Successful redo open thoracoabdominal aortic aneurysm surgery in a patient with vascular Ehlers-Danlos syndrome: A case report.

We report a case of a 63-year-old woman diagnosed with vascular Ehlers-Danlos syndrome (vEDS) who survived two prophylactic surgeries for the dilatation of a thoracoabdominal aortic aneurysm. She initially developed acute type B aortic dissection at the age of 44 years. Five years later, her dissected descending aorta was enlarged to 54 mm; thus, the descending aorta was replaced as the first surgery. Fortunately, the intra- and post-operative courses were uneventful. Fourteen years post her first surgery, the dissected thoracoabdominal aorta distal to the graft expanded to 53 mm; however, no anastomotic leakage was observed. Genetic testing revealed a COL3A1 abnormality, confirming the diagnosis of vEDS. Thoracoabdominal aorta replacement using deep hypothermia circulatory arrest was performed because of the high risk of aortic aneurysm rupture. The second surgery was performed without complications, and no complications were observed 13 months post-surgery. The major reason for a successful surgery in this patient was the relatively low vascular fragility associated with vEDS. This case demonstrates that there may be considerable individual differences in vascular fragility in patients with vEDS. Thus, surgical repair, along with endovascular therapy, might still be a beneficial option for patients with vEDS having large aortic aneurysms and a high risk of rupture. Learning objective: Prophylactic surgery for vascular lesions in Ehlers-Danlos syndrome (vEDS) is generally not recommended because of its high vascular fragility. However, if a patient with vEDS has an aortic aneurysm that is at a very high risk of rupture, aggressive treatment is a plausible option as there may be considerable individual differences in vascular fragility among patients with vEDS.

Descending thoracic aortic repair outcomes for chronic aortic dissection: a single-centre experience.

OBJECTIVES: Thoracic endovascular aortic repair is a widely accepted treatment for chronic aortic dissection because of good early results compared to open surgical repair. We provide early and long-term results of descending thoracic aortic repair for chronic aortic dissection. METHODS: Patients who underwent descending thoracic aortic repair for chronic aortic dissection between January 2012 and December 2020 at Kawasaki Aortic Centre were included in this analysis. RESULTS: Four hundred ninety-two patients (median age, 64 years; interquartile range, 52-75 years) were included. The median duration of follow-up was 3.2 years (interquartile range, 1.5-5.2 years). The early mortality rate was 2.0% (n = 10); strokes occurred in 17 patients (3.5%); and spinal cord injuries occurred in 30 patients (6.1%). Early major adverse events including early death, stroke, spinal cord injury, tracheostomy and haemodialysis at the time of discharge occurred in 62 patients. Multivariable analysis indicated that age > 70 years and non-elective surgery were predictors of early major adverse events. Among patients without both risk factors (i.e. low-risk patients), 1 early death (0.4%), 3 strokes (1.5%) and 1 spinal cord injury (0.4%) were observed, 2 tracheostomies were performed (0.8%) and no patients required haemodialysis at the time of hospital discharge. The 5-year survival rate was 87.2%. The cumulative incidence of chronic aortic dissection-related aortic reintervention at 5 years was 7.9%. CONCLUSIONS: Descending thoracic aortic repair for chronic aortic dissection resulted in good early and long-term results, and it can serve as the gold standard for low-risk patients.

Evaluation of the vertebrobasilar system in thoracic aortic surgery.

BACKGROUND: We evaluated the probability of vertebrobasilar system malperfusion due to occlusion of the left subclavian artery as assessed by preoperative magnetic resonance angiography in patients scheduled to undergo thoracic aortic surgery. METHODS: (Study 1) From January 2000 through March 2009, we studied variations of vertebral arteries in 301 patients scheduled to undergo thoracic aortic surgery. We classified vertebral artery variations into 3 categories according to the findings on preoperative magnetic resonance angiography: connection type, interrupted right vertebral artery, and interrupted left vertebral artery. (Study 2) From February 2007 through January 2010, we evaluated the cerebral complication in 41 patients who had occlusion of the left subclavian artery with a stent graft. RESULTS: (Study 1) On preoperative magnetic resonance angiography, the vertebral artery was classified as connection type in 247 patients, interrupted right vertebral artery in 34, and interrupted left vertebral artery in 20. (Study 2) We performed subclavian obstruction test, left-right subclavian artery bypass, or left subclavian artery-left common carotid artery bypass to the 3 patients with interrupted right vertebral artery, respectively. Forty patients (98%) out of 41 patients had no complication after occlusion of the left subclavian artery. CONCLUSIONS: Preoperative magnetic resonance angiography is useful for detection of the patients with high risk of vertebrobasilar system malperfusion due to occlusion of the left subclavian artery.

Fibromuscular dysplasia associated with simultaneous spontaneous dissection of four peripheral arteries in a 30-year-old man.

A 30-year-old man had a sudden bout of severe abdominal pain. An enhanced computed tomographic scan revealed dissections of the celiac artery, superior mesenteric artery, left renal artery, and right external iliac artery; stenosis of the right renal artery; and left kidney infarction. After careful evaluation, the patient was diagnosed with fibromuscular dysplasia (medial dysplasia), based on the findings obtained from the enhanced computed tomographic scan. This case is extremely rare because fibromuscular dysplasia occurred concurrently with simultaneous spontaneous dissections of four peripheral arteries in a young man.

Endovascular repair of ascending aortic rupture: effectiveness of a fenestrated stent-graft.

PURPOSE: To present a technique for endovascular treatment using a fenestrated stent-graft in a patient with ascending aortic rupture in the setting of methicillin-resistant Staphylococcus aureus infection. CASE REPORT: A 62-year-old woman had undergone mastectomy and radiotherapy twice for breast cancer and then coronary artery bypass grafting (CABG). She developed sternal osteomyelitis 5 years after the CABG. Sternectomy and negative-pressure wound drainage were performed, but the infection did not resolve. Ascending aortic rupture occurred 5 months after sternectomy. Endovascular therapy was considered the only effective means of achieving hemostasis. A custom-designed fenestrated stent-graft was deployed from the ascending aorta to the proximal descending aorta via a femoral artery approach without transient cardiac arrest. Bleeding completely stopped after surgery. The postoperative course was uneventful, and the inflammatory activity subsided on antibiotic therapy. At 7 months after surgery, the patient's recovery has been uneventful. CONCLUSION: Rupture of the ascending aorta associated with infection was successfully treated by stent-graft repair. The use of a custom-made, fenestrated stent-graft was an effective, lifesaving procedure for the management of this ascending aortic lesion.