Multimodal Imaging Directs Decision Making in Intraseptal L-AAOCA
Joseph Burns1, Lindsay F Eilers1, Stephen J Dolgner1
1Section of Pediatric Cardiology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas, USA.
Insights
Congenital coronary artery anomalies, though rare, can cause sudden cardiac death. This case highlights the importance of thorough evaluation and multimodal imaging for patients with intraseptal coronary artery courses.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Congenital coronary artery anomalies (CCAs) are uncommon causes of congenital heart disease.
- Intraseptal coronary artery courses have limited data regarding evaluation and management.
- CCAs carry a risk of sudden cardiac death, particularly in young individuals.
Observation:
- A 27-year-old woman presented with a single coronary trunk originating from the right sinus.
- The left main coronary artery had a 2.7 cm intraseptal course.
- She underwent stress cardiac magnetic resonance (CMR) and catheterization to assess for inducible ischemia.
Findings:
- The patient underwent successful surgical repair with transconal unroofing.
- Post-operative recovery was uneventful, with no residual symptoms.
- Multimodal imaging (CMR and catheterization) was crucial in guiding management decisions.
Implications:
- Comprehensive evaluation is essential for managing patients with CCAs.
- Further research is needed to establish optimal work-up, intervention indications, and timing.
- Multimodal imaging plays a critical role in diagnosing and managing CCAs, especially those presenting with exertional chest pain.
Background:
Congenital coronary artery abnormalities represent an uncommon class of congenital heart disease with an associated risk of sudden cardiac death. There is a paucity of data for the evaluation and management of affected patients with an intraseptal course.
Case Summary:
A 27-year-old woman was found to have a single coronary trunk arising from the right sinus with a 2.7 cm intraseptal course of the left main coronary artery. She underwent stress cardiac magnetic resonance and catheterization, to assess for inducible ischemia before surgical repair with transconal unroofing. Her recovery was uneventful, and she remains free of symptoms.
Discussion:
Management of congenital coronary artery anomalies requires an exhaustive evaluation. Further studies are warranted to define the ideal work-up, indication, and timing of intervention and the efficacy of medical management in improving symptoms.
Take-Home Messages:
Congenital coronary anomalies represent a rare but consequential cause of exertional chest pain. This diagnosis requires multimodal imaging to inform decision making.
More Related Videos
Related Concept Videos
01:19Imaging Studies VII: Vascular Imaging
01:21Imaging Studies for Cardiovascular System IV: CMRI
01:27Imaging Studies III: Computed Tomography