Optimal management strategies for patients with complex congenital heart disease.

The combination of critical pulmonary outflow obstruction with a large ventricular septal defect is symbolized by the diagnosis of tetralogy of Fallot, the most common form of cyanotic congenital heart disease. In many ways, the history of management for patients with tetralogy is representative of the evolution of management for all children with complex congenital cardiac diagnoses. Tetralogy was the first complex lesion addressed surgically with the palliative Blalock-Taussig shunt in 1945 and the first repaired completely with patients’ mothers serving as the bypass machine in 1954.1,2 The introduction of valved conduits in 1964 allowed repair of lesions not previously considered amenable to correction.3 The next 3 decades were marked by improved survival at open heart repair, recognition of the progressive deterioration of valved conduits necessitating early and repeated reoperation, and a progressive decrease in the age of primary repair to now the first few months of life.4,5 In the late 1970s, reports of arrhythmia and late sudden death among tetralogy survivors led to evaluation of tetralogy cohorts with residual right ventricular (RV) volume and/or pressure overload identified as predictors of late complications.6,7 Recognition of this problem heightened appreciation of the importance of arrhythmias in postoperative congenital heart disease patients and identified the need for informed long-term care and attention to residua of earlier procedures for all patients with congenital heart disease.8 Finally, the last 20 years have witnessed the dramatic rise in interventional procedures to address both correction of simple lesions and palliation/management of complex diagnoses.9