RocScholar (Rochester Regional Health)
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What Cardiologists Should Know About Amyloidosis
No full textBackground: Cardiac amyloidosis (CA) is an increasingly recognized but historically underdiagnosed cause of restrictive cardiomyopathy and heart failure with preserved ejection fraction (HFpEF). It results from the extracellular deposition of misfolded protein fibrils, most commonly transthyretin (ATTR) or immunoglobulin light chains (AL), leading to progressive myocardial dysfunction and multi-organ involvement.
Objective: This review provides a comprehensive, cardiology-centered overview of cardiac amyloidosis, with an emphasis on early recognition, diagnostic strategies, subtype differentiation, and the evolving therapies.
Content: We summarize the epidemiology, pathophysiology, and clinical manifestations of both ATTR and AL subtypes. Key diagnostic tools, including echocardiography, cardiac magnetic resonance imaging, bone scintigraphy, monoclonal protein screening, and endomyocardial biopsy, are reviewed in the context of a stepwise diagnostic approach. Special attention is given to clinical presentation, electrocardiographic and imaging red flags, and to differentiating CA from mimickers such as hypertrophic cardiomyopathy, hypertension-induced left ventricular hypertrophy, and aortic stenosis. Staging systems are detailed, highlighting the prognostic role of cardiac biomarkers. Therapeutic strategies are explored, including subtype-specific regimens (e.g., daratumumab-based therapy for AL; tafamidis and gene silencers for ATTR), the judicious use of conventional heart failure medications, and emerging therapies such as CRISPR-based gene editing.
Conclusions: Timely recognition and accurate diagnosis of cardiac amyloidosis are critical to improving outcomes. As diagnostic tools and disease-modifying therapies evolve rapidly, cardiologists must remain at the forefront of multidisciplinary care. A structured biomarker- and imaging-guided approach can enhance diagnostic yield, inform prognosis, and optimize patient-specific management
Efficacy of negative pressure wound therapy blowhole placement in alleviating severe subcutaneous emphysema and associated transient blindness following video-assisted thorascopic surgery: a case series
No full textVideo-assisted thorascopic surgery (VATS) lobectomy is widely used for treating lung cancer, but severe subcutaneous emphysema can be a rare complication, leading to distressing symptoms such as pain, temporary blindness, and voice changes. Traditional management strategies often require prolonged treatment, and blowhole incisions can result in infection and discomfort. This case series explores the use of negative pressure wound therapy (NPWT) applied via a unilateral blowhole incision to treat severe subcutaneous emphysema post-VATS lobectomy. In three cases, NPWT facilitated rapid resolution of pain, restored vision, and resolved voice changes within 8 h. No infection-related complications occurred. This approach not only accelerates recovery and alleviates patient discomfort but also reduces the burden on caregivers and healthcare teams. NPWT should be considered a valuable addition to the management of severe subcutaneous emphysema, improving patient outcomes and enhancing postoperative care
Listeria Bacteremia With Septic Brachial Artery Thrombosis and Pacemaker Infection in an Elderly Woman With a Prosthetic Aortic Valve and Recurrent Pacemaker Implantation
No full textA 77-year-old female with a history of coronary artery disease status post-coronary artery bypass grafting, aortic stenosis treated with transcatheter aortic valve replacement, and recent dual-chamber pacemaker implantation for high-grade atrioventricular block presented with recurrent syncope, right brachial artery thrombosis, and persistent bacteremia. Blood and thrombus cultures grew Listeria monocytogenes, leading to pacemaker extraction and a prolonged course of antibiotic therapy. This case illustrates the diagnostic challenges associated with systemic Listeria infections in patients with prosthetic devices and emphasizes the importance of early blood culture collection in patients with unexplained clinical symptoms
Anxiety: How Do I Get Through This Clinic Visit
No full textAnxiety: How Do I Get Through This Clinic Visit. Lorinda Parks, MD, Primary Care Regional Medical Director
Objectives:
Dr. Parks will discuss when patients come to clinic with a variety of forms of anxiety and how they often serve to derail the medical content of the visit, or risk quash any hope of the provider staying on schedule. There are tools and skills which can be deployed for anxiety similar to those used for HTN or DM but are not as widely taught. This presentation will strive to offer clinicians a usable skill set to educate patients about the message that anxiety is offering them, to remind patient and clinician that each of us can, in fact, manage the “season” of the life we are currently in and even find joy in the messages of anxiety. Participants should be able to utilize Emotional Tag Lines, THE WORK in 4 Questions and the Turnarounds in a formulaic way for anxiety; similar to the way pharmacologics are used in step wise approach to COPD or HTN. Select a tool which can generate understanding of anxiety and facilitate completion of the clinic visit while optimizing joy and connection
Controversies in the Indications for Surgery
No full textIt has been said “good surgeons know how to operate, better ones when to operate, and the best, when not to operate.” In addressing the treatment of craniosynostosis, this adage holds for both neurosurgeons and plastic surgeons. The nature and mechanisms of neurologic dysfunction in nonsyndromic craniosynostosis are subjects of great debate. Even in cases where there is a clear indication to operate, nuances of surgical management such as optimization of timing and technique are vigorously debated.
This chapter discusses the controversies in management of craniosynostosis, including the role of genetics and bony compression on neurologic outcome, the advisability, timing, and type of surgery, and the optimization of perioperative supports
COPD: Best Practices and New Horizons
No full textCOPD: Best Practices and New Horizons. Dr. Talal Kaiser, Pulmonary Unit
Objectives: Recognize COPD as common pulmonary disease with significant morbidity and mortality Understand the pathophysiology of obstructive lung disease Review best practice guidelines for COPD management and understand the evidence behind the guidelines Discuss new medication options available for COPD management including biologic therap
Characterization of bleeding in thrombotic thrombocytopenic purpura in the precaplacizumab era: a retrospective nationwide analysis
No full textBackground: The addition of caplacizumab to immune thrombotic thrombocytopenia (iTTP) treatment options has led to a renewed interest in characterizing the epidemiology and risk factors for bleeding in iTTP. Limited data exist on the bleeding risk in iTTP due to systemic underreporting in earlier cohorts.
Objectives: To describe the incidence, patterns, and predictors of bleeding in hospitalized iTTP patients independent of caplacizumab use.
Methods: We retrospectively analyzed the National Inpatient Sample database (2012-2019) and identified adult patients with a diagnosis of iTTP. Predictors of bleeding were determined by multivariable logistic regression analysis.
Results: We identified 3103 iTTP hospitalizations; bleeding occurred in 594 (19.1%), and 157 (5.1%) were characterized by major bleeding. Mucocutaneous bleeding (7.6%) was the most frequent type of bleeding and included heavy menstrual bleeding (2.6%), gingival (2.3%), epistaxis (1.4%), and skin/procedure-related bleeding (1.3%). This was followed closely by gastrointestinal bleeding (5.6%). Patients with bleeding were more likely to be Hispanic, have a weekend admission, and have a higher prevalence of comorbidities. In the multivariable analysis, Hispanic race (odds ratio [OR], 1.48; 1.14-1.91), Asian/Pacific Islander/Native American race (OR, 2.04; 1.51-2.76), coronary artery disease (OR, 1.70; 1.38-2.11), heart failure (OR, 1.39; 1.13-1.72), autoimmune disease (OR, 2.61; 2.08-3.26), Charlson Comorbidity Index ≥ 3 (OR, 2.08; 1.66-2.61), weekend admission (OR, 1.45; 1.22-1.72), and delay ≥2 days in plasma exchange initiation (OR, 1.63; 1.38-1.92), were significantly associated with major bleeding.
Conclusions: Bleeding is a relatively common issue in acute iTTP that has not been adequately addressed in existing literature. Further studies are needed to elucidate this risk and associated factors, especially given the incorporation of caplacizumab in the treatment of iTTP
