Repair of thoracic aortic aneurysm with bilateral aberrant subclavian artery.

We present a rare anatomical configuration of a 19-year-old woman, characterized by descending thoracic aortic aneurysm with right aberrant subclavian arteries with a Kommerell's diverticulum in a left aortic arch. The complexity of this vascular anomaly was accompanied by an anomalous origin of left subclavian artery. The patient underwent a single-stage open surgical repair via left thoracotomy under deep hypothermic circulatory arrest. The bilateral aberrant subclavian arteries were separately reconstructed in situ using hand-sewn branched grafts.

Insights From the Histopathologic Analysis of Acquired and Genetic Thoracic Aortic Aneurysms and Dissections.

OBJECTIVE: The purpose of this study was to apply contemporary consensus criteria developed by the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology to the evaluation of aortic pathology, with the expectation that the additional pathologic information may enhance the understanding and management of aortic diseases. METHODS: A scoring system was applied to ascending aortic specimens from 42 patients with heritable thoracic aortic disease and known genetic variations and from 86 patients from a single year, including patients with known genetic variations (n = 12) and patients with sporadic disease (n = 74). RESULTS: The various types of lesions of medial degeneration and the overall severity of medial degeneration overlapped considerably between those patients with heritable disease and those with sporadic disease; however, patients with heritable thoracic aortic disease had significantly more overall medial degeneration (P = .004) and higher levels of elastic fiber fragmentation (P = .03) and mucoid extracellular matrix accumulation (P = .04) than patients with sporadic thoracic aortic disease. Heritable thoracic aortic disease with known genetic variation was more prevalent in women than in men (27.2% vs 9.8%; P = .04), and women had more severe medial degeneration than men (P = .04). Medial degeneration scores were significantly lower for patients with bicuspid aortic valves than for patients with tricuspid aortic valves (P = .03). CONCLUSION: The study's findings indicate considerable overlap in the pattern, extent, and severity of medial degeneration between sporadic and hereditary types of thoracic aortic disease. This finding suggests that histopathologic medial degeneration represents the final common outcome of diverse pathogenetic factors and mechanisms.

Open repair with latissimus muscle flap coverage for treatment of infected thoracic endovascular aneurysm repair.

A male patient, 70 years of age, was evaluated for an infected thoracic endovascular aneurysm repair (TEVAR). After presenting with persistent fever, a positron emission tomography scan found an infected aortic stent graft. The patient underwent open repair with explantation of the infected TEVAR, extensive periaortic debridement, graft replacement with a Dacron graft, and complete coverage with a latissimus dorsi muscle flap. Tissue culture revealed Clostridium spp. He was discharged home with long-term ampicillin and sulbactam. A postoperative computed tomography scan showed no recurrence of infection. Open surgery with latissimus muscle flap coverage is an achievable option for infected TEVAR.

Outcomes of cardiothoracic surgery in women with Turner syndrome.

Background: To describe short- and mid-term surgical outcomes of patients with Turner syndrome (TS) after cardiovascular interventions. Methods: All individuals >12 years of age at the time of surgical repair for cardiovascular disease (valve or coarctation repairs, aortic disease, aortic dissection) from 2002 to 2022 were eligible. The primary endpoint was complications or death within 30 days of intervention. Secondary outcomes included late complications and reinterventions within six months. Combined data from the University of Texas Health Science Center at Houston and the Turner Syndrome Society of the United States were included in the analysis. Results: We identified 22 patients who met the inclusion criterion. The median age was 46 years (range, 21-75 years), with 86% having estrogen replacement therapy. The most common medical condition was hypertension (77%), followed by hypothyroidism (59%). The most frequent indication for surgery was aortic root or ascending aortic aneurysms (68%), followed by symptomatic aortic stenosis in patients with bicuspid aortic valve (64%), coarctation of aorta (45%), and acute aortic dissection (18%). Respiratory complications were the most common (68%). Pleural effusions were the most frequent found sign on imaging studies (68%). Thoracentesis, or chest tube placement, was required in 33% (5/15). Respiratory failure requiring specific support with high flow oxygen and/or thoracentesis occurred in 36% (8/22). Conclusions: Patients with TS may be at an increased risk for postoperative complications after aortic surgery. Bicuspid aortic valve (59%) and coarctation of the aorta (45%) were the most common congenital malformations among our study group. Our study showed that respiratory complications were the most common, with pleural effusions being the most common post-surgery complication.

Hybrid management of type B aortic dissection in a patient with right-sided aortic arch and aberrant left subclavian artery.

This report describes a patient with a right-sided aortic arch, aberrant left subclavian artery and Kommerell diverticulum, who presented with aneurysmal degeneration of the aortic root to the descending aorta, in addition to an acute type B2-10 aortic dissection. He underwent hybrid treatment with a valve-sparing aortic root replacement, transverse arch replacement with reattachment of the right subclavian artery, bilateral common carotid arteries, and thoracic endovascular aneurysm repair with left subclavian artery embolization and a left common carotid to subclavian artery bypass.

Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants.

Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck paragangliomas, which, in almost 20% of patients, might coexist with paragangliomas arising from other locations (eg, adrenal medulla, para-aortic, cardiac or thoracic, and pelvic). Given the higher risk of tumour multifocality and bilaterality for phaeochromocytomas and paragangliomas (PPGLs) because of SDHD pathogenic variants than for their sporadic and other genotypic counterparts, the management of patients with SDHD PPGLs is clinically complex in terms of imaging, treatment, and management options. Furthermore, locally aggressive disease can be discovered at a young age or late in the disease course, which presents challenges in balancing surgical intervention with various medical and radiotherapeutic approaches. The axiom-first, do no harm-should always be considered and an initial period of observation (ie, watchful waiting) is often appropriate to characterise tumour behaviour in patients with these pathogenic variants. These patients should be referred to specialised high-volume medical centres. This consensus guideline aims to help physicians with the clinical decision-making process when caring for patients with SDHD PPGLs.

Open Treatments for Thoracoabdominal Aortic Aneurysm Repair.

Thoracoabdominal aortic aneurysms (TAAA) represent a unique pathology that is associated with considerable mortality if untreated. While the advent of endovascular technologies has introduced new modalities for consideration, the mainstay of TAAA treatment remains open surgical repair. However, the optimal conduct of open TAAA repair requires careful consideration of patient risk factors and a collaborative team effort to mitigate the risk of perioperative complications. In this chapter, we briefly outline the history of treating TAAA, preoperative preparation and postoperative care, and our operative techniques for treatment.

Reappraisal of the role of motor and somatosensory evoked potentials during open distal aortic repair.

OBJECTIVE: Intraoperative motor and somatosensory evoked potentials have been applied to monitor spinal cord ischemia during repair. However, their predictive values remain controversial. The purpose of this study was to evaluate the impact of motor evoked potentials and somatosensory evoked potentials on spinal cord ischemia during open distal aortic repair. METHODS: Our group began routine use of both somatosensory evoked potentials and motor evoked potentials at the end of 2004. This study used a historical cohort design, using risk factor and outcome data from our department's prospective registry. Univariate and multivariable statistics for risk-adjusted effects of motor evoked potentials and somatosensory evoked potentials on neurologic outcome and model discrimination were assessed with receiver operating characteristic curves. RESULTS: Both somatosensory evoked potentials and motor evoked potentials were measured in 822 patients undergoing open distal aortic repair between December 2004 and December 2019. Both motor evoked potentials and somatosensory evoked potentials were intact for the duration of surgery in 348 patients (42%). Isolated motor evoked potential loss was observed in 283 patients (34%), isolated somatosensory evoked potential loss was observed in 18 patients (3%), and both motor evoked potential and somatosensory evoked potential loss were observed in 173 patients (21%). No spinal cord ischemia occurred in the 18 cases with isolated somatosensory evoked potential loss. When both signals were lost, signal loss happened in the order of motor evoked potentials and then somatosensory evoked potentials. Immediate spinal cord ischemia occurred in none of those without signal loss, 4 of 283 (1%) with isolated motor evoked potential loss, and 15 of 173 (9%) with motor evoked potential plus somatosensory evoked potential loss. Delayed spinal cord ischemia occurred in 12 of 348 patients (3%) with intact evoked potentials, 24 of 283 patients (8%) with isolated motor evoked potentials loss, and 27 of 173 patients (15%) with motor evoked potentials + somatosensory evoked potentials loss (P < .001). Motor evoked potentials and somatosensory evoked potentials loss were each independently associated with spinal cord ischemia. For immediate spinal cord ischemia, no return of motor evoked potential signals at the conclusion of the surgery had the highest odds ratio of 15.87, with a receiver operating characteristic area under the curve of 0.936, whereas motor evoked potential loss had the highest odds ratio of 3.72 with an area under the curve of 0.638 for delayed spinal cord ischemia. CONCLUSIONS: Somatosensory evoked potentials and motor evoked potentials are both important monitoring measures to predict and prevent spinal cord ischemia during and after open distal aortic repairs. Intraoperative motor evoked potential loss is a risk for immediate and delayed spinal cord ischemia after open distal aortic repair, and somatosensory evoked potential loss further adds predictive value to the motor evoked potential.

Multiple Interventions to Thoracoabdominal Aortic Aneurysm in a Child With Tuberous Sclerosis.

Thoracoabdominal aortic aneurysms in toddlers are extremely rare. However, we experienced an extent III thoracoabdominal aortic aneurysm in a boy with tuberous sclerosis who underwent 3 open repairs and 1 endovascular aortic repair between the ages of 4 years and 18 years. This case highlights the potential for severe recurrent vascular aneurysms in the thoracic and abdominal aorta as a complication of tuberous sclerosis in children. Although aortic aneurysms in children are rare, it is vital to recognize these cases to prevent death due to rupture.

Deep Hypothermic Circulatory Arrest With Retrograde Cerebral Perfusion: How Long Is Safe?

BACKGROUND: Cerebral protection strategies during proximal aortic repair remains controversial due to lack of evidence and large patient cohort studies. We herein evaluated our 3-decade experience using hypothermic circulatory arrest with retrograde cerebral perfusion (DHCA/RCP) to evaluate for its safety and safe duration during proximal aortic repair. METHODS: All proximal aortic repairs using DHCA/RCP from January 1991 to December 2020 performed at our institution were included in the analyses. Perioperative variables were evaluated for mortality and cerebrovascular accident (CVA; combined stroke and transient ischemic attack). RESULTS: In all, 1429 repairs were performed using DHCA/RCP. Of these, 464 (32%) were acute aortic dissection and 297 (21%) were resternotomy. The median age was 61 years (interquartile range 50-70 years). Operative mortality was 8.9% and CVA occurred in 8.4% (stroke 7.8%, transient ischemic attack 0.6%). There was a linear relationship between the RCP time and the incidence of immediate postoperative CVA. Incidence of CVA was less than 5% when RCP time was less than 20 minutes, 6.3% at 30 minutes, and 11.5% at 60 minutes. Multivariable analysis demonstrated acute type A aortic dissection (odds ratio 2.58, 95% CI1.49-4.48, P = .001) was the only predictor for postoperative CVA but RCP time was not (odds ratio 0.991, 95% CI 0.962-1.02, P = .527). CONCLUSIONS: DHCA/RCA provided satisfactory outcomes after proximal aortic operations. The safe duration of RCP with DHCA was up to 30 minutes in our experience. When the circulatory arrest time is expected to exceed 60 minutes, other adjuncts for cerebral protection should be recommended.

Rapid cooling is a safe technique in patients undergoing circulatory arrest for aortic repair.

Objective: To evaluate our institutional experience with rapid cooling for hypothermic circulatory arrest in proximal aortic repair. Methods: We retrospectively reviewed data from 2171 patients who underwent proximal aortic surgery requiring hypothermic circulatory arrest between 1991 and 2020. Cooling times were divided into quartiles and clinical outcome event rates were compared across quartiles using contingency table methods. Incremental effect of cooling time was assessed in the context of other perfusion time variables using multiple logistic regression analysis. Results: Median age was 61 years (interquartile range, 49-70 years) and 34.1% of patients were women. The procedure was emergent in 33.5% of patients, 22.9% had a previous sternotomy. The median circulatory arrest time was 22 minutes, with retrograde cerebral perfusion used in 94% of cases. Median cardiopulmonary bypass time was 149 minutes, with an aortic crossclamp time of 90 minutes. Patients were cooled to deep hypothermia. The first quartile had cooling times ranging from 5 to 13 minutes, second 14 to 18 minutes, third 19-23 minutes, and fourth 24-81 minutes. Overall, 30-day mortality was 9.4%, and was not significantly different across quartiles. There was a statistically significant trend toward lower rates of postoperative encephalopathy, gastrointestinal complications, and respiratory failure with shorter cooling times (P < .001, .006, and < .001, respectively). There was no significant difference in rates of postoperative stroke or dialysis. Conclusions: Rapid cooling can be performed safely in patients undergoing aortic surgery requiring circulatory arrest without increasing mortality or stroke. There were significantly lower rates of coagulopathy, respiratory failure, and postoperative encephalopathy with shorter cooling times.

Total Transfemoral Percutaneous Endovascular Aortic Arch Repair Using 3-Vessel Inner Branch Stent-Graft.

Endovascular repair has been introduced to decrease the morbidity and mortality associated with open surgical repair of aortic arch pathology. We illustrate total percutaneous transfemoral approach with a 3-vessel inner branch stent-graft to treat aortic arch aneurysm. (Level of Difficulty: Advanced.).

In vivo evaluation of a new hybrid graft using retrograde visceral perfusion for thoracoabdominal aortic repair in an animal model.

Objectives: The SPIDER technique for hybrid thoracoabdominal aortic aneurysm repair can avoid thoracotomy and extracorporeal circulation. To improve technical feasibility and safety, the new Thoracoflo graft, consisting of a proximal stent graft connected to a 7-branched abdominal prosthesis, was evaluated in a pig model for technical feasibility testing, before implantation in humans. Methods: Retroperitoneal exposure of the infradiaphragmatic aorta, including visceral and renal arteries, was performed in 7 pigs (75-85 kg). One iliac branch was temporarily attached to the distal aorta to maintain retrograde visceral and antegrade iliac perfusion after deployment of the thoracic stent graft segment (SPIDER technique). The proximal stent-grafted segment was deployed in the thoracic aorta via direct aortic puncture over the wire without fluoroscopy. The graft was deaired before flow via the iliac side branch to the visceral and iliac arteries was established. Visceral, renal, and lumbar arteries were subsequently sutured to the corresponding side branches of the graft. Technical feasibility, operating and clamping time, blood flow, and tissue perfusion in the related organs were evaluated before implantation and after 3 and 6 hours using transit-time flow measurement and fluorescent microspheres. Final angiography or postprocedural computed tomography angiography were performed. Results: Over-the-wire graft deployment was successful in 6 animals without hemodynamic alteration (P = n.s.). In 1 pig, the proximal stent graft section migrated as the guidewire was not removed, as recommended, before release of the proximal fixation wire. Angiography and computed tomography scan confirmed successful graft implantation and transit-time flow measurement confirmed good visceral and iliac blood flow. Fluorescent microspheres confirmed good spinal cord perfusion. Conclusions: Over-the-wire implantation of the Thoracoflo graft using the SPIDER technique is feasible in a pig model. No fluoroscopy was required. For safe implantation, it is mandatory to follow the single steps of implantation.