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.

Acute type A aortic dissection surgery in retrosternal gastric tube reconstruction patient.

BACKGROUND: Retrosternal gastric tube reconstruction is a common surgical technique for managing esophageal cancer, but it complicates acute type A aortic dissection repair and raises concerns about gastric tube damage. CASE PRESENTATION: A 73-year-old female, who underwent esophagectomy with retrosternal gastric tube reconstruction 6 months ago for esophageal cancer, presented with severe chest pain. Acute type A aortic dissection was confirmed by contrast-enhanced computed tomography, and emergency hemiarch replacement through a median sternotomy was performed, preserving the gastric tube without injury. The patient recovered well and was discharged after 3 weeks, showing no gastrointestinal symptoms or signs of mediastinitis.

Aortoesophageal fistula treated using one-stage total reconstruction: a case report from a high-volume center.

BACKGROUND: Aortoesophageal fistula (AEF) is a rare but typically life-threatening condition. Although several treatment strategies exist, including conservative treatment with intraluminal stent graft and open thoracic aortic replacement, the overall outcome remains poor, ranging from 16 to 39%. Furthermore, esophageal reconstruction methods vary between hospitals. Herein, we report a case of aortoesophageal fistula treated using one-stage total reconstruction. CASE PRESENTATION: This case involved a 58-year-old woman who developed acute type A aortic dissection and underwent successful total arch replacement at the other hospital. However, she developed AEF 1 year later and underwent urgent thoracic endovascular aortic repair, which eventually failed. We performed thoracic aortic replacement, total esophagectomy, gastric tube reconstruction, and omental flap in a one-stage operation. The patient was extubated the next day and transferred to the general ward on postoperative day 3. Computed tomography revealed favorable results. CONCLUSIONS: For postoperative AEF, dedicated debridement with reconstruction is more effective than conservative treatment. In an experienced center, post-procedure-related AEF can be easily treated using one-stage reconstruction.

Open after endovascular repair for previous extra anatomic bypass of adult coarctation.

BACKGROUND: During follow-up, aneurysm formation in adults with coarctation of aorta has been reported after undergoing extra-anatomical aortic bypass grafting. Endovascular repair was a reasonable treatment option but there were still complications. CASE PRESENTATION: A 48-year-old male who underwent extra-anatomical aortic bypass grafting, presented with severe back pain and hemoptysis. He had a diagnosed pseudoaneurysm with concealed rupture at the bypass grafting. He underwent endovascular repair and coil embolization. A postsurgical CT-angiogram showed there was extravasation from stent into the pseudoaneurysm. An open repair with endovascular stent removal instead of restenting was performed.

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.

Left Ventricular Hypertrophy Is More Prevalent in Type B than Type A Aortic Dissection.

OBJECTIVES: Several factors determining differences between types A and B aortic dissection (AD) have been reported; however, little data exist examining their differences in left ventricular hypertrophy (LVH). We compared the prevalence of LVH in patients with types A and B AD. METHODS: We retrospectively analyzed 334 patients with acute AD (227 type A; 107 type B). Concentric hypertrophy (CH; increased left ventricular mass index [LVMI] and relative wall thickness [RWT]) is one of four types of left ventricular (LV) geometry thought to be most associated with hypertension. We compared LVMI and the prevalence of CH in patients with types A or B AD. Multivariate logistic regression analyses of variables associated with type B AD were performed. RESULTS: Comparing type A and B AD, LVMI (95 ± 26 vs.107 ± 28, p <0.001) and prevalence of CH (26% vs. 44%, p = 0.001) were higher in type B AD. In multivariate analysis, CH was an independent factor associated with type B AD (odds ratio: 2.62, confidence interval: 1.54-4.47, p <0.001). CONCLUSIONS: Our data suggested LVH was more prevalent in type B than in type A AD. Considering LVH usually results from hypertension, patients with type B AD may be more affected by hypertension than those with type A.

Open surgery for descending thoracic aorta in an endovascular era.

BACKGROUND: Thoracic endovascular aortic repair has become the preferred treatment for a variety of descending thoracic aortic pathologies. However, there are unresolved issues such as morphologic appearance of chronic dissection, persistent false lumen perfusion, and adequacy of landing zone. Enthusiasm for improving the technique of open aortic repair and perioperative management is fading. In this study, we would like to demonstrate how we improve our surgical outcomes by establishing a dedicated aortic multidisciplinary team at the Kawasaki Aortic Center. METHOD: We performed a single-center retrospective study from January 2015 to December 2016. All patients with open descending thoracic aortic replacement were recruited. Preoperative patient demographic data, bypass strategies, operative details, and postoperative outcomes were reviewed. RESULT: From January 2015 to December 2016, we treated 168 cases of descending thoracic aortic repair using a left thoracotomy. Median age was 69.0 ± 21.8 years old, and 63.1% were aortic dissection (acute, 4.8%; chronic, 58.3%); 81.3% patients underwent elective operations. Left heart bypass, deep hypothermic circulatory arrest, and partial cardiopulmonary bypass were performed in 88.6%, 9.0%, and 2.4% of patients, respectively. Mean operative time was 312 ± 94 minutes. In-hospital mortality in total was 0.6%. The rate of transient spinal cord injury was 4.7%. CONCLUSIONS: Under a dedicated aortic multidisciplinary team, we demonstrated that open descending thoracic aorta replacement can be performed with excellent early outcomes with low reintervention rates, regardless of the nature of the aortic pathologies.

Open Thoracoabdominal Aortic Aneurysm Repair: Contemporary Outcomes for 393 Elective Cases.

BACKGROUND: The purpose of this study was to reveal the midterm and long-term mortality rates among patients with thoracoabdominal aortic aneurysm (TAAA) after open TAAA repair and to clarify the effect of aneurysm type on mortality. METHODS: We retrospectively analyzed data for 393 patients (290 men; age, 63.2 ± 12.7 years) who underwent open TAAA repair of elective Crawford extent I, II, or III TAAAs (62, 197, and 134 patients, respectively) between June 2003 and December 2015. The overall survival probability and differences according to aneurysm type were assessed using the Kaplan-Meier product limit method. Also, the effect of aneurysm type on mortality was assessed using the hazard ratio and Cox proportional hazards regression. RESULTS: The overall survival probabilities at 3 months, 1 year, 5 years, and 10 years were 90%, 84%, 78%, and 75%, respectively. The age-adjusted relative mortality rate was significantly higher for patients with dissecting aneurysms than for those with nondissecting aneurysms (relative risk, 1.62; 95% confidence interval, 1.03 to 2.55). In the multivariate Cox proportional hazard regression model, the hazard ratio for all-cause mortality did not differ between patients with dissecting and those with nondissecting aneurysms. However, those with dissecting aneurysms had increased mortality rates as their percentage vital capacity decreased (hazard ratio, 0.7; 95% confidence interval, 0.5 to 1.0); a similar trend was not observed for those with nondissecting aneurysms. CONCLUSIONS: Open TAAA repair can be safely performed with acceptable midterm and long-term results. Poor pulmonary function can impair the survival outcome of patients with dissecting aneurysms.

Surgical outcomes of aortic repair via transapical cannulation and the adventitial inversion technique for acute Type A aortic dissection.

OBJECTIVES: To evaluate the surgical outcomes of aortic repair via transapical cannulation and the adventitial inversion technique for acute Type A aortic dissection. METHODS: Between 2008 and 2015, a total of 300 patients with acute Type A aortic dissection underwent emergency surgery, consisting of 271 hemiarch repairs and 29 total aortic arch replacements, using transapical cannulation and the adventitial inversion technique at a distal anastomosis. The mean follow-up periods were 31.7 ± 25.2 months. Overall, 18% (54/300) of the patients were octogenarians, and 21.7% (65/300) had cardiac tamponade; 25% (75/300) had preoperative malperfusion. RESULTS: The in-hospital and 30-day mortality rates were 8.3% (25/300) and 6.7% (20/300), respectively. The 30-day mortality rate was 2.7% (6/225) among patients without preoperative malperfusion and 18.7% (14/75) among patients with malperfusion (P < 0.0001), 7.4% (4/54) among octogenarians and 6.5% (16/246) among patients aged less than 80 years (P = 0.81), and 6.3% (17/271) among patients treated with hemiarch repair and 10.3% (3/29) among patients treated with total aortic arch replacement (P = 0.403). Preoperative malperfusion was an independent predictor of perioperative mortality in a multivariable analysis. During the follow-up period, distal reintervention was performed in 11% (33/300) of the patients. The rates of freedom from reintervention at 1, 3 and 5 years were 95.9%, 88.9% and 80.0%, respectively. The overall survival rates at 1, 3 and 5 years were 88.7%, 86.7% and 82.0%, respectively. The in-hospital mortality rate for elective reintervention was 3.0% (1/33). CONCLUSIONS: Aortic repair via transapical cannulation and the adventitial inversion technique for acute Type A aortic dissection provides good early and mid-term results. The safety of elective distal reintervention can be guaranteed. To obtain better operative outcomes, effective treatment for cases with malperfusion is mandatory.