Pulmonary Valve Replacement

Monica E. Dugan, RN, MS, APRN, CRNFA, James S. Tweddell, MD


Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. Repair of TOF, first performed over half a century ago, is one of the great success stories of congenital heart surgery.[1] The goals of complete surgical repair of TOF include elimination of intracardiac shunting and relief of right ventricular (RV) outflow tract (OT) obstruction. Pulmonary regurgitation (PR) is a nearly constant feature of repaired TOF and was originally thought to be a benign[2]; however, long-term studies have shown that chronic PR will predictably result in right ventricular dilatation, biventricular dysfunction, arrhythmias, worsening exercise intolerance, and sudden death (Figure 1).[3],[4],[5] Proper timing and technique of pulmonary valve replacement (PVR) is essential to maintain the long-term survival and quality of life of individuals with TOF.[6]

Figure 1
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Long-term survival curve of 490 early survivors of repair of TOF. The curve shows 2 different phases that are distinct. The early, low risk phase lasts 25 years; thereafter, the risk increases significantly. Mortality risk (r) per year, as a linearized number, is calculated for each phase.

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Last updated: September 29, 2020