Abstract

Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart disease that encompasses an atrioventricular and ventriculoarterial discordance and accounts for less than 1 percent of congenital heart diseases. Objective: To present an atypical case of a man with complex congenital heart disease and conduction anomalies. Case Presentation: This is a case of a 34-year-old patient who came to the hospital with 1 week of dyspnea on exertion and episodes of lipothymia. The patient was referred to our hospital after an electrocardiogram from his primary care with evidence of blocked atrial fibrillation. During the initial evaluation blocked atrial fibrillation was confirmed with a rescue ventricular rate of 38 bpm. A magnetic resonance confirmed the presence of the atrioventricular and ventriculoarterial discordance, the aorta had a left anterior position, perimembranous ventricular septal defect with a right to left shunt, biventricular systolic dysfunction, moderate tricuspid, and mitral regurgitation, interventricular septal intramyocardial and biatrial fibrosis, left atrial dilation, and dilation of the pulmonary artery. After pacemaker placement, the patient has an improvement in his clinical symptoms and quality of life. Conclusions: Cardiac arrhythmias are CCTGA’s leading cause of death, mostly ventricular tachycardia, and atrial fibrillation. Right bundle branch block is a previously unreported and potentially very rare presentation of this disease. This, added to the fact that our patient was diagnosed at an advanced age, but without symptoms of heart failure, makes him an atypical case of CCTGA, with new potential treatment options.

Highlights

  • Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart disease that encompasses an atrioventricular and ventriculoarterial discordance and accounts for less than 1 percent of congenital heart diseases

  • Most of the patients with CCTGA are asymptomatic until adulthood, where the most common clinical presentation is due to heart failure exacerbations

  • The presence of right and left bundle branch blocks and ventricular arrhythmias are usually found in patients with heart failure and is thought to be a complication rather than part of CCTGA [1] [4]

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Summary

Introduction

Corrected transposition of the great arteries (CCTGA) is a rare heart disease that encompasses an atrioventricular and ventriculoarterial discordance. It has been hypothesized that the central cause which leads to heart failure is the incompetence of the right ventricle to maintain the systemic circulation through a person’s life [1] [2]. Conduction abnormalities are another common cause of decompensation [3]. The presence of right and left bundle branch blocks and ventricular arrhythmias are usually found in patients with heart failure and is thought to be a complication rather than part of CCTGA [1] [4]. It is recommended to give an implantable defibrillator to patients with heart failure with severely reduced ejection fraction [2] [6]

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