Mental Health Care Vital for Vascular EDS Patients Undergoing Great Stress, Case Report Says

Proper psychologial care for people with a history of vascular Ehlers-Danlos syndrome (EDS) who experience stress from a traumatic event is crucial for the prevention of complications.

The findings are from a Japanese case report of an EDS patient who suffered artery dissection shortly after her mother died.

The report, “Sudden onset of artery dissection in a 32-year-old woman with vascular Ehlers-Danlos syndrome due to psychological stress of her mother’s death: a case series,” was published in the journal JA Clinical Reports.

Patients with vascular EDS have a tendency to develop serious vascular complications, including aortic and visceral arterial ruptures, aneurysms, and dissection.

Dissection happens when a tear in the blood vessel allows blood to enter the space between the inner and outer layers of the vessel, causing it to narrow or be completely blocked.

A 57-year-old woman who had previously been diagnosed with vascular EDS arrived at the emergency department of a hospital in Takatsuki, Japan, due to a sudden onset of left lower back pain and left lower limb paralysis.

After an abdominal computed tomography (CT) scan, physicians discovered a left-sided retroperitoneal hematoma — a condition where blood accumulates in the abdominal cavity — and an artery dissection in her abdomen.

Physicians attempted to treat the dissection using a technique called stent graft repair.

Unfortunately, five hours after surgery, the woman underwent cardiac arrest, the resuscitation attempts failed, and she died.

The patient’s daughter, who was 32 and also diagnosed with vascular EDS, was told about her mother’s death at a customary end-of-life conference. But six hours after her mother’s death, she was also admitted to the same emergency department with sudden onset of left lower back pain.

Physicians conducted an abdominal CT and discovered a right-sided retroperitoneal hematoma and an artery dissection in the abdomen, similar to her mother, except the hematoma was on the right side.

She was admitted to the intensive care unit and physicians treated her using a conservative therapeutic combination of bed rest and nicardipine, a drug used to treat high blood pressure and angina.

The young woman did not develop any additional vascular complications requiring surgery, and she was discharged after 21 days.

Doctors believe the bleeding in the daughter could have been affected by the trauma of mother’s death, as it likely caused great psychological stress, which is known to have an impact on the cardiac health of patients.

The report emphasizes the importance of good communication between healthcare providers and family members of patients who are in critical care.

“Given that vascular EDS is a genetic syndrome, the importance of psychological care for the bereaved family should not be taken lightly,” the authors stated.

“Our report underscores the need for strategic management of vascular complications to prevent rupture, as well as the importance of psychological care for the bereaved family, given the hereditary nature of vascular EDS,” the clinical team wrote.