Impact of Noonan Syndrome on admissions for pediatric cardiac surgery


Advocate Children's Hospital


Background: Noonan Syndrome is the second most common genetic syndrome associated with congenital heart disease. Many patients with Noonan Syndrome will require a cardiac intervention. This study aimed to characterize the difference in cardiac surgery admissions in patients with and without Noonan Syndrome.

Methods: Data regarding hospital admissions was collected using the Kids' Inpatient Database from 1997 to 2012. A cross-sectional study was conducted comparing baseline characteristics, cardiac morphology, cardiac surgery, and other comorbidities between those with and without Noonan Syndrome. Regression analysis was conducted to determine factors related to Noonan Syndrome and risk factors for increased length of hospitalization, need for ECMO, and inpatient mortality using Noonan Syndrome as the independent variable.

Results: A total of 46,169 admissions with cardiac surgery under 18 years of age were included in the final analyses. Of these 778 (1.6%) had Noonan Syndrome. Pulmonary stenosis, coronary anomalies, and valvuloplasty without valve replacement were independently associated with Noonan Syndrome. Those with Noonan Syndrome were 90% more likely to have chylothorax. Pediatric cardiac surgery admissions tended to be 4.5 days longer and cost $54,296 more in total charges with Noonan Syndrome. Inpatient mortality is also increased by Noonan Syndrome.

Conclusions: Noonan Syndrome is present in a relatively small proportion of pediatric cardiac surgery admissions. Noonan Syndrome is independently associated with increased length and cost of such admissions as well as inpatient mortality.

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