Predictive value of a comprehensive atrial assessment with cardiac magnetic resonance in non-ischemic cardiomyopathy: keep it simple.

Cardiac magnetic resonance (CMR) can provide a multi-parametric evaluation of left atrial (LA) size and function. A complete CMR-based LA assessment might improve the risk stratification of patients with non-ischemic dilated cardiomyopathy (DCM). We performed a comprehensive CMR-based evaluation of LA size and function, in order to assess the prognostic impact of specific LA parameters in DCM. Secondary analysis of a prospective registry (UHSM-CMR study, NCT02326324) including 648 consecutive patients with DCM and CMR evaluation of LA area and LA length. Of these, 456 had complete LA assessment covering reservoir, conduit and booster pump function and including LA reservoir strain evaluated with feature tracking. The heart failure (HF) endpoint included HF hospitalizations, HF death and heart transplant. The arrhythmic endpoint included ventricular arrhythmias (VA) (sustained or treated by implantable defibrillator) and sudden death (SD). At median follow-up of 23 months, 34 patients reached the HF endpoint; in a multivariable model including NYHA class and LVEF, LA length had incremental predictive value. LA length ≥ 69 mm was the best cut-off to predict HF events (adjusted HR 2.3, p = 0.03). Among the 456 patients with comprehensive LA assessment, only LA length was independently associated with the HF endpoint after adjusting for LVEF and NYHA class. By contrast, no LA parameter independently predicted the arrhythmic risk. In DCM patients, LA length is an independent predictor of HF events, showing stronger association than other more complex parameters of LA function. No atrial parameter predicts the risk of VA and SD.

Magnetic Resonance Imaging of Intramyocardial Fat Deposition in Tuberous Sclerosis.

Tuberous sclerosis complex (TSC) is a rare autosomal dominant neurocutaneous syndrome. The phenotype is highly variable and may affect several organ systems, the hallmark of the disease being widespread hamartomas or abnormal growth of normal tissues. Although cardiac rhabdomyomas are the most common cardiac manifestation of TSC, being developed quite early, even during the second semester of the gestation, they tend to regress spontaneously over a period of months or years. On the other hand, the presence of intramyocardial fat deposition has been significantly associated with brain involvement and other extracardiac manifestations. We report the case of a 37-year-old man with TSC who presented to hospital with loss of consciousness, head injury and amnesia and in whom cardiac magnetic resonance imaging revealed the presence of multiple areas intramyocardial fat deposition.

Late-Onset Fabry Disease Presenting With Ventricular Tachycardia Originating From Typical Inferolateral Scar.

Late-onset cardiac Fabry disease is not uncommon among patients with unexplained left ventricular hypertrophy. Despite a less severe phenotype, life-threatening complications are possible in late-onset cardiac Fabry and may be the first presentation of the disease. Classical imaging features support the diagnosis; however, the presence of less common findings, such as ischemic features, should not lead to overlooking the diagnosis. Indeed, the coexistence of Fabry and ischemic heart disease is possible, even in the absence of obstructive coronary artery disease. Therefore, a high level of suspicion should be maintained, even in the presence of atypical presentations.