Chapter 20: Congenital Heart Disease

Key Points

• Congenital heart disease comprises a wide morphologic spectrum. In general, lesions can be conceptualized as those that can be completely repaired, those that should be palliated, and those that can be either repaired or palliated depending on particular patient and institutional characteristics.

• Percutaneous therapies for congenital heart disease are quickly becoming important adjuncts, and in some cases, alternatives, to standard surgical therapy. Important examples include percutaneous closure of atrial and ventricular septal defects, the hybrid approach to hypoplastic left heart syndrome, radiofrequency perforation of the pulmonary valve, and percutaneous pulmonary valve placement. Further studies are necessary to establish criteria and current benchmarks for the safe integration of these novel approaches into the care of patients with congenital heart surgery.

• Patients with critical left ventricular outflow tract obstruction, such as neonatal critical aortic stenosis, represent a challenging population. It is critical that the correct decision (whether to pursue univentricular or biventricular repair) be made prior to the initial operation, as attrition when the incorrect decision is made is high. There are several published criteria (Congenital Heart Surgeons’ Society critical stenosis calculator) to help surgeons decide which strategy to pursue.

• Optimum strategy for repair of total anomalous pulmonary venous connection (TAPVC) remains a topic of some contention. Sutureless repair, formerly reserved for initial restenosis after conventional repair, has evolved in many centers to be the primary approach for high-risk patients. Defining whether sutureless repair should be considered in all patients with TAPVC will require further study.

• Vascular rings and pulmonary artery slings often require multidisciplinary approaches for management. They can be associated with complete tracheal rings and tracheobronchomalacia.

• A recent prospective, randomized, multi-institutional trial sponsored by the National Institutes of Health, the Systemic Ventricle Reconstruction (SVR) trial, compared the outcomes of neonates with hypoplastic left heart syndrome having either a modified Blalock–Taussig shunt (MBTS) versus a right ventricle-to-pulmonary artery (RV-PA) shunt. The SVR trial demonstrated that transplantation-free survival 12 months after randomization was higher with the RV-PA shunt than with the MBTS. However, data collected over a mean follow-up period of 32 ± 11 months showed a nonsignificant difference in transplantation-free survival between the two groups.

• Outcomes have improved substantially over time in congenital heart surgery, and most complex lesions can be operated in early infancy. Neurologic protection, however, remains a key issue in the care of neonates undergoing surgery with cardiopulmonary bypass and deep hypothermic circulatory arrest. New monitoring devices and perioperative strategies are currently under investigation. Attention in the field has shifted from analyses of perioperative mortality, which for most lesions is under 10%, to longer-term outcomes, including quality of life and neurologic function.

• Pediatric mechanical circulatory support and heart transplantation is an upcoming and rapidly evolving component of congenital heart surgery. These are offering options for rescue, palliation, and treatment of complex defects or children who were palliated and failing.

• Public reporting has become an integral part of this subspecialty. The Society of Thoracic Surgeons Congenital Heart ...