A case of cardiogenic shock due to ventricular dyssynchrony resolved by atrial pacing.

We present a case of a man with ischemic cardiomyopathy and single chamber implantable cardioverter-defibrillator who developed sinus arrest creating sudden dependence on right ventricular (RV) pacing. He presented with cardiogenic shock secondary to abrupt onset ventricular dyssynchrony from RV pacing, which required emergent stabilization and completely resolved with atrial pacing. Learning objective: To establish a basic understanding of cardiogenic shock management. To reinforce the adverse effects associated with right ventricular pacing.

Epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States and factors predictive of outcome.

Background: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap syndrome are rare severe cutaneous adverse reactions associated with high mortality. Objectives: To estimate incidence and describe trends of SJS/TEN hospitalizations in the United States and to describe the clinical, demographic, and geographic characteristics of affected patients and risk factors for mortality. Methods: We utilized hospitalization data from the 2010 to 2020 National Inpatient Sample. SJS, SJS-TEN overlap syndrome, and TEN were identified by International Classification of Diseases, 9th Revision and International Classification of Diseases, 10th Revision codes and analyzed by logistic regression. Results: We identified 51,040 hospitalizations involving SJS/TEN. Amog those, 37,283 (73.0%) were for SJS only, 7818 (15.3%) were for SJS-TEN overlap syndrome, and 7160 (14.0%) were for TEN only. Overall, SJS/TEN hospitalization rates declined over time, 2010 to 2020 (P < .05). Mortality rates of the SJS group, SJS-TEN overlap syndrome group, and TEN group were 5.4%, 14.4%, and 15.3%, respectively. Increasing age, chronic kidney disease, pneumonia, sepsis, and malignant neoplasm were all significantly associated with increased odds of mortality (P < .05). Non-Hispanic White racial/ethnic identification was associated with decreased odds of mortality (P < .05). Limitations: Lack of standardization for diagnostic criteria. Conclusions: Risk factors identified in this study lay the groundwork for improvement in SJS/TEN mortality prediction scoring.

Transcatheter vacuum aspiration of valvular and lead related infective endocarditis.

BACKGROUND: Transcatheter aspiration is utilized for removal of thrombi and vegetations in inoperable patients and high-risk surgical candidates where medical therapy alone is unlikely to achieve desired outcome. A number of case reports and series have been published since the introduction of AngioVac system (AngioDynamics Inc., Latham, NY) in 2012 where this technology was used in the treatment of endocarditis. However, there is a lack of consolidated data reporting on patient selection, safety and outcomes. METHODS: PubMed and Google Scholar databases were queried for publications reporting cases where transcatheter aspiration was used for endocarditis vegetation debulking or removal. Data on patient characteristics, outcomes and complications from select reports were extracted and systematically reviewed. RESULTS: Data from 11 publications with 232 patients were included in the final analyses. Of these, 124 had lead vegetation aspiration, 105 had valvular vegetation aspiration, and 3 had both lead as well as valvular vegetation aspiration. Among the 105 valvular endocarditis cases, 102 (97 %) patients had right sided vegetation removal. Patients with valvular endocarditis were younger (mean age 35 years) vs. patients with lead vegetations (mean age 66 years). Among the valvular endocarditis cases, there was a 50-85 % reduction in vegetation size, 14 % had worsening valvular regurgitation, 8 % had persistent bacteremia and 37 % required blood transfusion. Surgical valve repair or replacement was subsequently performed in 3 % and in-hospital mortality was 11 %. Among patients with lead infection, procedural success rate was reported at 86 %, 2 % had vascular complications and in-hospital mortality was 6 %. Persistent bacteremia, renal failure requiring hemodialysis, and clinically significant pulmonary embolism occurred in about 1 % each. CONCLUSIONS: Transcatheter aspiration of vegetations in infective endocarditis has acceptable success rates in vegetation debulking as well as rates of morbidity or mortality. Large prospective multi-center studies are warranted to determine predictors of complications, thus helping identify suitable patients.

Circulating Biomarkers in Coronary Microvascular Dysfunction.

Coronary microvascular dysfunction is an underdiagnosed pathologic process that is associated with adverse clinical outcomes. Biomarkers, molecules measurable in the blood, could inform the clinician by aiding in the diagnosis and management of coronary microvascular dysfunction. We present an updated review of circulating biomarkers in coronary microvascular dysfunction representing key pathologic processes, including inflammation, endothelial dysfunction, oxidative stress, coagulation, and other mechanisms.

Severe cardiogenic shock and cardiac arrest due to fulminant cardiac sarcoidosis: a case report.

BACKGROUND: Cardiac sarcoidosis is found to occur in approximately 5% of patients with sarcoidosis. Its presentation can typically range from complete heart block to ventricular arrhythmias. This condition can rarely present with severe heart failure and cardiogenic shock requiring aggressive and timely management strategies. Advanced imaging techniques are usually required to assist with its diagnosis. CASE PRESENTATION: A 70-year-old woman with a history of pulmonary sarcoidosis presented with non-ST elevation myocardial infarction, congestive hepatopathy, and acute renal failure. Left heart catheterization showed evidence of non-obstructive coronary artery disease, and right heart catheterization revealed severely elevated filling pressures and depressed cardiac index. She underwent aggressive diuresis and placement of an intra-aortic balloon pump in addition to initiation of inotropic and vasopressor support. While in the cardiac intensive care unit, she experienced frequent episodes of ventricular tachycardia and went into cardiac arrest requiring cardiopulmonary resuscitation. High clinical suspicion for cardiac sarcoidosis was confirmed by cardiac magnetic resonance imaging findings. After starting immunosuppressive therapy for cardiac sarcoidosis, she demonstrated clinical improvement. CONCLUSION: Patients with cardiac sarcoidosis may remain asymptomatic or present with conduction abnormalities and arrhythmias. They rarely present with severe biventricular heart failure and cardiogenic shock, and in such cases, they require timely initiation of pharmacologic and device therapies, along with implementation of mechanical circulatory support.

Prescribing patterns of SGLT-2 inhibitors for patients with heart failure: A two-center analysis.

Background: Sodium glucose co-transporter 2 inhibitors (SGLT2i) have been proven to reduce the combined risk of cardiovascular death and hospitalizations in patients with heart failure (HF), irrespective of the presence or absence of diabetes. Despite class 1 and class 2A recommendations for their usage in HF with reduced ejection fraction (HFrEF) and HF with preserved ejection fraction (HFpEF) respectively by the American College of Cardiology, their prescription rate has remained low. Objective: The aim of this study is to analyze SGLT2i prescription patterns at two academic institutions, with the goal of identifying barriers to implementation. Design: A two-center retrospective analysis was conducted on patients ≥18 years old with a diagnosis of heart failure who were admitted to one of two hospital systems between 5/1/21 and 5/31/22. Patients with an eGFR ≥20 mL/min/1.73m2 and BNP ≥ 100 pg/mL were included. Results: SGLT2i was prescribed in only 19 out of 1081 HFpEF patients (1.8 %) and 51 out of 1596 HFrEF patients (3.2 %). A majority of SGLT2i prescriptions for the HFpEF population came from general medicine services (57.9 %) after obtaining approval from a cardiologist, which was required at our institutions. Adverse effects such as hypoglycemia and urinary tract infections were not significantly associated with SGLT2i use. Conclusions: Despite proven benefits of this class of medications as witnessed in large-scale clinical trials, the implementation of this drug class continues to be low.

Use of Multimodal Therapies to Treat Severe Orthostatic Hypotension From Autonomic Failure.

Orthostatic hypotension is one of the most debilitating features of autonomic failure. Presentations tend to be diverse and non-specific, ranging from dizziness and lightheadedness to loss of consciousness. Early recognition of this illness may prove to be difficult, and control of symptoms typically requires a multidisciplinary approach with patient education, pharmacologic, and device therapies. This condition tends to be associated with significant patient morbidity and poor quality of life, and treatment regimens typically require frequent adjustments before optimal symptom control is achieved. However, with a multidisciplinary approach, utilization of multimodal therapies, and proper identification of underlying pathophysiologic mechanisms, symptom improvement may be achieved. An 81-year-old male was admitted to our hospital following a syncopal episode. While hospitalized, he experienced severe syncope and was treated with physical therapy, midodrine, fludrocortisone, octreotide, erythropoietin, and intravenous iron infusions. He displayed minimal improvement and then had a biventricular pacemaker placed and had a resolution of all postural symptoms.

Spontaneous Coronary Artery Dissection-Induced Takotsubo Syndrome.

A case of an anxious 59-year-old woman, who presented with chest pressure, nausea, and vomiting, is described. After hours of symptoms that worsened despite medical management, cardiac catheterization was performed. Angiography revealed diffuse, long, tubular disease of multiple coronary vessels. Additionally, left ventriculography was consistent with Takotsubo syndrome. Based on both coronary angiography and left ventriculography, it was determined that this patient had concomitant spontaneous coronary artery dissection and Takotsubo syndrome.

Severe myopericarditis following the third dose of an mRNA COVID-19 vaccine: utility of a multimodal treatment approach.

We report a rare case of severe myopericarditis in a healthy man in his 20s after the third dose of an mRNA COVID-19 vaccine. His symptoms and troponinemia resolved with a beta-blocker in addition to standard anti-inflammatory therapy, highlighting the utility of multimodal therapy.

Multisystem organ failure secondary to acute generalised exanthematous pustulosis (AGEP) with atypical presentation resembling septic shock.

A woman was admitted for sepsis secondary to cellulitis. After clinical improvement of sepsis, non-follicular small pustules were observed on the trunk, limbs and face while vesicles/bullae and skin exfoliation were noted on upper extremities. Larger systemic manifestations included fever, hypertension and tachycardia. Laboratory results revealed neutrophilic leukocytosis, eosinophilia, mild transaminitis and acute renal failure. Despite treatment for potential sepsis and discontinuation of offending agents, her condition worsened leading to haemodynamic instability and renal failure requiring vasopressor support, intubation and continuous veno-venous haemodialysis. Skin biopsy revealed a diagnosis of acute generalised exanthematous pustulosis (AGEP), a rare condition usually caused by antibiotic treatment. The suspected offending drug was clindamycin, with possible combined effects by metronidazole and/or vancomycin. Improvement of skin manifestations were seen within 48 hours of starting systemic steroids. Here, we present an uncharacteristic case of AGEP clinically presenting with atypical skin lesions, severe systemic involvement mimicking septic shock, which culminated in multisystem organ failure.

Critical role of the coronary microvasculature in heart disease: From pathologic driving force to "innocent" bystander.

The coronary microvasculature is responsible for providing oxygen and nutrients to myocardial tissue. A healthy microvasculature with an intact and properly functioning endothelium accomplishes this by seemless changes in vascular tone to match supply and demand. Perturbations in the normal physiology of the microvasculature, including endothelial and/or vascular smooth muscle dysfunction, result in impaired function (vasoconstriction, antithrombotic, etc.) and structural (hypertrophic, fibrotic) abnormalities that lead to microvascular ischemia and potential organ damage. While coronary microvascular dysfunction (CMD) is the primary pathologic driving force in ischemia with non-obstructive coronary artery disease (INOCA), angina with no obstructive coronary arteries (ANOCA), and myocardial infarction with non-obstructed coronary arteries (MINOCA), it may be a bystander in many cardiac disorders which later become pathologically associated with signs and/or symptoms of myocardial ischemia. Importantly, regardless of the primary or secondary basis of CMD in the heart, it is associated with important increases in morbidity and mortality. In this review we discuss salient features pertaining to known pathophysiologic mechanisms driving CMD, the spectrum of heart diseases where it places a critical role, invasive and non-invasive diagnostic testing, management strategies, and the gaps in knowledge where future research efforts are needed.

Clinical mimickers of calciphylaxis: A retrospective study.

BACKGROUND: Calciphylaxis is an ischemic vasculopathy with high morbidity and mortality. Early and accurate diagnosis is critical to management of calciphylaxis. Clinical mimickers may contribute to delayed or misdiagnosis. OBJECTIVE: To assess the rate and risk factors for misdiagnosis and to identify clinical mimickers of calciphylaxis. METHODS: A retrospective medical record review was conducted of patients with calciphylaxis at a large urban tertiary care hospital between 2006 and 2018. RESULTS: Of 119 patients diagnosed with calciphylaxis, 73.1% were initially misdiagnosed. Of patients not initially misdiagnosed, median time to diagnosis from initial presentation was 4.5 days (interquartile range, 1.0-23.3), compared to 33 days (interquartile range, 13.0-68.8) in patients who were initially misdiagnosed (P = .0002). The most common misdiagnoses were cellulitis (31.0%), unspecified skin infection (8.0%), and peripheral vascular disease (6.9%). Patients who were misdiagnosed frequently received at least 1 course of antibiotics. Patients with end-stage renal disease were less likely to be misdiagnosed than those without this disease (P = .001). LIMITATIONS: Single-center, retrospective study. CONCLUSIONS: Understanding the risk factors for misdiagnosis of calciphylaxis is an opportunity for further education concerning this rare disease.

Penile calciphylaxis: A retrospective case-control study.

BACKGROUND: Calciphylaxis is a rare disorder characterized by skin necrosis caused by calcium deposition within vessels, thrombosis, and subsequent tissue ischemia. Penile involvement may rarely occur. OBJECTIVE: To identify risk factors, diagnosis, management, and mortality of patients with penile calciphylaxis. METHODS: A retrospective medical record review was conducted of 16 patients with penile calciphylaxis treated at 2 large urban tertiary care centers between January 2001 and December 2019. A control group of 44 male patients with nonpenile calciphylaxis at the same institution was included. RESULTS: The median survival of patients with penile calciphylaxis was 3.8 months (interquartile range, 27.0 months). Mortality was 50% at 3 months and 62.5% at 6 months for penile calciphylaxis, and 13.6% at 3 months and 29.5% at 6 months for controls (P = .008). Patients with penile calciphylaxis were less likely to be obese (P = .04) but more likely to have hyperparathyroidism (P = .0003) and end-stage renal disease (P = .049). LIMITATIONS: Retrospective study design and small sample size. CONCLUSIONS: This study further defines the disease course of penile calciphylaxis, which has high mortality. Imaging may be used to aid diagnosis. Risk factors include end-stage renal disease, hyperparathyroidism, and normal body mass index.

Assessment of outcomes of calciphylaxis.

BACKGROUND: Calciphylaxis is a rare thrombotic vasculopathy characterized by high morbidity and mortality. There is a paucity of studies examining longitudinal outcomes. OBJECTIVE: To assess mortality, days spent in the hospital, and amputations in patients with calciphylaxis. METHODS: A retrospective medical record review was conducted in 145 patients diagnosed with calciphylaxis at an urban tertiary care hospital from January 2006 to December 2018. RESULTS: Six-month mortality was 37.2%, and 1-year mortality was 44.1%. Patients with nephrogenic calciphylaxis had worse survival than those with nonnephrogenic calciphylaxis (P = .007). This difference in survival disappeared when limiting mortality to deaths due to calciphylaxis. Age (P = .003) and end-stage renal disease (P = .01) were risk factors associated with 1-year mortality. Diabetes mellitus was associated with greater total hospitalization days (coefficient, 1.1; 95% confidence interval, 1.01-1.4); bedside debridement was associated with fewer hospitalization days (coefficient, 0.8; 95% confidence interval, 0.7-0.9). Amputations were not associated with any of the examined risk factors. The use of warfarin followed by a transition to nonwarfarin anticoagulation was associated with decreased hazard of death (P = .01). LIMITATIONS: Retrospective nature. CONCLUSIONS: Calciphylaxis remains a complex, heterogeneous disease. Mortality is lower in patients with nonnephrogenic disease. These findings may be incorporated during discussions regarding the goals of care to facilitate informed shared decision making.