Abstract
Single ventricle physiology, including hypoplastic left heart syndrome, encompasses the most severe cardiac lesions. Despite improvements in palliation and care, failure to thrive remains a major concern specifically among the neonatal and infant population. This population may develop other nutritional challenges throughout the life span, which adversely affect growth, morbidity, and mortality. This review of the literature uniquely describes the staged surgical palliation and discusses clinical practice related to nutritional complications associated with the disease process, such as chylothorax and protein-losing enteropathy. A registered dietitian is an essential part of the multidisciplinary team to provide expert medical nutrition therapy for this challenging population.