Lehigh Valley Health Network

Previous single-center studies conducted in the United States in women with suspected preeclampsia indicated that serum soluble fms-like tyrosine kinase 1-to-placental growth factor ratio values of >38 measured on a widely available Elecsys immunoassay platform during the third trimester of pregnancy predicted the development of preeclampsia with severe features and adverse outcomes within 2 weeks of testing. This study aimed to validate the Elecsys soluble fms-like tyrosine kinase 1-to-placental growth factor ratio test for the prediction of preeclampsia with severe features in a United States population using the prospective Preeclampsia Risk Assessment: Evaluation of Cut-offs to Improve Stratification cohort that recruited women with a hypertensive disorder of pregnancy (gestational hypertension, chronic hypertension, de novo and superimposed preeclampsia) across 18 tertiary and community hospitals throughout the United States. We measured soluble fms-like tyrosine kinase 1-to-placental growth factor ratios using the Elecsys platform in archived serum samples from the Preeclampsia Risk Assessment: Evaluation of Cut-offs to Improve Stratification study that recruited hospitalized women with a hypertensive disorder of pregnancy between 23 0/7 and 34 6/7 weeks of gestation. The primary study outcome was prediction of preeclampsia with severe features within 2 weeks after testing. The secondary outcomes included a composite of adverse maternal and fetal/neonatal outcomes and prediction of delivery within 2 weeks. In the validation cohort of the Preeclampsia Risk Assessment: Evaluation of Cut-offs to Improve Stratification study (556 enrollments), the serum soluble fms-like tyrosine kinase 1-to-placental growth factor ratio at a cutoff value of >38 demonstrated a 67% positive predictive value (95% confidence interval, 61-73) and a 95% negative predictive value (95% confidence interval, 92-97) for the progression to preeclampsia with severe features within 2 weeks. Among women at <30 weeks of gestation (n=188), serum soluble fms-like tyrosine kinase 1-to-placental growth factor ratio at a cutoff value of >38 demonstrated a 78% positive predictive value (95% confidence interval, 68-86) and a 100% negative predictive value (95% confidence interval, 96-100) for the progression to preeclampsia with severe features within 2 weeks. The Elecsys soluble fms-like tyrosine kinase 1-to-placental growth factor ratio test performed better than standard-of-care clinical measures, with an area under the receiver operating characteristic curve of 0.92 (95% confidence interval, 0.89-0.90) for soluble fms-like tyrosine kinase 1-to-placental growth factor ratio vs <0.70 for standard-of-care tests, such as liver enzymes, platelet count, and serum creatinine (P<.001). Compared with women with a ratio of ≤38, those with a ratio of >38 had a higher risk of developing adverse maternal (relative risk, 4.9 [95% confidence interval, 2.6-9.9]; P<.001) and fetal/neonatal (relative risk, 3.2 [95% confidence interval, 2.6-4.0]; P<.001) outcomes and were more likely to deliver within 2 weeks (adjusted hazard ratio, 3.3 [95% confidence interval, 2.7-4.0]; P<.001). The soluble fms-like tyrosine kinase 1-to-placental growth factor ratio of >38 measured on the Elecsys platform predicted the development of preeclampsia with severe features, delivery, and adverse maternal and fetal/neonatal outcomes within 2 weeks of testing among preterm pregnant women hospitalized with a hypertensive disorder of pregnancy.

Introduction and Importance:Transdiaphragmatic intercostal hernias (TDIHs) are rare and typically posttraumatic. The coexistence of a TDIH with congenital anomalies, such as a Morgagni hernia (MH) and chest wall defect, is exceptionally uncommon. This report details a unique case of MH associated with an ipsilateral intercostal hernia in an adult, underscoring key diagnostic and surgical challenges.Case Presentation:A 71-year-old man with multiple comorbidities presented with acute abdominal pain and a right-sided chest wall bulge. Computed tomography revealed a large right lateral diaphragmatic and intercostal hernia between the right 9th and 10th ribs, with bowel loops protruding through the intercostal defect. The symptoms spontaneously improved, and repeat imaging showed a reduction of the hernia content. Thoracotomy revealed an omental herniation through an anteromedial diaphragmatic defect (MH) and a congenital chest wall defect with stretched intercostal muscles. The MH was primarily repaired using nonabsorbable sutures, and rib approximation was performed with absorbable sutures to address the chest wall defect.Clinical Discussion:This case is remarkable for the association of two congenital defects: an MH and a TDIH. Unlike typical posttraumatic TDIHs, the intercostal defect in this case stemmed from congenital chest wall maldevelopment. Preoperative diagnosis was challenging, with the MH initially missed on imaging. This emphasizes the importance of considering congenital variants in atypical hernias.Conclusion:To our knowledge, this is the first documented case of a TDIH occurring in conjunction with an MH, both likely congenital. Prompt recognition and tailored interventions are crucial for avoiding missed diagnoses and complications.

Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune disease that predominantly affects the optic nerves and spinal cord, which is a distinct disease process from multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease. The most common neuro-ophthalmologic manifestation of NMOSD is atypical optic neuritis (ON), characterized by severe vision loss with a higher propensity toward bilateral involvement, longitudinal enhancement of the optic nerve, and chiasmal involvement than MS-related optic neuritis. Due to the severity of disease, high recurrence rate, and significant risk of permanent deficits, early recognition of neuro-ophthalmologic signs and short- and long-term management with immunosuppressive therapies is critical. In this article, we will review the characteristic neuro-ophthalmologic findings in NMOSD, serologic markers, neuroimaging findings, and current approaches to both acute and long-term treatment.

Background: Acute myocardial infarction (AMI) remains a leading cause of morbidity and mortality worldwide. Ventricular arrhythmias (VAs), including ventricular tachycardia and ventricular fibrillation, are often life-threatening in setting of AMI. While advances in revascularization have improved outcomes, the burden of AMI and VA-related mortality across U.S. subpopulations remains unclear. We aimed to assess trends and demographic disparities in AMI and VA-related deaths in the United States. Methods: We analyzed national mortality data from the CDC WONDER database between 1999 and 2020. Deaths attributed to AMI and VAs were identified using ICD codes. Age-adjusted mortality rates (AAMRs) per 100,000 population and 95% confidence intervals (CIs) were calculated and stratified by sex, race/ethnicity, age group, and geographic region. Results: A total of 71,036 deaths were attributed to AMI and VAs over the study period. The AAMR declined sharply from 2.41 in 1999 to 1.08 in 2007 (APC: -9.91; 95% CI: -10.7 to -9.02). From 2007 to 2020, a gradual decline in mortality was observed (1.08 to 1.01) (APC: -0.83; 95% CI: -1.47 to -0.19). Men had consistently higher AAMR than women from 1999 (4.39 vs 1.88) to 2020 (1.84 v s0.68); moreover, since 2008, no significant decline in mortality was observed for males. Whites had the highest number of deaths (n=62,157; AAMR: 1.51 [95% CI: 1.50–1.52]), followed by Blacks or African Americans (1.43 [95% CI: 1.39–1.46]), American Indians (1.10 [95% CI: 0.98–1.22]), Hispanics (1.04 [95% CI: 1.00–1.07]), and Asian or Pacific Islanders (0.89 [95% CI: 0.85–0.94]). All races had a significant decline in mortality across years; however, Hispanics had a rise in mortality from 2011-2020 (APC: 3.06; 95% CI: 0.82 to 5.36). Mortality increased markedly with age: older adults (≥65 years) had a 49-fold higher AAMR (5.38 vs 0.11) compared to younger adults. There was also a significant state-wide difference in mortality as shown in Figure 1. Conclusion: AMI and VA-related mortality in the US from 1999–2020 remained substantial, with significant disparities by age, sex, race, and region. Older adults, males, and those residing in the South experienced the highest mortality burden. Hispanic had a increase in mortality since 2011. These findings underscore the continued need for targeted prevention, early recognition of electrical complications of AMI, improved post-AMI surveillance, and equitable access to life-saving interventions like ICDs.

Background: Patients with left ventricular assist devices (LVADs) experience high mortality and infection-related complications. While Glucagon-like peptide-1 receptor agonists (GLP-1 RAs) have shown cardiovascular benefits in various populations, their safety and efficacy in advanced heart failure patients on mechanical support remain understudied. Methods: We conducted a retrospective cohort study using the TriNetX Global Collaborative Network. Using ICD-10 and procedural codes, adults with a diagnosis of LVAD (ICD-10: Z95.811) and a history of LVAD-related procedures were identified. Only GLP-1 RAs with proven cardiovascular outcomes -liraglutide, dulaglutide, semaglutide, and tirzepatide- were included. Patients exposed to GLP-1 RAs after LVAD placement formed the GLP1 RA group. Those without GLP-1 RA exposure comprised the control group. Propensity score matching (1:1) was performed on demographics, comorbidities (diabetes, chronic kidney disease, obesity, ischemic heart disease, atrial fibrillation), and medications. Outcomes were assessed over a 5-year follow up period from the index date. The primary outcome was all-cause mortality. Secondary outcomes included hospitalization, device infections, endocarditis, and MRSA/MSSA bloodstream infections. Results: After matching , 543 patients were included in each cohort. The GLP1 group showed significantly lower mortality (17.3% vs. 33.9%, HR 0.40, 95% CI: 0.31–0.51, p&lt;0.001) and higher 5-year survival probability (70.3% vs. 45.6%). Hospitalization was also reduced (69.6% vs. 82.5%, HR 0.44, p&lt;0.001), with longer median time to first admission (241 vs. 34 days). Bloodstream infections were significantly lower in GLP-1 users (20.4% vs. 26.5%, HR 0.61, p=0.001). Device-specific infections was similar in both cohorts (HR 0.86, p=0.13). Endocarditis showed a favorable trend among the GLP-1 RA group but did not reach statistical significance (HR 0.52, p=0.050), possibly due to limited statistical power. Conclusion: In this large real-world analysis, GLP-1 RA use in patients with LVADs was associated with significantly lower long-term mortality, reduced hospitalizations, and fewer bloodstream infections. Although device-specific infections were unaffected, these findings provide observational evidence suggesting possible cardiovascular and infection-related benefits of GLP-1 RAs in advanced heart failure populations. Prospective studies are warranted to confirm safety and efficacy in this unique cohort.