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Cerebral Autosomal Dominant Arteriopathy (CADASIL) with cardiac involvement (ANOCA) and subcortical

Christian Langer1, Agne Adukauskaite2, Fabian Plank2

  • 1Department of Radiology, Innsbruck Medical University, Austria.

Journal of Cardiovascular Computed Tomography
|September 11, 2018
PubMed

Insights

This study presents a rare case of Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) syndrome involving cardiac issues. Fractional flow reserve CT (FFR-CT) proved more valuable than coronary CT angiography (CTA) for diagnosing coronary artery disease (CAD) in this patient.

Area of Science:

  • Cardiology
  • Neurology
  • Radiology

Background:

  • Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare genetic disorder affecting small blood vessels.
  • Cardiac involvement in CADASIL is uncommon, and its diagnosis can be challenging.

Observation:

  • A patient with CADASIL presented with angina in the absence of obstructive coronary artery disease (ANOCA).
  • Coronary CT angiography (CTA) was performed for initial assessment.
  • Non-invasive fractional flow reserve computed tomography (FFR-CT) was subsequently utilized.

Findings:

  • FFR-CT provided crucial diagnostic information that surpassed that of standard CTA in this ANOCA case.
  • The findings suggest occult coronary artery disease (CAD) may be present in CADASIL patients with cardiac symptoms.

Implications:

  • This case underscores the utility of FFR-CT in evaluating coronary artery disease in patients with CADASIL.
  • Coronary CT angiography (CTA) is recommended for routine screening of coronary artery disease in individuals with CADASIL syndrome.
  • Further research is warranted to understand the prevalence and management of cardiac involvement in CADASIL.

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