Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes

Aims Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021. Methods and results This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4–12) days, with a maximum dose of 120 (80–160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2–82.3%] and 43.3% (95% CI: 36–52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome. Conclusion Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes

Isolated subsegmental pulmonary embolism identification based on international classification of diseases (ICD)-10 codes and imaging reports

BackgroundIsolated subsegmental pulmonary embolism (issPE) is a commonly encountered diagnosis. Although the International Classification of Diseases (ICD)-10 codes are used for research, their validity for identifying issPE is unknown. Moreover, issPE diagnosis is challenging, and the findings from radiology reports may conflict with those from expert radiologists.MethodsBased on prespecified criteria, 1734 medical records of adult patients hospitalized within the Mass General Brigham health system (2016–2021) were selected in three equal groups: (1) patients with principal discharge diagnosis codes for PE, (2) patients with secondary discharge diagnosis codes for PE, and (3) patients with no PE codes. The accuracy of ICD-10 codes for issPE was verified by two independent physicians and weighted by total hospitalizations. In a randomly selected sample of 70 patients, the accuracy of initial radiology reports was determined through a blinded re-evaluation by two expert radiologists.ResultsIn weighted estimates, ICD-10 codes in primary or secondary discharge positions, compared with chart reviews, showed a low sensitivity (7.0 %) and positive predictive value (25.2 %). Evaluation by two expert radiologists noted that initial radiology reports were sensitive (97.1 %) for issPE but had a low specificity (40.0 %). Two (3.6 %) out of 55 patients with initial issPE reports did not have PE, while 19 (34.5 %) had more proximal PE.ConclusionsICD-10 codes for issPE have poor sensitivity and positive predictive value and should not be used for research or quality improvement. Radiology reports for issPE may be inaccurate regarding the location or, less often, the presence of PE.Thrombosis and HemostasisThrombosis and Hemostasi

Major Cardiovascular Events After COVID-19, Event Rates Post-vaccination, Antiviral or Anti-inflammatory Therapy, and Temporal Trends: Rationale and Methodology of the Corona-VTE-Network Study

BACKGROUND: Coronavirus disease 2019 (COVID-19) is associated with excess risk of cardiovascular and thrombotic events in the early post-infection period and during convalescence. Despite the progress in our understanding of cardiovascular complications, uncertainty persists with respect to more recent event rates, temporal trends, association between vaccination status and outcomes, and findings within vulnerable subgroups such as older adults (aged 65 years or older), or those undergoing hemodialysis. Sex-informed findings, including results among pregnant and breastfeeding women, as well as adjusted comparisons between male and female adults are similarly understudied. METHODS: Adult patients, aged ≥18 years, with polymerase chain reaction-confirmed COVID-19 who received inpatient or outpatient care at the participating centers of the registry are eligible for inclusion. A total of 10,000 patients have been included in this multicenter study, with Brigham and Women\u27s Hospital (Boston, MA) serving as the coordinating center. Other sites include Beth Israel Deaconess Medical Center, Anne Arundel Medical Center, University of Virginia Medical Center, University of Colorado Health System, and Thomas Jefferson University Health System. Data elements will be ascertained manually for accuracy. The two main outcomes are 1) a composite of venous or arterial thrombotic events, and 2) a composite of major cardiovascular events, defined as venous or arterial thrombosis, myocarditis or heart failure with inpatient treatment, new atrial fibrillation/flutter, or cardiovascular death. Clinical outcomes are adjudicated by independent physicians. Vaccination status and time of inclusion in the study will be ascertained for subgroup-specific analyses. Outcomes are pre-specified to be reported separately for hospitalized patients versus those who were initially receiving outpatient care. Outcomes will be reported at 30-day and 90-day follow-up. Data cleaning at the sites and the data coordinating center and outcomes adjudication process are in-progress. CONCLUSIONS: The CORONA-VTE-Network study will share contemporary information related to rates of cardiovascular and thrombotic events in patients with COVID-19 overall, as well as within key subgroups, including by time of inclusion, vaccination status, patients undergoing hemodialysis, the elderly, and sex-informed analyses such as comparison of women and men, or among pregnant and breastfeeding women

A Systematic Review of Passing as Non-Autistic in Autism Spectrum Disorder

While long described in anecdotal accounts of the lived experiences of autistic individuals, the phenomenon of behaving in ways that appear inconsistent with the presence of autism (or passing as non-autistic; PAN) has recently seen a dramatic increase in scrutiny in the published scientific literature. Increased research attention has coincided with a proliferation of methods, definitions, measures, and population assumptions associated with PAN. To date, however, no review has sought to systematically identify and synthesize the literature on PAN. This systematic review reflects the state of the PAN literature as of May 2020. Ninety articles were screened, 66 were identified for evaluation, and 46 met inclusion criteria and were reliably coded for study characteristics and participant characteristics. Results reveal that the PAN literature includes a relatively even mix of qualitative, quantitative, and mixed-method studies, and that a variety of terms are used for PAN (with masking and camouflage being the most frequent). Sample sizes varied widely (from one to 832 participants), with 63.06% of participants being categorized as autistic. Nearly all studies reported methods for confirming autism diagnoses, with community and clinical diagnoses being most common. The majority of studies reported participant gender, with slightly more females included than males on average, but fewer than half of all studies compared PAN across genders. Nearly all studies reported participant age, demonstrating a wide range of 2 to 79 years, with a mean age of 23.85. Conversely, only 23.91% of studies provided participant race or ethnicity data. Nearly all studies formally or informally excluded participants with intellectual disability. Finally, measures of internalizing symptoms, which are often thought to be linked to PAN, were reported in only 17.4% of studies. Implications for gaps in understanding of PAN and future directions for the field are discussed

International clinical practice guideline recommendations for acute pulmonary embolism: harmony, dissonance, and silence

Despite abundant clinical innovation and burgeoning scientific investigation, pulmonary embolism (PE) has continued to pose a diagnostic and management challenge worldwide. Aging populations, patients living with a mounting number of chronic medical conditions, particularly cancer, and increasingly prevalent health care disparities herald a growing burden of PE. In the meantime, navigating expanding strategies for immediate and long-term anticoagulation, as well as advanced therapies, including catheter-based interventions for patients with more severe PE, has become progressively daunting. Accordingly, clinicians frequently turn to evidence-based clinical practice guidelines for diagnostic and management recommendations. However, numerous international guidelines, heterogeneity in recommendations, as well as areas of uncertainty or omission may leave the readers and clinicians without a clear management pathway. In this review of international PE guidelines, we highlight key areas of consistency, difference, and lack of recommendations (silence) with an emphasis on critical clinical and research needs. Thrombosis and Hemostasi

Transition for patients with congenital heart disease in the UK: need for a universal model with adequate training and support

Abstract Background Adolescence is a vulnerable period for patients with congenital heart disease (CHD). Transition is a process that guides these patients through adolescence and ensures a smooth transfer to adult services, in order improve adherence to medical care and reduce loss to follow-up. While the importance of a formal Transition process is widely recognised and a requirement for specialist services in the UK, the optimal structure and delivery of Transition remains a matter of debate. Aims To examine the different models of Transition currently in place in specialist CHD centres around the UK. Methods A survey of Adult CHD centres in the UK was performed. A focus was placed on the structure of the Transition service, relevant training and areas of perceived improvement. Results There were 10 responses to our survey covering 10 specialist CHD centres. All respondents were consultant adult CHD specialists, looking after patients from the age of 16 [14–17] years. All centres have a specialised Transition service, which runs from the age 13 [11–15] to 18 [16–25] years (duration of transition 5 [2–13] years). The majority of centres (80%) report providing transition care “well before” transfer to adult care, whereas 20% provide transition care at or immediately before transfer (i.e. first adult CHD appointment). Transition is delivered by physicians and clinical specialist nurses in approximately equal numbers in 9 (90%) centres and exclusively by clinical nurse specialists in 1 (10%) centre. A median of 2 [1–5] visits are planned for each patient, with 7 (70%) centres seeing patients at least twice during transition. The majority, but not all centres (70%) provide a health passport during transition. A significant number of centres felt they werer not receiving sufficient support in the following domains: financial (50%), training (30%), clinical space (30%), referrals from paediatrics (50%). All respondents felt that their Transition service had room for improvement. Other areas of improvement highlighted included reduction in loss to follow-up, difficulties in providing a Transition service to patients followed in peripheral hospitals, the need for more support from paediatric services in referring all appropriate patients, and dedicated administrative support. The vast majority of respondents (9, 90%) felt equipped with the appropriate skills to care for transition patients. However, few (2, 20%) had completed formal training in more than one area related to adolescent health and transition. Conclusions While all CHD centres have a Transition service, Transition models and delivery differs significantly. There is urgent need for research in this area to develop a unified model, greater financial support and relevant training to optimise care. Figure 1. Participating UK centres Funding Acknowledgement Type of funding source: None </jats:sec

Microvascular Dysfunction in Dilated Cardiomyopathy A Quantitative Stress Perfusion Cardiovascular Magnetic Resonance Study

This work was supported by the National Institute for Health Research Cardiovascular Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, England. Drs. Arai and Hsu were funded by the Intramural Research Program of the National Heart, Lung, and Blood Institute, and National Institutes of Health (HL 006137–07). Dr. Gulati has received grant support from the National Institute for Health Research, CORDA, and Rosetrees Trust. Dr. Gatehouse has a research agreement with Siemens. Dr. Firmin has a research agreement with Siemens. Prof. Mathur received grant support from the Heart Cells Foundation and Barts and the London Charity. Prof. Pennell has received grant support from the National Institute for Health Research and the British Heart Foundation; has received research support from Siemens; served as a consultant to Siemens, Novartis, ApoPharma, AMAG, and Bayer; and served as the director and owns stock in Cardiovascular Imaging Solutions. Dr. Arai has a research agreement with Siemens and a clinical trial agreement with Bayer. Dr. Prasad has received grant support from the British Heart Foundation, CORDA, Rosetrees Trust, the Alexander Jansons Foundation, and the National Institute for Health Research; and has received speaking fees from Bayer Schering