wall thickness (LVWT) were significantly higher in ATTRwt in comparison to AL. Indexed ECV (ECVi) was also higher in ATTRwt (p = 0.041 for global and p = 0.001 for basal septal). In contrast, native T1- [1094 ms (1069-1127 ms) in ATTRwt vs. 1,122 ms (1076-1160 ms) in AL group, p = 0.040] and T2-values [57 ms (55-60 ms) vs. 60 ms (57-64 ms); p = 0.001] were higher in AL. Considering particularities in myocardial density, "total extracellular mass" (TECM) was substantially higher in ATTRwt whereas "total intracellular mass" (TICM) was rather similar between ATTRwt and AL. Consequently, the "ratio TICM/TECM" was lower in ATTRwt compared to AL (0.58 vs. 0.83; p = 0.007). Our data confirm the presence of a "native T1 vs. ECV paradox" with lower native T1 values in spite of higher myocardial mass and ECV in ATTRwt compared to AL. Importantly, this observation can be explained by particularities regarding myocardial density that result in a lower TICM/TECM "ratio" in case of ATTRwt compared to AL-since native T1 is determined by this ratio.

Serial Cardiovascular Magnetic Resonance Studies Prior to and After mRNA-Based COVID-19 Booster Vaccination to Assess Booster-Associated Cardiac Effects.

Background: mRNA-based COVID-19 vaccination is associated with rare but sometimes serious cases of acute peri-/myocarditis. It is still not well known whether a 3rd booster-vaccination is also associated with functional and/or structural changes regarding cardiac status. The aim of this study was to assess the possible occurrence of peri-/myocarditis in healthy volunteers and to analyze subclinical changes in functional and/or structural cardiac parameters following a mRNA-based booster-vaccination. Methods and Results: Healthy volunteers aged 18-50 years (n = 41; m = 23, f = 18) were enrolled for a CMR-based serial screening before and after 3rd booster-vaccination at a single center in Germany. Each study visit comprised a multi-parametric CMR scan, blood analyses with cardiac markers, markers of inflammation and SARS-CoV-2-IgG antibody titers, resting ECGs and a questionnaire regarding clinical symptoms. CMR examinations were performed before (median 3 days) and after (median 6 days) 3rd booster-vaccination. There was no significant change in cardiac parameters, CRP or D-dimer after vaccination, but a significant rise in the SARS-CoV-2-IgG titer (p < 0.001), with a significantly higher increase in females compared to males (p = 0.044). No changes regarding CMR parameters including global native T1- and T2-mapping values of the myocardium were observed. A single case of a vaccination-associated mild pericardial inflammation was detected by T2-weighted CMR images. Conclusion: There were no functional or structural changes in the myocardium after booster-vaccination in our cohort of 41 healthy subjects. However, subclinical pericarditis was observed in one case and could only be depicted by multiparametric CMR.

[de novo hATTR amyloidosis after domino transplantation of a donor's liver: a case report for the use of Patisiran]. / de novo hATTR Amyloidose nach Domino-Lebertransplantation: Ein Fallbeispiel für den Einsatz von Patisiran.

Hereditary transthyretin-mediated (hATTR) amyloidosis is a rare, rapidly progressing, and potentially life-threatening disease caused by one of more than 120 mutations in the transthyretin (TTR) gene. As a result of the cumulative amyloid deposits, especially in the peripheral nerves and the heart, the majority of patients develop progressive, peripheral sensorimotor polyneuropathy and biventricular cardiomyopathy over time.Since TTR - and its amyloidogenic variants too - is predominantly synthesized in the liver, early, orthotopic liver transplantation (LTx) is a treatment option that can be used to potentially stop the progression of hATTR amyloidosis.The actual case shows a patient with hepatocellular carcinoma who received the organ of a patient with hATTR as part of a domino liver transplantation. After approximately 10 years, the patient started to develop the characteristic symptoms of the metabolic disorder. Because of a further progression of the amyloidosis, therapy with the RNA interference therapeutic patisiran was initiated, which temporarily halted the progression.

Cardiovascular Magnetic Resonance-Guided Radiofrequency Ablation: Where Are We Now?

The possibilities of cardiovascular magnetic resonance (CMR) imaging for myocardial tissue characterization and catheter ablation guidance are accompanied by some fictional concepts. In this review, we present the available facts about CMR-guided catheter ablation procedures as well as promising, however unproven, theoretical concepts. CMR promises to visualize the respective arrhythmogenic substrate and may thereby make it more localizable for electrophysiology (EP)-based ablation. Robust CMR imaging is challenged by motion of the heart resulting from cardiac and respiratory cycles. In contrast to conventional "passive" tracking of the catheter tip by real-time CMR, novel approaches based on "active" tracking are performed by integrating microcoils into the catheter tip that send a receiver signal. Several experimental and clinical studies were already performed based on real-time CMR for catheter ablation of atrial and ventricular arrhythmias. Importantly, successful ablation of the cavotricuspid isthmus was already performed in patients with typical atrial flutter. However, a complete EP procedure with real-time CMR-guided transseptal puncture and subsequent pulmonary vein isolation has not been shown so far in patients with atrial fibrillation. Moreover, real-time CMR-guided EP for ventricular tachycardia ablation was only performed in animal models using a transseptal, retrograde, or epicardial access-but not in humans. Essential improvements within the next few years regarding basic technical requirements, such as higher spatial and temporal resolution of real-time CMR imaging as well as clinically approved cardiac magnetic resonance-conditional defibrillators, are ultimately required-but can also be expected-and will move this field forward.

Genome silencer therapy leading to 'regression' of cardiac amyloid load on cardiovascular magnetic resonance: a case report.

BACKGROUND: Hereditary or variant transthyretin amyloidosis (ATTRv) is a progressive disease manifesting with neuropathy and/or cardiomyopathy. An early and accurate diagnosis of cardiac amyloidosis is a pre-requisite for timely and appropriate patient management, including anti-amyloid therapies, as it is associated with heart failure, conduction disease, and arrhythmias, leading to reduced quality of life and early death. CASE SUMMARY: We present the case of an ATTRv male patient presenting with a mixed amyloidosis phenotype (neuropathy and cardiomyopathy). Cardiac disease manifestation comprised tachyarrhythmias (atrial fibrillation) and conduction abnormalities (atrio-ventricular block) in addition to segmental left ventricular (LV) hypertrophy (septal wall) due to regionally pronounced amyloid deposits in the basal LV myocardium. Interestingly, by means of serial cardiovascular magnetic resonance (CMR) studies, we were able to demonstrate an impressive and unexpected improvement of cardiomyopathy findings within a relatively short period-of-time after the implementation of genome-silencer therapies. DISCUSSION: This is our second case report that showed ATTRv cardiomyopathy reversal under anti-amyloid therapy-documented by multi-parametric CMR. Our findings support the hypothesis that amyloid infiltration leading to cardiomyopathy is not an irreversible pathological process-but rather a dynamic one, that cannot only be stopped but even reversed (to a certain degree) by currently emerging anti-amyloid therapies. Moreover, the role of serial multi-parametric CMR imaging for surveillance of cardiomyopathy dynamics under these therapies is nicely illustrated.

Diagnosis of Cardiac Involvement in Amyloid A Amyloidosis by Cardiovascular Magnetic Resonance Imaging.

Background: Diagnosis of cardiac involvement in amyloid A (AA) amyloidosis is challenging since AA amyloidosis is a rare disease and cardiac involvement even less frequent. The diagnostic yield of currently available non-invasive imaging methods is not well-studied and rather limited, and invasive endomyocardial biopsy (EMB) is rarely performed due to the potential risk of this procedure. Cardiovascular magnetic resonance (CMR)-based myocardial tissue characterization by late-gadolinium-enhancement (LGE) imaging and novel-mapping approaches may increase the diagnostic yield in AA amyloidosis. Methods: Two patients with AA amyloidosis in whom cardiac involvement was suspected based on CMR findings and subsequently proven by biopsy work-up are presented. CMR studies were performed on a 1.5-T system and comprised a cine steady-state free precession pulse sequence for ventricular function and a late-gadolinium-enhancement (LGE) sequence for detection of myocardial pathology. Moreover, a modified Look-Locker inversion recovery (MOLLI) T1-mapping sequence was applied in basal, mid and apical short-axes prior to contrast agent administration and ~20 min thereafter to determine native T1 and ECV values. Results: Both patients showed slightly dilated left ventricles (LV) with mild to moderate LV hypertrophy and preserved systolic function. Only a very subtle pattern of LGE was observed in both patients with AA amyloidosis. However, markedly elevated native T1 (max. 1,108 and 1,112 ms, respectively) and extracellular volume fraction (ECV) values (max. 39 and 48%, respectively) were measured in the myocardium suggesting the presence of cardiac involvement - with subsequent EMB-based proof of AA amyloidosis. Conclusion: We recommend a multi-parametric CMR approach in patients with AA amyloidosis comprising both LGE-based contrast-imaging and T1-mapping-based ECV measurement of the myocardium for non-invasive work-up of suspected cardiac involvement. The respective CMR findings may be used as gatekeeper for additional invasive procedures (such as EMB) and as a non-invasive monitoring tool regarding assessment and modification of ongoing treatments.

A cardiovascular magnetic resonance imaging-based pilot study to assess coronary microvascular disease in COVID-19 patients.

Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and is primarily characterised by a respiratory disease. However, SARS-CoV-2 can directly infect vascular endothelium and subsequently cause vascular inflammation, atherosclerotic plaque instability and thereby result in both endothelial dysfunction and myocardial inflammation/infarction. Interestingly, up to 50% of patients suffer from persistent exercise dyspnoea and a post-viral fatigue syndrome (PVFS) after having overcome an acute COVID-19 infection. In the present study, we assessed the presence of coronary microvascular disease (CMD) by cardiovascular magnetic resonance (CMR) in post-COVID-19 patients still suffering from exercise dyspnoea and PVFS. N = 22 patients who recently recovered from COVID-19, N = 16 patients with classic hypertrophic cardiomyopathy (HCM) and N = 17 healthy control patients without relevant cardiac disease underwent dedicated vasodilator-stress CMR studies on a 1.5-T MR scanner. The CMR protocol comprised cine and late-gadolinium-enhancement (LGE) imaging as well as velocity-encoded (VENC) phase-contrast imaging of the coronary sinus flow (CSF) at rest and during pharmacological stress (maximal vasodilation induced by 400 µg IV regadenoson). Using CSF measurements at rest and during stress, global myocardial perfusion reserve (MPR) was calculated. There was no difference in left ventricular ejection-fraction (LV-EF) between COVID-19 patients and controls (60% [57-63%] vs. 63% [60-66%], p = NS). There were only N = 4 COVID-19 patients (18%) showing a non-ischemic pattern of LGE. VENC-based flow measurements showed that CSF at rest was higher in COVID-19 patients compared to controls (1.78 ml/min [1.19-2.23 ml/min] vs. 1.14 ml/min [0.91-1.32 ml/min], p = 0.048). In contrast, CSF during stress was lower in COVID-19 patients compared to controls (3.33 ml/min [2.76-4.20 ml/min] vs. 5.32 ml/min [3.66-5.52 ml/min], p = 0.05). A significantly reduced MPR was calculated in COVID-19 patients compared to healthy controls (2.73 [2.10-4.15-11] vs. 4.82 [3.70-6.68], p = 0.005). No significant differences regarding MPR were detected between COVID-19 patients and HCM patients. In post-COVID-19 patients with persistent exertional dyspnoea and PVFS, a significantly reduced MPR suggestive of CMD-similar to HCM patients-was observed in the present study. A reduction in MPR can be caused by preceding SARS-CoV-2-associated direct as well as secondary triggered mechanisms leading to diffuse CMD, and may explain ongoing symptoms of exercise dyspnoea and PVFS in some patients after COVID-19 infection.

CMR-based T1-mapping offers superior diagnostic value compared to longitudinal strain-based assessment of relative apical sparing in cardiac amyloidosis.

Cardiac amyloidosis (CA) is an infiltrative disease. In the present study, we compared the diagnostic accuracy of cardiovascular magnetic resonance (CMR)-based T1-mapping and subsequent extracellular volume fraction (ECV) measurement and longitudinal strain analysis in the same patients with (a) biopsy-proven cardiac amyloidosis (CA) and (b) hypertrophic cardiomyopathy (HCM). N = 30 patients with CA, N = 20 patients with HCM and N = 15 healthy control patients without relevant cardiac disease underwent dedicated CMR studies. The CMR protocol included standard sequences for cine-imaging, native and post-contrast T1-mapping and late-gadolinium-enhancement. ECV measurements were based on pre- and post-contrast T1-mapping images. Feature-tracking analysis was used to calculate 3D left ventricular longitudinal strain (LV-LS) in basal, mid and apical short-axis cine-images and to assess the presence of relative apical sparing. Receiver-operating-characteristic analysis revealed an area-under-the-curve regarding the differentiation of CA from HCM of 0.984 for native T1-mapping (p < 0.001), of 0.985 for ECV (p < 0.001) and only 0.740 for the "apical-to-(basal + midventricular)"-ratio of LV-LS (p = 0.012). A multivariable logistical regression analysis showed that ECV was the only statistically significant predictor of CA when compared to the parameter LV-LS or to the parameter "apical-to-(basal + midventricular)" LV-RLS-ratio. Native T1-mapping and ECV measurement are both superior to longitudinal strain measurement (with assessment of relative apical sparing) regarding the appropriate diagnosis of CA.

Diagnostic value of the novel CMR parameter "myocardial transit-time" (MyoTT) for the assessment of microvascular changes in cardiac amyloidosis and hypertrophic cardiomyopathy.

BACKGROUND: Coronary microvascular dysfunction (CMD) is present in various non-ischemic cardiomyopathies and in particular in those with left-ventricular hypertrophy. This study evaluated the diagnostic value of the novel cardiovascular magnetic resonance (CMR) parameter "myocardial transit-time" (MyoTT) in distinguishing cardiac amyloidosis from other hypertrophic cardiomyopathies. METHODS: N = 20 patients with biopsy-proven cardiac amyloidosis (CA), N = 20 patients with known hypertrophic cardiomyopathy (HCM), and N = 20 control patients without relevant cardiac disease underwent dedicated CMR studies on a 1.5-T MR scanner. The CMR protocol comprised cine and late-gadolinium-enhancement (LGE) imaging as well as first-pass perfusion acquisitions at rest for MyoTT measurement. MyoTT was defined as the blood circulation time from the orifice of the coronary arteries to the pooling in the coronary sinus (CS) reflecting the transit-time of gadolinium in the myocardial microvasculature. RESULTS: MyoTT was significantly prolonged in patients with CA compared to both groups: 14.8 ± 4.1 s in CA vs. 12.2 ± 2.5 s in HCM (p = 0.043) vs. 7.2 ± 2.6 s in controls (p < 0.001). Native T1 and extracellular volume (ECV) were significantly higher in CA compared to HCM and controls (p < 0.001). Both parameters were associated with a higher diagnostic accuracy in predicting the presence of CA compared to MyoTT: area under the curve (AUC) for native T1 = 0.93 (95% confidence interval (CI) = 0.83-1.00; p < 0.001) and AUC for ECV = 0.95 (95% CI = 0.88-1.00; p < 0.001)-compared to the AUC for MyoTT = 0.76 (95% CI = 0.60-0.92; p = 0.008). In contrast, MyoTT performed better than all other CMR parameters in differentiating HCM from controls (AUC for MyoTT = 0.93; 95% CI = 0.81-1.00; p = 0.003 vs. AUC for native T1 = 0.69; 95% CI = 0.44-0.93; p = 0.20 vs. AUC for ECV = 0.85; 95% CI = 0.66-1.00; p = 0.017). CONCLUSION: The relative severity of CMD (measured by MyoTT) in relationship to extracellular changes (measured by native T1 and/or ECV) is more pronounced in HCM compared to CA-in spite of a higher absolute MyoTT value in CA patients. Hence, MyoTT may improve our understanding of the interplay between extracellular/intracellular and intravasal changes that occur in the myocardium during the disease course of different cardiomyopathies.

Diagnostic value of cardiovascular magnetic resonance in comparison to endomyocardial biopsy in cardiac amyloidosis: a multi-centre study.

BACKGROUND: Cardiac amyloidosis (CA) is an infiltrative disease characterised by accumulation of amyloid deposits in the extracellular space of the myocardium-comprising transthyretin (ATTR) and light chain (AL) amyloidosis as the most frequent subtypes. Histopathological proof of amyloid deposits by endomyocardial biopsy (EMB) is the gold standard for diagnosis of CA. Cardiovascular magnetic resonance (CMR) allows non-invasive workup of suspected CA. We conducted a multi-centre study to assess the diagnostic value of CMR in comparison to EMB for the diagnosis of CA. METHODS: We studied N = 160 patients characterised by symptoms of heart failure and presence of left ventricular (LV) hypertrophy of unknown origin who presented to specialised cardiomyopathy centres in Germany and underwent further diagnostic workup by both CMR and EMB. If CA was diagnosed, additional subtyping based on EMB specimens and monoclonal protein studies in serum was performed. The CMR protocol comprised cine- and late-gadolinium-enhancement (LGE)-imaging as well as native and post-contrast T1-mapping (in a subgroup)-allowing to measure extracellular volume fraction (ECV) of the myocardium. RESULTS: An EMB-based diagnosis of CA was made in N = 120 patients (CA group) whereas N = 40 patients demonstrated other diagnoses (CONTROL group). In the CA group, N = 114 (95%) patients showed a characteristic pattern of LGE indicative of CA. In the CONTROL group, only 1/40 (2%) patient showed a "false-positive" LGE pattern suggestive of CA. In the CA group, there was no patient with elevated T1-/ECV-values without a characteristic pattern of LGE indicative of CA. LGE-CMR showed a sensitivity of 95% and a specificity of 98% for the diagnosis of CA. The combination of a characteristic LGE pattern indicating CA with unremarkable monoclonal protein studies resulted in the diagnosis of ATTR-CA (confirmed by EMB) with a specificity of 98% [95%-confidence interval (CI) 92-100%] and a positive predictive value (PPV) of 99% (95%-CI 92-100%), respectively. The EMB-associated risk of complications was 3.13% in this study-without any detrimental or persistent complications. CONCLUSION: Non-invasive CMR shows an excellent diagnostic accuracy and yield regarding CA. When combined with monoclonal protein studies, CMR can differentiate ATTR from AL with high accuracy and predictive value. However, invasive EMB remains a safe invasive gold-standard and allows to differentiate CA from other cardiomyopathies that can also cause LV hypertrophy.

Clinical experience regarding safety and diagnostic value of cardiovascular magnetic resonance in patients with a subcutaneous implanted cardioverter/defibrillator (S-ICD) at 1.5 T.

BACKGROUND: Cardiovascular magnetic resonance (CMR) studies in patients with implanted cardioverter/defibrillators (ICD) are increasingly required in daily clinical practice. However, the clinical experience regarding the feasibility as well as clinical value of CMR studies in patients with subcutaneous ICD (S-ICD) is still limited. Besides safety issues, image quality and analysis can be impaired primarily due the presence of image artefacts associated with the generator. METHODS: Twenty-three patients with an implanted S-ICD (EMBLEM, Boston Scientific, Marlborough, Massachusetts, USA; MR-conditional) with suspected cardiomyopathy and/or myocarditis underwent multi-parametric CMR imaging. Studies were performed on a 1.5 T CMR scanner after device interrogation and comprised standard a) balanced steady state free precession cine, b) T2 weighted-edema, c) velocity-encoded cine flow, d) myocardial perfusion, e) late-gadolinium-enhancement (LGE)-imaging and f) 3D-CMR angiography of the aorta. In case of substantial artefacts, alternative CMR techniques such as spoiled gradient-echo cine-sequences and wide-band inversion-recovery LGE (wb-LGE) sequences were applied. RESULTS: Successful CMR studies could be performed in all patients without any case of unexpected early termination or relevant technical complication other than permanent loss of the S-ICD system beeper volume in 52% of our patients. Assessment of cine-CMR images was predominantly impaired in the left ventricular (LV) anterior, lateral and inferior wall segments and a switch to spoiled gradient echo-based cine-CMR allowed an accurate assessment of cine-images in N = 17 (74%) patients with only limited artefacts. Hyperintensity artefacts in conventional LGE-images were predominantly observed in the LV anterior, lateral and inferior wall segments and image optimisation by use of the wb-LGE was helpful in 15 (65%) cases. Aortic flow measurements and 3D-CMR angiography were assessable in all patients Perfusion imaging artefacts precluded a meaningful assessment in at least one half of the patients. A benefit in clinical-decision making was documented in 17 (74%) patients in the present study. CONCLUSION: Safe 1.5 T CMR imaging was possible in all patients with an S-ICD, though the majority had permanent loss of the S-ICD beeper volume. Achieving good image quality may be challenging in some patients - particularly for perfusion imaging. Using spoiled gradient echo-based cine-sequences and wb-LGE sequences may help to reduce the extent of artefacts, thereby allowing accurate cardiac assessment. Thus, 1.5 T CMR studies should not be withhold in patients with S-ICD for safety concerns and/or fear of extensive imaging artefacts precluding successful image analysis.

Surprising diagnosis in a patient with acute coronary syndrome: a case report of acute streptococcal pharyngitis-associated perimyocarditis.

BACKGROUND: Streptococcal pharyngitis is a common infection, with both suppurative and non-suppurative complications. Most importantly, a streptococcal infection can cause heart disease in different pathophysiological pathways. Acute non-rheumatic perimyocarditis appears to be a more frequent pathological entity associated with streptococcal pharyngitis as once thought, which is poorly understood and explored. CASE SUMMARY: We present the case of a middle-aged man with acute chest pain, electrocardiogram (ECG) abnormalities, and elevated cardiac enzymes following a recent episode of pharyngitis in which streptococcal-associated perimyocarditis was diagnosed. Cardiovascular magnetic resonance (CMR) imaging established the diagnosis and allowed cardiac disease monitoring after successful antibiotic therapy resulting in complete clinical recovery. DISCUSSION: Patients presenting with acute chest pain, ECG abnormalities, and cardiac enzyme elevations do not always suffer from an ischaemic heart attack. A thorough investigation comprising a detailed past medical history and non-invasive imaging such as CMR are the cornerstones for unravelling a correct diagnosis and implementing a proper treatment-as was shown in the present clinical case.