RESUMO
Hypertrophic cardiomyopathy (HCM) due to thick filament variants is more common; however, HCM due to thin filament variants (HCM-Thin) may be associated with a more malignant phenotype with an increased risk of sudden cardiac death. The aim of this study was to review all the published cases of HCM-Thin to better understand the natural history and clinical outcomes of this disease. A literature review of HCM-Thin identified 21 studies with a total of 177 patients that were suitable for analysis. There were three outcomes of interest, which included a heart failure composite, a ventricular arrhythmia composite and a heart failure and arrhythmia composite outcome. Kaplan-Meier (KM) survival analyses for freedom from each of the abovementioned composite outcomes were completed for the entire cohort and stratified by age of onset and sarcomeric variant. The heart failure composite occurred in 24 (13.6%) patients, the ventricular arrhythmia composite occurred in 30 patients (16.9%) and the combined heart failure and arrhythmia composite occurred in 50 patients (28.2%). In regard to left ventricular ejection fraction (LVEF), the majority of patients were preserved (LVEF > 50%) compared with mildly reduced (LVEF 41%-50%) and reduced (LVEF ≤ 40%) (respectively 26.6% vs. 0.6% vs. 3.4%). The median maximal left ventricular wall thickness (LVWT) was 19.0 mm [interquartile range (IQR) 5.3]. Only 10.7% of the cohort had evidence of left ventricular outflow tract (LVOT) obstruction. Those with paediatric-onset HCM had earlier onset and were at higher risk for each endpoint than their adult counterparts. When stratified by genetic variant, patients with TNNI3 and TPM1 were at a higher risk of the heart failure composite endpoint and the combined heart failure and arrhythmia composite endpoint in comparison with those with the other genetic variants. HCM-Thin is associated with significant morbidity and mortality, with a high arrhythmia burden despite low rates of cardiac obstruction and mild hypertrophy. The paediatric onset of disease and certain sarcomeric variants appear to be associated with a worse prognosis than their adult-onset and other sarcomeric variant counterparts. HCM-Thin seems to have a distinct phenotype, which may require a different management approach.
RESUMO
OBJECTIVES: This study sought to assess the impact of early versus delayed lead extraction in patients with an infected cardiovascular implantable electronic device (CIED). BACKGROUND: CIED infections are associated with poor outcomes. Prior studies have demonstrated improved survival with CIED extraction compared with antibiotic therapy alone. The impact of timing of CIED extraction has not been well characterized. METHODS: All infected CIED extraction cases at our medical center from 2006 to 2019 were reviewed. Patients were divided into 2 groups based on the presence of bacteremia or isolated pocket infection. We assessed the in-hospital morbidity and 1-year mortality for early versus delayed lead extraction, using hospitalization day 7 as cutoff. RESULTS: Of 233 patients who underwent CIED extraction, 127 patients had bacteremia and 106 patients had pocket infection. Delayed extraction (15.2 days) in bacteremic patients was associated with septic shock (odds ratio [OR]: 5.39; 95% confidence interval [CI]: 1.23 to 23.67; p = 0.026), acute kidney injury (OR: 5.61; 95% CI: 2.15 to 14.63; p < 0.001), respiratory failure (OR: 5.52; 95% CI: 1.25 to 24.41; p = 0.024), and decompensated heart failure (OR: 3.32; 95% CI: 1.10 to 10.05; p = 0.033). Locally infected patients with delayed extraction (10.7 days) were associated with acute kidney injury (OR: 3.45; 95% CI: 1.11 to 10.77; p = 0.033) and respiratory failure (OR: 10.29; 95% CI: 1.26 to 83.93; p = 0.030). Delayed CIED extraction in both groups was associated with increased 1-year mortality. CONCLUSIONS: Delayed infected CIED extraction is associated with worse outcomes. This underscores the importance of early detection and a strategy for prompt management including lead extraction.