Long term evolution of MRI characteristics in a case of atypical left lateral wall hypertrophic cardiomyopathy

We are reporting a long-time magnetic resonance imaging (MRI) follow-up in a rare case of cardiac left lateral wall hypertrophy. Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disorder and a significant cause of sudden cardiac death. Cardiac magnetic resonance (CMR) imaging can be a valuable tool for assessment of detailed information on size, localization, and tissue characteristics of hypertrophied myocardium. However, there is still little knowledge of long-term evolution of HCM as visualized by magnetic resonance imaging. Recently, our group reported a case of left lateral wall HCM as a rare variant of the more common forms, such as septal HCM, or apical HCM. As we now retrieved an old cardiac MRI acquired in this patient more than 20 years ago, we are able to provide the thrilling experience of an ultra-long MRI follow-up presentation in this rare case of left lateral wall hypertrophy. Furthermore, this case outlines the tremendous improvements in imaging quality within the last two decades of CMR imaging

Predictive value of assessing diastolic strain rate on survival in cardiac amyloidosis patients with preserved ejection fraction

Objectives: Since diastolic abnormalities are typical findings of cardiac amyloidosis (CA), we hypothesized that speckle-tracking-imaging (STI) derived longitudinal early diastolic strain rate (LSRdias) could predict outcome in CA patients with preserved left ventricular ejection fraction (LVEF >50%)./nBackground: Diastolic abnormalities including altered early filling are typical findings and are related to outcome in CA patients. Reduced longitudinal systolic strain (LSsys) assessed by STI predicts increased mortality in CA patients. It remains unknown if LSRdias also related to outcome in these patients./nMethods: Conventional echocardiography and STI were performed in 41 CA patients with preserved LVEF (25 male; mean age 65±9 years). Global and segmental LSsys and LSRdias were obtained in six LV segments from apical 4-chamber views./nResults: Nineteen (46%) out of 41 CA patients died during a median of 16 months (quartiles 5–35 months) follow-up. Baseline mitral annular plane systolic excursion (MAPSE, 6±2 vs. 8±3 mm), global LSRdias and basal-septal LSRdias were significantly lower in non-survivors than in survivors (all p<0.05). NYHA class, number of non-cardiac organs involved, MAPSE, mid-septal LSsys, global LSRdias, basal-septal LSRdias and E/LSRdias were the univariable predictors of all-cause death. Multivariable analysis showed that number of non-cardiac organs involved (hazard ratio [HR]  = 1.96, 95% confidence interval [CI] 1.17–3.26, P = 0.010), global LSRdias (HR = 7.30, 95% CI 2.08–25.65, P = 0.002), and E/LSRdias (HR = 2.98, 95% CI 1.54–5.79, P = 0.001) remained independently predictive of increased mortality risk. The prognostic performance of global LSRdias was optimal at a cutoff value of 0.85 S−1 (sensitivity 68%, specificity 67%). Global LSRdias <0.85 S−1 predicted a 4-fold increased mortality in CA patients with preserved LVEF./nConclusions: STI-derived early diastolic strain rate is a powerful independent predictor of survival in CA patients with preserved LVEF

Impact of regional left ventricular function on outcome for patients with AL amyloidosis

Objectives: The aim of this study was to explore the left ventricular (LV) deformation changes and the potential impact of/ndeformation on outcome in patients with proven light-chain (AL) amyloidosis and LV hypertrophy./nBackground: Cardiac involvement in AL amyloidosis patients is associated with poor outcome. Detecting regional cardiac/nfunction by advanced non-invasive techniques might be favorable for predicting outcome./nMethods: LV longitudinal, circumferential and radial peak systolic strains (Ssys) were assessed by speckle tracking imaging/n(STI) in 44 biopsy-proven systemic AL amyloidosis patients with LV hypertrophy (CA) and in 30 normal controls. Patients/nwere divided into compensated (n = 18) and decompensated (n = 26) group based on clinical assessment and followed-up/nfor a median period of 345 days./nResults: Ejection fraction (EF) was preserved while longitudinal Ssys (LSsys) was significantly reduced in both compensated/nand decompensated groups. Survival was significantly reduced in decompensated group (35% vs. compensated 78%,/nP = 0.001). LSsys were similar in apical segments and significantly reduced in basal segments between two patient groups./nLSsys at mid-segments were significantly reduced in all LV walls of decompensated group. Patients were further divided into/n4 subgroups according to the presence or absence of reduced LSsys in no (normal), only basal (mild), basal and mid/n(intermediate) and all segments of the septum (severe). This staging revealed continuously worse prognosis in proportion to/nincreasing number of segments with reduced LSsys (mortality: normal 14%, mild 27%, intermediate 67%, and severe 64%)./nMid-septum LSsys,11% suggested a 4.8-fold mortality risk than mid-septum LSsys$11%. Multivariate regression analysis/nshowed NYHA class and mid-septum LSsys were independent predictors for survival./nConclusions: Reduced deformation at mid-septum is associated with worse prognosis in systemic amyloidosis patients with/nLV hypertrophy