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A 29-year-old male was born with a transposition of the great arteries, a large ventricular septal defect, valvular pulmonary stenosis and a patent ductus arteriosus. Because of progressive cyanosis a Rashkind septostomy was performed on the first day of life. At one year of age he underwent a Mustard operation including closure of the ventricular septal defect and ductus arteriosus as well as an open commissurotomy of the pulmonary valve. He was asymptomatic during regular follow-up at our outpatient clinic. However, recently, 28 years after his operation, he started complaining of progressive fatigue and dizziness. Echocardiography revealed deterioration of his systemic right ventricular function. This was confirmed by magnetic resonance imaging (MRI), as a decrease in the ejection fraction from 48 to 37% within two years was noted. Subsequent Holter monitoring showed sinus rhythm with a prolonged PR interval up to 500 ms, periods of extreme sinus arrhythmia, sinus bradycardia and sinus arrests with frequent pauses of 2.6 sec. There were also episodes of atrioventricular junctional escape rhythm and high degree atrioventricular block with pauses of up to 5.4 sec. Because of the dizzy spells due to sinus node dysfunction in combination with atrioventricular conduction disturbances the patient underwent a transvenous double chamber pacemaker implantation. Through the left cephalic vein an active-fixation electrode was inserted and placed in the apex of the anatomic left (subpulmonary) ventricle. Subsequently, the atrial lead was inserted and screwed into the left atrial appendage. Sensing and pacing thresholds were excellent and a post-procedural X-ray showed adequate positioning of the leads (figure 1). The patient could be discharged uneventfully. At follow-up the patient had improved considerably and he did not have any complaints of fatigue or dizzy spells.
Figure 1
A) AP chest X-ray after pacemaker implantation to confirm the position of the pacemaker leads. The ventricular lead is situated in the anatomic left ventricle (LV), and the atrial lead in the left atrial appendage in the systemic venous baffle (SB). Ao=aorta, AP=pulmonary artery, RV=anatomic right ventricle. B) Lateral chest X-ray.
