Dead space fractions in neonates following first-stage palliation for hypoplastic left heart syndrome.

PURPOSE: (1) To characterise changes in dead space fraction during the first 120 post-operative hours in neonates undergoing stage 1 palliation for hypoplastic left heart syndrome, including hybrid procedure; (2) to document whether dead space fraction varied by shunt type (Blalock-Taussig shunt and Sano) and hybrid procedure; and (3) to determine the association between dead space fraction and outcomes. METHODS: Retrospective chart review in neonates undergoing stage 1 palliation for hypoplastic left heart syndrome in a cardiac intensive care unit over a consecutive 30-month period. A linear mixed model was used to determine the differences in dead space over time. Multivariable linear regression and a multivariable linear mixed model were used to assess the association between dead space and outcomes at different time points and over time, respectively. RESULTS: Thirty-four neonates received either a Blalock-Taussig shunt (20.5%), Sano shunt (59%), or hybrid procedure (20.5%). Hospital mortality was 8.8%. Dead space fractions in patients undergoing the hybrid procedure were significantly lower on day 1 (p = 0.01) and day 2 (p = 0.02) and increased over time. A dead space fraction >0.6 on post-operative days 3-5 was significantly associated with decreased duration of mechanical ventilation in all surgical groups (p 0.6 on post-operative days 3-5 was associated with lower duration of mechanical ventilation in all surgical groups. A more comprehensive, prospective assessment of dead space in this delicate patient population would likely be beneficial in improving outcomes.

Congenital Disorders of Red Blood Cells.

See Bonus NeoBriefs videos and downloadable teaching slides Understanding the physiologic process of red blood cell development in utero and subsequent erythropoiesis in the neonate is crucial as this determines red blood cell structure and therefore function, which is vital to neonatal health. Infants frequently experience anemia, and special consideration must be given to the evaluation of these infants to determine the correct etiology. Traditionally, anemia is conceptualized in terms of inadequate red blood cell production, increased red blood cell destruction, or whole blood loss. This framework translates well to inherited red blood cell defects, which include genetic abnormalities in bone marrow productivity or structure of the red blood cell membrane, enzymes, or hemoglobin. This article highlights fetal and neonatal erythropoiesis and the underlying etiologies of the inherited red blood cell disorders, as well as reviews the appropriate diagnostic evaluation and next steps in management. It is imperative that neonatal clinicians remain informed about these disorders to enable early recognition and treatment, and ultimately to improve outcomes in affected infants.