Background-Diastolic dysfunction is an independent predictor of mortality in patients with normal left ventricular ejection fraction. There are limited data, however, on whether worsening of diastolic function is associated with worse prognosis.

OBJECTIVES This study tested the diagnostic and prognostic utility of a rapid, visual T1 assessment method for identification of cardiac amyloidosis (CA) in a “real-life” referral population undergoing cardiac magnetic resonance for suspected CA. BACKGROUND In patients with confirmed CA, delayed-enhancement cardiac magnetic resonance (DE-CMR) frequently shows a diffuse, global hyperenhancement (HE) pattern. However, imaging is often technically challenging, and the prognostic significance of diffuse HE is unclear. METHODS Ninety consecutive patients referred for suspected CA and 64 hypertensive patients with left ventricular hypertrophy (LVH) were prospectively enrolled and underwent a modified DE-CMR protocol. After gadolinium administration a method for rapid, visual T1 assessment was used to identify the presence of diffuse HE during the scan, allowing immediate optimization of settings for the conventional DE-CMR that followed. The primary endpoint was all-cause mortality. RESULTS Among patients with suspected CA, 66% (59 of 90) demonstrated HE, with 81% (48 of 59) of these meeting pre-specified visual T1 assessment criteria for diffuse HE. Among hypertensive LVH patients, 6% (4 of 64) had HE, with none having diffuse HE. During 29 months of follow-up (interquartile range: 12 to 44 months), there were 50 (56%) deaths in patients with suspected CA and 4 (6%) in patients with hypertensive LVH. Multivariable analysis demonstrated that the presence of diffuse HE was the most important predictor of death in the group with suspected CA (hazard ratio: 5.5, 95% confidence interval: 2.7 to 11.0; p < 0.0001) and in the population as a whole (hazard ratio: 6.0, 95% confidence interval 3.0 to 12.1; p < 0.0001). Among 25 patients with myocardial histology obtained during follow-up, the sensitivity, specificity, and accuracy of diffuse HE in the diagnosis of CA were 93%, 70%, and 84%, respectively. CONCLUSIONS Among patients suspected of CA, the presence of diffuse HE by visual T1 assessment accurately identifies patients with histologically-proven CA and is a strong predictor of mortality.

The presence of even a large reversible perfusion defect does not alter the indices of mechanical dyssynchrony by phase analysis. Further, comparable information is obtained whether using a low dose or a high dose of the radiotracer.

Amyloidosis is an infiltrative disease characterized by deposition of amyloid fibrils within the extracellular tissue of one or multiple organs. Involvement of the heart, cardiac amyloidosis, is recognized as a common cause of restrictive cardiomyopathy and heart failure. The two major types of cardiac amyloidosis are cardiac amyloid light-chain (AL) and transthyretin-related cardiac amyloidosis (ATTR, mutant and wild types) (Nat Rev Cardiol 2010;7:398-408). While early recognition of cardiac amyloidosis is of major clinical importance, so is the ability to differentiate between subtypes. Indeed, both prognosis and therapeutic options vary drastically depending on the subtype. While endomyocardial biopsy with immunostaining is considered the gold standard, advances in imaging provide an attractive non-invasive alternative. Currently, electrocardiography, echocardiography, and cardiac magnetic resonance imaging are all used in the evaluation of cardiac amyloidosis with varying diagnostic and prognostic accuracy. Yet, none of these modalities can effectively differentiate the cardiac amyloid subtypes. Recent data with 99m Tc-phosphate derivatives, previously used as bone seeking radioactive tracers, have shown promising results; these radiotracers selectively bind ATTR, but not AL subtype, and can differentiate subtypes with high diagnostic accuracy. This review will initially present the non-radionuclide imaging techniques and then focus on the radionuclide imaging techniques, particularly 99m Tc-DPD and 99m Tc-PYP, mechanism of action, performance and interpretation of the study, diagnostic accuracy, prognostic value, future clinical perspective, and outlook.

Left ventricular ejection fraction as determined by echocardiography has a limited sensitivity in predicting risk for sudden cardiac death (SCD). Subsequent efforts to improve cost-effectiveness of device implantation and identify a better risk-stratifying tool have been quite desirable. The presence of scar and myocardial tissue heterogeneity has been linked to ventricular arrhythmia, which is believed to be the major cause of SCD. Cardiac magnetic resonance is a noninvasive imaging modality that visualizes and quantifies scar, with growing evidence delineating its additive value in identifying patients at higher risk for SCD.

Background:Obesity is a major public health epidemic and is associated with increased risk of heart failure and mortality. We evaluated the impact of body mass index (BMI) on the prevalence of diastolic dysfunction (DD).Methods:We reviewed clinical records and echocardiogram of patients with baseline echocardiogram between 1996 and 2005 that showed normal left ventricular ejection fraction (LVEF). Diastolic function was labeled as normal, stage 1, stage 2 or stage 3/4 dysfunction. Patients were categorized as normal weight (BMI <25 kg m−2), overweight (25–29.9 kg m−2), obese (30–39.9 kg m−2) and morbidly obese (⩾40 kg m−2). Multivariable ordinal and ordinary logistic regression were performed to identify factors associated with DD, and evaluate the independent relationship of BMI with DD.Results:The cohort included 21 666 patients (mean (s.d.) age, 57.1 (15.1); 55.5% female). There were 7352 (33.9%) overweight, 5995 (27.6%) obese and 1616 (7.4%) morbidly obese patients. Abnormal diastolic function was present in 13 414 (61.9%) patients, with stage 1 being the most common. As BMI increased, the prevalence of normal diastolic function decreased (P<0.0001). Furthermore, there were 1733 patients with age <35 years; 460 (26.5%) and 407 (23.5%) were overweight and obese, respectively, and had higher prevalence of DD (P<0.001). Using multivariable logistic regression, BMI remained significant in both ordinal (all stages of diastolic function) and binary (normal versus abnormal). Also, obesity was associated with increased odds of DD in all patients and those aged <35 years.Conclusions:In patients with normal LVEF, higher BMI was independently associated with worsening DD.

LV mechanical dyssynchrony by phase analysis is a predictor of mortality in patients with ESRD. It may have a role in risk-stratifying patients and should be incorporated in future studies using gated MPI.

The severity of LV dyssynchrony by phase analysis in patients with LV dysfunction, and ICD is associated with increased risk of death and appropriate ICD shock; a phase SD < 50 degrees was associated with no events at 1 year.