Dilated cardiomyopathy as the first presentation of coeliac disease: association or causation?

Global ventricular impairment is a frequent presentation in clinical practice, but dissection of causative mechanisms from clinical associations is challenging. We present the case of a 19-year-old man who presented with dilated cardiomyopathy as the first presentation of coeliac disease. The manifestation of iron deficiency anaemia prompted gastroenterology input and enabled accurate diagnosis. It is unclear whether coeliac disease was simply coexistent or directly implicated in pathophysiology. Mechanisms may relate to nutritional deficiencies or autoimmune myocarditis arising from cross-reactivity. We advocate early multidisciplinary involvement in such contexts to aid with management strategy. Despite adherence to a gluten-free diet, ventricular dysfunction persisted and he has been referred to a cardiac transplant centre.

Clinical experiences of delayed contrast enhancement with cardiac computed tomography: case series.

BACKGROUND: Myocardial delayed enhancement (MDE) by gadolinium-enhanced cardiac MRI is well established for myocardial scar assessment in ischemic and non-ischemic heart disease. The role of MDE by cardiac CT (CT-MDE) is not yet defined. FINDINGS: We reviewed all clinical cases of CT-MDE at a tertiary referral center to present the cases as a case series. All clinical cardiac CT exams which utilized CT-MDE imaging between January 1, 2005 and October 1, 2010 were collected as a series and their findings were also compared with available myocardial imaging to assess for myocardial abnormalities, including echocardiography (wall motion, morphology), cardiac MRI (delayed enhancement, morphology), SPECT MPI (perfusion defects). 5,860 clinical cardiac CT exams were performed during the study period. CT-MDE was obtained in 18 patients and was reported to be present in 9 patients. The indications for CT-MDE included ischemic and non-ischemic heart diseases. In segments positive for CT-MDE, there was excellent agreement of CT with other modalities: echocardiography (n=8) demonstrated abnormal morphology and wall motion (k=1.0 and k=0.82 respectively); prior MRI (n=2) demonstrated abnormal delayed enhancement (MR-MDE) (k=1.0); SPECT MPI (n=1) demonstrated fixed perfusion defects (k=1.0). In the subset of patients without CT-MDE, no abnormal segments were identified by echocardiography (n=8), MRI (n=1) and nuclear MPI (n=0). CONCLUSIONS: CT-MDE was performed in rare clinical situations. The indications included both ischemic and non-ischemic heart disease and there was an excellent agreement between CT-MDE and abnormal myocardium by echocardiography, cardiac MRI, and nuclear MPI.

The Prognostic Significance of Resting Regional Left Ventricular Function in Patients With Varying Degrees of Myocardial Ischemia.

BACKGROUND: Our aim was to determine whether regional left ventricular (LV) function on a resting transthoracic echo (TTE) provides prognostic information in patients with varying degrees of ischemia on myocardial perfusion imaging. METHODS: Between 2004 - 2009, we identified 503 patients (mean age 69 (SD 11); 79% male) with reversible ischemia on a myocardial SPECT scan who had a TTE within 30 days. We evaluated the rate of subsequent revascularization and death for all patients. RESULTS: Following the SPECT scan and TTE, 246/503(49%) patients underwent revascularization, 64/503 (13%) patients died, 369 (73%) patients had a normal left ventricular ejection fraction (LVEF), 242 (48%) patients had a resting wall motion abnormality (WMA), 21/261 (8%) with no WMA died compared to 43/242 (18%) in patients with a WMA. In patients with a WMA (n = 242) there was no significant difference in mortality when comparing patients with small (< 6 segments) and large (> 6 segments) WMA (P = 0.44). In patients with moderate/severe ischemia, the presence of a resting WMA was associated with a higher mortality rate (18% v 7%; P = 0.005). In a multivariable model, LVEF (< 50%) was associated with a hazard ratio of 2.2 (P = 0.002, 95% CI 1.34 - 3.68) however, WMA and number of abnormal segments did not reach statistical significance. CONCLUSION: A resting wall motion abnormality in patients with moderate/severe ischemia is associated with a higher mortality compared to patients with mild ischemia on myocardial perfusion imaging. Regional left ventricular dysfunction unlike LVEF was not an independent predictor of mortality.

The missing left atrial appendage.

A 34 year old man underwent a transesophageal echocardiogram (TEE) prior to implantation of a biventricular ICD and DC cardioversion, to exclude left atrium and left atrial appendage thrombus. He had a history of repaired tetralogy of Fallot as a child, Stickler syndrome, atrial flutter and was status post recent mitral valve replacement, pulmonary valve replacement and tricuspid valve repair. The left atrial appendage was not visualized on TEE. A cardiac CT clarified that there was a left atrial appendage and provided an explanation as to why it was not visualized on TEE, highlighting the importance of multimodality imaging in patients with complex congenital heart disease.

Association of frontal QRS-T angle--age risk score on admission electrocardiogram with mortality in patients admitted with an acute coronary syndrome.

Risk assessment is central to the management of acute coronary syndromes. Often, however, assessment is not complete until the troponin concentration is available. Using 2 multicenter prospective observational studies (Evaluation of Methods and Management of Acute Coronary Events [EMMACE] 2, test cohort, 1,843 patients; and EMMACE-1, validation cohort, 550 patients) of unselected patients with acute coronary syndromes, a point-of-admission risk stratification tool using frontal QRS-T angle derived from automated measurements and age for the prediction of 30-day and 2-year mortality was evaluated. Two-year mortality was lowest in patients with frontal QRS-T angles <38° and highest in patients with frontal QRS-T angles >104° (44.7% vs 14.8%, p <0.001). Increasing frontal QRS-T angle-age risk (FAAR) scores were associated with increasing 30-day and 2-year mortality (for 2-year mortality, score 0 = 3.7%, score 4 = 57%; p <0.001). The FAAR score was a good discriminator of mortality (C statistics 0.74 [95% confidence interval 0.71 to 0.78] at 30 days and 0.77 [95% confidence interval 0.75 to 0.79] at 2 years), maintained its performance in the EMMACE-1 cohort at 30 days (C statistics 0.76 (95% confidence interval 0.71 to 0.8] at 30 days and 0.79 (95% confidence interval 0.75 to 0.83] at 2 years), in men and women, in ST-segment elevation myocardial infarction and non-ST-segment elevation myocardial infarction, and compared favorably with the Global Registry of Acute Coronary Events (GRACE) score. The integrated discrimination improvement (age to FAAR score at 30 days and at 2 years in EMMACE-1 and EMMACE-2) was p <0.001. In conclusion, the FAAR score is a point-of-admission risk tool that predicts 30-day and 2-year mortality from 2 variables across a spectrum of patients with acute coronary syndromes. It does not require the results of biomarker assays or rely on the subjective interpretation of electrocardiograms.

An assessment of the concentration-related prognostic value of cardiac troponin I following acute coronary syndrome.

In 2004 the British Cardiac Society redefined myocardial infarction by cardiac troponin I (cTnI) concentration: ≤ 0.06 µg/L (unstable angina), >0.06 to < 0.5 µg/L (myocardial necrosis), and ≥ 0.5 µg/L (myocardial infarction). We investigated the effects of this classification on all-cause mortality in 1,285 patients from the Evaluation of the Methods and Management of Acute Coronary Events (EMMACE)-2 registry. There were 528 deaths (6.6-year all-cause mortality 41.1%). Survival was greatest in the cTnI ≤ 0.06-µg/L subgroup at 30 days (p = 0.005), 6 months (p = 0.015), 1 year (p = 0.002), and 6.6 years (p = 0.045). After adjustment there was no significant difference in survival between the cTnI >0.06- to < 0.5-µg/L and ≥ 0.5-µg/L subgroups. Increased mortality (hazard ratio, 95% confidence interval) was associated with ages 70 to 80 years (2.58, 1.17 to 3.91) and >80 years (3.30, 3.50 to 5.06), peripheral vascular disease (1.50, 1.16 to 1.94), heart failure (1.36, 1.05 to 1.83), diabetes mellitus (1.68, 1.36 to 2.07), severe left ventricular systolic dysfunction (1.50, 1.00 to 2.21), and creatinine per 10 µmol/L (1.65, 1.02 to 1.08), whereas ages 50 to 60 years (0.55, 0.32 to 0.96), ß blockers (0.53, 0.44 to 0.64), aspirin (0.80 0.65 to 0.99), angiotensin-converting enzyme inhibitors (0.67, 0.56 to 0.80), statins (0.73, 0.59 to 0.90), and revascularization (0.33, 0.12 to 0.92) were associated with a lower risk of death. In conclusion, although quantitative evaluation of cTnI concentration in patients with acute coronary syndrome with cTnI > 0.06 µg/L was associated with no added prognostic information, the dichotomization of patients by cTnI status ("positive" and "negative") facilitates acute coronary syndrome risk stratification.

Heart-type fatty acid-binding protein predicts long-term mortality and re-infarction in consecutive patients with suspected acute coronary syndrome who are troponin-negative.

OBJECTIVES: The purpose of this study was to establish the prognostic value of measuring heart fatty acid-binding protein (H-FABP) in patients with suspected acute coronary syndrome (ACS) (in particular, low- to intermediate-risk patients), in addition to troponin measured with the latest third-generation troponin assay. BACKGROUND: We have previously shown that H-FABP is a useful prognostic marker in patients with proven ACS. METHODS: Patients (n = 1,080) consecutively admitted to the hospital with suspected ACS were recruited over 46 weeks. Siemens Advia Ultra-TnI (Siemens Healthcare Diagnostics, Newbury, United Kingdom) and Randox Evidence H-FABP (Randox Laboratories, Ltd., Co., Antrim, United Kingdom) were analyzed on samples collected 12 to 24 h from symptom onset. After exclusion of patients with ST-segment elevation and new left bundle branch block, 955 patients were included in the analysis. RESULTS: The primary outcome measure of death or readmission with myocardial infarction after a minimum follow-up period of 12 months (median 18 months) occurred in 96 of 955 patients (10.1%). The H-FABP concentration was an independent predictor of death or myocardial infarction, after multivariate adjustment. Patients with H-FABP concentrations >6.48 microg/l had significantly increased risk of adverse events (adjusted hazard ratio: 2.62, 95% confidence interval: 1.30 to 5.28, p = 0.007). Among troponin-negative patients (which constituted 79.2% of the cohort), the aforementioned cutoff of 6.48 microg/l identified patients at very high risk for adverse outcomes independent of patient age and serum creatinine. CONCLUSIONS: We have demonstrated that the prognostic value of elevated H-FABP is additive to troponin in low- and intermediate-risk patients with suspected ACS. Other studies suggest that our observations reflect the value of H-FABP as a marker of myocardial ischemia, even in the absence of frank necrosis.

Diabetes mellitus and mortality after acute coronary syndrome as a first or recurrent cardiovascular event.

BACKGROUND: Diabetes Mellitus (DM) is associated with adverse cardiovascular prognosis. However, the risk associated with DM may vary between individuals according to their overall cardiovascular risk burden. Therefore, we aimed to determine whether DM is associated with poor outcome in patients presenting with Acute Coronary Syndrome (ACS) according to the index episode being a first or recurrent cardiovascular event. METHODS AND FINDINGS: We conducted a retrospective analysis of a prospective cohort study involving 2499 consecutively admitted patients with confirmed ACS in 11 UK hospitals during 2003. Usual care was provided for all participants. Demographic factors, co-morbidity and treatment (during admission and at discharge) factors were recorded. The primary outcome was all cause mortality (median 2 year follow up), compared for cohorts with and without DM according to their prior cardiovascular disease (CVD) disease status. Adjusted analyses were performed with Cox proportional hazards regression analysis. Within the entire cohort, DM was associated with an unadjusted 45% increase in mortality. However, in patients free of a history of CVD, mortality of those with and without DM was similar (18.8% and 19.7% respectively; p = 0.74). In the group with CVD, mortality of patients with DM was significantly higher than those without DM (46.7% and 33.2% respectively; p<0.001). The age and sex adjusted interaction between DM and CVD in predicting mortality was highly significant (p = 0.002) and persisted after accounting for comorbidities and treatment factors (p = 0.006). Of patients free of CVD, DM was associated with smaller elevation of Troponin I (p<0.001). However in patients with pre-existing CVD Troponin I was similar (p = 0.992). CONCLUSIONS: DM is only associated with worse outcome after ACS in patients with a pre-existing history of CVD. Differences in the severity of myocyte necrosis may account for this. Further investigation is required, though our findings suggest that aggressive primary prevention of CVD in patients with DM may have beneficially modified their first presentation with (and mortality after) ACS.

Heart-type fatty acid-binding protein predicts long-term mortality after acute coronary syndrome and identifies high-risk patients across the range of troponin values.

OBJECTIVES: Our aim was to determine if a high-performance assay for heart-type fatty acid-binding protein (H-FABP) has a role in predicting all-cause mortality after acute coronary syndrome (ACS). BACKGROUND: Heart-type fatty acid-binding protein is released into the circulation following myocardial ischemia and necrosis and therefore may be of value to physicians when caring for patients admitted to hospital with a clinical diagnosis of ACS. METHODS: This was a prospective observational study with a follow-up of 12 months. The H-FABP was measured 12 to 24 h after onset of symptoms in 1,448 patients admitted to hospital with ACS. The main outcome measure was all-cause mortality 1 year after index hospital admission. Multivariable analyses were conducted using the well validated GRACE (Global Registry of Acute Coronary Events) variables together with troponin I and highly sensitive C-reactive protein (hs-CRP). RESULTS: After 12 months of follow-up, 296 patients had died. Multivariable analysis demonstrated that H-FABP quartiles were strongly predictive of outcome: Q1 hazard ratio (HR) 1.0; Q2 HR 2.32 (95% confidence interval [CI] 1.25 to 4.30; p = 0.007); Q3 HR 3.17 (95% CI 1.73 to 5.82; p < 0.001); Q4 HR 4.88 (95% CI 2.67 to 8.93; p < 0.001). The crude all-cause 1-year mortality for unstable angina patients with H-FABP <5.8 microg/l was 2.1% compared with 22.9% for patients above this cutoff. The adjusted all-cause mortality HR in this group was 11.35 (95% CI 2.00 to 64.34; p = 0.006). CONCLUSIONS: Heart-type fatty acid-binding protein predicts long-term mortality after ACS and identifies high-risk patients in a manner that is additive to the GRACE clinical risk factors, troponin, and hs-CRP, possibly as a result of identifying the occurrence of myocardial ischemia with or without necrosis.