A Lethal Complication after Coronary Angiography in a Patient with Ehlers-Danlos Syndrome

Area of Science:

• Cardiology
• Vascular Surgery
• Genetics

Background:

• Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
• Vascular EDS (vEDS) is a severe subtype characterized by fragile blood vessels.
• Coronary angiography is an important diagnostic tool for acute coronary syndrome (ACS).

Observation:

• A 51-year-old man with EDS presented with chest tightness, diagnosed as ACS.
• Despite initial management, symptoms persisted, necessitating coronary angiography via the left radial artery.
• Complications included a left arm hematoma with compartment syndrome and later, a subarachnoid hemorrhage during general anesthesia.

Findings:

• The patient developed a significant left arm hematoma and compartment syndrome post-angiography due to delayed arterial leakage.
• A spontaneous subarachnoid hemorrhage occurred during general anesthesia, leading to a prolonged comatose state.
• These events highlight the increased risk of vascular rupture and bleeding in EDS patients undergoing invasive procedures.

Implications:

• Endovascular procedures in patients with vascular EDS should be reserved for life-threatening conditions.
• Vigilant monitoring and potentially prolonged manual compression or trans-radial band application are crucial for hemostasis post-angiography.
• This case underscores the critical need for individualized risk assessment and management strategies in EDS patients.