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Background Data on the prognostic value of left ventricular (LV) morphological and functional parameters including LV rotation in patients with dilated cardiomyopathy (DCM) using cardiovascular magnetic resonance (CMR) are currently scarce. In this study, we assessed the prognostic value of global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS) and LV torsion using CMR feature tracking (FT). Methods CMR was performed in 350 DCM patients and 70 healthy subjects across 5 different European CMR Centers. Myocardial strain parameters were retrospectively assessed from conventional balanced steady-state free precession cine images applying FT. A combined primary endpoint (cardiac death, heart transplantation, aborted sudden cardiac death) was defined for the assessment of clinical outcome. Results GLS, GCS, GRS and LV torsion were significantly lower in DCM patients than in healthy subjects (all p Conclusion LV strain assessed with CMR FT has a high prognostic value in patients with DCM, surpassing routine and dedicated functional parameters. Thus, CMR strain imaging may contribute to the improvement of risk stratification in DCM.

Background Functional coronary disorders such as coronary spasm and microvascular dysfunction (including microvascular spasm and impaired microvascular dilatation) are frequent findings among patients with angina and non-obstructed coronary arteries (ANOCA). In this study, we investigated a potential association of coronary spasm and myocardial perfusion abnormalities as well as predictors of such functional coronary disorders in ANOCA patients using a multimodality diagnostic strategy including adenosine stress CMR and intracoronary acetylcholine testing. Methods We enrolled 129 patients with ANOCA who underwent acetylcholine testing and adenosine stress perfusion CMR. Patients were allocated to 3 groups according to their spasm testing result with regard to standardized COVADIS criteria: 1) epicardial spasm, 2) microvascular spasm, and 3) no spasm. The myocardial perfusion reserve index (MPRI) was semiquantitatively determined from adenosine stress perfusion CMR. Multivariate regression analyses were performed to identify predictors of coronary functional disorders. Results Patients with epicardial spasm had lower MPRI than patients without, whereas MPRI was preserved in patients with microvascular spasm. Multivariate analyses revealed age, previous myocardial infarction, LVEF and epicardial spasm as independent predictors of diminished MPRI, whereas previous PCI was associated with epicardial spasm, and female sex was a strong predictor of microvascular spasm. Conclusions Our results demonstrate coexistence of different functional coronary disorder endotypes involving the macro- and microvascular level of the coronary circulation in patients with ANOCA. We demonstrate that epicardial spasm is associated with diminished myocardial perfusion reserve and report further predictors of coronary functional disorders.

Background Quantitative cardiovascular magnetic resonance T1-mapping is increasingly used for myocardial tissue characterization. However, the lack of standardization limits direct comparability between centers and wider roll-out for clinical use or trials. Purpose To develop a quality assurance (QA) program assuring standardized T1 measurements for clinical use. Methods MR phantoms manufactured in 2013 were distributed, including ShMOLLI T1-mapping and reference T1 and T2 protocols. We first studied the T1 and T2 dependency on temperature and phantom aging using phantom datasets from a single site over 4 years. Based on this, we developed a multiparametric QA model, which was then applied to 78 scans from 28 other multi-national sites. Results T1 temperature sensitivity followed a second-order polynomial to baseline T1 values (R2 > 0.996). Some phantoms showed aging effects, where T1 drifted up to 49% over 40 months. The correlation model based on reference T1 and T2, developed on 1004 dedicated phantom scans, predicted ShMOLLI-T1 with high consistency (coefficient of variation 1.54%), and was robust to temperature variations and phantom aging. Using the 95% confidence interval of the correlation model residuals as the tolerance range, we analyzed 390 ShMOLLI T1-maps and confirmed accurate sequence deployment in 90%(70/78) of QA scans across 28 multiple centers, and categorized the rest with specific remedial actions. Conclusions The proposed phantom QA for T1-mapping can assure correct method implementation and protocol adherence, and is robust to temperature variation and phantom aging. This QA program circumvents the need of frequent phantom replacements, and can be readily deployed in multicenter trials., Highlights • CMR T1 correlated with reference T1 and T2; this derives the QA model for T1-map. • The proposed QA model is robust to temperature variations and phantom aging. • This QA method requires no frequent phantom replacements. • The T1-map QA program can be readily deployed in multicenter trials.

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.

Background Gadolinium-based contrast agents were not approved in the United States for detecting coronary artery disease (CAD) prior to the current studies. Objectives The purpose of this study was to determine the sensitivity and specificity of gadobutrol for detection of CAD by assessing myocardial perfusion and late gadolinium enhancement (LGE) imaging. Methods Two international, single-vendor, phase 3 clinical trials of near identical design, “GadaCAD1” and “GadaCAD2,” were performed. Cardiovascular magnetic resonance (CMR) included gadobutrol-enhanced first-pass vasodilator stress and rest perfusion followed by LGE imaging. CAD was defined by quantitative coronary angiography (QCA) but computed tomography coronary angiography could exclude significant CAD. Results Because the design and results for GadaCAD1 (n = 376) and GadaCAD2 (n = 388) were very similar, results were summarized as a fixed-effect meta-analysis (n = 764). The prevalence of CAD was 27.8% defined by a ≥70% QCA stenosis. For detection of a ≥70% QCA stenosis, the sensitivity of CMR was 78.9%, specificity was 86.8%, and area under the curve was 0.871. The sensitivity and specificity for multivessel CAD was 87.4% and 73.0%. For detection of a 50% QCA stenosis, sensitivity was 64.6% and specificity was 86.6%. The optimal threshold for detecting CAD was a ≥67% QCA stenosis in GadaCAD1 and ≥63% QCA stenosis in GadaCAD2. Conclusions Vasodilator stress and rest myocardial perfusion CMR and LGE imaging had high diagnostic accuracy for CAD in 2 phase 3 clinical trials. These findings supported the U .S. Food and Drug Administration approval of gadobutrol-enhanced CMR (0.1 mmol/kg) to assess myocardial perfusion and LGE in adult patients with known or suspected CAD.

Objectives The aim of this study was to investigate the prognosis of a large cohort of patients with stable angina and unobstructed coronaries undergoing acetylcholine spasm testing. Background Coronary artery spasm can be found in up to 60% of patients with symptoms of myocardial ischemia despite unobstructed coronary arteries. Methods Consecutive symptomatic patients with unobstructed coronary arteries undergoing acetylcholine testing to detect epicardial or microvascular coronary spasm were prospectively enrolled. After a median follow-up period of 7.2 years (6.5 to 7.9 years), data regarding mortality, nonfatal myocardial infarction, stroke, repeat coronary angiography, recurrent symptoms, and quality of life were obtained in 736 patients (57% women, mean age 62 ± 12 years). Results In total, 55 deaths (7.5%), 8 nonfatal myocardial infarctions (1.4%), and 12 strokes (2.2%) occurred during the follow-up period. Recurrent symptoms were reported by 64% of patients, and repeat coronary angiography was performed in 12% of cases. Multivariate analysis revealed epicardial spasm as a predictor of nonfatal myocardial infarction (hazard ratio: 14.469; 95% confidence interval: 1.735 to 120.646) and repeat angiography (hazard ratio: 1.703; 95% confidence interval: 1.062 to 2.732), whereas patients with microvascular spasm more often had recurrent angina at follow-up (hazard ratio: 1.311; 95% confidence interval: 1.013 to 1.697). Conclusions In this long-term follow-up study, the overall prognosis of patients with coronary spasm was favorable. Patients with epicardial spasm were at increased risk for myocardial infarction and repeat angiography, while microvascular spasm was associated with recurrent angina. Acetylcholine testing may help identify patients at increased risk for adverse cardiac events among this overall low-risk population.

Slow coronary flow is frequently seen during angiography in patients with angina and unobstructed coronary arteries. However, the pathophysiology of this finding remains largely unclear. We report a case of a 52-year-old woman with slow coronary flow caused by acetylcholine-induced microvascular spasm, as confirmed by intracoronary flow measurements. (Level of Difficulty: Beginner.), Graphical abstract, Slow coronary flow is frequently seen during angiography in patients with angina and unobstructed coronary arteries. However, the pathophysiology of this…

The symptoms and signs of myocarditis are non-specific. Thus, myocarditis is a differential diagnosis in many patients with heart complaints. As myocarditis may accompany common viral infections of the upper respiratory and gastrointestinal tracts, and mild ECG changes are not uncommon in such patients, the diagnosis needs to be considered in large patient cohorts. Establishing the correct diagnosis is of importance as the disease may lead to sudden cardiac death or dilated cardiomyopathy. As clinical tools such as history taking, physical examination, blood tests, the ECG, and the chest X-ray are not sufficient to ascertain the diagnosis of myocarditis, additional information from cardiac imaging techniques, or endomyocardial biopsy are necessary to confirm or exclude the disease. In daily clinical routine, however, the use of biopsy is limited to severely ill patients with reduced left ventricular function due to its invasiveness and potential complications. Thus, this chapter reviews how non-invasive cardiac imaging techniques can be used in clinical practice to diagnose myocarditis.

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. 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

The diagnostic performance of adenosine stress cardiovascular magnetic resonance (CMR) for the detection of significant stenosis in infarct-related arteries is widely unknown. Two different types of perfusion defects can be observed: (a) larger than or (b) equal size as scar.We hypothesized that: (a) defectscar predicts significant coronary stenosis, and (b) defect=scar predicts an unobstructed infarct-related artery, and (c) angina symptoms might be of additional value in stratification.Patients with previous myocardial infarction referred for work-up of myocardial ischemia undergoing adenosine stress CMR were included if they had coronary angiography within 4 weeks of CMR.Two hundred patients with a mean age of 66±11 years, ischemic scars (subendocardial/transmural), and a mean left ventricular ejection fraction of 53% were included. In patients with defectscar, the positive predictive value was excellent (88%) and typical angina was reported only in the stenosis group (P=0.002). However, patients with defect=scar (with 50% showing subendocardial scar) had a prevalence of 37% for stenosis, yielding a low negative predictive value of 63%. In this group, symptoms of typical angina were independent of stenosis (P=1.0).A perfusion defect larger than scar is highly predictive for significant stenosis in infarct-related arteries. However, more than a third of the patients with perfusion defect of equal size as scar also showed significant coronary stenosis. As half of these patients showed still viable (subendocardial) scars, there is a high-risk of reinfarction. The addition of angina symptoms seems to increase diagnostic accuracy only in patients with perfusion defects larger than scar.

Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Robert Bosch Stiftung; Deutsche Forschungsgemeinschaft Background Sudden cardiac death (SCD) is an appalling complication of hypertrophic cardiomyopathy (HCM). There is an ongoing discussion about the optimal SCD risk stratification strategy in HCM since established SCD risk models have suboptimal discriminative power. Objective To evaluate the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for SCD risk stratification compared to the ESC SCD risk score and traditional SCD risk factors in an >10-year follow-up study. Methods 220 consecutive patients with HCM and LGE-CMR were enrolled. Follow-up data was available in 203 patients (median age 58 years, 61% male) after a median follow-up period of 10.4 years. Results LGE was present in 70% of patients with a median LGE amount of 1.6%, the median ESC 5-year SCD risk score was 1.84. In the overall cohort, SCD rates were 2.3% at 5 years, 4.8% at 10 years, and 15.7% at 15 years, independent from established risk models. A LGE amount of >5% (LV mass) portends the highest risk for SCD with SCD prevalences of 5.5% at 5 years, 13.0% at 10 years and 33.3% at 15 years. Conversely, patients with no or ≤5% LGE amount (of LV mass) have favorable prognosis. Conclusions LGE-CMR in HCM patients allows effective 10-year SCD risk stratification beyond established risk factors. LGE amount might be added to established risk models to improve its discriminatory power. Specifically, patients with >5% amount of LGE should be carefully monitored and might be adequate candidates for primary prevention ICD during the clinical long-term course. Abstract Figure.

The purpose of this study was to investigate the diagnostic value of simultaneous hybrid cardiac magnetic resonance (CMR) andLate gadolinium enhancement (LGE) CMR and FDG-PET are both established imaging techniques for the detection of CS. However, there are limited data regarding the value of a comprehensive simultaneous hybrid CMR/FDG-PET imaging approach that includes CMR mapping techniques.Forty-three patients with biopsy-proven extracardiac sarcoidosis (median age: 48 years, interquartile range: 37-57 years, 65% male) were prospectively enrolled for evaluation of suspected CS. After dietary preparation for suppression of myocardial glucose metabolism, patients were evaluated on a 3-T hybrid PET/MR scanner. The CMR protocol included T1 and T2 mapping, myocardial function, and LGE imaging. We assumed aCS if PET and CMR (ie, LGE or T1/T2 mapping) were both positive (PET+/CMR+), cCS if PET was negative but CMR was positive (PET-/CMR+), and no CS if patients were CMR negative regardless of PET findings.Among the 43 patients, myocardial glucose uptake was suppressed successfully in 36 (84%). Hybrid CMR/FDG-PET revealed aCS in 13 patients (36%), cCS in 5 (14%), and no CS in 18 (50%). LGE was present in 14 patients (39%); T1 mapping was abnormal in 10 (27%) and T2 mapping abnormal in 2 (6%). CS was diagnosed based on abnormal T1 mapping in 4 out of 18 CS patients (22%) who were LGE negative. PET FDG uptake was present in 17 (47%) patients.Comprehensive simultaneous hybrid CMR/FDG-PET imaging is useful for the detection of CS and provides additional value for identifying active disease. Our results may have implications for enhanced diagnosis as well as improved identification of patients with aCS in whom anti-inflammatory therapy may be most beneficial.

A fast and reliable diagnosis of cardiac amyloidosis requires a significant amount of clinical awareness. It is especially important to come to an early diagnosis in patients with cardiac AL amyloidosis in order to improve the otherwise unfavourable clinical course in these patients. There is a significant increase in the number of patients with cardiac amyloidosis of the ATTR wild-type variety. These patients are often elderly males presenting with predominantly right sided heart failure. We present a diagnostic pathway enabling a structured approach to these patients using multimodality cardiac imaging and endomyocardial biopsy. Early chemotherapy is the key to improving symptoms in patients with AL amyloidosis. In contrast, pharmacologic approaches for treating cardiac ATTR amyloidosis need further research and clinical trials.

Myocarditis is an important cause of arrhythmias and sudden cardiac death (SCD) in both physically active individuals and athletes. Elite athletes seem to have an increased risk for viral infection and subsequent myocarditis due to increased exposure to pathogens (worldwide traveling/international competition) or impaired immune system (continuing training during infections/resuming training early thereafter, strenuous exercise training or competition, and exercising in extreme weather conditions). Initial clinical presentation is variable, but athletes characteristically express non-specific symptoms of fatigue, muscle soreness, increased heart rate at rest, as well as during exercise and reduced overall exercise capacity. Beyond resting electrocardiogram (ECG), cardiac biomarkers, echocardiography, and 24-hour Holter ECG, diagnostic work-up should include cardiac magnetic resonance imaging (CMR) assessing inflammation, oedema, and fibrosis by late gadolinium enhancement (LGE), respectively, as these measures are crucial for prognosis and sports eligibility. For patients with insufficient cardiac recovery, endomyocardial biopsy is recommended to clarify differential diagnoses and initiate specific treatment options. In uncomplicated cases with normal left ventricular function during acute phase and absent LGE, eligibility for sports can be attested to three months after clinical recovery. In those with persistent pathological findings, even after six months, the risk for SCD remains increased and resuming exercise beyond recreational activities can only be recommended individually based on course of disease, left ventricular function, arrhythmias, pattern of LGE in CMR, as well as intensity and volume of exercise performed during training and competition. For all athletes, follow-up examination should be performed yearly.

Purpose Myocarditis is a common cardiac disease that is associated with significant mortality as demonstrated by several studies. Late gadolinium enhancement (LGE) by cardiac magnetic resonance imaging (CMR) is a valuable tool for risk stratification of patients with suspected myocarditis. Previous studies using CMR-LGE have reported a good negative predictive value over follow-up periods of 4–6 years, while its positive predictive value was only modest. However, there is a lack of data regarding the long-term prognosis (>10 years) of these patients. This study reports an extended long-term follow-up of a large cohort of patients with biopsy-proven viral myocarditis. Methods At initial presentation, all patients underwent endomyocardial biopsy and CMR for the work-up of suspected myocarditis or unclear cardiomyopathy and had evidence of virus in PCR analyses. The primary endpoints were: all-cause death, cardiac death, and the occurrence of sudden cardiac death (SCD). Results 183 patients with biopsy-proven viral myocarditis were followed for a median of 11.5 years. At baseline, patients were 52 years old, 31% were females, and the median ejection fraction was moderately reduced (44%). Initial CMR assessment revealed LGE in 101 (55%) patients, while 82 (45%) patients had LGE-negative CMR. During the follow-up period, the following endpoints occurred in the overall cohort: all-cause death (n=71, 39%), cardiac death (n=50, 27%) and SCD (n=20, 11%). Most importantly, only a single LGE-negative patient experienced a SCD during this 12-year follow-up, while all other SCDs occurred in patients with LGE-positive CMR (1% vs. 19%, p Conclusion This cohort of biopsy-proven viral myocarditis demonstrates substantial mortality (39% in 11.5 years). However, absence of LGE on CMR was associated with favorable prognosis. This was applicable regarding all-cause and cardiac mortality, but most importantly with regard to SCD with a NPV of 98% over almost 12 years median follow-up. Acknowledgement/Funding This work was funded by the Robert-Bosch-Stiftung, Stuttgart, Germany and the Berthold-Leibinger-Stiftung, Ditzingen, Germany.

Background Acute complete occlusion of a coronary artery results in progressive ischemia, moving from the endocardium to the epicardium (“wavefront”). Dependent on time-to-reperfusion and collateral flow, myocardial infarction (MI) will manifest, with transmural MI portending poor prognosis. Late gadolinium enhancement cardiac magnetic resonance (LGE-CMR) imaging can detect MI with high diagnostic accuracy. Primary percutaneous coronary intervention (PCI) is the preferred reperfusion strategy in patients with STEMI Purpose We sought to visualize time-dependent necrosis in a ST-segment elevation myocardial infarction (STEMI) population by LGE-CMR. Methods STEMI patients with: single-vessel disease, complete occlusion with Thrombolysis in Myocardial Infarction (TIMI) score 0, absence of collateral flow (Rentrop score 0) and symptom onset Results 164 patients (54±11 years, 80% male) were included. Receiver-operating-characteristic (ROC)-curve (area under the curve [AUC] = 0.81) indicating transmural necrosis revealed the best diagnostic cut-off for a symptom-to-balloon time of 121 minutes, i.e. patients with >121 minutes demonstrated increased IS, transmurality index, transmurality grade (all p-values Conclusions In myocardial infarction with no residual antegrade, and no collateral flow, immediate reperfusion is vital. A symptom-to-balloon time of >121 minutes causes a high grade of transmural necrosis. In the present, pure STEMI population, time to reperfusion to salvage myocardium was less than suggested by current guidelines.

Background Patients with signs and symptoms of myocardial ischemia yet unobstructed coronary arteries represent a diagnostic and therapeutic challenge. Coronary vasomotor disorders such as coronary epicardial or microvascular spasm are frequently found among these patients. They can be diagnosed using intracoronary acetylcholine testing (ACH-test). It has been shown that patients with epicardial spasm have a worse prognosis compared to patients with microvascular spasm. The reasons for this finding are however not apparent. We speculated in this study that patients with epicardial spasm have a worse vasomotor dysfunction compared to patients with microvascular spasm or normal ACH-test. To assess this hypothesis all patients in this study not only underwent ACH-testing but in addition also adenosine stress perfusion cardiac MRI (CMR) with calculation of the myocardial perfusion reserve index (MPRI). The latter method allows for assessment of vasodilatory function compared to the vasoconstrictor assessment using acetylcholine. Methods Between 2012 and 2016, 129 consecutive patients (mean age 64±13 years, 46% female) with signs and symptoms of myocardial ischemia yet unobstructed coronary arteries were enrolled in this study. All patients underwent ACH-testing as well as adenosine stress perfusion CMR. According to the results of the acetylcholine test, patients were allocated to 3 groups: a) epicardial spasm (angina, ischemic ECG changes and >75% coronary diameter reduction), b) microvascular spasm (angina, ischemic ECG changes and Results Epicardial and microvascular spasm was found in 31 (24%) and 69 (53%) patients, respectively, while 29 (22%) patients had no evidence of coronary spasm on ACH-testing. Women were more likely to have microvascular spasm than men (68% vs. 36%, p Conclusion MPRI determined by stress perfusion CMR was significantly reduced in patients with epicardial spasm compared to those with microvascular spasm or normal ACH-test. This could indicate that patients with epicardial spasm have a more generalized coronary vasomotor disorder compared to other patients. This may be the reason for the worse outcome observed and could lead to more aggressive medical therapy and closer follow-up. Acknowledgement/Funding This work was funded by the Robert-Bosch-Stiftung, Stuttgart, Germany and the Berthold-Leibinger-Stiftung, Ditzingen, Germany.

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.

Objectives Rheumatoid arthritis (RA) patients are at increased risk of suffering from adverse cardiovascular events. Cardiovascular magnetic resonance (CMR) mapping techniques might be appropriate tools to complement late gadolinium enhancement (LGE) for the assessment of myocardial involvement. This study aimed to perform advanced myocardial tissue characterisation in RA patients by a multicomponent CMR protocol. Methods 22 RA patients were prospectively enrolled and underwent CMR, including LGE and T1/T2 mapping sequences; 20 volunteers served as controls. Results Mean LV-EF was 66%; prevalence of LGE was 18%. RA patients had increased native T1 (985 vs. 959 ms, p = 0.03), expanded extracellular volume (ECV) (27 vs. 25%, p = 0.02) and higher T2 values (52 vs. 49 ms, p

Aims Intracoronary infusion of autologous nucleated bone marrow cells (BMCs) enhanced the recovery of left ventricular ejection fraction (LVEF) after ST-segment elevation myocardial infarction (STEMI) in the randomised-controlled, open-label BOOST trial. We reassessed the therapeutic potential of nucleated BMCs in the randomised placebo-controlled, double-blind BOOST-2 trial conducted in 10 centres in Germany and Norway. Methods and results Using a multiple arm design, we investigated the dose-response relationship and explored whether γ-irradiation which eliminates the clonogenic potential of stem and progenitor cells has an impact on BMC efficacy. Between 9 March 2006 and 16 July 2013, 153 patients with large STEMI were randomly assigned to receive a single intracoronary infusion of placebo (control group), high-dose (hi)BMCs, low-dose (lo)BMCs, irradiated hiBMCs, or irradiated loBMCs 8.1 ± 2.6 days after percutaneous coronary intervention (PCI) in addition to guideline-recommended medical treatment. Change in LVEF from baseline (before cell infusion) to 6 months as determined by MRI was the primary endpoint. The trial is registered at Current Controlled Trials (ISRCTN17457407). Baseline LVEF was 45.0 ± 8.5% in the overall population. At 6 months, LVEF had increased by 3.3 percentage points in the control group and 4.3 percentage points in the hiBMC group. The estimated treatment effect was 1.0 percentage points (95% confidence interval, -2.6 to 4.7; P = 0.57). The treatment effect of loBMCs was 0.5 percentage points (-3.0 to 4.1; P = 0.76). Likewise, irradiated BMCs did not have significant treatment effects. BMC transfer was safe and not associated with adverse clinical events. Conclusion The BOOST-2 trial does not support the use of nucleated BMCs in patients with STEMI and moderately reduced LVEF treated according to current standards of early PCI and drug therapy.

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 55yrs. 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.

In this study, we sought to investigate the impact of baseline calibration, which is used in quantitative cardiac MRI perfusion analysis to correct for surface coil inhomogeneity and noise, on myocardial perfusion reserve index (MPRI) and its contribution to previously reported paradoxical low MPRI

Background Adenosine is used in stress perfusion cardiac imaging to reveal myocardial ischemia by its vasodilator effects. Caffeine is a competitive antagonist of adenosine. However, previous studies reported inconsistent results about the influence of caffeine on adenosine's vasodilator effect. This study assessed the impact of caffeine on the myocardial perfusion reserve index (MPRI) using adenosine stress cardiovascular magnetic resonance imaging (CMR). Moreover, we sought to evaluate if the splenic switch-off sign might be indicative of prior caffeine consumption. Methods Semiquantitative perfusion analysis was performed in 25 patients who underwent: 1) caffeine-naïve adenosine stress CMR demonstrating myocardial ischemia and, 2) repeat adenosine stress CMR after intake of caffeine. MPRI (global; remote and ischemic segments), and splenic perfusion ratio (SPR) were assessed and compared between both exams. Results Global MPRI after caffeine was lower vs. caffeine-naïve conditions (1.09 ± 0.19 vs. 1.24 ± 0.19; p

The major guidelines on stable coronary artery disease recommend revascularizing patients with large areas of myocardium at risk. The algorithms on how to prove that such high risk is present differ considerably. The opinions on the use of coronary CT (calcium scoring and angiography) vary widely. This review aims to summarize the recommendations of the major guidelines, commenting on differences between the guidelines and discussing whether extending the role of coronary CT angiography should be considered in the light of new CT data.

Supplemental Digital Content is available in the text., Background: The common intronic deletion, MYBPC3Δ25, detected in 4% to 8% of South Asian populations, is reported to be associated with cardiomyopathy, with ≈7-fold increased risk of disease in variant carriers. Here, we examine the contribution of MYBPC3Δ25 to hypertrophic cardiomyopathy (HCM) in a large patient cohort. Methods: Sequence data from 2 HCM cohorts (n=5393) was analyzed to determine MYBPC3Δ25 frequency and co-occurrence of pathogenic variants in HCM genes. Case-control and haplotype analyses were performed to compare variant frequencies and assess disease association. Analyses were also undertaken to investigate the pathogenicity of a candidate variant MYBPC3 c.1224-52G>A. Results: Our data suggest that the risk of HCM, previously attributed to MYBPC3Δ25, can be explained by enrichment of a derived haplotype, MYBPC3Δ25/−52, whereby a small subset of individuals bear both MYBPC3Δ25 and a rare pathogenic variant, MYBPC3 c.1224-52G>A. The intronic MYBPC3 c.1224-52G>A variant, which is not routinely evaluated by gene panel or exome sequencing, was detected in ≈1% of our HCM cohort. Conclusions: The MYBPC3 c.1224-52G>A variant explains the disease risk previously associated with MYBPC3Δ25 in the South Asian population and is one of the most frequent pathogenic variants in HCM in all populations; genotyping services should ensure coverage of this deep intronic mutation. Individuals carrying MYBPC3Δ25 alone are not at increased risk of HCM, and this variant should not be tested in isolation; this is important for the large majority of the 100 million individuals of South Asian ancestry who carry MYBPC3Δ25 and would previously have been declared at increased risk of HCM.

A fast and reliable diagnosis of cardiac amyloidosis requires a significant amount of clinical awareness. It is especially important to come to an early diagnosis in patients with cardiac AL amyloidosis in order to improve the otherwise unfavourable clinical course in these patients. There is a significant increase in the number of patients with cardiac amyloidosis of the ATTR wild-type variety. These patients are often elderly males presenting with predominantly right sided heart failure. We present a diagnostic pathway enabling a structured approach to these patients using multimodality cardiac imaging and endomyocardial biopsy. Early chemotherapy is the key to improving symptoms in patients with AL amyloidosis. In contrast, pharmacologic approaches for treating cardiac ATTR amyloidosis need further research and clinical trials.