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Long-term survival after complete repair for tetralogy of Fallot is close to that of the general population, but not equal. This is probably the result of the combined effects of right ventricular failure and ventricular arrhythmias that may lead to sudden death." 2

In Leiden, the cardiothoracic surgeon Brom started the total correction of tetralogy of Fallot as early as the late 1950s. Now we are confronted with an increasing number ofpatients who present with symptomatic right ventricular failure, usually the result ofpulmonary regurgitation, sometimes 40 years after the initial repair. Predominant symptoms are diminished exercise capacity and supraventricular and/or ventricular arrhydhmias. A useful marker for ventricular arrhythmias and sudden death is the duration ofthe QRS complex on the surface ECG of 180 ms or more. Previously, it was thought that pulmonary regurgitation in Fallot patients was not really harmful. Recent accurate measurements of right ventricular volumes using magnetic resonance imaging (MRI) have visualised the enormous enlargement ofthe right ventricle in patients with severe pulmonary regurgitation.3 Replacement ofthe pulmonary valve in severe regurgitation has shown to lead to an impressive reduction of right ventricular volumes, an increase in validity class, and reduction ofthe QRS duration (the marker of severe arrhydhmias).46 In Leiden University Medical Centre, MRI is now the imaging tool of choice for the assessment of the cardiac status in the Fallot patient, since it allows measurement of both right and left ventricular fumction, with special focus on the right ventricular volume; measurement of the degree of pulmonary regurgitation; detection and quantification of central and peripheral pulmonary stenosis; measurement of the degree of tricuspid regurgitation; and quantification of shunt fraction in residual ventricular septal defect.3 A regurgitant pulmonary valve is usually replaced by a pulmonary homograft. Pulmonary homografts last longer than aortic homografts in the pulmonary position. Nevertheless, homografts may suffer from calcific degeneration and usually need replacing at a later stage.7 Some surgeons use mechanical valves in the pulmonary position, but since the patients are usually young, and females may develop pregnancy wish, we prefer to avoid life-long anticoagulation. Our experience with homograft pulmonary valve replacement late after correction of Fallot's tetralogy now consists of 60 patients. Patients with pulmonary atresia or a conduit inserted at initial repair, patients with absent pulmonary valve syndrome or those presenting with an important residual VSD or predominantly residual pulmonary stenosis are not included in this series. The mean interval between Fallot repair and later pulmonary homograft insertion is 19.3 years (range: 2.7-40.3 years). Hospital mortality is 1.7% while one patient died 18 months after uncomplicated surgery, probably because ofventricular rhythm disturbances. Symptomatic improvement was striking: after six months validity class improved from mean NYHA class 2.3 to 1.4. Comparison of preoperative and post-

Netherlands Heart Journal, Volume 9, Number 7, October 2001

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Editorial

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operative MRI scans showed significant reduction of right ventricular volumes one year after pulmonary valve insertion. The fraction of pulmonary regurgitant volume decreased from a mean 48% preoperatively to mean 4% after pulmonary homograft implantation.6 Optimal timing of pulmonary valve replacement is still a subject of debate as the amelioration of right ventricular function has to be weighed against the risk of later reoperation for homograft failure. A scoring system is under development to define the optimal moment of pulmonary valve replacement. Several parameters should be included, such as functional validity class, right ventricular dimensions and ejection fraction, degree of pulmonary regurgitation, degree of tricuspid regurgitation, degree and nature of arrhythmias, QRS duration, residual ventricular septal defect, and right ventricular outflow tract obstruction.

Much work is still to be done for this new and young adult population that has evolved from the dramatic progress of medicine and surgery. v

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H. W. Vliegen, M.G. Hazekamp. Leiden University Medical Centre, Leiden.

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Horneffer PJ, Zakha KG, Rowe SA, Man-olio TA, Gott VL, Reitz BA, et al. Long-term results of total repair of tetralogy in childhood. Ann Thorac Surg 1990;50:179-85. Gatzoulis MA, Till JA, Sommerville J, Reddington AN. Mechanoelectrical interaction in tetralogy ofFallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation 1995;92:231-7. Rebergen SA, Chin JG, Ottenkamp J, Wall EE van der, Roos A de. Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear resonance velocity mapping. Circulation 1993;88:2257-66. Conte S, Jashari R, Eyskens B, Gewillig M, Dumoulin M, Daenen W. Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction. EurJ Cardio Thorac Surg 1999;15:143-9. Yemets IM, Wllliams WG, Webb GD, Harrison DA, McLaughlin PR, Trusler GA, et al. Pulmonary valve replacement late after repair of tetralogy of Fallot. Ann Thorac Surg 1997; 64:526-30. Hazekamp MG, Kurvers MMJ, Schoof PH, Vliegen HW, Mulder BM, Roest AAW, et al. Pulmonary valve insertion late after repair of Fallot's tetralogy. EurJ Cardio Thorac Surg 2001 ;19:667-70. Niwaya K, Knott-Craig CJ, Lane MM, Chandrasekaren K, Overholt ED, Elkins RC Cryopreserved homograft valves in the pulmonary positionl: risk analysis for intermediate-term failure. J Thorac Cardiovasc Sur,g 1999;117:141-6.

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Netherlands Heart Journal, Volume 9, Number 7, October 2001