Aortic root dilation in acromegaly.

Previous studies have linked persistent elevations in growth hormone (GH) and insulin-like growth factor-1 (IGF-1) to cardiac abnormalities including aortic root dilation. Guidelines in the management of this dilation below the size recommended for surgery have not been well defined but follow-up and intervention when appropriate could be life-saving. We report the case of a man in his 60s who had been living with undiagnosed acromegaly for many years. His initial assessment through point-of-care ultrasound raised concerns about potential cardiac enlargement, prompting further investigation with a formal echocardiogram, which revealed a significant aortic root dilation measuring 4.5 cm. Subsequent blood tests confirmed elevated levels of IGF-1. Brain MRI showed a focal lesion in the pituitary gland, which was surgically resected, confirming the diagnosis of a GH-secreting pituitary adenoma. One year after surgery, a repeat CT angiogram of the chest demonstrated a stable size of the aortic root aneurysm.

Outcome Comparison in Hospitalized COVID-19 Patients With and Without AKI.

Aim: Patients hospitalized with COVID-19 have a higher incidence of Acute Kidney Injury (AKI) compared with non-COVID patients. Previous observational studies showed AKI in hospitalized patients with COVID-19 was associated with significant increased mortality rate. We conducted a retrospective cohort study in a large mid-Atlantic health system to investigate whether COVID-19 associated AKI during hospitalization would lead to worse outcomes in a predominant Black patient population, compared to COVID-19 without AKI. Methods: We reviewed health records of patients (aged≥18 years) admitted with symptomatic COVID-19 between March 5, 2020, and Jun 3, 2020, in 9 acute care facilities within the MedStar Health system. Patients were followed up until 3 months after discharge. Primary outcome was inpatient mortality. Secondary outcomes were need for ICU level of care, need for intubation, length of ICU stay, length of hospital stay, need for renal replacement therapy, recovery of renal function. Results: Among 1107 patients admitted with symptomatic COVID-19, the AKI incidence rate was 35 %. African American patients made up 63 % of the total patient population and 74 % of the total AKI population. Inpatient mortality in the AKI group and the non-AKI group was 163 (41.9 %) and 71 (9.9 %), respectively. COVID-19 patients with AKI had significant higher risk of in-patient mortality (OR, 4.71 [95 % CI, 3.38-6.62], P < 0.001), ICU admission (OR, 4.27 [95 % CI, 3.21-5.72], P < 0.001) and need of intubation (OR, 6.18 [95 % CI, 4.45-8.68], P < 0.001). Conclusions: AKI in hospitalized patients with COVID-19 was associated with higher mortality rate, need for intubation and ICU admission compared to COVID-19 patients without AKI group.

Real-world experience utilizing the nuvision 4D intracardiac echocardiography catheter for left atrial appendage closure.

Transesophageal echocardiography (TEE) has been the preferred imaging modality to help guide left atrial appendage closure. Newer technologies such as the Nuvision 4D Intracardiac echocardiography (ICE) catheter allow for real-time 3D imaging of cardiac anatomy. There are no direct comparison studies for procedural imaging between TEE and 4D ICE. To evaluate the performance and safety of left atrial appendage (LAA) closure procedures with the Watchman FLX and Amulet, guided by the Nuvision 4D ICE Catheter. This retrospective observational analysis was conducted on institutional LAAO National Cardiovascular Data Registry from January 2022 to March 2023. Patients had undergone LAA closure procedures with the Watchman FLX or Amulet device guided by TEE or a 4D ICE Catheter. The primary outcome evaluated was successful LAAO device placement. A total of 121 patients underwent LAAO device placement with 46 (38.0%) patients guided by 4D ICE during LAAO implantation. The 4D ICE group had a shorter procedural time compared with TEE guidance. Post procedural 45-day TEE post implant was also comparable for both groups with no patients in either group having incomplete closure of the left atrial appendage and peri-device leak > 5 mm. No device related complications (device related access, stroke, or pericardial effusion) occurred in either group at follow-up. There was no significant difference in device implant success or post procedural outcomes at 45 days in either the TEE or 4D ICE group. However, there was a noticeable improvement in procedural time with the 4D ICE catheter.

The Ultrasound Hepato-Jugular Reflux: Measuring the Hepato-Jugular Reflux with Ultrasound with Comparison to Invasive Right Heart Catheterization.

BACKGROUND: Ultrasound can overcome barriers to visualizing the internal jugular vein, allowing hepato-jugular reflux and jugular venous pressure measurement. We aimed to determine operating characteristics of the ultrasound hepato-jugular reflux and ultrasound jugular venous pressure predicting right atrial and pulmonary capillary occlusion pressures. METHODS: In a prospective observational cohort at three US academic hospitals the hepato-jugular reflux and jugular venous pressure were measured with ultrasound before right heart catheterization. Receiver operating curves, likelihood ratios, and regression models were utilized to compare the ultrasound hepato-jugular reflux and ultrasound jugular venous pressure to the right atrial and pulmonary capillary occlusion pressures. RESULTS: In 99 adults undergoing right heart catheterization, an ultrasound hepato-jugular reflux had a negative likelihood ratio of 0.4 if 0 cm and a positive likelihood ratio of 4.3 if ≥ 1.5 cm for predicting a pulmonary capillary occlusion pressure ≥ 15 mmHg. Regression modeling predicting pulmonary capillary occlusion pressure was not only improved by including the ultrasound hepato-jugular reflux (P < .001), it was the more impactful predictor compared with the ultrasound jugular venous pressure (adjusted odds ratio 2.6 vs 1.2). The ultrasound hepato-jugular reflux showed substantial agreement (kappa 0.76; 95% confidence interval, 0.30-1.21), with poor agreement for the ultrasound jugular venous pressure (kappa 0.11; 95% confidence interval, -0.37-0.58). CONCLUSION: In patients undergoing right heart catheterization, the ultrasound hepato-jugular reflux is reproducible, has modest impact on the probability of a normal pulmonary capillary occlusion pressure when 0 cm, and more substantial impact on the probability of an elevated pulmonary capillary occlusion pressure when ≥ 1.5 cm.

Generation and Characterization of a Human-Derived and Induced Pluripotent Stem Cell (iPSC) Line from an Alzheimer's Disease Patient with Neuropsychiatric Symptoms.

Agitation is one of the most eminent characteristics of neuropsychiatric symptoms (NPS) affecting people living with Alzheimer's and Dementia and has serious consequences for patients and caregivers. The current consensus is that agitation results, in part, from the disruption of ascending monoamine regulators of cortical circuits, especially the loss of serotonergic activity. It is believed that the first line of treatment for these conditions is selective serotonin reuptake inhibitors (SSRIs), but these are effective in only about 40% of patients. Person-specific biomarkers, for example, ones based on in vitro iPSC-derived models of serotonin activity, which predict who with Agitation responds to an SSRI, are a major clinical priority. Here, we report the generation of human-induced pluripotent stem cells (iPSCs) from a 74-year-old AD patient, the homozygous APOE ε4/ε4 carrier, who developed Agitation. His iPSCs were reprogrammed from peripheral blood mononuclear cells (PBMCs) using the transient expression of pluripotency genes. These display typical iPSC characteristics that are karyotypically normal and attain the capacity to differentiate into three germ layers. The newly patient-derived iPSC line offers a unique resource to investigate the underlying mechanisms associated with neuropsychiatric symptom progression in AD.

Artificial intelligence for dementia prevention.

INTRODUCTION: A wide range of modifiable risk factors for dementia have been identified. Considerable debate remains about these risk factors, possible interactions between them or with genetic risk, and causality, and how they can help in clinical trial recruitment and drug development. Artificial intelligence (AI) and machine learning (ML) may refine understanding. METHODS: ML approaches are being developed in dementia prevention. We discuss exemplar uses and evaluate the current applications and limitations in the dementia prevention field. RESULTS: Risk-profiling tools may help identify high-risk populations for clinical trials; however, their performance needs improvement. New risk-profiling and trial-recruitment tools underpinned by ML models may be effective in reducing costs and improving future trials. ML can inform drug-repurposing efforts and prioritization of disease-modifying therapeutics. DISCUSSION: ML is not yet widely used but has considerable potential to enhance precision in dementia prevention. HIGHLIGHTS: Artificial intelligence (AI) is not widely used in the dementia prevention field. Risk-profiling tools are not used in clinical practice. Causal insights are needed to understand risk factors over the lifespan. AI will help personalize risk-management tools for dementia prevention. AI could target specific patient groups that will benefit most for clinical trials.

Prime time for chronic kidney disease.

Chronic kidney disease (CKD) represents a public health burden worldwide and is associated with significant morbidity and mortality. Most patients with CKD are managed by primary care practitioners and this educational series hope to improve knowledge and delivery of care to this high-risk patient population with CKD.

Diagnostic dilemma: drug-induced vasculitis versus systemic vasculitis.

Drug-induced vasculitis can rarely cause inflammation and necrosis of blood vessel walls of both kidney and lung tissue. Diagnosis is challenging because of the lack of difference between systemic and drug-induced vasculitis in clinical presentation, immunological workup and pathological findings. Tissue biopsy guides diagnosis and treatment. Pathological findings must be correlated with clinical information to arrive at a presumed diagnosis of drug-induced vasculitis. We present a patient with hydralazine-induced antineutrophil cytoplasmic antibodies-positive vasculitis with a pulmonary-renal syndrome manifesting as pauci-immune glomerulonephritis and alveolar haemorrhage.

Spontaneous Retroperitoneal Hematoma in Vitamin C Deficiency.

Retroperitoneal hematoma is rare but potentially life-threatening. It is commonly caused by traumatic or iatrogenic vascular injury, retroperitoneal neoplasm, coagulopathy, chronic anticoagulation, or fibrinolytic therapy. However, retroperitoneal hematoma due to vitamin C deficiency is rare. Here, we report a case of 40 years old man who developed retroperitoneal hematoma in context of very low vitamin C. To our knowledge, this is the second described case of retroperitoneal hematoma from vitamin C deficiency.

The Effect of Race and Socioeconomic Status on Hospitalized Patients with COVID-19 Infection.

Background: There have been varying impacts of COVID-19 on racial, ethnic, and socioeconomic communities in the US. Recent literature suggests that Black Americans have the highest unadjusted and adjusted mortality rates from COVID-19, while White Americans have the lowest unadjusted and adjusted rates. However, the role of socioeconomic status and comorbidities in these disparities in health outcomes from COVID-19 are unclear. Thus, the purpose of this study is to evaluate how socioeconomic status and race impact COVID-19 outcomes in patients hospitalized with COVID-19 in a large health care system in the Mid-Atlantic region. Material and methods: We retrospectively analyzed the association of COVID-19 outcomes and race, ethnicity, and socioeconomic status using electronic medical records and the REDCap database from the time period of March 5, 2020 to June 3, 2020. The outcomes evaluated were intubation, ICU admission, and discharge destination. Multivariate logistic regression analysis was then performed to examine whether race and socioeconomic status were independent risk factors of mortality controlling for age, Charlson comorbidity index (CCI), and comorbidities. Results: Race was not found to be an independent predictor for COVID-19 inpatient mortality. Race was found to be an independent risk factor for ICU admission with odds of ICU admission for Black patients to be 1.5 times higher (odds ratio (OR) 1.4 1.07 to 2.04) compared to Non-Black/Non-White (72.4% identifying as Hispanic) but no difference between Black and White races. Race was found not to be an independent risk factor for intubation nor was race an independent risk factor for increased length of ICU LOS, hospital LOS or intubation days. Socioeconomic status was not an independent risk factor for inpatient mortality although high income groups were significantly less likely to be admitted to the ICU compared to middle income patients. Conclusion: Our cohort of patients in a large mid-Atlantic health system showed that there was no statistically significant difference between race or socioeconomic status and COVID-19 related inpatient mortality. However, Black patients and individuals in the lower to middle socioeconomic group had a higher rate of COVID-19 hospitalizations when accounting for age, sex, and comorbidities. With ongoing vaccination efforts, equitable administration of resources should focus on disproportionately affected populations.

Ruptured Sinus of Valsalva Aneurysm in Apert Syndrome: Case report.

Background: Sinus of Valsalva aneurysm (SOVA) is a rare anomaly of the aorta that can be congenital or acquired. It can be associated with syndromes such as Marfan syndrome and Ehlers-Danlos syndrome. However, to our knowledge, it has never been described in a patient with Apert syndrome. Although it often presents as an incidental finding on imaging, SOVA is associated with the risk of serious complications, including rupture. A possible connection between the conditions might be the FGFR2 gene mutation in Apert syndrome and the influence of a mutation in fibroblast growth factor 2 (FGF2) on heart development. Here we report a case of acute heart failure secondary to rupture of SOVA into the right atrium in a patient with Apert syndrome. Case presentation: A 47-year-old Caucasian woman with a history of Apert syndrome and rheumatoid arthritis presented with shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, and progressive bilateral lower extremity edema for 2 weeks. She was diagnosed with acute right heart failure due to ruptured SOVA. The patient underwent surgical repair of the ruptured SOVA. Unfortunately, her postoperative course was complicated by a stroke leading to brain death. Conclusion: Ruptured SOVA is a quite rare but serious condition that can cause life-threatening complications. In this case, SOVA occurred in a patient with Apert syndrome. The case may suggest that these two conditions may be related through the FGFR2 gene mutation associated with Apert syndrome and the related growth factor FGF2 involved in heart development.

Improving Primary Care Delivery for Patients Receiving Maintenance Hemodialysis.

The beneficial impact of primary care, focused on all aspects of a patient's health (rather than a disease-specific focus) is well established. Recognized benefits include greater receipt of preventive care and counseling, lower use of emergency care and hospitalization for ambulatory care-sensitive conditions, and decreased early mortality. Although the importance of primary care and care coordination at the primary care/specialty interface is well recognized, the role of primary care within traditional and emerging care models for patients receiving in-center maintenance hemodialysis remains ill-defined. In this perspective article, we will describe: (1) the role of primary care for patients receiving maintenance hemodialysis and the current evidence regarding the receipt of primary care among these patients; (2) the key challenges to delivery of primary care in these complex cases, including suboptimal care coordination between nephrology and primary care providers, the intensity of dialysis care, and the limited capacity of nephrologists and primary care providers to meet the broad health needs of hemodialysis patients; (3) potential strategies for improving the delivery of primary care for patients receiving hemodialysis; and (4) future research requirements to improve primary care delivery for this high-risk population.

Lamotrigine-associated hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of excessive immune activation. It is mostly seen in the paediatric population and is rarely observed in adults. HLH can be inherited or acquired and is commonly triggered by activation of the immune system by an underlying viral infection or in immune system deficiency such as malignancy or underlying rheumatological disease. HLH is a difficult entity to diagnose due to the rarity of this disorder, variable clinical presentation and non-specific clinical and laboratory findings. HLH carries a high mortality if left untreated, and therefore prompt diagnosis and initiation of immunosuppressive, immunomodulatory and cytostatic medications are critical to improve survival in affected patients. Here, we present a case of lamotrigine-associated HLH. To our knowledge, only eight other cases of lamotrigine-associated HLH have been reported in adult patients.

Catheter-Directed Therapy: Outcomes Versus Standard of Care and Evaluation of Current Practice.

BACKGROUND: Small studies have noted benefit with the use of catheter-directed therapy (CDT) versus standard of care in treatment of pulmonary embolism, but none have focused on the variability of clinical practice with this modality. METHODS: International Classification of Diseases (ICD) codes were used to retrospectively identify consecutive adult patients admitted to an intensive care unit (ICU) with pulmonary embolism over a 2-year period. We evaluated inpatient mortality and major bleeding and assessed treatment variation. RESULTS: Of 284 patients included, 46 underwent CDT (9 massive pulmonary embolism, 37 submassive pulmonary embolism). Significantly more patients who underwent standard treatment had a history of congestive heart failure and diabetes. Obesity, higher troponin levels, and right heart strain were significantly more likely in the CDT group. No significant difference in inpatient mortality or major bleeding events was observed between the treatment groups. Tissue plasminogen activator use varied widely in the CDT group, and inferior vena cava filter utilization was significantly more common in the CDT group (18; 41%) compared with the standard group (40; 17%) (P < 0.01). CONCLUSIONS: In this study, no significant difference in inpatient mortality or major bleeding was found in patients in the intensive care unit with pulmonary embolism who underwent CDT compared with standard care. It may be beneficial to standardize this procedure given the potential benefit of CDT in patients with submassive pulmonary embolism.

A rare case of spontaneous tumor lysis syndrome in multiple myeloma.

Spontaneous tumor lysis syndrome is an uncommon oncologic emergency. It occurs when a massive number of malignant cells release their contents to the blood stream without previous cancer treatment. TLS carries a mortality rate exceeding 15%. Because of the high mortality rate, the key to the management of TLS continues to be early recognition of high-risk patients and using prophylactic measures to prevent its occurrence. However, it remains difficult to completely eradicate TLS, as a small proportion of patients with aggressive tumors develop spontaneous TLS prior to receiving any therapy. We present a case of 58-year-old male with recently diagnosed multiple myeloma. He was found to have hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, elevated LDH levels, and acute renal failure, fulfilling the criteria of clinical TLS. He was treated with rasburicase, continuous renal replacement therapy, and dexamethasone.

High Scan Volume with Low Positive Scan Rate in Highest Utilizers: Computed Tomography Pulmonary Angiography in the Emergency Department.

BACKGROUND: We examined utilization patterns and predictors of computed tomography pulmonary angiography (CTPA) results in the emergency department (ED). OBJECTIVES: We retrospectively determined ED CTPA positive scan rate (PSR) among ED attendings based on a system that grouped attendings into scan quantity categories through >100. METHODS: We manually reviewed all scans ordered in 2017 in EDs in a multisite medical system. RESULTS: Of 10,032 ED CTPAs, 6168 were ordered by 153 ED attendings. Most attendings (123/153; 80%) ordered 60 or fewer scans with relatively high PSR (259/2927, PSR 8.8%; 95% confidence interval 7.8-9.9%). Of the ED attendings, 13 (3%) ordered more than 100 scans each (1981 scans; 32% of all scans), with PSR of 5.5% (95% confidence interval 4.5-6.5%). CONCLUSION: Most ED attendings were low- to mid-volume utilizers of CTPA and had a relatively high PSR. However, the small percentage of attendings who ordered more than 100 scans each accounted for a large percentage of the total scan volume and had a relatively low PSR. These findings suggest that sharing of performance feedback and best practices in the highest utilizers could help to improve CTPA PSR in the ED.

ACE inhibitor angioedema: characterization and treatment versus non-ACE angioedema in acute hospitalized patients.

Background: ACE angioedema has not been characterized in comparison with angioedema from other causes in acute hospitalized patients. Methods: We retrospectively compared ACE-angioedema and non-ACE angioedema patients from January 2013 to May 2017. Results: Of 855 cases screened, 575 met the inclusion criteria of angioedema diagnosis and an electronic medical record. Of these, 297 (51.7%) had ACE angioedema and 278 had angioedema from other causes, of these 31 who were taking an ACE inhibitor that was not considered to be the cause of angioedema (ACE other cause). At least 80% of cases in all groups were African American. Epinephrine was prescribed in 21% of ACE angioedema cases. One-third of patients in all groups were admitted to the ICU, and about 25% required intubation. Previous history of ACE inhibitor-induced angioedema was found in 63 of 278 non-ACE cause angioedema patients (23%) and in 23 (8%) in the ACE cause group. Conclusion: ACE angioedema was the cause of half of angioedema admissions over a 4.5-year period. Mortality, morbidity, and treatment did not differ between the groups. Patients on ACE inhibitors were often treated with medications known not to be effective for ACE angioedema. Over one-fourth of patients not taking an ACE inhibitor had a previous history of ACE angioedema, and 31 patients taking ACE inhibitors were diagnosed with non-ACE angioedema. Regardless of the etiology of angioedema, 25% of patients required airway protection in the form of intubation.