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Loeys-Dietz Syndrome Complicated by Right Coronary Artery Pseudoaneurysm

Yasir Jawaid1, Obadah Aqtash1, Kanaan Mansoor1

  • 1MU Internal Medicine Residency Program, Marshall University, 1249 15th Street, Huntington, WV 25701, USA.

Case Reports in Cardiology
|January 25, 2019
PubMed

Insights

Loeys-Dietz syndrome (LDS) patients face high risks of vascular aneurysms. This case highlights a rare coronary artery pseudoaneurysm after aortic root repair in LDS2.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Connective Tissue Diseases

Background:

  • Loeys-Dietz syndrome (LDS) is a rare genetic disorder characterized by progressive vascular aneurysms and craniofacial abnormalities.
  • Patients with LDS have an elevated risk of aneurysm rupture and dissection at earlier ages than other aneurysmal syndromes.
  • Prompt surgical intervention is crucial for preventing life-threatening vascular events in LDS patients.

Observation:

  • Coronary artery involvement, particularly post-aortic root repair, is an underreported complication in LDS.
  • This report details a unique case of Loeys-Dietz syndrome type 2 (LDS2) presenting with chest pain.

Findings:

  • The patient was diagnosed with a pseudoaneurysm stemming from a right coronary artery graft dehiscence.
  • This represents a rare manifestation of postoperative complications following aortic root repair in LDS2.

Implications:

  • This case underscores the importance of considering coronary artery complications in LDS patients post-surgery.
  • Awareness of such rare pseudoaneurysms is vital for timely diagnosis and management in Loeys-Dietz syndrome.
  • Further research into coronary artery complications in LDS is warranted to improve patient outcomes.

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