ABSTRACT
Introduction: We report a series of operated tetralogy of Fallot (TOF) patients focusing on complications and outcomes. Materials and Methods: Data from TOF patients seen at our centre’s adult congenital heart disease clinic was analysed. Results: There were 21 patients: the mean age was 32.2 ± 12.4 years; the age at first operation was 9.0 ± 7.9 years; the mean postoperative follow-up duration was 23.5 ± 12.1 years; and the current New York Heart Association (NYHA) status: I, 82%; II, 4%; III, 14%. Fourteen patients had complete operative notes. All these patients underwent total TOF correction; 2 had staged aortopulmonary shunt with total correction at a mean of 3.2 years later, pulmonary artery patch augmentation in 8 patients and pulmonary valvotomy in 8 patients. Three patients required pulmonary valve homograft replacement for severe pulmonary regurgitation (PR) at 13, 28 and 36 years after the initial corrective operation. Current investigations: RBBB on ECG (91%), QRS duration 137 ± 29 ms. Echocardiography showed dilated right ventricular end-diastolic (RVED) diameters (3.2 ± 0.8 cm); severe PR (67%), residual right ventricular outflow tract obstruction (RVOTO) (42%) and VSD patch leakage (9%). Cardiac magnetic resonance (CMR) (8 patients) showed dilated RVED volumes 252.6 ± 93.8 mL, indexed RV volume 165.7 ± 34.8 mL; RV systolic function was preserved in most patients with a RV ejection fraction of 49.5 ± 5.7%. One patient had atrial tachycardia and another had frequent non-sustained ventricular tachycardia that required radiofrequency ablation. Conclusion: Patients with TOF who had full corrective surgery during childhood are now surviving into adulthood. Many challenges arising from complications in the postoperative period remain. It is imperative that adult TOF patients should have regular follow-up to monitor development and subsequent management of these complications.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease with an incidence of approximately 0.5/1000 live births (5% to 7% of congenital heart lesions). In Singapore with a live birth of over 37, 000 per year,1 the expected number of new TOF cases will be approximately 19 per year. Based on data obtained from the National Birth Defects Registry, there were 164 TOF cases amongst all live and stillbirths between 1994 and 2000.2 In classical TOF, an anterior and cephalic displacement of the infundibular septum results in a large ventricular septal defect (VSD) and the development of infundibular pulmonary stenosis. Right ventricular (RV) hypertrophy is associated with both pulmonary stenosis and VSD (Fig. 1). Without surgical intervention, most patients die in childhood with a rate of survival of 66% at 1 year of age, 40% at 3 years, 11% at 20 years and 3% at 40 years .3 The advent of surgical repair which includes closure of the VSD and relief of RV outflow tract (RVOT) obstruction has greatly improved the long-term survival of TOF patients. Complete repair of TOF in early childhood is now routinely available in tertiary centres in Singapore. High survival rates in TOF patients who underwent complete repair have been reported.4,5
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