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Survival, neurocognitive, and functional outcomes after completion of staged surgical palliation in a cohort of patients with hypoplastic left heart syndrome

Journal of the American Heart Association Feb 17, 2020

Atallah J, Guerra GG, Joffe AR, et al. - By analyzing 117 consecutive patients with hypoplastic left heart syndrome who underwent surgical palliation at a single center from 1996 to 2010, researchers assessed demographic as well as perioperative factors from each surgical stage for their link with survival and neurocognitive outcomes among these patients in this cohort study. Survival to the Fontan stage was seen in 70 patients (60%), and 68 patients (58%) survived to experience neurocognitive evaluation at a mean (SD) age of 56.6 months (6.4 months). The full-scale, performance, and verbal intelligence quotient, and visual-motor integration mean (SD) scores were estimated to be 86.7 (16.1), 86.3 (15.8), 88.8 (17.2), and 83.2 (14.8), respectively. A strong link with lower scores for all intelligence quotient domains was displayed by the following factors in multivariable analysis: older age at Fontan, sepsis peri-Norwood, lowest arterial partial pressure of oxygen postbidirectional cavopulmonary anastomosis, and presence of neuromotor disability pre-Fontan. In patients with hypoplastic left heart syndrome, poor neurocognitive outcomes were independently predicted by older age at Fontan and sepsis.
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