Challenging Management of Pulmonary Regurgitation in an Adult Patient With Tetralogy of Fallot and Pulmonary Arterial Hypertension

Pulmonary regurgitation following correction of tetralogy of Fallot (ToF) is a common postoperative complication associated with progressive right ventricular (RV) enlargement and dysfunction, and is an important determinant of late morbidity and mortality. Usually, pulmonary regurgitation is well tolerated for many years following surgery, but it can lead to progressive exercise intolerance, heart failure, tachyarrhythmias, late sudden death requiring often re-intervention. The appropriate timing of such intervention can be a challenging topic given the risk of prosthetic valve degeneration, the increased risk of reoperation and the decision depends on assessment of right ventricle size and function. Pulmonary hypertension (PH) is not an usual finding in ToF patient and it can be caused by pulmonary agenesis, hypoplasia and/or thrombosis, residual ventricular septal defect, or a large prior systemic-to-pulmonary shunt. Association of pulmonary arterial hypertension in a patient with ToF and pulmonary regurgitation who needs valve correction involves higher surgical risks and the management must be taken in a multidisciplinary team.