Publications by authors named "Selina Hein"

Article Synopsis
  • * The study involved 308 wildtype and 31 hereditary ATTR-CM patients, revealing significant health metrics deterioration in most, but only 9% showed disease progression based on ESC criteria.
  • * Conclusions indicate that while most patients did not experience significant disease progression within a year, treatment response did not correlate with improved survival, and higher NT-proBNP levels at diagnosis predicted poorer outcomes.
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Article Synopsis
  • Cardiac transthyretin amyloidosis (ATTR) is a deadly heart disease caused by amyloid fibril buildup, with two forms: hereditary (ATTRv) and wild-type (ATTRwt).
  • Current guidelines suggest using ACE inhibitors and beta-blockers for heart failure treatment in symptomatic patients, regardless of the cause, but their effectiveness specifically for ATTR remains unclear.
  • A study of 403 patients found that while heart failure therapy improved survival for those with ATTRwt and certain comorbidities, survival was significantly shorter for those with ATTRv undergoing the same treatment.
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Background: COVID-19 is associated with a prothrombotic state. Current guidelines recommend prophylactic anticoagulation upon hospitalization.

Methods: COVID-PREVENT, an open-label, multicenter, randomized, clinical trial enrolled patients (≥ 18 years) with moderate to severe COVID-19 and age-adjusted D-dimers > 1.

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Purpose: Low plasma concentrations of the amino acid homoarginine (HA) have been shown to correlate with adverse cardiovascular outcome, particularly in patients with chronic kidney disease. The present study sought to investigate the effect of HA treatment on cardiac remodeling in rats undergoing artificially induced renal insufficiency by 5/6 nephrectomy (5/6 Nx).

Methods: A total of 33 male Wistar rats were randomly divided into sham and 5/6 Nx groups, receiving either placebo treatment or 400 mg·kg·day HA over a 4-week period.

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Spontaneous pneumothorax is a potentially life-threatening situation. Therefore, it is mandatory to treat it safely. The incidence is approximately 10 out of 100 000 residents per year.

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Article Synopsis
  • The COVID-19 pandemic led to a shift from traditional in-person medical education to e-learning, posing challenges for student motivation and engagement.
  • A team of medical students and physicians created a series of interactive e-learning modules, simulating real patient scenarios within a 10-week curriculum to maintain relevance in clinical education.
  • Student feedback indicated that the case-based, engaging format improved their motivation and allowed them to apply clinical knowledge effectively, suggesting this method can enhance learning even in socially isolated conditions.
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  • Elevated interleukin-6 (IL-6) levels are linked to worse outcomes in patients with cardiac ATTR amyloidosis (ATTR-CM) and are more pronounced in those with more severe clinical presentations.
  • The study involved measuring IL-6 levels in 106 ATTR-CM patients, along with asymptomatic carriers and healthy controls, finding significant differences in levels particularly between wild-type patients and healthy controls.
  • While high IL-6 levels are associated with poor outcomes, it did not enhance risk prediction beyond existing established prognostic factors.
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Article Synopsis
  • The study investigates how toxic effects from transthyretin amyloid in patient plasma affect heart cells (cardiomyocytes) and their growth response, particularly when stimulated with phenylephrine.
  • A novel measure called the Hypertrophic Index was introduced to assess changes in cell size after treatment, revealing that plasma from certain patient groups resulted in reduced growth response compared to healthy controls.
  • The findings showed that this reduced growth response was a significant independent risk factor for various clinical outcomes, such as heart failure and death, even when accounting for other established risk factors.
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Background: Hereditary transthyretin amyloidosis is caused by pathogenic variants in the gene and typically manifests, alongside cardiac and other organ dysfunctions, with a rapidly progressive sensorimotor and autonomic polyneuropathy (ATTRv-PN) leading to severe disability. While most prospective studies have focussed on endemic ATTRv-PN, real-world data on non-endemic, mostly late-onset ATTRv-PN are limited.

Methods: This retrospective study investigated ATTRv-PN patients treated at the Amyloidosis Centre of Heidelberg University Hospital between November 1999 and July 2020.

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Cardiovascular diseases are a major cause of morbidity and mortality, and there are significant sex differences therein. However, the underlying mechanisms are poorly understood. The steroid hormone 17β-estradiol (E2) is thought to play a major role in cardiovascular sex differences and to be protective, but this may not hold true for males.

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Background: Carpal tunnel syndrome (CTS) and spinal canal stenosis can be frequently observed in the medical history of patients with transthyretin amyloidosis (ATTR), both in the hereditary (mt-ATTR) and wild-type (wt-ATTR) form. The aim of this retrospective single-center analysis was to determine the prevalence of these findings, delay to diagnosis of systemic amyloidosis and the prognostic value in a large cohort of patients with wt-ATTR and mt-ATTR amyloidosis.

Methods: Medical records of 253 patients diagnosed with wt-ATTR, 136 patients with mt-ATTR and 77 asymptomatic gene carriers were screened for history of CTS and spinal canal stenosis and laboratory analysis, electrocardiography and echocardiographic results, respectively.

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Introduction: Cardiopulmonary exercise testing (CPET) has repeatedly been reported to reliably predict adverse outcomes in different forms of heart failure. However, it has not been elucidated in detail in cardiac amyloidosis (CA). Therefore, we evaluated the predictive value of CPET parameters in patients with CA regarding disease severity and prediction of mortality.

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Zebrafish is a widely used model to evaluate genetic variants and modifiers that can cause heart muscle diseases. Surprisingly, the β-adrenergic receptor (β-AR) pathway in zebrafish is not well characterized, although abnormal β-AR signaling is a major contributor to human heart failure (HF). Chronic β-AR activation in the attempt to normalize heart function in the failing heart results in a reduction of the β-ARs expression and receptor desensitization, largely mediated through G-protein coupled receptor kinase 2 (GRK2) upregulation.

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Background: Cardiac amyloid load has not been analyzed for its effect on mortality in patients with amyloid light-chain (AL) cardiac amyloidosis.

Objectives: This study retrospectively compared histological amyloid load with common clinical predictors of mortality.

Methods: This study assessed 216 patients with histologically confirmed cardiac amyloidosis at a single center with electrocardiography, echocardiography, and laboratory testing.

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Aims: Regulatory proteins of the sarcomere are pivotal for normal heart function and when affected by mutations are frequently causing cardiomyopathy. The exact function of these regulatory proteins and how mutations in these translate into distinct cardiomyopathy phenotypes remains poorly understood. Mutations in the essential myosin light chain (ELC) are linked to human cardiomyopathy characterized by a marked variability in disease phenotypes and high incidences of sudden death.

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Translucent zebrafish larvae represent an established model to analyze genetics of cardiac development and human cardiac disease. More recently adult zebrafish are utilized to evaluate mechanisms of cardiac regeneration and by benefiting from recent genome editing technologies, including TALEN and CRISPR, adult zebrafish are emerging as a valuable in vivo model to evaluate novel disease genes and specifically validate disease causing mutations and their underlying pathomechanisms. However, methods to sensitively and non-invasively assess cardiac morphology and performance in adult zebrafish are still limited.

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Progressive cardiomyopathy is a major cause of death in Duchenne muscular dystrophy (DMD) patients. Coupling between Ca(2+) handling and contractile properties in dystrophic hearts is poorly understood. It is also not clear whether developing cardiac failure is dominated by alterations in Ca(2+) pathways or more related to the contractile apparatus.

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