Hagan, Grace

A 63-year-old female with mixed connective tissue disease on long-term immunosuppression (prednisone, azathioprine, hydroxychloroquine (HCQ)) was referred to a tertiary care center for evaluation of progressive dyspnea and exertional chest discomfort. Cardiac history included transient complete heart block and ventricular tachycardia with dual-chamber AICD, and severe aortic stenosis with bicuspid aortic valve. Laboratory evaluation was notable for NT pro-BNP > 5000 pg/mL and elevated but adynamic troponin (300-500 ng/L). Electrocardiogram revealed atrial pacing with first degree atrioventricular block. Echocardiogram confirmed severe aortic stenosis, elevated filling pressures, abnormal left ventricular longitudinal strain and LV hypertrophy. Cardiac MRI, sarcoid PET, and PYP SPECT/CT were without any evidence of edema, scar, inflammation or amyloid deposition. Catheterization revealed severe pulmonary hypertension and elevated filling pressures, without significant coronary disease. Medical treatment included empagliflozin (discontinued due to urosepsis), bumetanide, spironolactone, and metoprolol succinate. Neither TAVR nor transition to biventricular pacing improved LV function. Ultimately, endomyocardial biopsy was performed, revealing histologic features of HCQ-induced cardiotoxicity, including myocyte hypertrophy and interstitial fibrosis. HCQ was discontinued. She was referred to Rheumatology for alternative treatments. HCQ induced cardiomyopathy represents <2% of adverse events of patients treated with HCQ, presenting as hypertrophic or restrictive cardiomyopathy with conduction abnormalities. Mean treatment duration in HCQ cardiomyopathies is approximately 12.7 years. Confirmation of diagnosis requires endomyocardial biopsy, as imaging findings are often non-specific. Management involves discontinuing the offending agent and optimizing heart failure therapy. Around 45% of patients with HCQ cardiomyopathy exhibit resolution of cardiac function following medication discontinuation. This case emphasizes the importance of a systematic approach to unexplained heart failure, particularly in patients with long-term HCQ use. Infiltrative or toxic cardiomyopathies should be considered when imaging suggests myocardial dysfunction. Multimodal imaging and biopsy are crucial for diagnosis. Early recognition is essential to prevent irreversible myocardial damage and improve long-term outcomes.

Abstract Background The incidence, epidemiology, and clinical characteristics of enterococcal bloodstream infection (BSI) have not previously been studied on a population-based level in the United States. Methods We utilized the Expanded Rochester Epidemiology Project medical records linkage system to conduct a contemporary 5-year, retrospective, population-based study of monomicrobial enterococcal (ME) BSI among adult residents of 8 counties in southeast Minnesota from January 1, 2018 to December 31, 2022. Results A total of 109 cases of ME-BSI were identified. The overall age- and sex-adjusted incidence rate was 6.7 per 100 000 person-years (95% confidence interval, 5.4–7.9). Multivariable analysis revealed that male sex (P &lt; .001) and older age (P &lt; .001) were each independently associated with increased incidence, whereas no temporal association was demonstrated (year effect, P = .475). The median age of incident cases was 76.8 years (interquartile range, 65.0–83.3). Urinary tract source was the most common presumed source (42.2%), and Enterococcus faecalis was the predominant (83.5%) pathogen. Overall, 14.4% of patients met modified Duke criteria for definite infective endocarditis. Cumulative mortality was 28.4% at 12 weeks and 43.1% at 1 year. Conclusions In this population-based study, ME-BSI was significantly associated with older age and male sex. There was a high rate of mortality at 12 weeks and 1 year, with ∼1 out of 7 cases complicated by definite infective endocarditis. These findings underscore the importance of ongoing investigation of this syndrome.

Abstract Background The Rochester Epidemiology Project (REP) has been used to examine numerous infectious and non-infectious entities in a population-based approach in Olmsted County, Minnesota. To further investigate patterns of enterococcal bloodstream infection (BSI), we used the Expanded Rochester Epidemiology Project (E-REP) to include additional surrounding counties to conduct a retrospective population-based study of adult patients who developed enterococcal BSI, with or without infective endocarditis (IE). Methods The E-REP population under study included eight counties in southeastern Minnesota (including Olmsted County), representing a total population of over 250,000 individuals. Adults (age ≥ 18 years) diagnosed with an initial episode of enterococcal BSI with or without enterococcal IE between January 1, 2018 and December 31, 2022 were included; patients with either polymicrobial BSI with enterococci or a previous bout of enterococcal BSI before the study period were excluded. Cases of enterococcal BSI and IE were identified using ICD-10 codes and underwent complete medical record review. Patient characteristics, microbiology, antibiotic regimens, infectious complications, and outcomes were collected. Results A total of 113 cases of enterococcal BSI were identified over the five-year study period. The age- and sex-adjusted incidence rate of enterococcal BSI was 6.9 per 100,000 person-years, with a significantly higher rate among males than females (Figure 1). BSI incidence rate varied by age, with patients 80 years or older having the highest incidence rate (Figures 1 and 2). Twenty-two cases of enterococcal IE were identified, yielding an overall IE prevalence of 20.8% (Figure 3). The cumulative mortality rate at 12 months was high at 41.7% (Figure 3). Five patients (4.4%) had a relapse of enterococcal BSI, defined as recurrence of BSI after negative follow-up blood cultures and within 12 weeks of the initial BSI. Conclusion This is the first population-based investigation that has focused on the epidemiology and clinical profile of patients with enterococcal BSI and complicating IE in a US population. This included an IE prevalence of 20.8%, which should be considered when evaluating and managing patients with enterococcal BSI. Disclosures Larry M. Baddour, MD, UpToDate, Inc.: Royalty payments (authorship duties).