Similarly to the first case there was phasic reversal flow through the fenestration that was no longer detected during atrial pacing. Function of the systemic ventricle and atrioventricular valve were normal. ECG disclosed competitive retrogradely conducted junctional rhythm. Few hours after cardiopulmonary bypass weaning he developed hypotension and low cardiac output syndrome requiring inotropic and vasopressor support. TCPC was planned because of progressive desaturation and effort intolerance. The second patient was a 5 years old child who had undergone neonatal systemic to pulmonary shunt and cavo-pulmonary connection at the age of 9 months, due to severe symptomatic Ebstein anomaly. Based on this findings she was scheduled for permanent atrial pacing owing to persisting junctional rhythm. Upon atrial pacing the reverse component of flow disappeared with a slight increase in aortic velocity time integral (VTI).Ĭlinically, these echocardiographic changes were accompanied by resolution of pleural effusion and progressive increase of oxygen saturation and albumin normalization during the following days (Fig. This observation was consistent with the evidence of giant ‘atrial’ wave one the jugular venous pressure tracing. Colour-Doppler interrogation of the Fontan conduit and supra-hepatic veins, disclosed phasic hepatopetal signal consistent with a reversal flow through the fenestration on time with atrial retrograde conduction (Fig. Transthoracic echocardiogram showed normal ventricular function and mild AV valve regurgitation. Blood chemistry disclosed increased liver enzymes and low albumin. ![]() 1a) and progressive desaturation were observed. Weaning from the bypass was uneventful and the patient was successfully extubated after 12 h.ĭuring the first postoperative week abdominal congestion, right pleural effusion (Fig. Preoperatively the patient had a well-tolerated junctional rhythm with constant retrograde conduction with preserved heart rate excursions. Due to progressive cyanosis the patient was scheduled for tricuspid valve repair and TCPC. She previously had undergone neonatal Norwood-Sano palliation and bidirectional cavo-pulmonary connection at the age of 3 months. The first patient was a 4 years old girl with hypoplastic left heart syndrome. Likewise, efficiency of temporary atrial pacing should be granted and surgeons should have a low threshold for epicardial lead implantation. In the absence of any hemodynamic target, hearth rhythm should be systematically checked after TCPC irrespective of adequacy of heart rate. The patients rapidly improved after atrial pacing, allowing discharge with a minimal dose of diuretic. We describe two cases of acute Fontan circulatory failure due to postoperative retrogradely conducted junctional escape rhythm despite an adequate heart rate and circadian variation. The clinical impact of arrhythmic disturbance on the follow up of patients who had undergone total cavo-pulmonary connection is well recognized but the role of, transient periods of retrogradely conducted junctional rhythm on the immediate post-operative course is less defined. Echocardiographic evaluation can reveal clues of this hemodynamic condition. Although bradycardia is usually well tolerated, retrogradely conducted junctional rhythm may acutely increase atrial pressure impairing cardiac output. ![]() ![]() Sequential atrioventricular activation plays a critical role in the physiology of Fontan circulation.
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