Clinical course long after atrial switch: a novel risk score for serious clinical events. (25th November 2020)

• Record Type: Journal Article
• Authors: Woudstra, O; Zandstra, T.E; Vogel, R.F; Van Dijk, A.P.J; Vliegen, H.W; Kies, P; Jongbloed, M.R.M; Egorova, A.D; Doevendans, P.A; Konings, T.C; Mulder, B.J.M; Tanck, M.W.T; Meijboom, F.J; Bouma, B.J
• Is Part Of: European heart journal. Volume 41:(2020)Supplement 2
• Publication Date: 2020-11-25
• Subjects: Congenital Heart Disease - Epidemiology, Prognosis, Outcome; Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/ehjci/ehaa946.2175
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: Background: Patients after atrial switch surgery for transposition of the great arteries (TGA-AtrSO) experience serious clinical events during adulthood, mainly heart failure and arrhythmias, but data on the emerging risks remain scarce. Purpose: To assess the risk for events during the clinical course in adulthood of TGA-AtrSO patients and provide a novel risk score for event-free survival. Methods: We reviewed medical records of TGA-AtrSO patients from five hospitals. Endpoints were all-cause mortality, heart failure (HF), defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and symptomatic ventricular arrhythmias (VA). Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques. Results: We followed 169 TGA-AtrSO patients (60% Mustard, age 28 [IQR 24–36] years) for 13 [IQR 9–16] years, during which 17 (10%) died, 34 (20%) had HF events, and 15 (9%) had VA events. Five-year risk of mortality, first HF event, and first VA increased from 1% each at age 25, to 7% (95% CI 4–10%), 17% (95% CI 10–25%), and 4% (95% CI 2–8%), respectively, at age 50. A prediction model combining age >30, prior VA, age >1 year at repair surgery, QRS duration >120ms, ≥mild LV dysfunction, and severe tricuspid regurgitation discriminated well between patients at low (<5%), medium (5–20%) and high (>20%) 5-year risk (optimism corrected C-statistic=0.84). Observed 5- and 10-year survival in low-risk patients were 100% and 99%, compared to only 45% and 19% in high-risk patients. Conclusion: The clinical course of atrial switch patients increasingly consists of serious clinical events, especially heart failure. A novel risk score stratifying patients as low, medium, and high risk for event-free survival is presented, providing information on absolute individual risks which may support decisions for pharmacological and interventional management. Funding Acknowledgement: Type of funding source: Foundation. Main funding source(s): Dutch Heart Foundation; Amsterdam University Fund