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Coarctation and Cerebral Aneurysms — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Coarctation and Cerebral AneurysmsEECC

A 20-year-old man with surgically repaired coarctation presents with headache and epistaxis. His BP is 175/100 mmHg in the right arm and 170/95 in the left arm (no gradient). MRI shows no re-coarctation but identifies multiple cerebral aneurysms. What is the association?

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Correct answer: E — Coarctation of the aorta is associated with intracranial (Berry) aneurysms in 5-10% of patients — the underlying vascular wall abnormality (cystic medial necrosis) affects cerebral arteries as well; combined with hypertension, this creates a significant risk of subarachnoid haemorrhage

Coarctation of the aorta is associated with intracranial (Berry) aneurysms: prevalence ~5-10% (5-10 times higher than the general population). The mechanism: the same vascular wall pathology (cystic medial degeneration affecting the arterial media) that causes coarctation also weakens cerebral arterial walls. Combined with hypertension (very common in coarctation patients even after repair), this creates a significant risk of subarachnoid haemorrhage (SAH). Other associated vascular abnormalities: BAV (50-85% of coarctation patients), ascending aortic aneurysm, and coronary artery anomalies. The 2020 ESC ACHD Guidelines recommend: (1) awareness of cerebral aneurysm risk; (2) prompt investigation of severe headache (SAH presentation); (3) some experts recommend screening MRA of the cerebral circulation, though this is not universally standardised. BP control is essential to reduce rupture risk.

Reference: ESC (2020): ACHD Guidelines