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

• Published in: European journal of preventive cardiology Vol. 30; no. 13; pp. 1335 - 1342
• Main Authors: Krishnathasan, Kaushiga, Dimopoulos, Konstantinos, Duncan, Neill, Ricci, Piera, Kempny, Alexander, Rafiq, Isma, Gatzoulis, Michael A, Heng, Ee Ling, Blakey, Sarah, Montanaro, Claudia, Babu-Narayan, Sonya V, Francis, Darrel P, Li, Wei, Constantine, Andrew
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 20-09-2023
• Subjects: Adult; Aftercare; Female; Heart Defects, Congenital - complications; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - therapy; Heart Failure - diagnosis; Heart Failure - etiology; Heart Failure - therapy; Humans; Male; Middle Aged; Patient Discharge; Renal Insufficiency, Chronic - complications; Renal Insufficiency, Chronic - diagnosis; Renal Insufficiency, Chronic - therapy; Retrospective Studies; Heart failure; Chronic kidney disease; Bayesian analysis; Adult congenital heart disease; Renal failure
• ISSN: 2047-4873; 2047-4881
• DOI: 10.1093/eurjpc/zwad094

Abstract
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.
Lay Summary
Renal dysfunction was associated with more advanced disease, higher diuretic doses, and a longer hospital inpatient stay.
Chronic kidney disease was common and tripled the risk of death, transplantation, or ventricular assist device.
Renal dysfunction in adults with congenital heart disease and heart failure should prompt intensified monitoring, optimization of medical therapy, and collaborative management with renal physicians.