Results The 598 patients (age 59 ± 12 years, 20% female underwent stress CMR a median of 93 days (IQR: 41, 224 days) after coronary stenting with follow-up for 1.4 years (IQR: 0.6-2.7). Inducible perfusion defects were identified in 294 (49%) patients of whom 18 (6%) died during follow-up compared with 6 (2.0%) patients with no perfusion defects (p = 0.01). Of the 294 patients with perfusion defects, 70 (24%) were revascularised (PCI 55, CABG 27) of whom 5 (7%) died during follow-up compared with 13 (6%) who were not revascularised(p = 0.68). K-M survival analysis confirmed that revascularisation was unassociated with survival benefit, regardless of the severity of ischaemia (Figure 1).
Cardiac death is the leading cause of mortality in patients with sarcoidosis, yet cardiac involvement often remains undetected. Cardiovascular magnetic resonance imaging (CMR) and 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) have been used to diagnose cardiac sarcoidosis (CS) yet never simultaneously in a cohort. This study sought to assess the diagnostic and prognostic utility of simultaneous hybrid cardiac PET/MR.Fifty-one consecutive patients with suspected CS (age 50 ± 13 years, 31 males) underwent simultaneous PET/MR following a high-fat/low-carbohydrate diet and 12-h fast. Blinded image analysis of FDG uptake and late gadolinium enhancement (LGE) was performed using the American Heart Association (AHA) 16-segment model. The sensitivity and specificity of PET/MR for diagnosing CS was estimated using the Japanese Ministry of Health and Welfare guidelines. The primary endpoint was a composite of death, aborted sudden cardiac death, sustained ventricular arrhythmia, complete heart block, and hospital admission with decompensated heart failure. The secondary endpoints were a fall in left ventricular ejection fraction (LVEF) >10%, non-sustained ventricular tachycardia and other cardiac-related hospital admission. The prevalence of CS was 65% (n = 33). The sensitivity of PET and CMR alone for detecting CS was 0.85 and 0.82, respectively. Hybrid PET/MR was superior for detecting CS with sensitivity, specificity, positive, and negative predictive values of 0.94, 0.44, 0.76, and 0.80, respectively. There was poor inter-modality agreement for the location of cardiac abnormalities (k = 0.02). Over the median follow-up of 2.2 years, there were 18 (35%) adverse events. Cardiac RV PET abnormalities and presence of LGE were independent predictors of adverse events. Abnormalities found on both PET and magnetic resonance imaging was the strongest predictor of major adverse cardiac events.Simultaneous PET/MR is an accurate method for diagnosing CS. FDG-PET and CMR combined offers complementary information on disease pathophysiology. The presence of LGE and FDG uptake on PET/MR identifies patients at higher risk of adverse events. PET and CMR should therefore be considered in the assessment of disease presence, stage, and prognosis in CS.
Background: Apical hypertrophic cardiomyopathy (HCM) is commonly associated with drug-refractory chest pain. We sought to determine whether, in apical HCM, coronary perfusion time is abbreviated by the diastolic persistence of apical contraction, resulting in impaired myocardial perfusion and chest pain. Methods: 62 apical HCM patients had cardiac magnetic resonance (CMR) scans assessed for stress perfusion (myocardial perfusion reserve index (MPRi)), late gadolinium enhancement (LGE; % of myocardial volume) left ventricular (LV) volumes and LV contractile persistence (% total cardiac cycle) at the LV apex and base. Radial and circumferential strain were assessed. Patients were divided into three groups on the basis of severity of contractile persistence. The interval between earliest and latest systolic peaks was measured from strain data from each of the apical segments. Results: Compared to subjects with the least contractile persistence (C1), those with the most (C3) were more likely to have chest pain (94% vs 63%, p<0.05) and lower MPRi (0.90±0.24 vs 1.43±0.50, p<0.05). Multiple regression analyses included contractile persistence, LVH, %LGE, age and gender. Contractile persistence was independently associated with chest pain (0.4 per 10% cardiac cycle, CI 95%; 0.1 to 0.8, p<0.05) and a reduction in apical MPRi (-0.09 per 10% cardiac cycle, CI 95%; -0.04 to -0.15, p<0.01). There were striking differences in systolic strain between C1 and C3. First, radial strain was almost absent in C3, with only post-systolic contraction detected. Second, temporal dispersion in circumferential strain was greater in C3 than C1 (230±101ms vs 114±44ms, p<0.05). Using the convention >130ms as a threshold, circumferential dyssynchrony was present in 25% of C1 and 81% of C3 patients (p<0.001) and radial dyssynchrony in 65% of C1 and 95% of C3 patients (p<0.05). In patients with radial dyssynchrony, the earliest peak was most often in the inferior or anterior segments (60%) and the latest in the lateral segment (33%). In patients with circumferential dyssynchrony, the earliest peak was most often in the inferior or anterior segments (59%) and the latest in the lateral segment (41%). Conclusion: In apical HCM, regional persistence of contractility into diastole causes myocardial ischaemia and chest pain. This is the first description of contractile persistence and dyssynchrony as a mechanism for myocardial perfusion abnormalities and presents novel therapeutic opportunities for drug-refractory chest pain in apical HCM.
Abstract Introduction Desmoplakin (DSP) gene mutations are reported to cause arrhythmogenic right ventricular cardiomyopathy (ARVC), but recent evidence suggests that they manifest as a much broader range of phenotypes. Purpose To describe the phenotypic characteristics of DSP mutation carriers in a consecutive cohort of patients with heart muscle disease. Methods A retrospective analysis of consecutive patients with heart muscle disease and their relatives that underwent diagnostic or research genetic testing. Only DSP variants classified as pathogenic by the American College of Medical Genetics and Genomics criteria were considered. Dilated cardiomyopathy (DCM), hypokinetic non-dilated cardiomyopathy (HNDC) and ARVC were diagnosed in accordance with current criteria. Results 109 mutation carriers were identified: 34 (31%) had DCM and 23 (21%) HNDC. ARVC diagnosis was borderline and definite in 39 (36%) and 39 (36%) individuals, respectively. Sixteen (15%) presented with clinically suspected myocarditis. 23 patients met both definite AC and DCM criteria and 13 patients met definite AC and HNDC criteria. In 87 patients with cardiac magnetic resonance imaging, a spectrum of disease was observed (Table) including an intermediate phenotype characterised by subepicardial LV fibrosis in the absence of ventricular dilatation or dysfunction. Ventricular dysfunction was manifest as left dominant disease (n=22, 48%) and biventricular disease (n=24, 52%); none had right dominant disease. Conclusions DSP mutations are associated with left and biventricular phenotypes and can present as myocarditis. Current ARVC and DCM diagnostic criteria inadequately describe the clinical subtypes of DSP related disease. A novel approach to disease classification is proposed. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): British Heart Foundation
Objectives: To determine whether access to rapid access chest pain clinics of people with recent onset symptoms is equitable by age, socioeconomic status, ethnicity and gender, according to need. Design: Retrospective cohort study with ecological analysis. Setting: Patients referred from primary care to five rapid access chest pain clinics in secondary care, across England. Participants: Of 8647 patients aged $35 years referred to chest pain clinics with new-onset stable chest pain but no known cardiac history, 7570 with documented census ward codes, age, gender and ethnicity comprised the study group. Patients excluded were those with missing date of birth, gender or ethnicity (n¼782) and those with missing census ward codes (n¼295). Outcome measures: Effects of age, gender, ethnicity and socioeconomic status on clinic attendance were calculated as attendance rate ratios, with number of attendances as the outcome and resident populationyears as the exposure in each stratum, using Poisson regression. Attendance rate ratios were then compared with coronary heart disease (CHD) mortality ratios to determine whether attendance was equitable according to need. Results: Adjusted attendance rate ratios for patients aged >65 years were similar to younger patients (1.1, 95% CI 1.05 to 1.16), despite population CHD mortality rate ratios nearly 15 times higher in the older age group. Women had lower attendance rate ratios (0.81, 95% CI 0.77 to 0.84) and also lower population CHD mortality rate ratios compared with men. South Asians had higher attendance rates (1.67, 95% CI 1.57 to 1.77) compared with whites and had a higher standardised CHD mortality ratio of 1.46 (95% CI 1.41 to 1.51). Although univariable analysis showed that the most deprived patients (quintile 5) had an attendance rate twice that of less deprived quintiles, the adjusted analysis showed their attendance to be 13% lower (0.87, 95% CI 0.81 to 0.94) despite a higher population CHD mortality rate.
Diagnostic models play an important role in the management of suspected angina but provide no explicit information about prognosis. The objective of this study was to develop a prognostic model to predict 10-year coronary mortality in patients presenting for the first time with suspected angina to complement the updated Diamond-Forrester diagnostic model of disease probability.1
Methods
A multicentre cohort of 8762 patients with suspected angina was followed up for a median of 10 years during which 233 coronary deaths were observed. Developmental (n = 4412) and validation (n = 4350) prognostic models based on clinical data available at first presentation showed good performance with close agreement and the final model utilised all 8762 patients to maximize power.
Results
The prognostic model showed strong associations with coronary mortality forage, sex, typicality of chest pain, smoking status, diabetes, pulse rate and ECG findings. Model discrimination was good (C statistic 0.83), patients in the highest risk quarter accounting for 173 coronary deaths during follow-up (10 year risk of death: 8.7%) compared with a total of 60 deaths in the three lower risk quarters. Observed 10-year coronary mortality increased with increasing estimates of disease probability, ranging from 0.2% to 25.4% with CAD probability of <10% and >90%, respectively. However, when our prognostic model was simplified to incorporate only those factors used by the updated Diamond-Forrester (age, sex and character of symptoms) it under-estimated coronary mortality risk, particularly in patients with risk factors.
Conclusion
For the first time in patients with suspected angina, a prognostic model is presented based on simple clinical factors available at the initial cardiological assessment. The model discriminated powerfully between patients at high risk and at lower risk of coronary death during the 10-year follow-up period. Its potential clinical utility was reflected in the prognostic value it added to the updated Diamond-Forrester diagnostic model of disease probability.
Reference
Genders TS et al. CAD Consortium. A clinical prediction rule for the diagnosis of coronary artery disease: validation, updating, and extension. Eur Heart J. 2011;32(11):1316-– 30
Background The first MRI conditional pacemaker was introduced in 2008. Though, they represent the minority of all devices implanted, their use has increased. Similarly, CMR imaging in the UK has increased from just over 20,000 studies in 2008 to nearly 40,000 scans in 2010. However, there is limited data describing the frequency, safety and diagnostic quality of CMR scans in patients with MR conditional devices. Our aim was to describe device scanning in our clinical practice and identify reasons why MR conditional pacemakers were selected.
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