Surgically repaired tetralogy of Fallot in the 7th decade: a late presentation of severe pulmonic regurgitation

Egypt Heart J. 2024 Apr 14;76(1):46. doi: 10.1186/s43044-024-00477-3.

Abstract

Background: Surgically repaired tetralogy of Fallot (TOF) is a congenital heart disease with a cumulative survival rate of 72% in the 4th decade of life in longitudinal single-cohort studies. Debate surrounds conservative versus surgical management in adults with TOF once pulmonary regurgitation occurs.

Case presentation: A 73-year-old male with surgically corrected TOF presented with heart failure symptoms. He underwent ToF repair with a classic right Blalock-Taussig shunt at 2 years of age with transannular patching at 18 years of age. Echocardiography revealed elevated right ventricular systolic pressures, severe right ventricular dilatation, and pulmonary regurgitation. Our patient's new-onset right-sided heart failure was managed medically with diuresis. He received a new pulmonic valve via percutaneous approach on a later planned hospitalization with resolution of symptoms and improved tricuspid regurgitation.

Conclusion: It is a class I recommendation for pulmonic valve intervention once greater than moderate PR occurs; however, medical optimization should take place first. Following adequate RV load optimization, our patient underwent successful transcatheter pulmonic valve implantation with resolution of symptoms and cessation of diuretic.

Keywords: Adult congenital heart disease; Guidelines; Heart failure; Pulmonic regurgitation; Seventh decade; Surgical repair; Tetralogy of Fallot.