The impact of a dedicated single-ventricle home-monitoring program on interstage somatic growth, interstage attrition, and 1-year survival

Objective: There has been considerable improvement in survival after the first stage of palliation for single-ventricle heart disease. Yet, interstage mortality continues to plague this population. Home monitoring has been proposed to reduce interstage mortality. We review our experience after creation of a Single Ventricle Program.

Methods: All infants with a single ventricle heart defect who were admitted to Texas Children's Hospital from the inception of the Single Ventricle Program on September 1, 2007, to January 1, 2010, were included in the Single Ventricle Program cohort. Infants with a single ventricle presenting between January 1, 2002, and August 31, 2007, comprised the pre-Single Ventricle Program group. Anatomic, operative, and postoperative details were noted for all patients. End points included in-hospital death after the first stage of palliation, interstage death (defined as after discharge from the first stage of palliation and before the second stage of palliation), and death or heart transplantation by 1 year of age. Interstage weight gain was also compared.

Results: A total of 137 infants with a single ventricle were included in the pre-Single Ventricle Program cohort, and 93 infants were included in the Single Ventricle Program cohort. Anatomic subtypes were similar between groups. There was significant improvement in rate of interstage weight gain, whereas age at the second stage of palliation was significantly reduced in the Single Ventricle Program group. In-house mortality decreased during the Single Ventricle Program era (P = .021). Interstage mortality did not significantly decrease in the Single Ventricle Program group. However, 1-year transplant-free survival improved during the Single Ventricle Program era (P = .002).

Conclusions: The Single Ventricle Program improved interstage weight gain, thereby allowing for early second-stage palliation at an equivalent patient weight. Interstage mortality was not significantly reduced by our program. However, 1-year transplant-free survival was significantly improved in patients in the Single Ventricle Program.