Pediatric Cardiology and Cardiac Surgery
Vol.25 No.1 2009 (45-52)

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Departments of 1)Pediatrics, 2)Cardiovascular Surgery, Graduate School of Medical Science, Kyushu University, Fukuoka 3)Department of Surgery,Yokohama City University School of Medicine, Yokohama and 4)Division of Pediatrics, Kyushu Koseinenkin Hospital, Fukuoka, Japan


Background: Fatal arrhythmia late after repair of tetralogy of Fallot (TOF) is related to right ventricular volume load caused by pulmonary valve regurgitation (PR). However, the timing and clinical outcomes of pulmonary valve replacement (PVR) have been unclear in these patients.
Objective: To evaluate the clinical outcomes of PVR in patients late after repair of TOF.
Patients and Methods: Since 2003, 13 patients have undergone PVR due to PR after the total repair of TOF. We evaluate clinical data obtained by chest X-ray, electrocardiogram, echocardiogram, and cardiac catheterization in these patients.
Results: Primary total repair of TOF and PVR with Carpentier-Edwards pericardial valve was performed at 4.9 ±4.7 (1.0-18.3) years of age and 23.4 ±11.5 (7.9-45.3) years of age, respectively. The interval between primary repair and PVR was 18.4 ±9.3 (3.8-35.2) years. Follow-up duration was 6.4 ±6.3 (1.8-25.3) years after PVR. There was no early or late death related to PVR, and structural failure of the bioprosthesis valve was not observed. There was a significant decrease in the cardiothoracic ratio and right ventricular end-diastolic volume index after PVR compared with those before PVR, but no significant change has been observed between just after PVR and late after PVR. Before PVR, ventricular tachyarrhythmia/ventricular premature contraction (VPC) were documented in 6 patients, atrial fibrillation (Af)/atrial flutter (AFL) in 2, and complete atrioventricular block in one. Although 6 patients underwent cryoablation during PVR, 2 patients had VPC and another 2 patients had Af in post-PVR. Another patient who had no arrhythmia before PVR developed AFL after PVR. Two patients needed pacemaker implantations including an implantable cardioverter defibrillator (ICD).
Conclusions: PVR effectively improves right ventricular load, but it could not completely inhibit ventricular arrhythmia. Atrial arrhythmia was a residue in the majority of patients who had right heart volume load associated with tricuspid regurgitation and residual left-to-right shunt before PVR. Electrophysiological mapping should be performed before and after PVR, and pacemaker implantation including ICD might be needed in such patients.