Background: There is growing interest in the observed significant incidence of transthyretin cardiac amyloidosis in elderly patients with aortic stenosis. Approximately 16% of patients with severe aortic stenosis undergoing aortic valve replacement have transthyretin cardiac amyloidosis. Outcomes after aortic valve replacement appear worse in patients with concomitant transthyretin cardiac amyloidosis. Method: Publications in PubMed, Cochrane Library, and Embase databases were systematically searched from January 2012 to September 2018 using the keywords transthyretin, amyloidosis, and aortic stenosis. All studies published in English that reported the prevalence, association and outcomes of transthyretin cardiac amyloidosis in patients with aortic stenosis undergoing were included. Results/Conclusion: The relationship between aortic stenosis and transthyretin cardiac amyloidosis is not well understood. A few studies have proven successful surgical management when both conditions coexist. This systematic review suggests that transthyretin cardiac amyloidosis is common in elderly patients with aortic stenosis and tend to have high mortality rates after AVR. The significant incidence of the two diseases occurring simultaneously warrants further investigation to improve management strategies in the future.
Adrenal-renal fusion is a rare entity wherein the capsule of the adrenal gland is fused to the kidney. Here, we report a case of adrenal-renal fusion making intraoperative dissection challenging. We also report on four other cases of adrenal-renal fusion at our institution and a review of the literature. Although rare, radiologists and surgeons must be aware of this condition and consider it as a possibility, especially when dealing with upper pole renal lesions in order to avoid misdiagnosis and unnecessary resections.
Objectives: To determine the association of right heart invasive hemodynamic parameters with post-percutaneous coronary intervention (PCI) acute kidney injury (AKI). Background: AKI after PCI is associated with a high morbidity and mortality. Various mechanisms are implicated in AKI after PCI. However, the association between filling pressures and invasive hemodynamic measures of right heart function with post-PCI AKI has not been described. Methods: This is a retrospective single-center analysis of patients of who underwent simultaneous right heart catheterization (RHC) and left heart catheterization with PCI at the Einstein Medical Center, Philadelphia, between January 2010 and December 2016. We included patients who had hemodynamic parameters from the concomitant RHC as well as measurements of kidney function up to 1 month after the procedure. We excluded patients with ST elevation myocardial infarction, end-stage renal disease, cardiogenic shock, and PCI with a need for mechanical circulatory device support. Multivariate linear regression analysis was used to analyze the association between the various right ventricular hemodynamic parameters and eGFR within 1 week and 1 month after catheterization after adjusting for age, race, gender, diabetes and hypertension, contrast volume, cardiac index, and baseline eGFR. Results: Right atrial (RA) pressure was inversely associated with eGFR within 1 week (β = –1.66; 95% CI –3.06 to –0.25; p = 0.021) and 1 month after PCI (β = –2.14; 95% CI –4.08 to –0.20; p = 0.031). Conclusion: Elevated RA pressure is associated with a worsening kidney function after cardiac catheterization and PCI.
The incidence of Infective Endocarditis (IE) is higher in dialysis patients compared to the general population. A major risk factor for IE in this group stems from bacterial invasion during repeated vascular access. Previous studies have shown increased risk of bacteremia in patients with indwelling dialysis catheters compared to permanent vascular access. However, association between the development of IE and the type of dialysis access is unclear. We aimed to examine the associated types of intravascular access and route of infection in dialysis patients who were admitted with infective endocarditis at our center. All patients admitted to Albert Einstein Medical Center in Philadelphia with a diagnosis of infective endocarditis who were on chronic hemodialysis were identified from the hospital database for the period of 1/1/07 to 12/31/18. Modified Duke criteria was used to confirm the diagnosis of infective endocarditis. A total of 96 cases were identified. Of those, 57 patients had an indwelling dialysis catheter while the other 39 had permanent dialysis access. In 82% of patients with dialysis catheters, their dialysis access site was identified as the primary source of infection compared to 30% in those with permanent dialysis access (p<0.001). The number of dialysis catheters placed in the preceding 6 months was strongly associated with endocarditis resulting from the dialysis access site (OR = 3.202, p=0.025). Dialysis catheters are more likely to serve as the source of infection in dialysis patients developing IE compared to permanent dialysis access. Increased awareness of risk of IE associated with dialysis catheters is warranted.
Background: Atrial flutter is an infrequent yet potentially fatal arrhythmia. Digoxin is the preferred first-line treatment for fetal atrial flutter due to its efficacy and favorable safety profile. The optimal digoxin serum target level for neonatal atrial flutter management remains uncertain, with the standard target level ranging from 1.0 to 2.0 ng/mL due to potential toxicity concerns above this threshold. Case Presentation: We present a case of atrial flutter in a fetus within a monochorionic diamniotic (MCDA) twin pregnancy that was successfully managed using a higher-than-standard target level of digoxin. A 34-year-old nulliparous woman was referred to our institution at 31 + 3 weeks of gestation due to fetal distress in an MCDA twin pregnancy. Fetal echocardiography revealed a ventricular rate of 214 bpm in twin A, while twin B exhibited no abnormal findings. Conclusions: Our case highlights a distinct correlation between the serum digoxin level and its impact on atrial flutter. A higher target serum level of digoxin may be necessary to achieve sinus conversion due to the unique maternal and fetal circulatory characteristics in MCDA pregnancies.
Background: Peripheral artery disease (PAD) is prevalent in patients with chronic heart disease, and portends a worse cardiovascular outcome. Edinburgh Claudication Questionnaire (ECQ) is a commonly used screening tool aimed to identify PAD in general practice. Despite its common use, its accuracy had not been validated in a cardiac population in the USA. We aimed to evaluate the diagnostic accuracy of the ECQ as a means of identifying PAD in an outpatient cardiology clinic. Method: Subjects were recruited at outpatient cardiology clinic at Loma Linda University Medical Center between 2017 and 2019. Patients with previously diagnosed PAD were excluded. 119 patients completed ECQ during their routine clinic visit, and were subsequently referred for duplex ultrasound and Ankle-Brachial Index (ABI) measurement. ABI was used as a reference standard for the diagnosis of PAD. Result: rom a total of 119 patients, 53% were male with mean age was 68. Majority (87%) of patients had hypertension, and 16% were active smokers. There were 16% of patients who tested positive on the ECQ and 47% of them had positive ABI. In patients with a negative ECQ, 23% had a positive ABI result. Receiver operating characteristic curve analysis showed ECQ had sensitivity of 28% (95% CI 13.7-46.7), specificity of 89% (95% CI 79.8-94.3), PPV of 2.5% (95% CI 1.1-5.5) and NPV of 77.8% (0.7 to 1.0), which equates to a positive and negative likelihood ratio of 2.6 and 0.8 respectively. On subgroup analyses, the ECQ performed better among current smokers (AUC=0.64 95% CI 0.43 to 0.84) and among male patients (AUC=0.66 95% CI 0.53 to 0.78). In the subgroup of male patients who were currently smoking, the ECQ performed better with 50% sensitivity and 99% specificity (AUC=0.75 95% CI 0.56 to 0.94). Conclusion: The ECQ had good specificity but poor sensitivity in the cardiology outpatient setting making it a poor tool for screening on its own. Sensitivity was higher in male current smokers. Further study of its utilization in high-risk patients such as in male smokers may be of benefit.
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