Follow-up started from CMR study and was continued until the first MACE or to surgical repair (patients were censored at surgery n=31 39%) or to the last clinical visit for the remainder of patients. Heart failure admission was defined as admission for diuresis of fluid overload not secondary to acute arrhythmia presentation. Ventricular tachyarrhythmia was defined as ventricular tachycardia (VT) associated with presyncope/syncope, sustained VT (≥30 s) or ventricular fibrillation. The prespecified clinical endpoint of major adverse cardiovascular events (MACEs) consisted of new-onset clinically documented sustained ventricular tachyarrhythmia/heart failure hospital admission/transplantation or death. Patients with permanent pacemaker/automated implantable defibrillator (n=10/2) were not included in this study due to relative contraindication to CMR. EA was defined as apical displacement of the septal leaflet of the tricuspid valve by at least 8 mm/m 2 body surface area in relation to the insertion of the anterior mitral valve leaflet. Seven patients were lost to follow-up, thus the final study cohort consisted of 79 patients including 4 patients with prior atrial septal defect closure (surgical n=2, catheter n=2). We aimed to study the prognostic significance of CMR for significant adverse cardiac events in a large, prospective, single-centre and contemporary cohort of adult patients with unrepaired EA.Įighty-six consecutive patients with unrepaired EA underwent protocolised CMR and were prospectively followed for events from November 2002 until July 2014. 10–13 Recent studies correlated CMR-derived measures in EA with known heart failure markers and/or exercise capacity, 14 15 but its value to guide prognosis is not reported. 8 9 Cardiovascular magnetic resonance (CMR) is used to image adults with Ebstein’s anomaly due to unrestricted views of heart structures and its place as the gold standard for quantification of left ventricular (LV) and right ventricular (RV) volumes and function without geometrical assumption. 1–7 The onset of atrial tachyarrhythmia (AT) in adults is associated with significant morbidity. Several predictors of adverse outcomes have been reported such as age at presentation, anatomic severity, grade of tricuspid regurgitation, cyanosis, male gender, increased cardiothoracic ratio (CTR), prolonged/fragmented QRS, reduced exercise capacity and New York Heart Association (NYHA) functional class have been reported. Mortality in Ebstein’s anomaly (EA) of the tricuspid valve relates to ventricular tachyarrhythmia, congestive heart failure and sudden cardiac death. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).ĬMR variables associated with first-onset AT (n=17 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041) the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007). Results CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037) all remained significant when tested solely for mortality. 6 Città della Salute e della Scienza Hospital, University of Turin, Turin, Italyĭr Sonya V Babu-Narayan, NIHR Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, England S.5 Department of Cardiovascular Medicine, Division of Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.4 National Heart and Lung Institute, Imperial College, London, England.Luke’s International Hospital, Tokyo, Japan 2 Department of Molecular Medicine and Surgery, Section of Clinical Physiology, Karolinska Institutet, Stockholm, Sweden.1 NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, England.
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