Cerebral Blood Flow Following Hybrid Stage I Palliation in Infants with Hypoplastic Left Heart Syndrome
Hypoplastic left heart syndrome (HLHS) palliation may result in altered cerebral blood flow with subsequent neurodevelopmental implications. The purpose of the study was to assess blood flow in the middle cerebral artery (MCA) and investigate the relationship with early neurodevelopmental outcomes in infants with HLHS after hybrid stage I. Transcranial Doppler (TCD) was performed to obtain peak systolic, end-diastolic, and mean velocities, as well as pulsatility index of the MCA in infants with HLHS (n = 18) at baseline and at 2, 4, and 6 months of age. Developmental assessment was performed at 6 months of age. Results of TCD and development were compared to healthy control subjects (n = 6) and normative data. Overall, peak systolic velocity (p = 0.0031), end-diastolic velocity (p < 0.0001), and mean velocity (p < 0.0001) were significantly lower and pulsatility index (p = 0.0011) significantly higher in the HLHS group compared to the control group. A significant increase in change over time was noted for peak systolic velocity (p < 0.0016) and mean velocity (p < 0.0046). There was no significant correlation between TCD variables and development scores. TCD values in 5–6 months old infants with HLHS who undergo hybrid stage I palliation had consistently lower blood flow velocities than control infants; however, pulsatility index was slightly higher. No correlation between TCD measurements and measures of cognitive, language, and motor skills were noted.
KeywordsCongenital heart disease Hypoplastic left heart syndrome Neurodevelopment Hybrid
Congenital heart disease
Hypoplastic left heart syndrome
Patent ductus arteriosus
Mental development index
Psychomotor development index
Bayley scales of infant and toddler development, 3rd edition
Centimeters per second
Peak systolic velocity
Sharon L. Cheatham: Dr. Cheatham conceptualized and designed the study, coordinated and provided data collection for both groups, carried out the initial analyses, prepared the manuscript, and approved the final manuscript as submitted. Joanne L. Chisolm: Ms. Chisolm coordinated data collection, critically reviewed the manuscript for important intellectual content, and approved the final manuscript as submitted. Nicole O’Brien: Dr. O’Brien provided data collection for the HLHS group, critically reviewed and revised the manuscript for important intellectual content, and approved the final manuscript as submitted.
Compliance with Ethical Standards
Conflict of interest
The authors have no other conflict of interest.
This study was approved by the Institutional Review Board and has therefore been performed in accordance with ethical standards. Informed consent was obtained on all subjects prior to their inclusion in the study.
- 1.Akintuerk H, Michel-Behnke I, Valeske K, Mueller M, Thul J, Bauer J, Hagel KJ, Kreuder J, Vogt P, Schranz D (2002) Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart. Circulation 105(9):1099–1103CrossRefPubMedGoogle Scholar
- 6.Bacha EA, Daves S, Hardin J, Abdulla RI, Anderson J, Kahana M, Koenig P, Mora BN, Gulecyuz M, Starr JP, Alboliras E, Sandhu S, Hijazi ZM (2006) Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thorac Cardiovasc Surg 131(1):163–171.e2CrossRefPubMedGoogle Scholar
- 10.Bayley N (2006) The Bayley scales of infant development, 3rd edn. The Psychological Corporation, San AntonioGoogle Scholar
- 13.Knirsch W, Liamlahi R, Dave H, Kretschmar O, Latal B (2016) Neurodevelopmental outcome of children with hypoplastic left heart syndrome at one and four years of age comparing hybrid and Norwood procedure. Ann Thorac Cardiovasc Surg 22(6):375–377. https://doi.org/10.5761/atcs.lte.16-00106 CrossRefPubMedPubMedCentralGoogle Scholar
- 15.Newburger JW, Sleeper LA, Bellinger DC et al. (2012) Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial. Circulation 125(17):2081–2091. https://doi.org/10.1161/CIRCULATIONAHA.111.064113 CrossRefPubMedPubMedCentralGoogle Scholar
- 18.Ravishankar C, Zak V, Williams IA et al (2013) Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial. J Pediatr 162(2):250–256.e2. https://doi.org/10.1016/j.jpeds.2012.07.048 CrossRefPubMedGoogle Scholar
- 20.McElhinney DB, Benson CB, Brown DW, Wilkins-Haug LE, Marshall AC, Zaccagnini L, Tworetzky W (2010) Cerebral blood flow characteristics and biometry in fetuses undergoing prenatal intervention for aortic stenosis with evolving hypoplastic left heart syndrome. Ultrasound Med Biol 36(1):29–37/. https://doi.org/10.1016/j.ultrasmedbio.2009.09.004 CrossRefPubMedPubMedCentralGoogle Scholar
- 23.Marino BS, Lipkin PH, Newburger JW et al. (2012) American Heart Association Congenital Heart Defects Committee, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 126(9):1143–1172CrossRefPubMedGoogle Scholar