TY - JOUR
T1 - Clinical course long after atrial switch
T2 - A novel risk score for major clinical events
AU - Woudstra, Odilia I.
AU - Zandstra, Tjitske E.
AU - Vogel, Rosanne F.
AU - van Dijk, Arie P.J.
AU - Vliegen, Hubert W.
AU - Kiès, Philippine
AU - Jongbloed, Monique R.M.
AU - Egorova, Anastasia D.
AU - Doevendans, Pieter A.F.M.
AU - Konings, Thelma C.
AU - Mulder, Barbara J.M.
AU - Tanck, Michael W.T.
AU - Meijboom, Folkert J.
AU - Bouma, Berto J.
N1 - Funding Information: This work was supported by the Dutch Heart Foundation (CVON project CONCOR-genes) and Amsterdam University Fund (8532). Publisher Copyright: © 2021 The Authors.
PY - 2021
Y1 - 2021
N2 - BACKGROUND: Patients with transposition of the great arteries corrected by an atrial switch operation experience major clinical events during adulthood, mainly heart failure (HF) and arrhythmias, but data on the emerging risks remain scarce. We assessed the risk for events during the clinical course in adulthood, and provided a novel risk score for event-free survival. METHODS AND RESULTS: This multicenter study observed 167 patients with transposition of the great arteries corrected by an atrial switch operation (61% Mustard procedure; age, 28 [interquartile range, 24–36] years) for 13 (interquartile range, 9–16) years, during which 16 (10%) patients died, 33 (20%) had HF events, defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and 15 (9%) had symptomatic ventricular arrhythmias. Five-year risk of mortality, first HF event, and first ventricular arrhythmia increased from 1% each at age 25 years, to 6% (95% CI, 4%–9%), 23% (95% CI, 17%–28%), and 5% (95% CI, 2%–8%), respectively, at age 50 years. Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques. A prediction model combining age >30 years, prior ventricular arrhythmia, age >1 year at repair, moderate or greater right ventricular dysfunction, severe tricuspid regurgitation, and mild or greater left ventricular dysfunction discriminated well between patients at low (<5%), intermediate (5%–20%), and high (>20%) 5-year risk (optimism-corrected C-statistic, 0.86 [95% CI, 0.82–0.90]). Observed 5-and 10-year event-free survival rates in low-risk patients were 100% and 97%, respectively, compared with only 31% and 8%, respectively, in high-risk patients. CONCLUSIONS: The clinical course of patients undergoing atrial switch increasingly consists of major clinical events, especially HF. A novel risk score stratifying patients as low, intermediate, and high risk for event-free survival provides information on absolute individual risks, which may support decisions for pharmacological and interventional management.
AB - BACKGROUND: Patients with transposition of the great arteries corrected by an atrial switch operation experience major clinical events during adulthood, mainly heart failure (HF) and arrhythmias, but data on the emerging risks remain scarce. We assessed the risk for events during the clinical course in adulthood, and provided a novel risk score for event-free survival. METHODS AND RESULTS: This multicenter study observed 167 patients with transposition of the great arteries corrected by an atrial switch operation (61% Mustard procedure; age, 28 [interquartile range, 24–36] years) for 13 (interquartile range, 9–16) years, during which 16 (10%) patients died, 33 (20%) had HF events, defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and 15 (9%) had symptomatic ventricular arrhythmias. Five-year risk of mortality, first HF event, and first ventricular arrhythmia increased from 1% each at age 25 years, to 6% (95% CI, 4%–9%), 23% (95% CI, 17%–28%), and 5% (95% CI, 2%–8%), respectively, at age 50 years. Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques. A prediction model combining age >30 years, prior ventricular arrhythmia, age >1 year at repair, moderate or greater right ventricular dysfunction, severe tricuspid regurgitation, and mild or greater left ventricular dysfunction discriminated well between patients at low (<5%), intermediate (5%–20%), and high (>20%) 5-year risk (optimism-corrected C-statistic, 0.86 [95% CI, 0.82–0.90]). Observed 5-and 10-year event-free survival rates in low-risk patients were 100% and 97%, respectively, compared with only 31% and 8%, respectively, in high-risk patients. CONCLUSIONS: The clinical course of patients undergoing atrial switch increasingly consists of major clinical events, especially HF. A novel risk score stratifying patients as low, intermediate, and high risk for event-free survival provides information on absolute individual risks, which may support decisions for pharmacological and interventional management.
KW - Adult
KW - Atrial switch
KW - Mustard repair
KW - Prediction model
KW - Senning repair
KW - Transposition of the great arteries
UR - http://www.scopus.com/inward/record.url?scp=85102537761&partnerID=8YFLogxK
U2 - https://doi.org/10.1161/JAHA.120.018565
DO - https://doi.org/10.1161/JAHA.120.018565
M3 - Article
C2 - 33615824
SN - 2047-9980
VL - 10
SP - 1
EP - 16
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
IS - 5
M1 - e018565
ER -