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.

Heritable thoracic aortic disease: a literature review on genetic aortopathies and current surgical management.

Heritable thoracic aortic disease puts patients at risk for aortic aneurysms, rupture, and dissections. The diagnosis and management of this heterogenous patient population continues to evolve. Last year, the American Heart Association/American College of Cardiology Joint Committee published diagnosis and management guidelines for aortic disease, which included those with genetic aortopathies. Additionally, evolving research studying the implications of underlying genetic aberrations with new genetic testing continues to become available. In this review, we evaluate the current literature surrounding the diagnosis and management of heritable thoracic aortic disease, as well as novel therapeutic approaches and future directions of research.

Direct and indirect activation of the adenosine triphosphate-sensitive potassium channel to induce spinal cord ischemic metabolic tolerance.

OBJECTIVES: The mitochondrial adenosine triphosphate-sensitive potassium channel is central to pharmacologically induced tolerance to spinal cord injury. We hypothesized that both direct and nitric oxide-dependent indirect activation of the adenosine triphosphate-sensitive potassium channel contribute to the induction of ischemic metabolic tolerance. METHODS: Spinal cord injury was induced in adult male C57BL/6 mice through 7 minutes of thoracic aortic crossclamping. Pretreatment consisted of intraperitoneal injection 3 consecutive days before injury. Experimental groups were sham (no pretreatment or ischemia, n = 10), spinal cord injury control (pretreatment with normal saline, n = 27), Nicorandil 1.0 mg/kg (direct and indirect adenosine triphosphate-sensitive potassium channel opener, n = 20), Nicorandil 1 mg/kg + carboxy-PTIO 1 mg/kg (nitric oxide scavenger, n = 21), carboxy-PTIO (n = 12), diazoxide 5 mg/kg (selective direct adenosine triphosphate-sensitive potassium channel opener, n = 25), and DZ 5 mg/kg+ carboxy-PTIO 1 mg/kg, carboxy-PTIO (n = 23). Limb motor function was assessed using the Basso Mouse Score (0-9) at 12-hour intervals for 48 hours after ischemia. RESULTS: Motor function was significantly preserved at all time points after ischemia in the Nicorandil pretreatment group compared with ischemic control. The addition of carboxy-PTIO partially attenuated Nicorandil's motor-preserving effect. Motor function in the Nicorandil + carboxy-PTIO group was significantly preserved compared with the spinal cord injury control group (P < .001), but worse than in the Nicorandil group (P = .078). Motor preservation in the diazoxide group was similar to the Nicorandil + carboxy-PTIO group. There was no significant difference between the diazoxide and diazoxide + carboxy-PTIO groups. CONCLUSIONS: Both direct and nitric oxide-dependent indirect activation of the mitochondrial adenosine triphosphate-sensitive potassium channel play an important role in pharmacologically induced motor function preservation.

The fate of the downstream aorta after total arch replacement.

OBJECTIVES: The goal of this study was to evaluate the fate of the downstream aorta following total arch replacement. METHODS: Between October 1999 and March 2018, a total of 740 patients underwent total arch replacement. After excluding connective tissue disease, previous descending or thoracoabdominal aortic surgery, patients without adequate preoperative images or operative mortality, late outcomes consisting of additional surgery for distal dilation and distal aortic events were evaluated in 623 survivors (240 aortic dissections, including 139 patients with acute dissection and 383 with a non-dissection aneurysm). The mean follow-up was 5.0 ± 4.0 years. RESULTS: The mean preoperative maximum diameter of the descending aorta was 36.9 ± 8.0 mm. An elephant trunk was inserted in 232 patients, including 183 patients with aortic dissection. Freedom from additional surgery for distal dilation was 88.5% at 5 years and 80.2% at 10 years. Freedom from distal aortic events was 81.9% at 5 years and 70.5% at 10 years. Multivariable regression analysis demonstrated that the preoperative diameter of the descending aorta was a significant risk factor for unfavourable distal aortic events. Computed tomography evaluation demonstrated a significant increase in the descending aortic diameter over time (P < 0.001). Positive aortic remodelling was observed in the proximal descending (P < 0.001) to mid-descending (P < 0.001) aorta exclusively in patients with acute aortic dissection. CONCLUSIONS: The diameter of the descending aorta increased significantly after total arch replacement, particularly in the distal descending aorta. The preoperative descending aortic diameter portended a significant risk for unfavourable distal aortic events.

Reoperative aortic valve replacement after bio-Bentall using a double sewing ring technique.

We report an 89-year-old haemodialysis patient with a structural valve deterioration following a Bentall-de Bono operation with a tissue valve using the double sewing ring technique. The bioprosthesis was extracted easily, and the aortic valve replacement was performed without any complications in a high-risk patient. This procedure may be feasible given the prospects of reoperation.

Repair of insufficient aortic valve and single coronary artery with intramural course.

We report about a patient with severe bicuspid aortic valve insufficiency and a single coronary artery with an intramural course. Aortic valve repair with double plication and coronary translocation was satisfactory.

Optimizing Nicorandil for Spinal Cord Protection in a Murine Model of Complex Aortic Intervention.

There are currently no clinically utilized pharmacological agents for the induction of metabolic tolerance to spinal cord ischemia-reperfusion injury in the setting of complex aortic intervention. Nicorandil, a nitric oxide donor and ATP-sensitive potassium (KATP) channel opener, has shown promise in neuroprotection. However, the optimized clinical application of the drug and its mechanism of neuroprotection remains unclear. We hypothesized that 3-days pretreatment would confer the most effective neuroprotection, mediated by mitochondrial KATP channel activation. Spinal cord injury was induced by 7 minutes of thoracic aortic cross-clamping in adult male C57BL/6 mice. Time course: mice received 0.1 mg/kg nicorandil for 10 min, 4 hours, and 3 consecutive days prior to ischemia compared with control. Dose challenge: mice received 3-days nicorandil pretreatment comparing 0.1 mg/kg, 1.0 mg/kg, 5.0 mg/kg, and saline administration. Mitochondrial KATP channel blocker 5-hydroxy-decanoate (5HD) was co-administered to elucidate mechanism. Limb motor function was evaluated, and viable anterior horn neurons quantified. Nicorandil pretreatment at 4 hours and 3 days before ischemia demonstrated significant motor function preservation; administration 10 minutes before ischemia showed no neuroprotection. All nicorandil doses showed significant motor function preservation. Three days administration of Nicorandil 1.0 mg/kg was most potent. Neuroprotection was completely abolished by 5HD co-administration. Histological analysis showed significant neuron preservation with nicorandil pretreatment, which was attenuated by 5HD co-administration. Three days administration of Nicorandil 1.0 mg/kg showed near-total motor function preservation in a murine spinal cord ischemia-reperfusion model, mediated by the mitochondrial KATP channel.

Aortic endograft sizing and endoleak, reintervention, and mortality following endovascular aneurysm repair.

OBJECTIVE: Endograft sizing for endovascular abdominal aortic aneurysm repair (EVAR) is not consistent despite published instructions for use (IFU). We sought to identify factors associated with over/undersizing, determine sex influence on sizing, and examine sizing effects on endoleak, reintervention, and mortality by analyzing data obtained from the W.L. Gore & Associates Global Registry for Endovascular Aortic Treatment (GREAT). METHODS: All patients enrolled in GREAT undergoing EVAR were included for analysis. Proximal/distal aortic landing zones were compared with device implanted to assess sizing as related to IFU. χ2/Fisher exact tests were used to evaluate associations between IFU sizing and demographics. Logistic regression modeling was used to identify predictors of outside IFU sizing. Cox proportional hazards regression analyzed the relationship between sizing and endoleak, device-related reinterventions, and all-cause/aortic mortality. RESULTS: There were 3607 EVAR subjects enrolled in GREAT as of March 2020. Of them, 1896 (53%) were within IFU for sizing, 791 (22%) were oversized, 540 (15%) were undersized, and 380 (10%) had both over- and undersized components. Factors predictive of use outside of IFU included female sex (P = .001), non-white race (P = .0003), decreased proximal neck length (P < .061), or larger iliac diameters (P < .0001). Women were more likely than men to have proximal neck undersizing and iliac limb oversizing, and men were more likely to have iliac limb undersizing. On multivariate analysis, undersizing of the proximal graft was associated with endoleak (hazard ratio [HR], 1.8) and aortic (HR, 60.5) and all-cause (HR, 18.0) mortality. Undersizing of iliac limbs was associated with endoleak (HR, 1.5) and device-related reintervention (HR, 1.4). Iliac limb outside IFU sizing was associated with aortic (HR, 2.6) and all-cause (HR, 1.3) mortality. Proximal and distal oversizing was not associated with adverse outcomes. Female sex was associated with mortality on univariate but not multivariate analysis. CONCLUSIONS: Women undergoing EVAR with GORE EXCLUDER abdominal aortic aneurysm Endoprosthesis (W.L. Gore & Associates Inc, Flagstaff, Ariz) are more likely to have proximal stent-graft undersizing and iliac limb oversizing, whereas men are more likely to have undersized iliac limbs. Proximal aortic graft undersizing is associated with endoleak and all-cause/aortic mortality, whereas undersizing of iliac limbs is associated with endoleak and device-related reintervention. Oversizing was not associated with adverse outcomes.

Reactive Oxygen Species Mediate Nicorandil-induced Metabolic Tolerance to Spinal Cord Injury.

BACKGROUND: Spinal cord injury remains a devastating complication of thoracoabdominal aortic surgery. We previously demonstrated that pretreatment with nicorandil preserved motor function in a murine spinal cord injury model through mitochondrial adenosine triphosphate-sensitive potassium channel activation. We hypothesized that the neuroprotective effect of nicorandil is mediated by downstream generation of reactive oxygen species. METHODS: Spinal cord injury was induced by 7 minutes of thoracic aortic cross-clamping in adult male C57BL/6 mice. Five groups were evaluated: ischemic control (n = 19); nicorandil 1.0 mg/kg (n = 17); nicorandil 1.0 mg/kg plus N acetyl L-cysteine (NAC [reactive oxygen species scavenger, n = 18)]) 150 mg/kg; NAC 150 mg/kg (n = 13); and sham (n = 10). Limb motor function and the number of viable neurons within the anterior horn of the spinal cord were evaluated. RESULTS: Mice in the sham group showed no functional deficits after surgery. Compared with ischemic control, motor function was significantly preserved in the nicorandil pretreatment group at every timepoint after ischemia. In the nicorandil plus NAC group, the motor-preserving effect of nicorandil was completely abolished (P < .001). Viable neuron quantification showed significant neuron preservation in the nicorandil group (29.± 2.6) compared with the ischemic control group (18.5 ± 2.1, P = .024) and nicorandil plus NAC group (14 ± 8.3, P = .001); no significant difference was observed between the ischemic control group and nicorandil plus NAC group (P = 0.768). CONCLUSIONS: Reactive oxygen species generation plays a key role in the nicorandil-induced metabolic tolerance to spinal cord injury. Manipulation of mitochondrial adenosine triphosphate-sensitive potassium channels may lead to improvement in preventing spinal cord injury after thoracoabdominal aortic interventions.

The fate of aortic root and aortic regurgitation after supracoronary ascending aortic replacement for acute type A aortic dissection.

BACKGROUND: The aim of this study was to evaluate the fate of the preserved aortic root after supracoronary aortic replacement for acute type A aortic dissection. METHODS: Between October 1999 and March 2018, 339 patients underwent supracoronary aortic replacement for acute type A aortic dissection at our institution. Late outcomes were evaluated, including overall survival, aortic-related death, and aortic root-related reoperation. The median follow-up was 3.7 years (1.4-8.4 years). RESULTS: Operative mortality was 46 patients (13.6%). The cumulative incidences at 5 years for aortic root-related reoperation, aortic-related death, and non-aortic related death were 2.5%, 14.5% and 12.4%, respectively. Multivariable Cox hazard regression analysis demonstrated greater sinus of Valsalva diameter and number of commissural detachments to be significant risk factors for a composite outcome consisting of aortic-related death or aortic root-related reoperation. Mixed-effects regression demonstrated that sinus of Valsalva diameter significantly increased with time (P < .001), and aortic regurgitation significantly worsened (P < .001). CONCLUSIONS: Sinus of Valsalva diameter and commissural detachment were independent predictors of unfavorable outcomes after supracoronary aortic replacement. Close follow-up is particularly necessary for these patients, and aortic root replacement at the time of initial operation may lead to more favorable late outcomes.

Utility of neuromonitoring in hypothermic circulatory arrest cases for early detection of stroke: Listening through the noise.

OBJECTIVE: Stroke remains a potentially devastating complication of aortic arch intervention. The value of neurophysiologic intraoperative monitoring (NIOM) in the early identification of stroke is unclear. We evaluated the utility of NIOM for early stroke detection in aortic arch surgery. METHODS: Across 8 years at our institution, 365 patients underwent aortic arch surgery with hypothermic circulatory arrest, and 224 cases utilized NIOM. One patient was excluded for intraoperative death. In the remaining cohort, we reviewed the incidence, timing, and location of strokes, and the incidence and nature of NIOM alerts. RESULTS: Hemiarch was performed in 154 patients and total arch replacement in 69 patients. Stroke occurred in 6.3% of all cases (14 out of 223), 15.9% of total arches (11 out of 69), and 2.0% of hemiarches (3 out of 154). There were 33 NIOM alerts (14.8%), and 9 patients had both alerts and stroke. Of these, NIOM deficits plausibly correlated with imaging findings in 7 cases (78%). Of the 5 stroke patients without NIOM alerts, 2 developed neurologic symptoms 3 days or more postoperatively, and infarcts in 3 patients did not result in sensory or motor deficits. Excluding 2 patients with late stroke, the sensitivity of NIOM for stroke detection was 75%, specificity was 88.5%, positive predictive value was 27.3%, and negative predictive value was 97.4%. CONCLUSIONS: Despite a low positive predictive value requiring a high level of discrimination when interpreting abnormal findings, NIOM has high sensitivity and specificity for the early stroke detection. Furthermore, its high negative predictive valve is reassuring for low risk of stroke in the absence of alerts.

Right Thoracoscopic Aberrant Right Subclavian Artery Division and Subclavian-Carotid Transposition.

Video-assisted thoracoscopic surgical division of obstructive congenital vascular rings has been demonstrated to be a safe technique in the setting of right-sided or double aortic arch, requiring a left-sided thoracic approach. This report describes 2 cases of aberrant right subclavian artery division using right video-assisted thoracoscopic surgery and subclavian-carotid transposition. This approach is safe and feasible in the management of symptomatic aberrant right subclavian artery without the morbidity of a thoracotomy incision, although patients require ongoing surveillance.

Total arch replacement in octogenarians and nonagenarians: A single-center 18-year experience.

BACKGROUND: This study evaluates our 18-year experience of total arch replacement in the octogenarian and nonagenarian population. METHODS: Between October 1999 and March 2018, a total of 740 patients underwent total arch replacement at our institution. A total of 139 patients were aged 80 years or more (83.1 ± 2.8 years), and 601 patients were aged less than 80 years (66.9 ± 11.3 years). Early and late outcomes were compared between the groups. RESULTS: In the group aged 80 years or more, operative mortality occurred in 12 patients (8.6%) and significantly improved over time (P = .010). Operative mortality was significantly higher in the group aged 80 years or more (P = .033) when compared with the group aged less than 80 years (4.0%). Regarding postoperative complications, deep sternal wound infection, pneumonia, and tracheostomy occurred in significantly more patients in the group aged 80 years or more. In the group aged 80 years or more, there were 52 late deaths, with 4 aortic-related deaths. Overall survival was 55.4% ± 5.0% at 5 years and 32.2% ± 6.1% at 8 years. Multivariable Cox-hazard regression analysis demonstrated that chronic kidney disease, nonelective surgery, and concurrent procedures were significant risk factors for overall survival in the group aged 80 years or more. Cumulative incidence for reoperation was significantly lower in the group aged 80 years or more (8.7% at 5 years) compared with the group aged less than 80 years (14.2% at 5 years). CONCLUSIONS: Total arch replacement was performed with an acceptable overall survival in octogenarians and nonagenarians, although operative mortality was higher than in younger patients. However, older patients had a lesser burden of reoperation compared with younger patients.

A successful report of mitral valve repair for parachute-like mitral valve in adult.

A 65 year-old man was diagnosed with congestive heart failure secondary to severe mitral regurgitation resulting from a parachute-like asymmetrical mitral valve. We performed mitral annuloplasty and triangular resection of the thick tissue. The postoperative course was uneventful with no recurrence of mitral regurgitation.

Impact of shaggy aorta on outcomes of open thoracoabdominal aortic aneurysm repair.

BACKGROUND: The aim of this study was to evaluate the impact of diffuse aortic atherosclerosis-related thrombosis, or "shaggy aorta" on the outcomes of open thoracoabdominal aortic aneurysm repair (TAAA). METHODS: From October 1999 to March 2018, 251 patients underwent open TAAA repair using segmental-staged aortic clamping. Twenty-eight patients (11.2%) received emergent or urgent operations. Patients were classified into 3 groups: dissection aneurysm (139 patients, 55.4%), degenerative aneurysm without shaggy aorta (76 patients, 30.3%), and degenerative aneurysm with shaggy aorta (36 patients, 14.3%). Shaggy aorta was assessed using enhanced computed tomography and defined as patients with atheroma thickness ≥5 mm with irregular atheroma surface. Mean follow-up was 4.3 ± 4.1 years. RESULTS: Operative mortality was 8% (20 patients) and spinal cord injury occurred in 25 patients (10.0%), 16 of whom (6.4%) had permanent neurologic dysfunction. Operative mortality was significantly worse in patients with shaggy aorta (dissection: 2.2%, non-shaggy: 6.6%, and shaggy: 33.3%, P < .001) and shaggy aorta was a significant risk factor for spinal cord injury (dissection: 7.2%, non-shaggy: 6.6%, and shaggy: 27.8%, P < .003). Multivariable analysis demonstrated that shaggy aorta was a significant risk factor for composite outcome consisted of operative mortality, spinal cord injury, and acute renal failure (odds ratio, 4.78; 95% confidence interval, 1.91-12.3, P < .001). CONCLUSIONS: Preoperative enhanced computed tomography assessment of shaggy aorta could predict high-risk patients for open TAAA repair.

Congenital vascular ring.

A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. With the development of new technologies, multi-detector computed tomography (MDCT) has become a good diagnostic modality for pre- and postoperative evaluation. MDCT provides an excellent image of aortic arch abnormalities and the related anatomy, as well as the tracheal pathology. For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.

Results of staged repair of aortic disease in patients with Marfan syndrome.

OBJECTIVE: We present our open surgical strategies for staged replacement of the thoracic and thoracoabdominal aorta in patients with Marfan syndrome. METHODS: Between October 1999 and December 2017, 82 patients with Marfan syndrome underwent 118 aortic repairs. We divided the aorta into 4 segments for categorization: (1) the aortic root, (2) aortic arch, (3) descending thoracic, and (4) abdominal aorta. Procedures were categorized according to the types of surgery. Staged repair was defined as a subsequent operation on a different segment of the aorta after initial repair (n = 111, 94.1%), and reoperation was defined as an operation on the same segment (n = 7, 5.9%). RESULTS: The mean age at initial operation was 41.7 ± 14.9 years. Staged repairs included aortic root replacement (n = 42, 36%), total arch replacement (n = 11, 9.3%), combined aortic root and total arch replacement (n = 13, 11%), descending aorta replacement (n = 4, 3.4%), thoracoabdominal aortic repair (n = 36, 31%), and extensive arch-descending or thoracoabdominal repair (n = 5, 4.2%). Four patients received 3 staged repairs. Operative mortality was 0.8% (1/118). Stroke occurred in 1.7% (2/118), and spinal cord injury occurred in 1.7% (2/117). Overall survival was 95.8 ± 2.4% at 10-years. Twenty-four patients underwent replacement of the whole aorta after 2.5 ± 3.8 years following initial repair. CONCLUSIONS: Our strategies for staged replacement of the thoracic and thoracoabdominal aorta in patients with Marfan syndrome resulted in excellent early- and long-term outcomes.