How Often Does Apical Sparing of Longitudinal Strain Indicate the Presence of Cardiac Amyloidosis?

Echocardiographic diagnosis of cardiac amyloidosis (CA) is frequently suggested by the presence of a left ventricular (LV) apical sparing pattern (ASP) on longitudinal strain (LS) assessment, the so-called "cherry on top" pattern, defined by strain magnitude preserved exclusively at the apex. However, it is unclear how frequently this strain pattern truly represents CA. This study aimed to evaluate the predictive value of ASP in the diagnosis of CA. We retrospectively identified consecutive adult patients who had the following studies performed within an 18-month period: (1) transthoracic echocardiogram and (2) either (a) cardiac magnetic resonance imaging, (b) Technetium-Pyrophosphate (PYP) imaging, or (c) endomyocardial biopsy. LS was retrospectively measured in the apical 4-, 3-, and 2-chamber views in patients who had adequate noncontrast images (n = 466). An apical sparing ratio (ASR) was calculated as (average apical strain)/[(average basal strain) + (average midventricular strain)]. Patients with ASR ≥1 were evaluated for the presence/absence of CA, using established criteria. Basic LV parameters were also measured. A total of 33 patients (7.1%) had ASP. Nine of these patients (27%) had "confirmed" CA, 2 (6.1%) "highly probable" CA, 1 (3.0%) "possible" CA, and 21 (64%) no evidence of CA. When comparing patients with and without confirmed CA, there were no significant differences in ASR, average global LS, ejection fraction, or LV mass. Patients with confirmed CA were older (76 ± 9 vs 59 ± 18 years, p = 0.01) and had thicker posterior wall (15 ± 3 vs 11 ± 3 mm, p = 0.004) with a trend toward thicker septal wall (15 ± 2 vs 12 ± 4 mm, p = 0.05). In conclusion, the presence of ASP on LS represents confirmed or highly probable CA in only 1/3 of patients and is more likely to indicate true CA in older patients with increased LV wall thickness. Although a larger, prospective study is needed to confirm these findings, 1/3 should be considered as a large diagnostic yield that justifies further testing, given the poor outcomes associated with CA diagnosis.

Considerations in the management of giant frontal mucoceles with significant intracranial extension: A systematic review.

BACKGROUND: Giant frontal mucoceles, characterized by significant intracranial and/or intraorbital extension, can present with significant neurologic symptoms. Although typical mucoceles are managed endoscopically, giant mucoceles are often treated with an open or combined approach due to various concerns, including frontal lobe displacement, size, and rapid decompression of the intracranial component. The impact of significant intracranial extension on outcomes is not well described. OBJECTIVE: This review studied key neurologic considerations in the management of giant frontal sinus mucoceles and analyzed outcomes of different management strategies. METHODS: Systematic literature review by following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. RESULTS: Thirty-two articles, which represented 85 cases, met inclusion criteria. Neurologic symptoms on presentation ranged from headache (24.7%) and vision loss (12.9%) to extremity weakness (1.2%), frontal lobe syndrome (2.4%), and seizures (4.7%). Twenty-eight patients were treated endoscopically (34.1%), and 54 (65.9%) were treated with an external approach. Twenty-five of the open procedures included a craniotomy. Indications for the open approach included subdural empyema, enucleation, or large anterior table defects. Perioperative antibiotics were not consistently used. No perioperative seizures were reported with any approach. There were six cerebrospinal fluid leaks, all in the patients who underwent open procedures. The overall recurrence rate was 3.5%, with no recurrences reported in patients treated with an open approach. Follow-up ranged from 1 week to 8 years. CONCLUSIONS: Giant frontal mucoceles often present with various neurologic symptoms. Both open and endoscopic techniques offer excellent results. Endoscopic management is effective and preferred, except in special circumstances. An interdisciplinary team approach should be used to optimize surgical planning.

Teaching professionalism in graduate medical education: What is the role of simulation?

BACKGROUND: We systematically reviewed the literature concerning simulation-based teaching and assessment of the Accreditation Council for Graduate Medical Education professionalism competencies to elucidate best practices and facilitate further research. METHODS: A systematic review of English literature for "professionalism" and "simulation(s)" yielded 697 abstracts. Two independent raters chose abstracts that (1) focused on graduate medical education, (2) described the simulation method, and (3) used simulation to train or assess professionalism. Fifty abstracts met the criteria, and seven were excluded for lack of relevant information. The raters, 6 professionals with medical education, simulation, and clinical experience, discussed 5 of these articles as a group; they calibrated coding and applied further refinements, resulting in a final, iteratively developed evaluation form. The raters then divided into 2 teams to read and assess the remaining articles. Overall, 15 articles were eliminated, and 28 articles underwent final analysis. RESULTS: Papers addressed a heterogeneous range of professionalism content via multiple methods. Common specialties represented were surgery (46.4%), pediatrics (17.9%), and emergency medicine (14.3%). Sixteen articles (57%) referenced a professionalism framework; 14 (50%) incorporated an assessment tool; and 17 (60.7%) reported debriefing participants, though in limited detail. Twenty-three (82.1%) articles evaluated programs, mostly using subjective trainee reports. CONCLUSION: Despite early innovation, reporting of simulation-based professionalism training and assessment is nonstandardized in methods and terminology and lacks the details required for replication. We offer minimum standards for reporting of future professionalism-focused simulation training and assessment as well as a basic framework for better mapping proper simulation methods to the targeted domain of professionalism.