Clinical Outcomes of Pulmonary Valve Replacement in Patients Late after Repair of Tetralogy of Fallot

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1 PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL. 25 NO. 1 (45 52) Clinical Outcomes of Pulmonary Valve Replacement in Patients Late after Repair of Tetralogy of Fallot 1) Jun Muneuchi, Yoshiaki Kanaya, 1) Yoshihisa Tanoue, 2) Yuichi Shiokawa, 2) Ken-ichiro Yamgaguchi, 1) Kazuyuki Ikeda, 1) Munetaka Masuda, 3) Takuro Ohno, 4) and Ryuji Tominaga 2) 1) Departments of 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 ( ) years of age and ( ) years of age, respectively. The interval between primary repair and PVR was ( ) years. Follow-up duration was ( ) 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. TOF PR PR TOF PR PVR 2003 PVR Carpentier-Edwards pericardial 13 X

2 PVR PVR PVR /PVR PVR PVR / VPC 6 Af /AFL 2 1 cryoablation 5 1 VPC 2 Af 2 1 AFL 1 1 ICD TOF PR PVR TR ICD tetralogy of Fallot TOF pulmonary regurgitation PR TOFPR pulmonary valve replacement PVR 1 2 TOF PVR PVR TOF PR PVR PVR 5 TOF PR PVR TOF PR PVR 2003 PRTOF PVR transannular patch 2 Blalock-Taussig PVR PR X cardiothoracic ratio CTR 55 / right ventricular pressure/ left ventricular pressure RVp/LVp > PVR PRGoldberg PR 6 atrial fibrillation Af atrial flutter AFL ventricular tachycardia VT 10 atrial premature contraction APC ventricular premature contraction VPC 2 APC VPC PVR PVR Carpentier-Edwards pericardial 1 Hancock II 21mm 3 23mm 7 25mm 2 27mm PVR VPC/VT PVR cryoablation cyoablation PVR X QRS ms 50mmHg

3 47 /right ventricular end-diastolic volume index RVEDVI RVp/LVp right ventricular end-diastolic pressure RVEDP RVEDVI Heart II PVR t PVR13 Table PVR PVR PR pulmonary stenosis PS50mmHg 6 46tricuspid regurgitation TR 3 23atrial septal defect ASD 2 15 partial anomalous pulmonary venous connection PAPVC 1 PVR PVR PVR NYHA I II 9 III IV 3 PVR I II PVR Table 2 X CTR PVR p = CTR 56 5 PVR PVR QRS complete right bundle blanch block CRBBB 11 PVR CRBBBQRS PVR PVRPVR ms ms ms PVR 7 RVEDVI PVR ml/m ml/m 2 p = 0.04 RVp/LVp RVEDP PVR p = mmhg mmhg p = 0.003Fig. 1 7 VT/VPC 6 Af/AFL 2 complete atrioventricular block CAVB 1 VT/VPC 6 2 CARTO VT/VPC PVR VT/VPC 6 4 VT/VPC 1 5 cryoablation VT/VPC cryoablation PVR VT/VPC 6 4 PVR 2 5 VPC 2 VPC VPC 2 AFL 1 13 PVR CARTO radiofrequency ablation PVR Af PVR VT implantable cardioverter defibrillator ICD 6 PVR AFL 2 voltage mappingfig. 2 6 AFL APC Af 13 PVR Af PVR TR ASD PAPVC 4 3 PVR 2 PR TOF PVR 13 12PVR CTR RVEDVI TR

4 48 Table 1 Clinical data of 13 patients who underwent PVR due to PR after the total correction of TOF Case Sex Age Age Follow-up Symptoms Residual PR Arrhythmia lesions ICR (y) PVR (y) post PVR (y) Pre PVR Post PVR Late PVR Pre PVR Post PVR Late PVR PMI ABL 1 M None (CTR ) PS Severe Mild 2 F None (PS ) PS Mod Mild 3 M None (CTR ) Severe Mild 4 F None (CTR ) Severe Mild VPC Cryo 5 F None (VPC ) PS, TR Mod Tr Tr VPC, CAVB VPC, CAVB VPC, CAVB Cryo + 6 M None (CTR ) PS, TR, PAPVC Mod Tr Mild SND, AFL SND, APC Cryo, RF 7 F Fatigue Severe Mild VPC 8 F Prone to infection PS Severe 9 M Syncope ASD Severe Mild VT 10 M None (CTR ) Mod Tr Mild 11 F Fatigue Severe Mild Mild VPC VPC VPC Cryo 12 F Cyanosis PS, ASD, TR Severe Mild Af Af Af Cryo 13 M Syncope Severe VT, AFL SND, Af SND, Af RF, Cryo + TOF: tetralogy of Fallot, PVR: pulmonary valve replacement, ICR: intracardiac repair, PR: pulmonary regurgitation, ABL: ablation, PMI: pacemaker implantation, CTR: cardiothoracic ratio, PS: pulmonary stenosis, TR: tricuspid valve regurgitation, PAPVC: partial anomalous pulmonary venous connection, ASD: atrial septal defect, Mod: moderate, Tr: trivial, VT: ventricular tachycardia, VPC: ventricular premature contraction, CAVB: complete atrioventricular block, Af: atrial fibrilation, AFL: atrial flutter, SND: sinus node dysfunction, Cryo: cryoablation, RF: radiofrequency ablation

5 49 Table 2 CTR, QRS width, RVEDVI, RVp/LVp, and RVEDP before and after PVR in 13 patients Case CTR (%) QRS width (ms) RVEDVI (ml/m 2 ) RVp/LVp RVEDP (mmhg) Pre PVR Post PVR Late PVR Pre PVR Post PVR Late PVR Pre PVR Post PVR Pre PVR Post PVR Pre PVR Post PVR CTR: cardiothoracic ratio, RVEDVI: right ventricular end-diastolic volume index, RVp: right ventricular pressure, LVp: left ventricular pressure, RVEDP: right ventricular end-diastolic pressure, PVR: pulmonary valve replacement CTR N = 13 QRS width N = p = NS NS NS Pre PVR Post PVR Late PVR Pre PVR Post PVR Late PVR RVEDVI N = 8 RVp/LVp N = 8 RVEDP N = 8 p = p = p = Pre PVR Post PVR.2 Pre PVR Post PVR 2 Pre PVR Post PVR Fig. 1 Changes in CTR and QRS width are shown before PVR, just after PVR, and late after PVR. Changes in hemodynamics are shown before PVR and after PVR. CTR: cardiothoracic ratio, RVEDVI: right ventricular end-diastolic volume index, RVp: right ventricular pressure, LVp: left ventricular pressure, RVEDP: right ventricular end-diastolic pressure, PVR: pulmonary valve replacement 13 Kawachi St. Jude Medical

6 50 Fig. 2 Activation mappings of the right atrium in cases 6 and 13 show surgical incisions form central obstacles of atrial tachyarrhythmia CTR RVEDVI PVR Therrien TOF PR PVR PVR RVEDVI 170ml/m 2 PVR RVEDVI PVR 5 TOF PR PR 8 10 PR RVEDVI RVEDVI 170ml/m 2 PVR 4 RVEDVI PVR VT/ VPC 6 2 PVR VPC PVR Hooft van Huysduynen PVR RVEDV spatial QRS-T axis QRS 13 TOF QRS 180msec T T wave alternance VT PVR QRS 180msec 13 1 VPC 4 PVR VT VT 13 3 TR ASD PAPVC Therrien TOF PVR 5 TOF Li Fontan 22 TOF 20 ASD 17 Mustard

7 TOF Waterston Potts TR PVR PRTR AFL CARTO cryoablation 23 in vivo 24 Lukac AFL cryoablation cryoablation AFL/SND sinus node dysfunction PVR PVR CARTO 6 PAPVC SND Geva TOF PR PVR 26 PR MRI 25 RVEDVI 160ml/m 2 right ventricular end-systolic volume index RVESVI 70ml/m 2 left ventricular end-diastolic volume index LVEDVI 65ml/m 2 right ventricular ejection fraction RVEF 45 sustained VT2 PVR TR PVR MRI MRI TOF PR PVR PVR CARTO TOF 13 PVR PVR PVR ICD 1 Gatzoulis MA, Balaji S, Webber SA, et al: Risk factors for ar

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