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Article

Ebstein's anomaly with life-threatening arrhythmia: an unusual cardiovascular manifestation of Williams syndrome - a case report.

BMC cardiovascular disorders - 2 Mar 2026

Schouw M E, Koopmann T T, Blom N A, Stöger J L, Barge-Schaapveld D Q C M, Bökenkamp R

Abstract excerpt

BACKGROUND: Williams syndrome (WS) is a rare genetic multisystem disorder that often affects the cardiovascular system. However, an association with Ebstein’s anomaly (EA) is extremely uncommon. Previously, only two such cases have been reported. Here, we present a third patient with concurrent WS and EA, manifesting with life-threatening ventricular arrhythmias. CASE PRESENTATION: Our case involves an...

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Identifiers

PMID: 41772445
DOI: 10.1186/s12872-026-05653-8
PMCID: PMC13059330

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