TY - JOUR
T1 - Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure
AU - Van De Bruaene, Alexander
AU - Hickey, Edward J.
AU - Kovacs, Adrienne H.
AU - Crean, Andrew M.
AU - Wald, Rachel M.
AU - Silversides, Candice K.
AU - Redington, Andrew N.
AU - Ross, Heather J.
AU - Alba, Ana Carolina
AU - Billia, Filio
AU - Nair, Krishnakumar
AU - Benson, Lee
AU - Horlick, Eric
AU - Osten, Mark
AU - Colman, Jack
AU - Heggie, Jane
AU - Oechslin, Erwin N.
AU - Roche, S. Lucy
N1 - Publisher Copyright:
© 2017 Elsevier B.V.
PY - 2018/2/1
Y1 - 2018/2/1
N2 - 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 ± 13 years. Patients were grouped as follows: cyanotic ACHD 10%, 1 V 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 3 years. 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 > 164 pg/ml (p = 0.003) and sodium < 136 mmol/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.
AB - 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 ± 13 years. Patients were grouped as follows: cyanotic ACHD 10%, 1 V 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 3 years. 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 > 164 pg/ml (p = 0.003) and sodium < 136 mmol/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.
KW - Adult congenital heart disease
KW - B-type natriuretic peptide
KW - Heart failure
KW - Transplant
KW - Ventricular assist device
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U2 - 10.1016/j.ijcard.2017.10.086
DO - 10.1016/j.ijcard.2017.10.086
M3 - Article
C2 - 29103857
AN - SCOPUS:85032882934
SN - 0167-5273
VL - 252
SP - 80
EP - 87
JO - International Journal of Cardiology
JF - International Journal of Cardiology
ER -