Rhythm disturbances and treatment strategies in children with
congenitally corrected transposition of the great arteries
Taner Kasar MD
Pelin Ayyildiz MD
Gulhan Tunca Sahin MD
Erkut Ozturk MD
Selman Gokalp MD
Sertac Haydin MD
Alper Guzeltas MD
Yakup Ergul MD
Department of Pediatric Cardiology, Saglik
Bilimleri University Istanbul Mehmet Akif
Ersoy Thoracic and Cardiovascular Surgery
Research and Training Hospital, Istanbul,
Department of Pediatric Cardiovascular
Surgery, Saglik Bilimleri University Istanbul
Mehmet Akif Ersoy Thoracic and
Cardiovascular Surgery Research and
Training Hospital, Istanbul, Turkey
Yakup Ergul, Department of Pediatric
Cardiology, Saglik Bilimleri University
Istanbul Mehmet Akif Ersoy Thoracic and
Cardiovascular Surgery Research and
Istasyon Mah. Turgut
Ozal Bulvarı No:11, K
Istanbul, Halkali, Istanbul, Turkey.
Background: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected
transposition of the great arteries (ccTGA) and associated treatment strategies.
Patients and Methods: This retrospective cohort study included 65 pediatric patients with ccTGA
who were admitted to the clinic between 2009 and 2017. The patients were divided into two
groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electro-
physiological data, and device implantation data on the two groups were compared.
Results: Group I (n 5 53, 82%) consisted of patients with significant associated lesions, and Group
II (n 5 12, 18%) consisted of those with minor or no associated lesions (isolated ccTGA). Rhythm
abnormalities were diagnosed in 22 (34%) of the patients based on initial ECG findings and Holter
ECG recordings. Eleven (17%) of these patients had atrioventricular (AV) block of different degrees,
and the other 11 (17%) had supraventricular arrhythmia (SVA). The median follow-up was 49
months (range, 9–89 months), and the rhythm remained normal in 26 (42%) of the patients. Three
patients died on follow-up. Of 40 patients with normal initial findings, nine required pacemaker
implantation due to complete heart block, and SVA developed in seven patients on follow-up. No
ventricular tachycardia was seen initially or on follow-up. Ablation was performed in four patients.
During the follow-up period, pacemakers were implanted in 12 (23%) of patients in Group I and 4
(33%) of patients in Group II due to complete heart block. Cardiac resynchronization therapy (CRT)
was performed in four patients due to systemic ventricular dysfunction. Notably, all four of these
patients had a pacemaker implanted postoperatively.
arrhythmia, children, congenitally corrected transposition of the great arteries, rhythm disturbances
Congenitally corrected transposition of the great arteries (ccTGA)
is a rare pathology, which accounts for less than 1–1.4% of all con-
genital heart defects.
Although frequently associated with other
cardiac defects, such as ventricular septal defects (VSDs), pulmonary
stenosis (PS), and anomalies of the tricuspid valve, it can occur in
Previous studies revealed that spontaneous complete
heart block was present from birth in around 4% of patients and that
progressive deterioration of the condition was common.
presence and severity of associated lesions in ccTGA and conduction
disturbances alter the timing of the clinical presentation of the
There are a limited number of studies of rhythm disturbances and
their management in children with ccTGA.
The aim of the present
study was to investigate rhythm and conduction disturbances in a
relatively large group of pediatric ccTGA patients from a single center
and associated treatment strategies.
2018 Wiley Periodicals, Inc. wileyonlinelibrary.com/journal/chd Congenital Heart Disease. 2018;13:450–457.
Received: 18 October 2017
Revised: 12 January 2018
Accepted: 23 January 2018