Improved outcomes for pediatric patients with congenital heart disease have created a growing population of surviving adults, with increasingly complex treatment requirements for their adult congenital heart disease (ACHD). Over the past two decades, the spectrum of complexity for ACHD patients has evolved from late primary repairs of patients with simple lesions including coarctation, patent ductus, septal defects, and tetralogy of Fallot, to nth time reoperations on survivors of complex multistaged palliations of one- and two-ventricle hearts. Internationally, significant efforts to define the optimal program resources necessary to effectively treat ACHD patients have been made, yet many congenital heart patients continue to suffer from poor continuity of care as they enter adulthood.1 This chapter describes several current treatment strategies and results for adult patients undergoing congenital heart surgery.
The optimal venue for ACHD treatment is unknown, and it is fair to say that no two programs are the same. The need for a coordinated and comprehensive programmatic strategy has been well described, and should encompass not only the lifetime of a generation of patients with congenital heart defects from fetal to adult life, but also the health of pregnant mothers with congenital heart disease who will give birth to a new generation.2 Our congenital heart program philosophy is to reduce the cumulative trauma of care for each patient with congenital heart disease over his or her lifetime. To achieve this, we discuss each adult patient referred to our program for surgery or intervention in a combined conference with participation from adult and pediatric interventional cardiologists and surgeons, dedicated cardiac intensivists, cardiac anesthesiologists, cardiac imaging specialists, and the cardiac nursing, pharmacy, and social work teams. Treatment options are selected so that the least traumatic form of therapy, with a reasonable chance of success, is chosen as the initial approach. Failure to achieve a good result leads to an escalation to the next least traumatic therapeutic option. As an example, a patient with secundum ASD would be put forward for device closure, and if this failed, the patient would then be given the option of a minimally invasive partial sternotomy. If this approach was not felt to be safe, the approach would be escalated to a full sternotomy repair.
ACHD patients with complex lesions often have complex medical histories, stored in multimedia formats, which expose the weaknesses of chart-based medical record systems. Use of an electronic medical record (EMR) by medical teams, and personal health record (PHR) by patients and families, may improve outcomes in patients with chronic disease.3 Patients and their families often have not been educated about their heart defect and what to expect over time. The PHR could be used to enhance patients' understanding of their disease and treatment. These information systems may also facilitate development of national and international registries designed to measure clinical outcomes and performance of centers treating adult congenital heart patients.4
Our program goal is to empower patients ...