|Institution:||University of Cincinnati|
|Keywords:||Surgery; Hypoplastic left heart syndrome; lymphopenia; mortality; infections; chylous leak|
|Full text PDF:||http://rave.ohiolink.edu/etdc/view?acc_num=ucin1439305259|
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect, occurring in 2-3 infants per 10,000 births. These patients have a high mortality rate of about 40% within the first year of life, with death occurring between sequential, palliative surgeries in the newborn period. Recently, infants with HLHS have also been identified with T cell lymphopenia (TCL) by newborn screening for severe combined immunodeficiency; however, the incidence and impact of lymphopenia in newborns with HLHS is unknown. Following referral of an infant with HLHS, recurrent infections and T cell lymphopenia, we noted numerous iatrogenic causes for lymphopenia in the inter-surgical period. To determine the frequency of lymphopenia in infants with HLHS at our institution, we performed a retrospective review of patients evaluated between 2008 and 2014. Sixty-eight infants with HLHS were included, 44 of them had absolute lymphocyte counts (ALC) done in the inter-surgical period (0.7-1.9 months of age). Inter-surgical lymphopenia (34.1%) was far more frequent than pre-surgical lymphopenia (5.3%) (p= 0.0003). Several risk factors were found to be associated with inter-surgical lymphopenia, including chylous leak (p=0.01) and hydrocortisone stress dose (p=0.03). Lymphopenia was found to be associated with an increased risk for mortality (OR 4.36, CI 1.1-17.0, p=0.03) and a higher number of post-operative infections (OR 11.74, CI 1.9-71.0, p=0.01). Further on, ALC was found to be inversely correlated with superior vena cava pressure, suggesting that the association between lymphopenia and poor postoperative outcomes may be modulated by the severity of cardiac disease. These results suggest that inter-surgical lymphopenia in infants with HLHS is a marker for adverse postoperative outcomes during the first year of life. Advisors/Committee Members: Haynes, Erin Nicole (Committee Chair).