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

Sex‐ and age‐based differences in the natural history and outcome of dilated cardiomyopathy

Aim: To evaluate the relationship between sex, age and outcome in dilated cardiomyopathy (DCM). Methods and results: We used proportional hazard modelling to examine the association between sex, age and all‐cause mortality in consecutive patients with DCM. Overall, 881 patients (290 women, median age 52 years) were followed for a median of 4.9 years. Women were more likely to present with heart failure (64.0% vs. 54.5%; P = 0.007) and had more severe symptoms (P 60 years of age was driven by non‐sudden death. Conclusion: Women with DCM have better survival compared to men, which may partly be due to less severe left ventricular dysfunction and a smaller scar burden. There is increased mortality driven by non‐sudden death in patients >60 years of age that is less marked in women. Outcomes with contemporary treatment were favourable, with a low incidence of sudden death

Multifaceted Fontan patients and their response to pregnancy

We present four patients with Fontan circulation who underwent successful pregnancies, albeit with complications that required close monitoring and timely intervention. Each Fontan patient presents with a unique clinical picture making risk stratification challenging but all the more important

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