Thoracoabdominal aortic replacement in a 6-year-old boy with Loeys-Dietz syndrome.

Connective tissue disorders such as Marfan- and Loeys-Dietz syndrome (LDS) can lead to aortic aneurysms and aortic dissections in children. Patients with LDS often necessitating multiple aortic surgeries throughout their lives to extend their lifespan. A boy with LDS underwent Bentall procedure at the age of three for aortic aneurysm. At the age of six, this boy was referred to the hospital again due to severe abdominal pain. Computed tomographic angiography (CTA)indicates aortic dissection (DeBakey Type III, Stanford Type B). After a multidisciplinary team discussion, a successful thoracoabdominal aortic replacement was performed.

Single-stage total aortic replacement in a young patient with chronic type A aortic dissection, Marfan and mega-aorta syndromes.

Single-stage total aortic replacement represents a comprehensive approach for patients at high risk of aorta-related complications between procedures. It not only avoids staged surgical treatment but also facilitates quicker rehabilitation. Opting for a radical surgery in such cases can yield superior outcomes compared with a staged approach, making it particularly suitable for young patients with aorta-related risk factors. Moreover, a single-stage aorta repair reduces the likelihood of subsequent aortic interventions.

Clinical study of reoperation for acute type A aortic dissection.

Objective: The initial operation for type A aortic dissection has limitations, and there may be a need for reoperation in cases such as giant pseudoaneurysm formation and reduced blood supply to the distal vessels. In this study, we collected case data of patients who underwent cardiac major vascular surgery at our hospital to analyze the effectiveness of reoperation treatment options for type A aortic dissection and to summarize our treatment experience. Method: Between June 2018 and December 2022, 62 patients with type A aortic dissection (TAAD) underwent reoperation after previous surgical treatment. Of these, 49 patients (45 males) underwent endovascular aortic repair (EVAR) with a mean age of (49.69 ± 10.21) years (30-75 years), and 13 patients (11 males) underwent thoracoabdominal aortic replacement (TAAR) with a mean age of (41.00 ± 11.18) years (23-66 years). In this study, we retrospectively analyzed the recorded data of 62 patients. In addition, we summarized and analyzed their Computed Tomographic Angiography (CTA) results and perioperative complications. Outcome: In the EVAR group, 47 patients (95.92%) were successfully implanted with overlapping stents, and 2 patients died in the perioperative period. Postoperative complications included cerebral infarction (4.08%), acute renal insufficiency (30.61%), pulmonary insufficiency and need for ventilator (6.12%), poor wound healing (2.04%), postoperative reoperation (16.33%), and lower limb ischemia (2.04%). In the TAAR group, 12 patients (92.31%) were successfully revascularized and 1 patient died in the perioperative period. Postoperative complications included cerebral infarction (7.69%), acute kidney injury (46.15%), pulmonary insufficiency and need for ventilator (15.38%), poor wound healing (30.77%) and postoperative reoperation (15.38%). Conclusion: According to the results of the study, compared with TAAR, EVAR was less invasive, faster recovery, and offered a better choice for some high-risk and high-age patients with comorbid underlying diseases. However, the rate of revascularization was higher after EVAR than TAAR due to vascular lesions. Compared with the use of ascending aortic replacement + hemi-aortic arch replacement for acute type A aortic dissection in many countries and regions, the use of ascending aortic replacement + aortic arch replacement + elephant trunk stent is more traumatic in China, but facilitates reoperation. For young patients, the choice of treatment should be individualized combining vascular lesions and long-term quality of life.

Debranching abdominal aortic hybrid surgery for aortic diseases involving the visceral arteries.

Objective: Aortic diseases involving branches of the visceral arteries mainly include thoracoabdominal aortic aneurysm (TAAA), aortic dissection (AD) and abdominal aortic aneurysm (AAA). The focus of treatment is to reconstruct the splanchnic arteries and restore blood supply to the organs. Commonly used methods include thoracoabdominal aortic replacement, thoracic endovascular aortic repair and hybrid approaches. Hybrid surgery for aortic disease involving the visceral arteries, consisting of visceral aortic debranching with retrograde revascularization of the celiac trunk and renal arteries and using stent grafts, has been previously described and may be considered particularly appealing in high-risk patients. This study retrospectively analyzed recorded data of patients and contrasted the outcomes with those of a similar group of patients who underwent conventional open repair surgery. Methods: Between 2019 and 2022, 72 patients (52 men) with an average age of 61.57 ± 8.66 years (range, 36-79 years) underwent one-stage debranching abdominal aortic hybrid surgery. These patients, the hybrid group, underwent preoperative Computed Tomographic Angiography (CTA) and had been diagnosed with aortic disease (aneurysm or dissection) involving the visceral arteries and were at high risk for open repair. The criteria used to define these patients as high-risk group who are in the need of hybrid treatment were American Society of Anesthesiologists (ASA) class 3 or 4. In all cases, we accomplished total visceral aortic debranching through a previous visceral artery retrograde revascularization with synthetic grafts (customized Y or four-bifurcated grafts), and aortic endovascular repair with one of two different commercially produced stent grafts (Medtronic® and Lifetech®). In some cases, we chose to connect the renal artery to the artificial vessel with a stent graft (Viabahn) and partly or totally anastomosed. We analyzed the results and compared the outcomes of the hybrid group with those of a similar group of 46 patients (36 men) with an average age 54.15 ± 12.12 years (range, 32-76). These 46 patients, the conventional open group, were selected for having had thoracoabdominal aortic replacement between 2019 and 2022. Results: In the hybrid group, 72 visceral bypasses were completed, and endovascular repair was successful in all cases. No intraoperative deaths occurred. Perioperative mortality was 2.78%, and perioperative morbidity was 9.72% (renal insufficiency in 1, unilateral renal infarction in 5, Intestinal ischemia in 1). At 1-month postoperative CTA showed 2 endoleaks, one of which was intervened. At follow-up, there were unplanned reoperation rate of 4.29% and 5 (7.14%) deaths. The remaining patients' grafts were patent at postoperative CTA and no endoleak or stent graft migration had occurred. In the conventional open group, 1 died intraoperatively, 4 died perioperatively, perioperative mortality was 10.87% and complications were respiratory failure in 5, intestinal paralysis/necrosis in 4, renal insufficiency in 17, and paraplegia in 2. At follow-up, 5 (12.20%) patients presented with synthetic grafts hematoma 4 (9.76%) patient died, and 6 (14.63%) patients required unplanned reoperation intervention. Conclusion: Hybrid surgery is technically feasible in selected cases. For aortic diseases involving the visceral arteries, the application of hybrid abdominal aorta debranching can simplify the operation process, decrease the risks of mortality and morbidity in high-risk and high-age populations and decrease the incidence of various complications while achieving ideal early clinical efficacy. However, a larger series is required for valid statistical comparisons, and longer follow-ups are necessary to evaluate the long-term efficacy of hybrid surgery.

Thoracoabdominal Aortic Replacement Together with Curative Oncological Surgery in Retroperitoneal and Spinal Tumours.

Malignancies with an extended encasement or infiltration of the aorta were previously considered inoperable. This series demonstrates replacement and subsequent resection of the thoracoabdominal aorta and its large branches as an adjunct to curative radical retroperitoneal and spinal tumor resection. Five consecutive patients were enrolled between 2016 and 2020, suffering from cancer of unknown primary, pleomorphic carcinoma, chordoma, rhabdoid sarcoma, and endometrial cancer metastasis. Wide surgical resection was the only curative option for these patients. For vascular replacement, extracorporeal membrane oxygenation (ECMO) was used as a partial left-heart bypass. The early technical success rate was 100% for vascular procedures and all patients underwent complete radical tumour resection with negative margins. All patients required surgical revision (liquor leak, n = 2; hematoma, n = 3; bypass revision, n = 1; bleeding, n = 1; biliary leak, n = 1). During follow-up (average 47 months, range 22-70) primary patency rates of aortic reconstructions and arterial bypasses were 100%; no patient suffered from recurrent malignant disease. Thoracoabdominal aortic replacement with rerouting of visceral and renal vessels is feasible in oncologic patients. In highly selected young patients, major vascular surgery can push the limits of oncologic surgery further, allowing a curative approach even in extensive retroperitoneal and spinal malignancies.

Intervention rates and outcomes in medically managed uncomplicated descending thoracic aortic dissections.

OBJECTIVE: To evaluate the long-term incidence and outcome of aortic interventions for medically managed uncomplicated thoracic aortic dissections. METHODS: Between January 2012 and December 2018, 91 patients were discharged home with an uncomplicated, medically treated aortic dissection (involving the descending aorta with or without aortic arch involvement, no ascending involvement). After a median period of 4 (first quartile: 2, third quartile: 11) months, 30 patients (33%) required an aortic intervention. Patient characteristics, radiographic, treatment, and follow-up data were compared for patients with and without aortic interventions. A competing risk regression model was analyzed to identify independent predictors of aortic intervention and to predict the risk for intervention. RESULTS: Patients who underwent aortic interventions had significantly larger thoracic (P = .041) and abdominal (P = .015) aortic diameters, the dissection was significantly longer (P = .035), there were more communications between both lumina (P = .040), and the first communication was significantly closer to the left subclavian artery (P = .049). A descending thoracic aortic diameter exceeding 45 mm was predictive for an aortic intervention (P = .001; subdistribution hazard ratio: 3.51). The risk for aortic intervention was 27% ± 10% and 36% ± 11% after 1 and 3 years, respectively. Fourteen patients (47%) underwent thoracic endovascular aortic repair, 11 patients (37%) thoracic endovascular aortic repair and left carotid to subclavian bypass, 3 patients (10%) total arch replacement with the frozen elephant trunk technique, and 2 patients (7%) thoracoabdominal aortic replacement. We observed no in-hospital mortality. CONCLUSIONS: The need for secondary aortic interventions in patients with initially medically managed, uncomplicated descending aortic dissections is substantial. The full spectrum of aortic treatment options (endovascular, hybrid, conventional open surgical) is required in these patients.

Open descending thoracic aorta aneurysm repair with removal of multiple previous endovascular devices.

False lumen patency is a poor prognostic factor for favorable aortic remodeling in patients with chronic aortic dissection. Several endovascular techniques are available to obliterate the false lumen; however, they are not always successful. We present the case of a 55-year old male with a chronic type B dissection and a large descending thoracic aortic aneurysm with rapid growth, up to 90 mm, despite attempted control of the false lumen with several endovascular devices (thoracic aortic stent graft, Amplatzer device, glue and candy plug). Successful aneurysmal open repair was achieved by removing these devices during the procedure. This case demonstrates the technical challenges of explanting an aortic stent graft and multiple other devices during distal aortic repair.

Staged graft replacement with thoracic endovascular aneurysm repair for an extensive thoracoabdominal aortic aneurysm after total arch replacement.

BACKGROUND: Open surgery for thoracoabdominal aortic aneurysm is highly invasive. Staged repair for extensive TAAA is effective because it has low morbidity and mortality, and preserves spinal cord perfusion. An initial total arch replacement can create a proximal landing zone for thoracic endovascular aneurysm repair. CASE PRESENTATION: We performed a staged hybrid thoracoabdominal aortic aneurysm repair after total arch replacement, which consisted of a primary open repair procedure as Crawford Extent III and IV thoracoabdominal aortic aneurysms, and a secondary thoracic endovascular aneurysm repair for the residual lesions for four patients. No spinal cord injury was observed. In one patient, the residual descending aortic aneurysm ruptured six months after the primary open surgery. CONCLUSIONS: Overall, staged hybrid repair is effective and shows low morbidity and mortality. Secondary thoracic endovascular aneurysm repair should be performed as soon as possible to reduce the risk of residual aneurysm rupture.

Total Aortic Arch Replacement and Frozen Elephant Trunk.

Aortic arch pathologies have been a surgical challenge, involving cerebral, visceral and myocardial protection. Innovative techniques including total arch replacement and frozen elephant trunk had evolved over last decades with promising mid-term outcomes. We evaluate our mid-term outcomes on total arch replacement with frozen elephant trunk and the role of timely second staged interventions. Between August 2014 and April 2020, 41 patients with aortic arch pathologies underwent total arch replacement with frozen elephant trunk with Thoraflex-Hybrid-Plexus device (Vascutek, Inchinnan, Scotland). Patients' perioperative, clinical and radiological outcomes were reviewed. Post discharge survival (n = 37) at 3 year was 100%. Overall survival of 85.3% over a median follow up of 3.3 years, inpatient mortality of 9.8%. Distribution of aortic pathologies with acute type A dissection or intramural hematoma (n = 15, 36.6%), thoracic aortic aneurysm, including arch and descending aortic aneurysm (n = 9, 22%) and chronic aortic dissection including chronic type A and type B dissections (n = 13, 31.7%). Mean operative, circulatory arrest, and antegrade cerebral perfusion time were 417 ± 121 minutes, 89 ± 28 minutes, and 154 ± 43 minutes, respectively. Second stage procedures were performed in 32% and distal stent graft induced new entry was observed in 19% of patients. We reported an Asian series of Thoraflex with outstanding midterm clinical outcomes, given descending aortic pathologies were tackled with a timely second stage interventions. The observation of aortic remodeling and distal stent graft induced new entry requires further investigations.

Left Atrial to Femoral Artery Full Cardiopulmonary Bypass: A Novel Technique for Descending and Thoracoabdominal Aortic Surgery.

Left atrial-femoral artery (LA-FA) bypass with a centrifugal pump and no oxygenator is commonly used for descending and thoracoabdominal aortic (DTAA) operations, mitigating the deleterious effects of cross-clamping. We present our initial experience performing DTAA replacement under LA-FA (left-to-left) cardiopulmonary bypass (CPB) with an oxygenator. DTAA replacement under LA-FA bypass with an oxygenator was performed in 14 consecutive patients (CPB group). The pulmonary vein and femoral artery (or distal aorta) were cannulated and the full CPB machine were used, including oxygenator, roller pump, pump suckers, and kinetically enhanced drainage. The CPB group was compared with 50 consecutive patients who underwent DTAA replacement utilizing traditional LA-FA bypass without an oxygenator (LA-FA group). Perioperative data were collected and statistical analyses were performed. All CPB patients maintained superb cardiopulmonary stability. The pump sucker permitted immediate salvage and return of shed blood. Superb oxygenation was maintained at all times. High-dose full CPB heparin was reversed without difficulty. The CPB group required markedly fewer blood transfusions than the LA-FA group (2.21 vs. 5.88 units, p < 0.004). The 30-day mortality rate was 7.1% ( n = 1) and there were no paraplegia cases in the CPB group versus 7 (14%) deaths and 3 (6%) paraplegia cases in the LA-FA group. Traditional LA-FA bypass without an oxygenator avoids high-dose heparin. In the present era, heparin reversal is more secure. Our experience finds that the novel application of LA-FA CPB with an oxygenator is safe and suggests improved hemodynamics (immediate return of shed blood) and a hemostatic advantage (avoidance of loss of coagulation factors in the cell saver).

Treatment strategy selection of Marfan syndrome combined with thoracoabdominal aortic diseases / 中国胸心血管外科临床杂志

@#Objective    To summarize the surgical treatment plan and experience of patients with Marfan syndrome complicated with Stanford type B aortic dissection, and to explore the treatment strategy selection. Methods    A retrospective analysis was conducted on 27 patients with Marfan syndrome complicated with thoracoabdominal aortic diseases who were treated in the department of cardiovascular surgery of our hospital from January 2013 to June 2019, including 13 males and 14 females, with an average age of 32.2±8.6 years. According to the patients' conditions, 19 of them received single pump-assisted blood transfusion combined with total thoracoabdominal aortic replacement (TAAAR), and 8 received thoracic endovascular aortic repair (TEVAR) in critically ill and pregnant patients. The patients were followed up in the outpatient clinic, and the thoracoabdominal aortic CT angiography was reexamined at 3 months, 6 months, 12 months and annually. The outcome of surgery, the incidence of intermediate cardiovascular adverse events, defined as the reoperation due to aortic or cardiac diseases, and intermediate survival rate were studied. Results    All 27 patients successfully completed the operation, the operation time was 60-852 (395.10±222.60) min, the spinal cord ischemia time was 14-26 (19.33±3.44) min, and the abdominal viscera ischemia time was 16-23 (19.83±1.94) min. Eight patients of TEVAR were all operated in acute phase and 19 patients of TAAAR in chronic phase. Two early postoperative deaths occurred in TEVAR patients. One died of puerperal infection and multiple organ dysfunction after cesarean section at the same time. After TEVAR, type A dissection re-ocurred in one patient. The family member gave up the treatment, and the patient died of the dissection ruptured after cesarean section. During the average follow-up of 47.6±36.7 months, 1 patient died of cerebrovascular accident and 9 patients were reoperated for adverse cardiovascular events, including 4 in TEVAR and 5 in TAAAR. Conclusion    TAAAR is the first choice for the treatment of Marfan syndrome combined with thoracoabdominal aortic diseases. TEVAR is easy to operate, with a low incidence of early mortality and complications, but has the risk of internal leakage and avulsion, and a high reoperation rate in the middle stage, so it can be used for high-risk elderly patients not suitable for open surgery, or as a bridge therapy for emergency patients before open surgery.

Thoracoabdominal aortic replacement in patients aged 50 and younger.

OBJECTIVE: Open repair of a thoracoabdominal aortic aneurysm (TAAA) has been regarded as one of the most invasive procedures in cardiovascular surgery. Conversely, endovascular technology currently enables the repair of the thoracoabdominal aorta, and this approach is less invasive. However, the long-term durability of this method of endovascular repair remains unknown. This investigation retrospectively analyzed the long-term durability of thoracoabdominal aorta repair in patients aged 50 and younger. PATIENTS AND METHODS: Since 1995, 100 patients aged 50 and younger underwent thoracoabdominal aortic replacement at our institution. There were 63 males, and the average age was 38 ± 7. Ninety-six had aortic dissection as an aortic pathology. Marfan syndrome was found in 61 patients, Loeys-Dietz in 10, Acta 2 mutations in 4, aortitis in 2, and Ehlers-Danlos syndrome in 1. There were 2 patients with a type I TAAA, 56 with a type II, 33 with a type III, and 9 with a type IV. RESULTS: There were 3 hospital deaths (3%), of which 2 were emergent cases. Spinal cord injury occurred in 1 patient (1%). Two patients (2%) had wound complications. Three patients suffered cerebral hemorrhage and 1 had an intramedullary infection, of which all were associated with cerebrospinal fluid drainage. The 3-year survival rate after the operation was 94%, that of 5 years was 94%, and that of 10 years was 91%. CONCLUSIONS: Results of thoracoabdominal aortic replacement in patients aged 50 and younger were favorable. While spinal cord complications were rare, cerebrospinal drainage revealed several complications in this series. Evolving endovascular repair needs to be compared with these results, especially in patients aged 50 and younger.

Thoracoabdominal aortic replacement for Crawford extent II aneurysm after thoracic endovascular aortic repair.

BACKGROUND: The surgical treatment of Crawford extent II aneurysms after thoracic endovascular aortic repair (TEVAR) remains challenging, because of the need to remove the failed endograft and the complexity of the aortic reconstruction. We retrospectively reviewed our experience with surgical management of Crawford extent II aneurysms after TEVAR using thoracoabdominal aortic replacement (TAAR). METHODS: Eleven patients (10 males, 1 female) with Crawford extent II aneurysm after TEVAR were treated with TAAR between August 2012 and May 2015. The indications included: diameter >5.0 cm; persistent pain; size increase >0.5 cm/year; and no suitable landing zone for re-TEVAR. Five patients underwent surgery under deep hypothermic cardiac arrest, two under mild hypothermic cardiopulmonary bypass, and four under direct aortic cross-clamping under normothermia. Two patients had Marfan syndrome. RESULTS: There were no in-hospital deaths. Continuous renal replacement therapy was required in three patients. One patient needed re-intubation, and two patients had prolonged intubation (>72 h). One patient sustained paraplegia after surgery but recovered during follow-up. Cerebrospinal fluid drainage were used in four patients (3 immediately in the operation room, and 1 in the intensive care unit when the patient suffered paraplegia). One patient died during follow-up. CONCLUSIONS: TAAR represents a feasible option for the treatment of Crawford extent II aneurysms after TEVAR, with acceptable surgical risks and favorable results.

Hybrid Thoracic Endovascular Aortic Repair for Intercostal Patch Aneurysm after Thoracoabdominal Aortic Replacement.

We report a case of hybrid thoracic endovascular aortic repair for intercostal patch aneurysm after thoracoabdominal aortic replacement. Eighteen years ago, a 63-year-old woman with Marfan syndrome had undergone thoracoabdominal aortic replacement with reimplantation of the intercostal artery in an island fashion. Follow-up computed tomography (CT) revealed a remaining intercostal patch aneurysm of diameter 60 mm 17 years after the last operation. Hybrid thoracic endovascular aortic repair for exclusion of this intercostal patch aneurysm was successfully performed, with visceral artery bypasses. Postoperative CT showed no anastomotic stenosis or endoleak.

Thoracoabdominal aortic aneurysm repair after frozen elephant trunk procedure.

OBJECTIVES: To evaluate the feasibility and the outcomes of second-stage thoracoabdominal (TA) repair after previous frozen elephant trunk (FET) implantation. METHODS: Between 2005 and 2013, 41 patients underwent open TA aortic repair in our institution. Of these, 9 patients (78% male) underwent second-stage TA repair after previous FET implantation. Feasibility and outcomes were evaluated. RESULTS: The mean interval between FET implantation and second-stage TA repair was 423 days (19-1979 days). Indications for second-stage TA repair were progression in aortic diameter of atherosclerotic aneurysms in the downstream segments in 6 patients, diameter progression in post-dissection aneurysms in 2 patients and giant cell aortitis with aneurysm formation in another patient. There were no in-hospital deaths. The median intensive care unit stay was 3.5 days (range: 1-12 days) and median hospital stay was 22 days (range: 14-132 days). We did not observe symptomatic spinal cord ischaemia or stroke. One patient (11%) developed acute renal failure requiring haemodialysis. CONCLUSION: Second-stage TA aortic repair after previous frozen elephant implantation is a feasible and effective treatment modality for patients with various pathologies of downstream aortic segments. This approach adds additional value to the conventional elephant trunk technique by providing an excellent landing zone not only for additional stent graft procedures but also for subsequent open TA repair.

Heparin Anticoagulation during Cardiopulmonary Bypass for Thoracoabdominal Aorta Replacement in a Patient with a History of Heparin-Induced Thrombocytopenia / 日本心臓血管外科学会雑誌

Immune heparin-induced thrombocytopenia (HIT) is a crucial side effect of heparin therapy. We report the case of a 52-year-old man who was strongly suspected of having HIT after urgent descending aorta replacement. This case required continuous hemodiafiltration (CHDF) anticoagulated with unfractionated heparin (UFH) for acute renal failure after the operation. The patient developed thrombocytopenia and thrombus emphraxis in the circuit on the seventh day and was suspected of having HIT. UFH was ceased and replaced with argatroban. After then, thrombus emphraxis was not seen in the circuit and the platelet count was recovered promptly. He tested positive in an enzyme-linked immunosorbent assay for anti-platelet factor 4/heparin antibodies (anti-PF4/H Abs). Six months later, we found, an expanding thoracoabdominal aortic aneurysm and performed thoracoabdominal aorta replacement. We selected heparin anticoagulation for cardiopulmonary bypass because anti-PF4/H Abs were negative at that time. Thrombus emphraxis was not found during the operation. The patient developed neither thrombocytopenia nor thrombosis in the perioperative period.