ABSTRACT
Atrial septal restriction is estimated to occur in up to 22% of patients with hypoplastic
left heart syndrome and is associated with poor prognosis postnatally. Atrial septal
aneurysms are known to be more prevalent in patients with congenital heart disease
and atrial septal restriction due to elevated atrial pressures in fetal life. We report
the case of a newborn with hypoplastic left heart syndrome, restrictive interatrial
communication, and atrial septal aneurysm. The latter led to formation of a large
intralimbic hematoma within it, which resulted in complete obstruction of the interatrial
communication after birth, which led to death. We suggest that in addition to the
known associated problems encountered in patients with hypoplastic left heart syndrome
and restrictive interatrial communication, aneurysmal formation of the interatrial
septum should alert the fetal and postnatal echocardiographer further, and careful
assessment and follow-up is recommended.
KEYWORDS
Intralimbic hematoma - atrial septal aneurysm - hypoplastic left heart syndrome -
atrial septal restriction
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Dr. Aphrodite Tzifa
Department of Congenital Heart Disease, Evelina Children's Hospital
St Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH, UK
Email: aphrodite.tzifa@kcl.ac.uk