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Klin Padiatr 2018; 230(01): 24-30
DOI: 10.1055/s-0043-120526
Original Article
© Georg Thieme Verlag KG Stuttgart · New York

Neurodevelopmental Outcome in Children With Single Ventricle After Total Cavopulmonary Connection

Psychologisches Funktionsniveau bei Kindern und Jugendlichen mit univentrikulärer Zirkulation nach operativer Palliation
Nicole Vahsen
1   German Paediatric Heart Centre Sankt Augustin, Paediatric Cardiology, Sankt Augustin, Germany
,
Arndt Bröder
2   University of Mannheim, School of Social Sciences, Mannheim, Germany
,
Viktor Hraska
3   Childrenʼs Hospital of Wisconsin, Cardiothoracic Surgery, Milwaukee, United States
,
Martin Schneider
1   German Paediatric Heart Centre Sankt Augustin, Paediatric Cardiology, Sankt Augustin, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
19 December 2017 (online)

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Abstract

Background The purpose of the present study was to assess a broad range of neuropsychological outcome variables in children with functionally single ventricle hearts after a total cavopulmonary connection and to examine potential risk factors for impaired neurodevelopment.

Patients & Method A total of 104 patients aged 2 to 20 years underwent follow-up standardized psychological testing, including measures of intelligence, motor function, visuospatial abilities, behavioural outcome and health-related quality of life.

Results With a mean fluid intelligence score of 93.0 and a mean crystallized intelligence score of 92.3, patients scored significantly lower on tests of intelligence compared to the general population (p<0.001). Reduced motor function was found in 34% of the patients (p<0.001), impaired visuospatial abilities in 51%; parents reported significantly more behavioural problems (p<0.001). There was no difference in the self-reported quality of life compared to the general population. Risk factors for reduced fluid intelligence were deep hypothermic circulatory arrest times (p=0.03) and complications causing suboptimal brain perfusion prior to the total cavopulmonary connection (e. g. seizures; p=0.04).

Conclusions Patients with functionally single ventricle hearts palliated with a total cavopulmonary connection are at an increased risk of neurodevelopmental delays and behavioural disorders. Nevertheless, they adapt well in terms of quality of life. Early diagnostics and interventions are necessary when developmental delays are suspected.

Zusammenfassung

Hintergrund In der vorliegenden Studie wurde das Funktionsniveau von Patienten mit funktionell univentrikulärem Herzen nach Anlage einer totalen cavopulmonalen Anastomose untersucht. Außerdem wurden mögliche Zusammenhänge zwischen dem Funktionsniveau und verschiedenen medizinischen Risikofaktoren überprüft.

Patienten & Methode Bei insgesamt 104 Kindern zwischen 2 und 20 Jahren wurde das intellektuelle Leistungsvermögen, die koordinativen und visuell-räumlichen Fähigkeiten, Verhaltensauffälligkeiten und die gesundheitsbezogene Lebensqualität erfasst.

Ergebnisse Mit einer mittleren fluiden Intelligenz von 93,0 und einer kristallinen Intelligenz von 92,3 schnitten die Patienten signifikant schlechter im Test ab als die Normstichprobe (p<0,001). 34% wiesen eine motorische Koordinationsstörung, 51% eine Störung der visuell-räumlichen Fähigkeiten auf. Laut Elternurteil war außerdem die Prävalenz von Verhaltenssauffälligkeiten signifikant erhöht (p<0,001). Die selbst eingeschätzte Lebensqualität unterschied sich nicht von der Lebensqualität gesunder Kinder. Hinsichtlich medizinischer Risikofaktoren ergab sich ein signifikant negativer Zusammenhang zwischen der kumulierten Dauer des hypothermen Herzkreislaufstillstands und der fluiden Intelligenz (p=0,03). Außerdem wiesen Patienten mit präoperativen Komplikationen, die eine suboptimale zerebrale Perfusion bewirken, eine signifikant niedrigere fluide Intelligenz auf, als Patienten ohne solche präoperativen Komplikationen (p=0,04).

Schlussfolgerung Insgesamt ist das Risiko für Entwicklungs- und Verhaltensstörungen bei Kindern mit funktionell univentrikulärem Herzen nach Anlage einer totalen cavopulmonalen Anastomose erhöht. Diagnostische und interventive Maßnahmen sollten frühzeitig eingeleitet werden.

Key words

Total cavopulmonary connection - follow-up studies - neurodevelopmental outcome - risk factors - quality of life

Schlüsselwörter

Totale cavopulmonale Anastomose - Folgeuntersuchung - Entwicklungsstörung - Risikofaktoren - Lebensqualität
 

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