ArticlePDF Available

What is the diagnosis?

Authors:
Murad Almasri at Arkansas Children's Hospital
Murad Almasri
Hamza A. Salim at An-Najah National University
Hamza A. Salim
Jeffrey Orcutt
Jeffrey Orcutt
Jeffrey Orcutt
  • This person is not on ResearchGate, or hasn't claimed this research yet.
Download full-text PDF
Read full-text
Download citation

Abstract

In this manuscript, we present an ECG that is diagnostic of congenitally corrected transposition of great arteries (ccTGA) and ask about the arrhythmias associated as well as the management options.
Available via license: CC BY-NC 4.0
Content may be subject to copyright.
What is the diagnosis?
Murad Almasri
1
, Hamza Salim
2
*, and Jeffrey Orcutt
1
1
Pediatric Cardiology, Arkansas Children’s Hospital, 1 Children’s Way, Little Rock, AR 72202, USA; and
2
Faculty of Medicine, An-Najah National University, Sultan Abdul Hamid Street,
00972 Nablus, Palestine
Received 22 July 2023; accepted 4 September 2023; online publish-ahead-of-print 6 September 2023
In this manuscript, we present an ECG that is diagnostic of congenitally corrected transposition of great arteries (ccTGA) and ask about the arrhythmias
associated as well as the management options.
---------------------------------------------------------------------------------------------------------------------------------------------------------------
Keywords ccTGA • arrhythmia • paediatrics
* Corresponding author. Tel: +972595122548, Email: Hamza.sleeem@gmail.com
Handling Editor: Stefano Bordignon
© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.
This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits
non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
Clinical vignette
A 6-year-old child is followed in the paediatric cardiology clinic.
The patient has been receiving periodic assessments including
electrocardiograms (ECGs), echocardiograms (ECHOs), and Holter
monitoring. The child’s medical history does not include any surgical
interventions.
The child’s overall health has been stable, with no symptoms reported
during routine clinical visits. As part of the ongoing evaluation, an ECG
was performed during the latest visit which is shown below:
ECG
European Heart Journal - Case Reports (2023) 7, 1–2
https://doi.org/10.1093/ehjcr/ytad446
ECG CHALLENGE
Downloaded from https://academic.oup.com/ehjcr/article/7/9/ytad446/7261695 by guest on 16 September 2023
1. What is the diagnosis based on
the clinical picture and ECG
ndings?
a. Tetralogy of Fallot
b. D—Transposition of the great arteries
c. congenitally corrected transposition of the great arteries (ccTGA)
d. Ventricular septal defect (VSD)
e. Atrial septal defect (ASD)
Explanation:
The correct answer is C
In ccTGA (congenitally corrected transposition of the great arter-
ies), the ECG ndings show the absence of initial Q waves that are typ-
ically seen in leads V5 and V6 (lateral precordial leads), as well as the
abnormal presence of initial Q waves in leads V3R and V1 (anterior
precordial leads). These ndings represent the abnormal depolariza-
tion sequence of the interventricular septum present in ventricular
inversion.
1
The other options have different ECG ndings: Tetralogy of Fallot
can show right ventricular hypertrophy and strain pattern,
D-Transposition of the great arteries has non-specic ECG ndings,
VSD may show signs of ventricular hypertrophy, and ASD can have a
normal ECG or signs of right atrial enlargement.
2. Which arrhythmia is most
commonly associated with the
diagnosis?
a. Complete heart block
b. Supraventricular tachycardia (SVT)
c. Wolff–Parkinson–White syndrome (WPW)
d. Bradyarrhythmia
e. Junctional rhythm
Explanation:
The correct answer is A
Complete heart block is the most common arrhythmia associated with
ccTGA. Other options are also associated with ccTGA but are less com-
mon. WPW is caused by an abnormal accessory pathway, which can lead
to supraventricular tachyarrhythmias. Bradyarrhythmias and complete
heart block can occur due to abnormalities in the conduction system.
2–3
3. What is the best treatment
option for this case?
a. Observation
b. Pulmonary artery (PA) banding
c. Double switch procedure
d. Heart transplantation
e. Balloon valvuloplasty
The correct answer is A
The best treatment option for the presented case is observation.
The patient has been stable with no signicant symptoms, and the pro-
vided electrocardiogram (ECG) as well as the normal reported echo-
cardiogram do not indicate an immediate need for intervention.
4
ccTGA patients who remain unoperated are at risk to develop sys-
temic atrioventricular valve (SAVV) regurgitation, which can lead to sys-
temic ventricle (SV) dysfunction. However, the decision for surgical
intervention depends on the severity of regurgitation and signs of ven-
tricular dysfunction, which are not evident in this case.
4
The other options are not indicated in this case based on the absence
of specic indications or evidence for these interventions.
Acknowledgements
The authors acknowledge the effort and dedication put forth by
McKinsey Jansen in acquiring the data and ECG for this submission.
Consent: The patient agreed to have this ECG published online.
Conict of interest: The authors have nothing to disclose.
Funding: This project was not funded.
Data availability
There are no new data associated with this article.
References
1. Fernández F, Laurichesse J, Scébat L, Lenègre J. Electrocardiogram in corrected transpos-
ition of the great vessels of the bulbo-ventricular inversion type. Br Heart J 1970;32:
165–171.
2. Shalganov TN, Balabanski TL, Lazarov SD. Radiofrequency catheter ablation in a child
with Wolff-Parkinson-White syndrome and congenitally corrected transposition of the
great arteries. Cardiol J 2007;14:500–503.
3. Berman DA, Adicoff A. Corrected transposition of the great arteries causing complete
heart block in an adult. Treatment with an articial pacemaker. Am J Cardiol 1969;24:
125–129.
4. Beauchesne LM, Warnes CA, Connolly HM, Ammash NM, Tajik AJ, Danielson GK.
Outcome of the unoperated adult who presents with congenitally corrected transpos-
ition of the great arteries. J Am Coll Cardiol 2002;40:285–290.
2 ECG Challenge
Downloaded from https://academic.oup.com/ehjcr/article/7/9/ytad446/7261695 by guest on 16 September 2023
ResearchGate has not been able to resolve any citations for this publication.
Radiofrequency catheter ablation in a child with Wolff-Parkinson-White syndrome and congenitally corrected transposition of the great arteries
Article
Full-text available
  • Jan 2007
The case is presented of a five-year-old boy with Wolff-Parkinson-White syndrome, who had undergone three surgical palliations because of congenitally corrected transposition of the great arteries, ventricular septal defect and pulmonary trunk near-atresia. Successful and uncomplicated radiofrequency catheter ablation of an accessory pathway located across the systemic atrioventricular valve ring was performed. The procedure was motivated by forthcoming corrective surgery, which would preclude venous access to the heart. This case emphasizes the need for precise timing of ablative therapy in such patients and shows that surgery itself may facilitate catheter ablation. (Cardiol J 2007; 14: 500-503).
View
Electrocardiogram in corrected transposition of the great vessels of the bulboventricular inversion type
Article
  • Apr 1970
  • Br Heart J
Twenty cases of corrected transposition of the great vessels of the bulbo-ventricular inversion type, either lone or combined with other intracardiac anomalies, were analysed. Rhythm and/or atrio-ventricular conduction disturbances were common to all groups of cases. QRS pattern changes were found to be related both to ventricular inversion and to ventricular hypertrophy. Isolated corrected transposition and corrected transposition with systemic ventriculo-atrial regurgitation give rise to tracings suggestive of systemic ventricular hypertrophy. Corrected transposition of the great vessels with pulmonary stenosis or pulmonary artery hypertension is usually accompanied by the electrocardiographic signs of a venous-ventricular hypertrophy, with a characteristic inversion of the normal praecordial pattern. The conventional criteria of ventricular hypertrophy may be applied in corrected transposition of the great vessels but are less reliable than in cases without ventricular inversion. The so-called electrocardiographic pattern of `ventricular inversion' in this anomaly is related not only to the inverted position of the ventricles but to a greater extent to the predominant, anatomically left, venous-ventricular hypertrophy which re-establishes the normal weight ratio between the anatomically right and anatomically left ventricles.
View
Corrected transposition of the great arteries causing complete heart block in an adult. Treatment with an artificial pacemaker
Article
  • Aug 1969
  • AM J CARDIOL
Corrected transposition of the great arteries was found in a 40 year old man with congestive heart failure and complete heart block. Treatment with an implanted artificial pacemaker resulted in temporary subjective improvement, but hemodynamic measurements essentially were unchanged and the patient eventually died with congestive heart failure. Autopsy disclosed, in addition to corrected transposition of the great arteries, multiple small pulmonary emboli and an abnormality of the left atrioventricular valve. This report illustrates the natural history of corrected transposition in an adult patient.
View
Outcome of unoperated patients who present with congenitally corrected transposition of the great arteries
Article
  • Jul 2002
  • J AM COLL CARDIOL
The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries. The presentation of this disorder and the outcome in unoperated adults have not been well defined. All unoperated patients > or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome. Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age > or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade > or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index > or =2/4). In 16 (53%), SAVV regurgitation > or =3/4 and ventricular dysfunction had been documented for >6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001). Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.
View
Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries
  • Jan 2002
  • 285
  • Beauchesne