Open Access

ARTICLE

Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator

Madalena Coutinho Cruz¹, André Viveiros Monteiro¹, Guilherme Portugal¹, Sérgio Laranjo², Ana Lousinha¹, Bruno Valente¹, Paulo Osório¹, Pedro Silva Cunha¹, Lídia de Sousa¹, José Alberto Oliveira¹, Ana Agapito¹, Mário Martins Oliveira¹, Fátima Pinto², Rui Cruz Ferreira¹

1 Cardiology Department, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
2 Pediatric Cardiology Department, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal

* Corresponding Author: Madalena Coutinho Cruz, Cardiology Department, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Rua de Santa Marta 50, Lisboa 1169‐024, Portugal. Email:

Congenital Heart Disease 2019, 14(4), 525-533. https://doi.org/10.1111/chd.12767

Abstract

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.

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