Congenital heart surgery in the adult.

We are only at the “tip of the iceberg” with respect to our recognition and treatment of the burgeoning population of adults with congenital heart disease (ACHD). The latest data place the number of adults with congenital heart disease in the United States at 1 million, and that number is growing at 5% per year.1 Unfortunately, many ACHD patients do not receive routine follow-up care, so exact numbers do not exist—but recognition is growing that healthcare delivery for ACHD patients needs to improve. In 2008, the American College of Cardiology (ACC) and the American Heart Association (AHA) published guidelines for the management of ACHD patients.2 These guidelines stressed the need for the development and strengthening of transition clinics for adolescents and young adults with CHD. They also mandated a more formal education program for ACHD specialists, to ensure optimal management of patients. This is a fundamental issue that will need to be confronted as the population of ACHD patients grows. The number of sufficiently trained ACHD cardiologists is inadequate, and appropriately trained congenital heart surgeons are even scarcer. The importance of having a trained congenital heart surgeon to perform surgery in ACHD patients was highlighted in a recent study that examined national practice patterns for the surgical management of ACHD patients.3 The sample included 30,250 index cardiac operations: 73% were operations on children and 27% were operations on ACHD patients. When looking at the same diagnostic group, these investigators found that congenital heart surgeons performed the pediatric operations 68% of the time, and non-congenital heart surgeons performed the ACHD operations 95% of the time. Also, the hospital mortality rate was higher in ACHD patients who were operated on by non-congenital heart surgeons (4.84%; 95% confidence interval, 4.30–5.38) than in ACHD patients who were operated on by congenital heart surgeons (1.87%; 95% confidence interval, 0.62–3.13) (P <0.0001). Other specific recommendations from the ACC/AHA guidelines included the eventual issuance of a “medical passport” to provide a mechanism for the ready availability of patient history information, the designation of medical guardians, collaboration between specialists, access to specialized follow-up care, and the identification of regional centers of excellence for ACHD. Examples of case scenarios that warrant consultation and treatment at an ACHD center include admission for urgent care, or for diagnostic or interventional procedures; procedures requiring general anesthesia or conscious sedation; acute care of a cardiac problem; and acute care of a non-cardiac problem in high-risk patients. Currently, at Texas Children's Hospital, the management of ACHD patients is in evolution. Congenital heart surgery at this institution has a long history that began in 1955 with Dr. Denton Cooley. The program was reorganized as a dedicated congenital heart surgery service in 1995 and has continued to grow through the years. Most of our patients are newborns and infants. Outcomes for this cohort of patients have improved drastically in the last 20 years, and, as a result, more children with congenital heart disease are surviving into adulthood. One particular condition, hypoplastic left heart syndrome, has seen great improvement in surgical survival in the last decade. Not only are these patients surviving—they are guaranteed further surgical care, because their treatment involves a series of staged palliative operations. To provide a mechanism for transitioning the care of the ACHD patients to specialists within the region who can offer appropriate follow-up care, the Texas Adult Congenital Heart (TACH) Center was formed in 2004. The center comprises internists and pediatricians who focus on ACHD patients and congenital heart surgeons who are trained in both adult cardiovascular surgery and pediatric cardiac surgery. The center involves a network of adult and pediatric institutions that provide varying degrees of physical and human resources and support by specialists. Since the inception of the center, we have observed that a strong ACHD program requires strong support from all involved institutions. Although there are benefits to this institutional collaboration, some challenges remain. Issues that require further development and agreement are identifying the best hospitals for congenital heart surgery in ACHD patients and standardizing appropriate cardiopulmonary bypass strategy, anesthetic management, intraoperative monitoring, and intensive care unit monitoring. Moreover, it is important to determine how to accommodate the many ACHD patients who have no insurance. Information sharing can also become problematic when records are located at multiple facilities. There are 4 common paradigms in which ACHD patients present for surgical consideration. The first is as an undiagnosed or incorrectly diagnosed patient with a noncyanotic lesion, such as complete atrioventricular (AV) canal, partial anomalous pulmonary veins, and anomalous coronary artery origin. The second is the previously “cured” patient with tetralogy of Fallot, transposition of the great arteries, corrected transposition, and coarctation. The third is the previously palliated patient, such as one with a shunted single ventricle or one who has undergone an atriopulmonary Fontan procedure. The fourth and final category includes patients with heart failure who are often listed for heart transplantation before their referral. These last 3 paradigms arise in the setting of decreased ventricular function. To illustrate one of these scenarios, consider the following case of a 17-year-old boy with corrected transposition of the great arteries, a ventricular septal defect, pulmonary stenosis, and a displaced left AV valve, who presented at our institution with heart failure, cyanosis, decreased exercise tolerance, and 3rd-degree heart block.4 Hemodynamic data from a cardiac catheterization revealed a well-protected pulmonary vasculature, low pulmonary vascular resistance, reasonable biventricular function, apical displacement of the left AV valve with at least moderate insufficiency, and a nonrestrictive ventricular septal defect. After careful review of the cardiac anatomy, we concluded that the most reasonable option for a biventricular repair in this individual would be a double-switch procedure, most likely a Senning atrial switch with a Rastelli repair, which would commit the morphologic left ventricle to the systemic circulation. After a median sternotomy and aortobicaval cannulation, we performed a right atriotomy, confirmed the anatomy, and decided that the creation of an unobstructed left ventricle-to-aorta baffle was possible (Fig. 1). An appropriate baffle was sewn in place through a longitudinal right ventriculotomy. After this, inspection revealed that the combination of the baffle and the dysplastic left-sided AV valve had reduced the effective size of the right ventricle to the extent that it might not accommodate the entire systemic venous return. This was remedied by performing a hemi-Mustard atrial switch procedure and a bi-directional Glenn anastomosis, cutting back the coronary sinus in such a manner as to include it in the systemic venous atrium (Fig. 2). The main pulmonary artery was transected and oversewn, a 22-mm valved conduit was placed from the morphologic right ventricle to the pulmonary artery bifurcation (Fig. 3), and a dual-chamber epicardial pacemaker was placed. The patient's recovery was uncomplicated except for a persistent pericardial effusion, which was managed with a pericardial drain. This patient is currently monitored by the TACH Center. He is fully saturated and asymptomatic. He has full exercise capacity and reports an excellent quality of life. Fig. 3 The completed repair. Fig. 2 Both a hemi-Mustard atrial switch procedure and a bi-directional Glenn anastomosis were performed to accommodate the entire systemic venous return. Fig. 1 Illustration of the patient's cardiac anatomy, which included corrected transposition of the great arteries, a ventricular septal defect, pulmonary stenosis, and a dysplastic atrioventricular valve. In conclusion, excellent surgical outcomes and a high quality of life can be achieved in ACHD patients. The challenge lies in replicating these outcomes consistently through an effective care-delivery model for ACHD patients. Educational programs must respond to the increased demand for specialized ACHD practitioners, ACHD centers must have the proper resources, and specialists must collaborate. Finally, we need more data on outcomes in this population, so that we can refine expectations and optimize care.