Natural history of children after tetralogy of Fallot repair.

BACKGROUND

Our knowledge of tetralogy of Fallot repair (rTOF) outcomes is derived from older reports employing evolving surgical and pre/post-operative care strategies. We sought to describe the "natural history" of rTOF patients repaired in the contemporary era.

METHODS

Single center retrospective cohort study of TOF patients repaired between 2000 and 2015. Patients had clinically directed follow-up pre-operatively and post- until age 18-years with ECG, echocardiography, Holter, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing.

RESULTS

95 rTOF patients (61% male) were followed for a median of 15.7 (range 8-18) years. Median age at repair was 6-months [IQR 5-6 months] with 52 patients (55%) undergoing valve-sparing repair (VSR). One patient (1%) had post-operative atrioventricular block, three (3.2%) had atrial arrhythmias, and four additional patients (4.2%) had ventricular tachycardia. There were no deaths. Nine patients (9.5%) underwent pulmonary valve replacement. Exercise capacity (p < 0.001), right and left ventricular ejection fraction (p = 0.03 for both) decreased significantly between 8 and 18 years. VSR patients had lower right ventricular volumes and pulmonary insufficiency (p < 0.001).

CONCLUSIONS

Despite normal growth and excellent survival, rTOF patients exhibit decreased right ventricular function, progressive exercise intolerance and incident ventricular and atrial arrhythmias in adolescence.

IMPACT: Our understanding of tetralogy of Fallot repair (rTOF) outcomes is derived from older cohorts, repaired at an older age with different surgical approaches. Patients with rTOF experience declining exercise capacity throughout childhood and incident arrhythmias starting in adolescence. Valve-sparing repair technique was associated with lower right ventricular volumes and pulmonary insufficiency, but no difference in ventricular function or exercise capacity. This is the first report of outcomes of a contemporary cohort of rTOF patients followed throughout childhood with guideline-based multi-modal testing. This study informs regarding prognosis after repair and guides interventions to preserve exercise tolerance and ongoing surveillance.