BACKGROUNDLeft ventricular outflow tract (LVOT) obstruction is an important determinant of the management of hypertrophic cardiomyopathy (HCM). With a nationwide shortage of amyl nitrite in 2019, we ...implemented a 'repetitive squat-to-stand' manoeuvre to provoke LVOT obstruction during echocardiography. CASE SUMMARYA 64-year-old female was referred with symptomatic HCM refractory to pharmacologic therapy. Transthoracic echocardiography showed minor LVOT obstruction with conventional imaging at rest and during Valsalva manoeuvre, but severe obstruction was confirmed with the repetitive squat-to-stand manoeuvre. Alcohol septal ablation via the first septal perforator was performed with subsequent resolution of symptoms. DISCUSSIONDue to the dynamic nature of LVOT obstruction, a series of provocative manoeuvres including Valsalva manoeuvre, inhalation of amyl nitrite, and exercise are often necessary to maximally augment ventricular obstruction. The recent unavailability of amyl nitrite during a nationwide shortage prompted the implementation of a protocol of repetitive squat-to-stand manoeuvre in our echocardiography laboratory. Rising from the squatting position decreases preload and afterload, both of which augment dynamic LVOT obstruction. Repetition of squatting and standing appears to enhance the sensitivity of the manoeuvre, particularly when exertional symptoms are reproduced. In this case, repetitive squat-to-stand manoeuvre led to the identification of severe LVOT obstruction which may not have been diagnosed otherwise, alteration of treatment to septal reduction therapy, and subsequent resolution of symptoms.
Background: One of the common causes of sudden cardiac death (SCD) in young people is hypertrophic cardiomyopathy (HCM) and the primary prevention of SCD is with an implantable cardioverter ...defibrillators (ICD). Concerning the incidence of appropriate ICD therapy and the complications associated with ICD implantation and discharge, patients with implanted ICDs are closely monitored and interrogation reports are generated from clinical consultations. Methods: In this study, we compared the performance of structured device data and unstructured interrogation reports for extracting information of ICD therapy and heart rhythm. We sampled 687 reports with a gold standard generated through manual chart review. A rule-based natural language processing (NLP) system was developed using 480 reports and the information in the corresponding device data was aggregated for the task. We compared the performance of the NLP system with information aggregated from structured device data using the remaining 207 reports. Results: The rule-based NLP system achieved F-measure of 0.92 and 0.98 for ICD therapy and heart rhythm while the performance of aggregating device data was significantly lower with F-measure of 0.78 and 0.45 respectively. Limitations of using only structured device data include no differentiation of real events from management events, data availability, and disparate perspectives of vendor and data granularity while using interrogation reports needs to overcome non-representative keyword/pattern and contextual errors. Conclusions: Extracting phenotyping information from data generated in real-world requires the incorporation of medical knowledge. It is essential to analyze, compare, and harmonize multiple data sources for real-world evidence generation.
BACKGROUNDHistoplasma capsulatum is an extremely rare cause of prosthetic valve endocarditis (PVE) and can present with non-specific symptoms leading to a delay in diagnosis with unfavourable ...outcomes. CASE SUMMARYA 65-year-old male patient with a history of a bioprosthetic aortic valve replacement and non-obstructive coronary artery disease was admitted for altered mentation, failure to thrive, and a 20-pound unintentional weight loss over the past 4 months. Upon examination, he was lethargic but afebrile and haemodynamically stable. A late peaking ejection murmur was heard on exam. Skin exam was significant for embolic phenomenon involving the extremities. Inflammatory markers and serum calcium were elevated. A bedside echocardiogram showed severe obstruction across the aortic valve prosthesis. Two years prior, he had an echocardiogram with a normal functioning prosthesis. Routine blood cultures were negative and serologic screening was unrevealing. Urine Histoplasma antigen screen was positive on hospital day 3 and on hospital day 10, fungal blood cultures were positive for H. capsulatum. Unfortunately, the patient died shortly afterwards as a result of multiorgan failure from embolic manifestations of the infection. DISCUSSIONBased on our patient's findings and those of previously reported cases in the literature, H. capsulatum PVE should be strongly considered in patients from endemic areas with non-specific symptoms and negative routine blood cultures.
Background Aficamten, a novel cardiac myosin inhibitor, reversibly reduces cardiac hypercontractility in obstructive hypertrophic cardiomyopathy. We present a prespecified analysis of the ...pharmacokinetics, pharmacodynamics, and safety of aficamten in SEQUOIA‐HCM (Safety, Efficacy, and Quantitative Understanding of Obstruction Impact of Aficamten in HCM). Methods and Results A total of 282 patients with obstructive hypertrophic cardiomyopathy were randomized 1:1 to daily aficamten (5–20 mg) or placebo between February 1, 2022, and May 15, 2023. Aficamten dosing targeted the lowest effective dose for achieving site‐interpreted Valsalva left ventricular outflow tract gradient <30 mm Hg with left ventricular ejection fraction (LVEF) ≥50%. End points were evaluated during titration (day 1 to week 8), maintenance (weeks 8–24), and washout (weeks 24–28), and included major adverse cardiac events, new‐onset atrial fibrillation, implantable cardioverter‐defibrillator discharges, LVEF <50%, and treatment‐emergent adverse events. At week 8, 3.6%, 12.9%, 35%, and 48.6% of patients achieved 5‐, 10‐, 15‐, and 20‐mg doses, respectively. Baseline characteristics were similar across groups. Aficamten concentration increased by dose and remained stable during maintenance. During the treatment period, LVEF decreased by −0.9% (95% CI, −1.3 to −0.6) per 100 ng/mL aficamten exposure. Seven (4.9%) patients taking aficamten underwent per‐protocol dose reduction for site‐interpreted LVEF <50%. There were no treatment interruptions or heart failure worsening for LVEF <50%. No major adverse cardiovascular events were associated with aficamten, and treatment‐emergent adverse events were similar between treatment groups, including atrial fibrillation. Conclusions A site‐based dosing algorithm targeting the lowest effective aficamten dose reduced left ventricular outflow tract gradient with a favorable safety profile throughout SEQUOIA‐HCM. Registration URL: https://www.clinicaltrials.gov ; Unique Identifier: NCT05186818.
Lassa Fever in Post-Conflict Sierra Leone Shaffer, Jeffrey G; Grant, Donald S; Schieffelin, John S ...
PLoS neglected tropical diseases,
03/2014, Letnik:
8, Številka:
3
Journal Article
Recenzirano
Odprti dostop
Background Lassa fever (LF), an often-fatal hemorrhagic disease caused by Lassa virus (LASV), is a major public health threat in West Africa. When the violent civil conflict in Sierra Leone (1991 to ...2002) ended, an international consortium assisted in restoration of the LF program at Kenema Government Hospital (KGH) in an area with the world's highest incidence of the disease. Methodology/Principal Findings Clinical and laboratory records of patients presenting to the KGH Lassa Ward in the post-conflict period were organized electronically. Recombinant antigen-based LF immunoassays were used to assess LASV antigenemia and LASV-specific antibodies in patients who met criteria for suspected LF. KGH has been reestablished as a center for LF treatment and research, with over 500 suspected cases now presenting yearly. Higher case fatality rates (CFRs) in LF patients were observed compared to studies conducted prior to the civil conflict. Different criteria for defining LF stages and differences in sensitivity of assays likely account for these differences. The highest incidence of LF in Sierra Leone was observed during the dry season. LF cases were observed in ten of Sierra Leone's thirteen districts, with numerous cases from outside the traditional endemic zone. Deaths in patients presenting with LASV antigenemia were skewed towards individuals less than 29 years of age. Women self-reporting as pregnant were significantly overrepresented among LASV antigenemic patients. The CFR of ribavirin-treated patients presenting early in acute infection was lower than in untreated subjects. Conclusions/Significance Lassa fever remains a major public health threat in Sierra Leone. Outreach activities should expand because LF may be more widespread in Sierra Leone than previously recognized. Enhanced case finding to ensure rapid diagnosis and treatment is imperative to reduce mortality. Even with ribavirin treatment, there was a high rate of fatalities underscoring the need to develop more effective and/or supplemental treatments for LF.
PDF
