Thorac Cardiovasc Surg 2022; 70(S 02): S67-S103
DOI: 10.1055/s-0042-1743000
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Long-Term Neurodevelopmental Outcome and Serial Cerebral MRI Assessment in Fontan Patients at School Age

W. Knirsch
1   Children's Hospital Zurich - Eleonore Foundation, Zürich, Switzerland
,
S. Schwan
2   Pediatric Cardiology, University Children's Hospital, Giessen, Deutschland
,
T. Logeswaran
2   Pediatric Cardiology, University Children's Hospital, Giessen, Deutschland
,
K. Heye
3   Pediatric Cardiology, University Children's Hospital, Zürich, Switzerland
,
B. Latal
4   Child Development Center, University Children's Hospital, Zürich, Switzerland
,
A. Hahn
5   Pediatric Neurology, University Children's Hospital, Giessen, Deutschland
,
A. Goetschi
6   Epidemiology, Biostatistics and Prevention Institute, Zürich, Switzerland
,
U. Held
6   Epidemiology, Biostatistics and Prevention Institute, Zürich, Switzerland
,
R. Kottke
7   Center of MR Research, University Children's Hopital, Zürich, Switzerland
,
B. Reich
2   Pediatric Cardiology, University Children's Hospital, Giessen, Deutschland
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Background: Infants with hypoplastic left heart syndrome/complex congenital heart defect (CHD) undergoing staged surgical palliation are at risk for impaired neurodevelopmental (ND) outcome. Little is known about the long-term effects on brain growth and relation to ND outcome until primary school age.

Method: In a prospective two-center study of consecutive patients undergoing stage I (hybrid or Norwood) to stage III (Fontan procedure) was evaluated by two serial cerebral MRI (cMRI) examinations, neurodevelopmental testing before Fontan procedure at 2 years of age (Bayley's grade III) and after Fontan procedure at 6 to 8 years of age (WPSSI III). MRI findings were compared with a healthy control group. Clinical characteristics were documented and related to cerebral and neurodevelopmental findings.

Results: Thirty-three infants (16 females) were compared with eight age- and gender-matched healthy controls. Total brain volume (p < 0.001), total gray matter (GM) volume (p = 0.002), total deep GM volume (p = 0.001), and total white matter volume (p < 0.001) were smaller in CHD patients. Surgical procedure at stage I (Norwood, n = 5 vs. hybrid, n = 24) had no significant influence on brain volumes at primary school age, nor on ND outcome. Body weight gain was severely impaired until stage II followed by a catch-up growth until primary school age. Pathologic brain lesions at 2 years of age (39%), including cerebral stroke, white matter injury, ventriculomegaly, and brain matter loss, were related to lower APGARscore, male gender, cumulative length of hospital/ICU stay, and multiple interventions. At primary school age, pathologic cerebral findings (53%) were related to neurodevelopmental outcome (p = 0.03) evaluated by WPSSI III (full-scale score of 96 without pathological cMRI versus 85 with pathological cMRI).

Conclusion: Smaller brain volumes and structural brain lesions in complex CHD patients at primary school age resulting in impaired neurodevelopmental outcome. For evaluation of predictive surgical or clinical factors, larger cooperative multicenter studies are needed.



Publication History

Article published online:
12 February 2022

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