Familial Spontaneous Coronary Artery Dissection Involving the Left Main Coronary Artery in a Young Male: A Case
Murat Çap1, İsmail Tatli1, Adnan Duha Comert1
1Department of Cardiology, University of Health Sciences Diyarbakir Gazi Yaşargil Education and Research Hospital, Diyarbakır, Turkey.
Insights
Spontaneous coronary artery dissection (SCAD) can run in families. A TTN gene variant may be linked to SCAD, suggesting conservative management is effective for complex cases.
Area of Science:
- Cardiology
- Genetics
Background:
- Spontaneous coronary artery dissection (SCAD) is a significant cause of acute coronary syndrome, especially in younger individuals lacking traditional risk factors.
- Familial clustering of SCAD suggests an underlying genetic predisposition.
- SCAD often presents acutely, necessitating prompt diagnosis and management.
Observation:
- A 33-year-old male presented with acute chest pain and ST-segment elevation, indicative of acute coronary syndrome.
- Coronary angiography revealed spontaneous dissection of the left main, left anterior descending, and circumflex arteries with substantial thrombus.
- The patient reported a family history of SCAD in two siblings.
Findings:
- A conservative management strategy, including tirofiban, dual antiplatelet therapy, and anticoagulation, was employed due to high thrombus burden and procedural risks.
- Intravascular ultrasound confirmed significant thrombus and intramural hematoma.
- Genetic analysis identified a heterozygous TTN gene variant in the patient and his affected brother.
Implications:
- This case highlights the potential familial inheritance of SCAD.
- A novel association between TTN gene mutations and coronary artery dissection is suggested.
- Conservative management with antiplatelet and anticoagulant therapy can be successful in complex SCAD cases with high thrombus burden.
Abstract:
Spontaneous coronary artery dissection (SCAD) is a rare but important cause of acute coronary syndrome, particularly in young patients without conventional risk factors. While often sporadic, familial clustering has been increasingly recognized, indicating a genetic predisposition. We report a case of a 33-year-old male presenting with acute chest pain and ST-segment elevation. Coronary angiography showed spontaneous dissection with a large thrombus burden involving the distal left main coronary artery and extending into the proximal left anterior descending artery and circumflex artery. The patient had a family history of SCAD involving 2 siblings. Given the high thrombus burden and risk of procedural complications, a conservative approach was chosen. Management included tirofiban infusion, dual antiplatelet therapy, and anticoagulation. Intravascular ultrasound confirmed thrombus and intramural hematoma. Follow-up showed near-complete healing. Genetic testing identified a heterozygous TTN gene variant in the patient and his brother, a gene associated with cardiomyopathies. In conclusion, this case emphasizes the potential familial nature of SCAD and suggests a possible, previously unrecognized, association between TTN gene mutations and coronary dissection, indicating that conservative management with antiplatelet and anticoagulant therapy may be effective even in complex SCAD cases with high thrombus burden.
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