Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation |
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| Affiliation: | 1. Division of Cardiology, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada;2. Paediatric Cardiology, McMaster University Medical Centre, Hamilton, Ontario, Canada;1. Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, New York, New York, USA;2. Humanitas Research Hospital IRCCS, Rozzano, Milan, Italy;3. Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York, USA;4. Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy;5. Albany Medical College, Albany, New York, USA;6. Department of Cardiology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA;1. Division of Cardiac Surgery, University of Ottawa Heart Institute, Ottawa, Ontario, Canada;2. Division of Cardiology, Department of Medicine, Memorial University, St. John''s, Newfoundland, Canada;3. Division of Cardiovascular Surgery, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada;4. Division of Cardiac Anesthesiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada;5. Department of Surgery, New Brunswick Heart Center, Saint John, New Brunswick, Canada;6. Faculté de pharmacie, Université Laval, Institut universitaire de cardiologie et de pneumologie de Québec, Québec City, Québec, Canada;7. Département de Chirurgie, Université Laval, Institut universitaire de cardiologie et de pneumologie de Québec, Québec City, Québec, Canada;8. Peter Munk Cardiac Centre and Heart and Stroke Richard Lewar Centre, University of Toronto, Toronto, Ontario, Canada;9. Division of Cardiac Surgery, QEII Health Sciences Centre, Halifax, Nova Scotia, Canada;10. Department of Cardiovascular Surgery, Maine Medical Center, Portland, Maine, USA;11. Division of Cardiac Surgery, St. Mary’s General Hospital, Kitchener, Ontario, Canada;12. Keenan Research Centre for Biomedical Science, UnityHealth, University of Toronto, Toronto, Ontario, Canada;13. Department of Cardiac Sciences, Section of Cardiac Surgery, Libin Cardiovascular Institute, University of Calgary, Calgary, Alberta, Canada;14. Department of Medicine, New Brunswick Heart Center, Saint John, New Brunswick, Canada;15. Department of Surgery, McMaster University, Hamilton, Ontario, Canada;p. Department of Medicine and Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, Alberta, Canada;q. Department of Surgery, Section of Cardiac Surgery, Max Rady College of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada;1. Division of Cardiac Surgery, Nova Scotia Health Authority, Dalhousie University, Halifax, Nova Scotia, Canada;2. School of Health Administration, Dalhousie University, Halifax, Nova Scotia, Canada;3. Critical Care Medicine, University of British Columbia, Vancouver, British Columbia, Canada;4. Division of Cardiology, Nova Scotia Health Authority, Dalhousie University, Halifax, Nova Scotia, Canada;5. Department of Cardiovascular Surgery, Maine Medical Center, Portland, Maine, USA;1. Division of Cardiac Surgery, St Michael''s Hospital, University of Toronto, Toronto, Ontario, Canada;2. Department of Otolaryngology-Head & Neck Surgery, Health Sciences Centre, University of Manitoba, Manitoba, Winnipeg, Canada;3. Division of Cardiac Surgery, Royal Jubilee Hospital, Victoria, British Columbia, Canada;4. Division of Cardiac Surgery, New Brunswick Heart Centre, Saint John, New Brunswick, Canada;5. Division of Cardiac Surgery, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada;6. Division of Cardiovascular Surgery, St. Mary’s General Hospital, Kitchener, Ontario, Canada;7. Department of Surgery, Section of Cardiac Surgery, Max Rady College of Medicine, University of Manitoba, Manitoba, Winnipeg, Canada;1. Centre for Outcomes Research and Evaluation, McGill University Health Centre Research Institute, Montréal, Québec, Canada;2. Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada;3. Department of Translational Medicine, University of Ferrara, Ferrara, Italy;4. University Centre for Studies on Gender Medicine University of Ferrara, Ferrara, Italy;5. Faculty of Medicine, University of Alberta, Edmonton, Alberta, Canada;6. Cardiovascular and Stroke Strategic Clinical Network, Alberta Health Services, Edmonton, Alberta, Canada;7. Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada;8. Department of Medicine, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada |
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| Abstract: | BackgroundLeft ventricular noncompaction (LVNC) is associated with genetic and phenotypic variability that influences outcomes. We aimed to identify risk factors for death or heart transplantation (HTx) in a paediatric LVNC cohort.MethodsWe reviewed patients < 18 years of age (2001-2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC), and at least mildly reduced left ventricular ejection fraction (EF). Patients with dilated cardiomyopathy (DCM) were included as control subjects. Descriptive statistics, multivariate analysis, and time-to-event analysis were used.ResultsWe included 188 patients, 34 (18%) with I-LVNC, 37 (20%) with D-LVNC, and 117 (62%) with DCM. Overall median age at diagnosis was 1.08 years (interquartile range [IQR] 0.22-10.65) and median follow-up was 1.4 years (IQR 0.2-5.2) years. I-LVNC patients’ median baseline LVEF was 47%, compared with 33% for D-LVNC, and 21% for DCM (P < 0.0001); 62% of I-LVNC patients developed moderate to severe LV dysfunction during follow-up. The incidence of death or transplantation was 43.6% in the overall cohort. Freedom from death or HTx at 10 years after diagnosis was 88.6% (95% CI 76%-100%) for I-LVNC, 47% (95% CI 29%-65%) for D-LVNC, and 42.3% (95% CI 33%-52%) for DCM. On multivariable analysis, baseline LVEF and LV end-diastolic diameter (LVEDD) z-score were associated with death or transplantation. Patients with a baseline LVEDD z-score > 4 and moderate to severe LV dysfunction had a transplantation-free survival of 38%.ConclusionsBaseline LV dilation and systolic dysfunction were independently associated with progression to death or HTx in LVNC patients. |
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