Cardiac resynchronization therapy revealed first promising results in patients with a congenital heart disease and a systemic right ventricle.
Contrast enhanced magnet resonance imaging showed accessibility of the coronary sinus in an 18 year old male patient with mirror dextrocardia, d-transposition of the great arteries and ventricular septal defect (VSD) after Mustard operation and VSD patch closure. Entering the CS from the inferior vena cava, a well-developed vein draining from the apex and the right ventricular anterior wall could be identified. From the left subclavian vein an atrial and a left ventricular lead (Medtronic , 3830-59) were placed, followed by a CS-lead (Biotronik, Corox) (Figure 1), which initially caused diaphragmal contractions. Retraction could solve this problem with persistent good sensing (atrial lead 4.5 mV, sub pulmonic lead 13.4 mV) and pacing thresholds (atrial 0.4 V/0.4 ms, sub pulmonic 0.4 V/0.4 ms, sub aortic CS 1.1 V/0.4 ms). All system parameters were stable at 3 months follow-up. Bicycle test showed mild improvement in peak performance from 1.9 W/kg to 2.5 W/kg. Pro-brain natriuretic peptide decreased from 649 ng/l to 239 ng/l [standard normal: < 64 ng/l].
In the view of an increasing number of adult patients after atrial switch operation (Mustard/Senning) presenting with cardiac failure and interventricular dyssynchrony, the least invasive transvenous approach to CRT is feasible also if additional anomalies are present and should be considered in the pre-procedural evaluation.