A case of heart failure due to multiple late complications after repair of tetralogy of Fallot in adulthood

Abstract

The prognosis of tetralogy of Fallot (TOF) has improved in recent years, but complications in the late postoperative period remain a serious problem. These complications, combined with specific hemodynamic and structural abnormalities, make it difficult to determine the optimal treatment plan. A man in his early 60s had been diagnosed with TOF in his early teens; he had undergone ventricular septal defect closure and right ventricular outflow tract repair in his mid-20s. Approximately 40 years after surgery, he was referred to our hospital because of worsening heart failure due to moderate aortic regurgitation with left ventricular dysfunction caused by a residual ventricular septal defect, marked continuous right ventricular dilation and dysfunction, and severe pulmonary and tricuspid regurgitation. The patient had clearly missed the optimal time for surgery and had a high surgical risk score. After the patient had been provided sufficient information regarding treatment and risks, he underwent pulmonary and aortic valve replacement, tricuspid annuloplasty, and shunt closure. No obvious perioperative complications were observed, and the heart failure had remained stable for 4 years following reoperation. We report this complicated case of TOF repaired in adulthood with marked biventricular remodeling, associated with a residual shunt and progression of valvular disease.

Learning objective

Surgical interventions for the multiple complications that occur during the long-term course after TOF repair, particularly in the context of right ventricular remodeling, may be associated with risks. In the field of adult congenital heart disease, rather than applying a single cut-off to determine the optimal timing for intervention, clinicians should consider factors such as the patients' age, sex, and other individual characteristics, paying particular attention to the hemodynamic status.