Neurologische und psychomotorische Entwicklung von Feten und Neugeborenen mit angeborenen Herzfehlern – Ursachen und Prävalenz von Störungen im Langzeitverlauf

Zusammenfassung

Kinder mit schweren angeborenen Herzfehlern, die im Neugeborenen- oder Säuglingsalter mittels Herz-Lungen-Maschine operiert werden müssen, zeigen ein erhöhtes Risiko für neurologische und psychomotorische Entwicklungsstörungen. Defizite betreffen in der Regel Teilbereiche der Entwicklung und weniger die formale Intelligenz, sie treten in dieser Gruppe – abhängig von Schwere und Art des Herzfehlers – in 15% bis über 50% auf. Kinder mit leichteren Herzfehlern, die den wesentlichen Anteil angeborener Herzerkrankungen ausmachen, zeigen eine deutlich bessere Entwicklungsprognose als diejenigen mit komplexen Herzfehlern. In dieser Übersichtsarbeit werden der Einfluss schwerer Herzfehler auf das sich entwickelnde Zentralnervensystem des Feten und Neugeborenen diskutiert, aktuelle klinische und bildgebende Studien zusammengefasst und eine Übersicht über den neurologischen Langzeitverlauf gegeben. Bereits intrauterin ist – abhängig von Perfusion und Oxygenierung des Zentralnervensystems – die zerebrale Ausreifung verzögert und das Risiko für hypoxämische Schäden der weißen Substanz erhöht. Präoperativ finden sich relevante morphologische und neurologische Auffälligkeiten, die mit einer erhöhten Vulnerabilität der weißen Substanz korrelieren. Intrauterine, präoperative und zusätzliche perioperative Einflussfaktoren können langfristig zu einer relevanten Störung der psychomotorischen Entwicklung führen, wobei die subjektive Lebensqualität als gut empfunden wird. Neue therapeutische Ansätze zielen somit auf eine Optimierung der intrauterinen und perinatalen Versorgung von Feten und Neugeborenen mit angeborenen Herzfehlern. Die Vermeidung von negativen Einflussfaktoren auf die psychomotorische Entwicklung, konsequente Früherkennung und eine spezifische Förderung sollen zur Verbesserung des Langzeitverlaufes beitragen.

Abstract

Children with severe congenital heart defects (CHD) requiring open heart surgery in the first year of life are at high risk for developing neurological and psychomotor abnormalities. Depending on the type and severity of the CHD, between 15 and over 50% of these children have deficits, which are usually confined to distinct domains of development, although formal intelligence tends to be normal. Children with mild CHD, who comprise the majority of congenital heart defects, have a far better developmental prognosis than those with complex CHD. This review concentrates on the impact of severe CHD on the developing brain of the foetus and infant. It also provides a summary of recent clinical and neuroimaging studies, and an overview of the long-term neurological prognosis. Advanced neuroimaging modalities indicate that, related to altered cerebral blood flow and oxygenation, foetuses with severe CHD show delayed third trimester brain maturation and increased vulnerability for hypoxic injury. Morphological and neurological abnormalities are present before surgery, commonly affecting the white matter. In the long-term, impaired neurological and developmental outcomes are related to the combination of prenatal, perinatal and additional perioperative risk factors. Therefore, new therapeutic approaches aim to optimise the intra- and perinatal management of foetuses and newborns with congenital heart defects. Identification and avoidance of risk factors, early neurodevelopmental assessment and therapy may optimise the long-term outcome in this high-risk population.

Schlüsselwörter

angeborene Herzfehler - Entwicklung - psychomotorische Entwicklungsstörung - Langzeitverlauf

Key words

congenital heart disease - neurodevelopment - psychomotor impairment - outcome

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