Dearani, Joseph A.

Successful outcome in the care of patients with congenital heart disease depends on a comprehensive multidisciplinary team. Surgery is offered for almost every heart defect, despite complexity. Early mortality for cardiac surgery in the neonatal period is ≈10% and beyond infancy is <5%, with 90% to 95% of patients surviving with a good quality of life into the adult years. Advances in imaging have facilitated accurate diagnosis and planning of interventions and surgical procedures. Similarly, advances in the perioperative medical management of patients, particularly with intensive care, has also contributed to improving outcomes. Arrhythmias and heart failure are the most common late complications for the majority of defects, and reoperation for valvar problems is common. Lifelong surveillance for monitoring of recurrent or residual structural heart defects, as well as periodic assessment of cardiac function and arrhythmia monitoring, is essential for all patients. The field of congenital heart surgery is poised to incorporate new innovations such as bioengineered cells and scaffolds that will iteratively move toward bioengineered patches, conduits, valves, and even whole organs.

The Fontan procedure is now considered the final common pathway for patients with anatomical or functional single ventricle. These patients initially have their systemic and pulmonary circulations in parallel, supported by one functional ventricular chamber. The ultimate goal with this procedure is to separate the two circulations, to prevent mixing of venous and arterial blood, and to provide adequate tissue oxygenation. The objective of this article is to review the Fontan procedure with its various modifications and refinements since its introduction to clinical practice in 1971, by Fontan and Baudet.

Pulmonary artery translocation has been described as an alternative surgical strategy to treat anomalous aortic origin of the coronary artery from the wrong sinus of Valsalva. We describe another application of this strategy in a patient who underwent complete repair of pulmonary atresia with ventricular septal defect and presented years later with ischemic symptoms due to compression of the left main coronary artery by the right ventricular-to-pulmonary artery conduit. To our knowledge, this is the first application of the pulmonary artery translocation technique to treat such a problem.

A 60-year-old man with a dual-chamber implantable cardioverter defibrillator and severe dyspnea on exertion due to apical hypertrophic cardiomyopathy underwent a septal myectomy and radiofrequency maze procedure. Following the procedure a persistent delay in atrial sensing was observed and was most likely a result of iatrogenic conduction delay from right atrial ablation lines. These observations suggest that atrial conduction properties can be altered during the surgical maze procedure and should be considered in the differential diagnosis of sensing or pacing malfunction.

In patients with congenitally corrected transposition of the great arteries, the main concern has been the long-term performance of the morphologic right ventricle in association with tricuspid valve regurgitation when it remains as the systemic ventricle. Deterioration in ventricular function can occur slowly over many years, even without associated cardiac anomalies or previous surgical interventions. This review summarizes the authors' experience and provides a thorough review of the literature addressing the management of the failing systemic right ventricle as well as the tricuspid valve regurgitation with congenitally corrected transposition. This includes different surgical options, the authors' preferred management algorithm, and the late outcome.