Background: The atriopulmonary Fontan circulation leads to arrhythmias, heart failure, or protein-losing enteropathy, eventually requiring conversion. In hesitant patients, we evaluated the effect of dual chamber pacing as a time-buying measure.
Methods: Between 1997 and 2004, 9 patients (aged, 6 to 18 years) with an atriopulmonary Fontan connection and sinus node dysfunction received dual chamber epicardial pacemaker systems. Indications included refractory arrhythmias (n = 5), protein-losing enteropathy (n = 2), heart failure with effusions (n = 1), and exercise intolerance (n = 2). Data were compared between hospital discharge after pacemaker implantation and last follow-up.
Results: There was no mortality or morbidity. At a follow-up of 3.3 +/- 1.0 years, lead survival was 100%. Both atrial (impedance = 683 +/- 40 Ohm; threshold = 0.8 +/- 0.1 V at 0.5 ms; sensing P waves = 3.3 +/- 0.8 mV) and ventricular (impedance = 630 +/- 68 Ohm; threshold = 1.3 +/- 0.3 V at 0.5 ms; sensing R waves = 8.7 +/- 2.5 mV) leads retained satisfactory pacing characteristics at last control, better than those at discharge. Arrhythmias subsided in all instances and no longer required medication in 3 patients. Protein-losing enteropathy improved temporarily in 1 patient and disappeared in another. Exercise intolerance diminished, and heart failure was controlled.
Conclusions: Although most atriopulmonary Fontan circulations will need conversion with arrhythmia surgery, patients may delay out of fear. Dual chamber pacing improves single ventricle hemodynamics and can help decompensated Fontan patients. In a multiple-redo setting, a left lateral thoracotomy provides safe access and allows for quantitatively reliable and durable epicardial pacing.