A case report of upgrading to cardiac resynchronization therapy in a patient with congenitally corrected transposition of great arteries and dextrocardia

Abstract

Background: Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart anomaly. Physiological correction may be associated with a long pre-symptomatic period in many patients and delayed accidental diagnosis. Additional related congenital malformations may increase the complexity of cardiac interventions.

Case summary: A 59-year-old man with known dextrocardia, situs viscerum inversus, and CCTGA was scheduled for upgrading of a dual-chamber pacemaker to cardiac resynchronization therapy to treat heart failure related to a progressive systolic dysfunction of the systemic right ventricle (RV). Because of the specific anatomy of this patient, the therapeutic procedure was complicated by the cannulation of the Marshall vein. Nevertheless, the left ventricular lead was successfully implanted into the coronary sinus lateral branch. At the 3-month follow-up, the patient remarkably reported a significant functional improvement, despite no favourable reverse remodelling of the systemic RV.

Discussion: Upgrade of a pacemaker to biventricular pacing was feasible in this patient, who had CCTGA and dextrocardia, which resulted in symptomatic improvement at follow-up. Pre-implant contrast cardiac computed tomography angiography was essential for visualizing the venous-specific anatomy in this patient, who suffered from congenital heart disease. Conduction system pacing represents a potential alternative for the patient to prevent or treat pacing-related heart failure.

Keywords: Cardiac resynchronization therapy; Case report; Congenitally corrected transposition of the great arteries; Dextrocardia; Heart failure.

Figure 1

Three-dimensional reconstruction rendered from the electrocardiogram-gated contrast computed tomography of the heart in the arterial phase, demonstrating the anatomical condition in the patient with congenitally corrected transposition of the great arteries and dextrocardia. (A) The anterior position rotated by 28° up and by 23° to the left. (B) The posterior position rotated by 67° down and by 130° to the right. AO, aorta; CS, coronary sinus; LV, left ventricle; PA, pulmonary artery; RV, right ventricle; white solid arrow, ventricular electrode; white dashed arrow, atrial electrode.

Figure 2

Implantation of a cardiac resynchronization therapy system. (A) Coronary sinus cannulation followed by occlusive venogram that clarifies the position in the Marshall vein (yellow solid arrow). (B) Venogram of the coronary sinus. Only one lateral branch is suitable for implanting the left ventricular electrode (yellow dashed arrow). (C) RAO projection after the completion of the procedure, a new bipolar systemic right ventricle electrode in the coronary sinus (RV e), and previously implanted devices: an original right atrium electrode implanted in 1994 (RA e 1), a right atrium electrode implanted in 2003 (RA e 2), and a subpulmonary left ventricle electrode implanted in 1994 (LV e).

Figure 3

(A) Electrocardiogram levels recorded during pacing of the morphological left ventricle, standard placement of limb and chest leads, and a QRS duration of 220 ms. (B) Electrocardiogram levels recorded during pacing of the morphological left ventricle, standard placement of limb leads, chest leads placed inversely (V₁R–V₆R), and a QRS duration of 205 ms. (C) Electrocardiogram taken during biventricular pacing, standard placement of limb leads, reversed placement of chest leads, and a QRS duration of 160 ms.

Figure 4

Transthoracic echocardiography after upgrading to cardiac resynchronization therapy and a modified apical four-chamber view from the right hemithorax. The objective parameters persisted despite the therapy. Thickness of the systemic right ventricle free wall 13 mm, right ventricle ejection fraction 25%, left ventricle ejection fraction 50%, moderate regurgitation of the morphological tricuspid valve, and mild regurgitation of the morphological mitral valve. LA, left atrium; LV, subpulmonary left ventricle; RA, right atrium; RV, systemic right ventricle; solid arrow, left ventricle electrode.

Figure 5

Electrocardiogram strip obtained during device pre-implant integration presenting a 2:1 second-degree atrioventricular block and a native narrow QRS complex.