Research Appraisals
Evidence-based critical appraisals of the latest medical research, systematically evaluated using Oxford CEBM methodology.
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Relative apical sparing of left ventricular longitudinal strain for the diagnosis of cardiac amyloidosis: a systematic review and meta-analysis
BACKGROUND: Cardiac amyloidosis (CA) is underdiagnosed due to non-specific clinical and echocardiographic features. This systematic review and meta-analysis evaluated the diagnostic accuracy of the left ventricular relative apical sparing (RELAPS) pattern on speckle-tracking echocardiography. METHODS: A literature search of PubMed, Scopus and Cochrane was conducted through August 2025. RELAPS was defined as the average apical longitudinal strain divided by the sum of average basal and average middle left ventricular longitudinal strains. Diagnostic accuracy was assessed using a bivariate random-effects model. Subgroup analyses by CA subtype and echocardiogram software, and meta-regression for clinical variables, were performed. Optimal cut-offs were determined by maximising the Youden index. Analyses were conducted in R V.4.4.1. RESULTS: Forty-one studies (3473 CA; 4525 comparators) were included. Pooled analysis yielded an area under the receiver operating characteristic curve (AUC-ROC) of 0.818, with sensitivity 65.9% (95% CIs 59.2% to 72.0%) and specificity 83.1% (CI 78.5% to 86.8%). The diagnostic oods ratio (DOR) was 9.54 (CI 7.41 to 12.10). In subtype analyses, immunoglobulin light chains (AL-CA) showed AUC-ROC 0.876, sensitivity 59.8% (CI 41.1% to 76.0%) and specificity 92.7% (CI 83.6% to 96.9%); transthyretin (ATTR-CA) showed AUC-ROC 0.822, sensitivity 63.8% (CI 51.2% to 74.8%) and specificity 84.4% (CI 76.7% to 89.9%), with no significant differences between subtypes (sensitivity p=0.760; specificity p=0.172). Three echocardiography software systems were evaluated. GE EchoPAC (26 studies) achieved an AUC-ROC of 0.822 (sensitivity 70.4% (CI 64.1% to 76.0%), specificity 81.2% (CI 75.7% to 85.7%)). Philips QLAB (four studies) performed comparably (AUC-ROC 0.904; sensitivity 67.6% (CI 31.2% to 90.6%), specificity 92.7% (CI 73.6% to 98.3%)). In contrast, TomTec (four studies) had an AUC-ROC of 0.806, but its sensitivity (31.1% (CI 9.4% to 66.3%)) was significantly lower than GE EchoPAC's (p<0.001), despite a similar specificity (93.6% (CI 78.8% to 98.3%)). CONCLUSION: RELAPS provides moderate diagnostic accuracy for identifying CA, with good specificity and modest sensitivity. Performance varies by analysis software and threshold, underscoring the need for standardised measurement and prespecified cut-offs.
27 Apr 2026
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Assessing the real-world safety of vutrisiran for transthyretin-mediated amyloidosis with polyneuropathy: Based on WHO-VigiAccess and FAERS databases
BACKGROUND: Vutrisiran is a double-stranded siRNA targeting transthyretin mRNA, primarily for the treatment of transthyretin-mediated amyloidosis (ATTR) with polyneuropathy. Understanding its safety in real-world settings is crucial for achieving optimal treatment outcomes for patients. METHODS: This study assessed the safety of vutrisiran for clinical use by analyzing all adverse drug reaction (ADR) reports in WHO-VigiAccess and U.S. Food and Drug Administration's Adverse Event Reporting System (FAERS) databases with vutrisiran as the primary suspected drug and by using four disproportionality analysis and Bayesian information component. Weibull distribution was also utilized to predict the time of occurrence of ADR. RESULTS: During the research process, WHO-VigiAccess reported 721 adverse reaction reports related to vutrisiran, while FAERS documented 732 ADR reports. The analysis revealed that: this study not only demonstrated known adverse reactions such as injection site reactions, such as redness, pain, or swelling at the injection site; systemic reactions like fatigue and myalgia; pain in extremity; and potential risks associated with reduced vitamin A levels, but also detected potential safety signals related to drug such as night blindness and cardiac pacemaker insertion. Furthermore, it was observed that in both the WHO-VigiAccess and FAERS databases, the highest reporting rates of ADR were found in the category of general disorders and administration site conditions. It also confirms the importance of monitoring the occurrence of adverse reactions within one year after drug treatment. CONCLUSION: The study identified several known ADRs and detected potential positive signals that may be associated with the drug. These research results provide clinicians with more information to consider and monitor adverse reactions in patients with ATTR-related polyneuropathy after treatment with vutrisiran, offering more evidence for its safety in real-world settings.
16 Apr 2026
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