Pulmonary valve replacement based solely on right ventricular size is appropriate in the asymptomatic tetralogy patient

Abstract

Although the long term outcome of patients with repaired tetralogy of Fallot (TOF) is favorable [1], the initial repair to relieve the right ventricular (RV) outflow tract obstruction often results in significant pulmonary regurgitation (PR). PR in the repaired TOF patient is usually well tolerated for long periods of time. The low resistance, high capacitance reservoir of the pulmonary circulation minimizes the actual regurgitant volume in the face of severe PR. However, over time, chronic PR results in volume overloading of the RV [2]. Volume overloading of the RV eventually leads to an increase in right ventricular end diastolic volume (RVEDV), right ventricular end systolic volume (RV-ESV), and RV systolic dysfunction [3]. The ensuing RV dilatation and dysfunction for one predispose the patient to exercise intolerance [4], congestive heart failure, and atrial and ventricular arrhythmias, and may contribute to the incidence of sudden cardiac death [5]. In order to know whether pulmonary valve replacement (PVR) based solely on RV size is appropriate in the asymptomatic tetralogy of Fallot (TOF) patient we will review: 1) the effect of long term PR in patients with repaired TOF; 2) the results and hemodynamic effect of surgical as well as percutaneous PVR; 3) what we know about timing of PVR in these patients; and, finally, 4) the known determinants of morbidity and mortality in repaired TOF patients with severe PR.