title: Cardiac resynchronization therapy in adults with congenital heart disease.
abstract: In adults with congenital heart disease (CHD) heart failure is one of the leading causes of morbidity and mortality but experience with and reported outcome of cardiac resynchronization therapy (CRT) is limited. We investigated the efficacy of CRT in adults with CHD.
  
  
  
  This was a retrospective study including 48 adults with CHD who received CRT since 2003 in four tertiary referral centres. Responders were defined as patients who showed improvement in NYHA functional class and/or systemic ventricular ejection fraction by at least one category. Ventricular function was assessed by echocardiography and graded on a four point ordinal scale. Median age at CRT was 47 years (range 18-74 years) and 77% was male. Cardiac diagnosis included tetralogy of Fallot in 29%, (congenitally corrected) transposition of great arteries in 23%, septal defects in 25%, left sided lesions in 21%, and Marfan syndrome in 2% of the patients. The median follow-up duration after CRT was 2.6 years (range 0.1-8.8). Overall, 37 out of 48 patients (77%) responded to CRT either by improvement of NYHA functional class and/or systemic ventricular function. There were 11 non-responders to CRT. Of these, three patients died and four underwent heart transplantation.
  
  
  
  In this cohort of older CHD patients, CRT was accomplished with a success rate comparable to those with acquired heart disease despite the complex anatomy and technical challenges frequently encountered in this population. Further studies are needed to establish appropriate guidelines for patient selection and long term outcome.
authors.affiliation: Department of Cardiology, Academic Medical Center Amsterdam, The Netherlands.|Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
authors.forename: Zeliha
authors.initials: Z
authors.lastname: Koyak
authors.affiliation: Department of Cardiology, Academic Medical Center Amsterdam, The Netherlands.
authors.forename: Joris R
authors.initials: JR
authors.lastname: de Groot
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center for Adults, and University of Toronto, Toronto, Canada.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Krimly
authors.affiliation: Department of Cardiology, Academic Medical Center Amsterdam, The Netherlands.
authors.forename: Tara M
authors.initials: TM
authors.lastname: Mackay
authors.affiliation: Department of Cardiology, Academic Medical Center Amsterdam, The Netherlands.
authors.forename: Berto J
authors.initials: BJ
authors.lastname: Bouma
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center for Adults, and University of Toronto, Toronto, Canada.
authors.forename: Candice K
authors.initials: CK
authors.lastname: Silversides
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center for Adults, and University of Toronto, Toronto, Canada.
authors.forename: Erwin N
authors.initials: EN
authors.lastname: Oechslin
authors.affiliation: Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Ulas
authors.initials: U
authors.lastname: Hoke
authors.affiliation: Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Lieselot
authors.initials: L
authors.lastname: van Erven
authors.affiliation: Department of Cardiology, UZ Leuven, Leuven, Belgium.
authors.forename: Werner
authors.initials: W
authors.lastname: Budts
authors.affiliation: Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Isabelle C
authors.initials: IC
authors.lastname: Van Gelder
authors.affiliation: Department of Cardiology, Academic Medical Center Amsterdam, The Netherlands.|Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
authors.forename: Barbara J M
authors.initials: BJM
authors.lastname: Mulder
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center for Adults, and University of Toronto, Toronto, Canada.
authors.forename: Louise
authors.initials: L
authors.lastname: Harris
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D058406:Cardiac Resynchronization Therapy / Q000009:adverse effects; D058409:Cardiac Resynchronization Therapy Devices; D000066491:Clinical Decision-Making; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D006333:Heart Failure / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D018579:Patient Selection; D020127:Recovery of Function; D012189:Retrospective Studies; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right; D055815:Young Adult
keywords: Adult congenital heart disease; Cardiac resynchronization therapy