Alexander Van De Bruaene1, Edward J Hickey2, Adrienne H Kovacs3, Andrew M Crean4, Rachel M Wald3, Candice K Silversides3, Andrew N Redington5, Heather J Ross3, Ana Carolina Alba3, Filio Billia3, Krishnakumar Nair3, Lee Benson2, Eric Horlick3, Mark Osten3, Jack Colman3, Jane Heggie6, Erwin N Oechslin2, S Lucy Roche7. 1. Peter Munk Cardiac Centre, University Health Network, Toronto, Canada; Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium. 2. Peter Munk Cardiac Centre, University Health Network, Toronto, Canada; University of Toronto, Canada; The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Canada. 3. Peter Munk Cardiac Centre, University Health Network, Toronto, Canada; University of Toronto, Canada. 4. Peter Munk Cardiac Centre, University Health Network, Toronto, Canada; University of Toronto, Canada; The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Canada; Joint Department of Medical Imaging, University Health Network, Toronto, Canada. 5. Heart Institute, Cincinnati Children's Hospital, Cincinnati, United States. 6. University of Toronto, Canada; Department of Anaesthesia, University Health Network Toronto, Canada. 7. Peter Munk Cardiac Centre, University Health Network, Toronto, Canada; University of Toronto, Canada; The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Canada. Electronic address: lucy.roche@uhn.ca.
Abstract
OBJECTIVE: Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. METHODS: Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. RESULTS: Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8-2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup (p=0.001), NYHA class (p=0.002) B-type natriuretic peptide >164pg/ml (p=0.003) and sodium <136mmol/L (p=0.036) as independently associated with death, transplant or VAD. CONCLUSIONS: Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.
OBJECTIVE: Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. METHODS: Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. RESULTS: Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8-2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup (p=0.001), NYHA class (p=0.002) B-type natriuretic peptide >164pg/ml (p=0.003) and sodium <136mmol/L (p=0.036) as independently associated with death, transplant or VAD. CONCLUSIONS: Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.
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