Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure

Alexander Van De Bruaene, Edward J. Hickey, Adrienne Kovacs, Andrew M. Crean, Rachel M. Wald, Candice K. Silversides, Andrew N. Redington, Heather J. Ross, Ana Carolina Alba, Filio Billia, Krishnakumar Nair, Lee Benson, Eric Horlick, Mark Osten, Jack Colman, Jane Heggie, Erwin N. Oechslin, S. Lucy Roche

Research output: Contribution to journalArticle

6 Citations (Scopus)

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 ± 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.

Original languageEnglish (US)
Pages (from-to)80-87
Number of pages8
JournalInternational Journal of Cardiology
Volume252
DOIs
StatePublished - Feb 1 2018
Externally publishedYes

Fingerprint

Heart Diseases
Heart Failure
Phenotype
Heart-Assist Devices
Heart Ventricles
Transplants
Premature Mortality
Brain Natriuretic Peptide
Proportional Hazards Models
Disease-Free Survival
Young Adult
Cause of Death
Hospitalization
Hemodynamics
Sodium
Population

Keywords

  • Adult congenital heart disease
  • B-type natriuretic peptide
  • Heart failure
  • Transplant
  • Ventricular assist device

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure. / Van De Bruaene, Alexander; Hickey, Edward J.; Kovacs, Adrienne; Crean, Andrew M.; Wald, Rachel M.; Silversides, Candice K.; Redington, Andrew N.; Ross, Heather J.; Alba, Ana Carolina; Billia, Filio; Nair, Krishnakumar; Benson, Lee; Horlick, Eric; Osten, Mark; Colman, Jack; Heggie, Jane; Oechslin, Erwin N.; Roche, S. Lucy.

In: International Journal of Cardiology, Vol. 252, 01.02.2018, p. 80-87.

Research output: Contribution to journalArticle

Van De Bruaene, A, Hickey, EJ, Kovacs, A, Crean, AM, Wald, RM, Silversides, CK, Redington, AN, Ross, HJ, Alba, AC, Billia, F, Nair, K, Benson, L, Horlick, E, Osten, M, Colman, J, Heggie, J, Oechslin, EN & Roche, SL 2018, 'Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure', International Journal of Cardiology, vol. 252, pp. 80-87. https://doi.org/10.1016/j.ijcard.2017.10.086
Van De Bruaene, Alexander ; Hickey, Edward J. ; Kovacs, Adrienne ; Crean, Andrew M. ; Wald, Rachel M. ; Silversides, Candice K. ; Redington, Andrew N. ; Ross, Heather J. ; Alba, Ana Carolina ; Billia, Filio ; Nair, Krishnakumar ; Benson, Lee ; Horlick, Eric ; Osten, Mark ; Colman, Jack ; Heggie, Jane ; Oechslin, Erwin N. ; Roche, S. Lucy. / Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure. In: International Journal of Cardiology. 2018 ; Vol. 252. pp. 80-87.
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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 ± 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.",
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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

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

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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