A novel method for the evaluation and treatment of obstructive sleep apnea in four adults with complex congenital heart disease and Fontan repairs.

INTRODUCTION: Advances in cardiac diagnosis, medical therapy, and surgery have resulted in greater survival for patients with congenital heart disease (CHD). As a result, increasing numbers of CHD patients are surviving to adulthood with little knowledge regarding the consequences of comorbid medical conditions. Obstructive sleep apnea (OSA) is common and associated with hypertension, heart failure, and arrhythmias. Adults with certain types of CHD are at a higher risk for these and other cardiovascular complications than the general population. Therefore, OSA in adults with CHD may have a greater adverse impact on cardiovascular health than in the general population.
MATERIALS AND METHODS: We report four patients with CHD and OSA. All had conditions resulting in a hypoplastic right ventricle causing a single ventricle physiology, and all were treated surgically with a Fontan repair. RESULTS AND DISCUSSION: Because of their unique cardiac physiology, our cases were at risk for a further decrease in cardiac output and systemic perfusion with the application of continuous positive airway pressure (CPAP) therapy for OSA. We describe a method of identifying safe CPAP thresholds in the cardiac catheterization laboratory prior to titration of CPAP in the sleep laboratory in these patients.