TY - EJOU
AU - Cruz, Madalena Coutinho
AU - Monteiro, André Viveiros
AU - Portugal, Guilherme
AU - Laranjo, Sérgio
AU - Lousinha, Ana
AU - Valente, Bruno
AU - Osório, Paulo
AU - Cunha, Pedro Silva
AU - Sousa, Lídia de
AU - Oliveira, José Alberto
AU - Agapito, Ana
AU - Oliveira, Mário Martins
AU - Pinto, Fátima
AU - Ferreira, Rui Cruz
TI - Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator
T2 - Congenital Heart Disease
PY - 2019
VL - 14
IS - 4
SN - 1747-0803
AB - Objective: Sudden cardiac death is common in the adult congenital heart disease
(ACHD) population. Knowledge and experience about the use of implantable cardio‐
verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize
a cohort of patients with ACHD and ICDs.
Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a
single tertiary center were evaluated. Data on baseline clinical features, heart defect,
indication for ICD, type of device, appropriate therapies, ICD‐related complication,
and mortality during follow‐up were collected.
Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular
tachycardia (VT) or ventricular fibrillation (VF). The rate of inappropriate therapies
and device‐related complications was 33.3%. Secondary prevention and primary pre‐
vention patients with class I indications for ICD had more appropriate therapies than
complication, but this relationship was reversed for patients with class II indications.
Remote monitoring played an important role in diagnosing new atrial arrhythmias be‐
fore scheduled visits in 46.2% of patients, leading to a change in medication. VT/VF
episodes were associated with a composite of death, cardiac transplantation, and hos‐
pital admission (OR 13.0; 95% CI: 2.1‐81.5).
Conclusion: ICDs are not only useful in preventing SCD, but also have a major role in
diagnosing atrial tachyarrhythmias ahead of scheduled visits. Although improve‐
ments in ICD technology might reduce complications and inappropriate therapies,
adequate selection of candidates for primary prevention still remains difficult be‐
cause of the lack of clear indications.
KW - congenital heart defect
KW - implantable cardioverter defibrillator
KW - supraventricular tachycardia
KW - ventricular fibrillation
KW - ventricular tachycardia
DO - 10.1111/chd.12767