Timing and type of surgery for severe pulmonary regurgitation after repair of tetralogy of Fallot Periklis A. Davlouros a,b , Ageliki A. Karatza a,b , Michael A. Gatzoulis a, * , Darryl F. Shore c a Adult Congenital Heart Programme, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK b Congenital Heart Programme, Department of Cardiology and Department of Paediatrics, Patras University Hospital, Rion, Patras, Greece c Department of Cardiac Surgery, Royal Brompton Hospital, London, UK Abstract Repaired tetralogy of Fallot (rTOF) has an excellent long-term prognosis; however, survival is somewhat less than normal. Of all the residual lesions and sequellae after rTOF, pulmonary regurgitation (PR) is the most important, correlating with right ventricular (RV) size, exercise intolerance and serious ventricular arrhythmias. Pulmonary valve replacement (PVR) has beneficial effects on RV size and function, provided it is performed early, before irreversible RV dysfunction ensues. Moreover, PVR is associated with an improvement in patients’ symptoms and exercise tolerance and combined with arrhythmia surgery (cryoablation) it leads to a dramatic decrease in the incidence of fatal ventricular arrhythmias. Associated lesions, especially branch pulmonary artery stenosis, which aggravates PR, and tricuspid regurgitation, which further impacts on RV size and function, need addressing. Large right ventricular outflow (RVOT) akinetic and aneurysmal regions are frequent and further compromise RV function; therefore, resection during PVR should be attempted. Despite excellent mid-term results, homografts and xenografts, usually used for RVOT reconstruction, suffer late dysfunction and failure, committing patients and surgeons to further operations. Therefore, the decision to operate should be based on the balance between progressive RV dilatation, exercise intolerance, symptoms, arrhythmias and the fact that further reoperations will be needed. Research on the ideal valve for RVOT reconstruction is ongoing. Prospective follow-up of patients with rTOF with exercise testing and assessment of RV size and function, preferably with magnetic resonance, will define better the natural history of the disease and will probably provide firm guidelines for PVR timing especially in asymptomatic patients. D 2004 Elsevier Ireland Ltd. All rights reserved. Keywords: Tetralogy of Fallot; Pulmonary regurgitation; Pulmonary valve replacement 1. Introduction Tetralogy of Fallot (TOF) has an incidence of approximately 0.5/1000 live births (5–7% of congenital heart lesions). Untreated, 25% of patients die in the first year of life [1]; however, cardiac surgery has clearly transformed the outcome with an operative mortality 0– 2% in many recent series [2–4]. Repair has conveyed excellent long-term results with most of these patients remaining well and leading normal lives [5]. Twenty-five- year survival rates have been reported to be as high as 94%, for hospital survivors of the early era of open-heart surgery [6,7]. With increasing duration of follow-up, however, significant late morbidity and mortality has been observed in a proportion of patients [8,9]. This is mainly due to right ventricular (RV) dysfunction, exercise intolerance, arrhythmia and sudden death [8–10]. Pulmo- nary regurgitation (PR) seems to be the principal haemodynamic substrate associated with the above complications [11]. 0167-5273/$ - see front matter D 2004 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2004.08.013 * Corresponding author. Tel.: +44 207 351 8872; fax: +44 207 351 8629. E-mail address: m.gatzoulis@rbh.nthames.nhs.uk (M.A. Gatzoulis). International Journal of Cardiology 97 (2004) 91 – 101 www.elsevier.com/locate/ijcard