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Small for Gestational Age Is An Important Pre-Operative Risk Factor for the Management of Patients with Hypoplastic Left Heart Syndrome

Friday, October 19, 2012
Room 275-277 (Morial Convention Center)
Anthony Sochet, MD1, Katherine Braley, MD1, Mark Ayers, MD1, Emilio Quezada, MD1, Elimarys Perez-Colon, MD2, Jennifer Leshko, RN3, David Cooper, MD4, Jeffrey Jacobs, MD5 and Gul Dadlani, MD1, (1)Pediatrics, All Children's Hospital and University of South Florida, St. Petersburg, FL, (2)Internal medicine/Pediatrics, All Children's Hospital and University of South Florida, St. Petersburg, FL, (3)Pediatric Cardiology, All Children's Hospital, St. Petersburg, FL, (4)Pediatric Cardiology, Cincinnati Chilren's Hospital, Cincinnati, OH, (5)Cardiac Surgery, All Children's Hospital and University of South Florida, St. Petersburg, FL

Purpose:    Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease associated with high mortality and morbidity.  Previous studies have linked decreased survival in HLHS with low birth weight and prematurity.  Fetal growth restriction is associated with end-organ changes that may increase the morbidity or mortality in infants undergoing cardiopulmonary bypass such as:  a decrease in pancreatic islet cell, loss of renal glomeruli and underdevelopment of pulmonary alveoli.  We performed a retrospective review of the morbidity and mortality associated with small for gestational age (SGA, birth weight <10th %) versus appropriate/large for gestational age (AGA/LGA, birth weight >10th %) infants born with HLHS undergoing Stage 1 Norwood surgical palliation. 

Methods: After IRB approval, a single institution review of all patients undergoing surgical palliation of HLHS from January 2007 to December 2011 was performed using the CardioAccess Database.   Patient demographics, cardiac anatomy, operative details, comorbidities and anomalies were recorded and analyzed.  Morbidities evaluated included: length of stay, genetic disorders/syndromes, abnormal brain imaging, seizures, duration of ventilatory support, extracorporeal membrane oxygenation (ECMO), vocal cord paralysis, renal abnormalities, renal failure requiring dialysis and G-tube placement.  P-values were calculated for markers of morbidity comparing neonates born SGA and AGA/LGA as shown in the table below.

Results:  Of the 47 patients identified with HLHS, 9 were SGA and 38 AGA/LGA.  The average birth weight for the SGA cohort was 2.61 kg and 3.3 kg for AGA/LGA infants.  Average gestational age was 38.6 weeks for SGA and 38.4 for AGA/LGA.  Compared to infants born AGA, SGA had a higher mortality (44.4% vs. 21.1%). Postoperatively, infant born SGA spent an average of 14.4 additional days mechanically ventilated than their AGA/LGA counterparts (25.2 vs. 10.8).  Although mortality, days of mechanical ventilation and need for ECMO support were higher in the SGA group our p-values were not statistically significant secondary to the small sample size.

Conclusion:  Small for gestational age is an important pre-operative risk factor to consider prior to staged surgical palliation in patients with HLHS.  Pre-operative family counseling and education may be beneficial to families with SGA neonates due to the potentially increased risk of mortality, length of mechanical ventilation and need for ECMO support. 



SGA , n=9

AGA/LGA, n=38


Avg Birth Weight (kilograms)




Avg Gestational Age (weeks)





4 (44.4%)

8 (21.1%)


Genetic Disorders/Syndrome

1 (11.1%)

4 (10.5%)


Abnormal Brain Imaging

5 (55.6%)

18 (47.4%)


Seizure Activity

2 (22.2%)

12 (31.6%)


Duration of intubation (days)




Need for ECMO

3 (33.3%)

8 (21.1%)


Vocal Cord Paralysis

2 (22.2%)

11 (28.9%)


Abnormal Renal Ultrasound

2 (22.2%)

7 (18.4%)


Required Dialysis

1 (11.1%)

1 (2.6%)


G-tube Placement

4 (44.4%)

26 (68.4%)