In Vivo Zinc Finger Nuclease-mediated Targeted Integration of a Glucose-6-phosphatase Transgene Promotes Survival in Mice With Glycogen Storage Disease Type IA.

Glycogen storage disease type Ia (GSD Ia) is caused by glucose-6-phosphatase (G6Pase) deficiency in association with severe, life-threatening hypoglycemia that necessitates lifelong dietary therapy. Here we show that use of a zinc-finger nuclease (ZFN) targeted to the ROSA26 safe harbor locus and a ROSA26-targeting vector containing a G6PC donor transgene, both delivered with adeno-associated virus (AAV) vectors, markedly improved survival of G6Pase knockout (G6Pase-KO) mice compared with mice receiving the donor vector alone (P < 0.04). Furthermore, transgene integration has been confirmed by sequencing in the majority of the mice treated with both vectors. Targeted alleles were 4.6-fold more common in livers of mice with GSD Ia, as compared with normal littermates, at 8 months following vector administration (P < 0.02). This suggests a selective advantage for vector-transduced hepatocytes following ZFN-mediated integration of the G6Pase vector. A short-term experiment also showed that 3-month-old mice receiving the ZFN had significantly-improved biochemical correction, in comparison with mice that received the donor vector alone. These data suggest that the use of ZFNs to drive integration of G6Pase at a safe harbor locus might improve vector persistence and efficacy, and lower mortality in GSD Ia.

Practice patterns and predictors of followup imaging after a negative bone scan in men with castration resistant prostate cancer: results from the SEARCH database.

PURPOSE: We investigated imaging practice patterns in men with nonmetastatic (M0) castration resistant prostate cancer. MATERIALS AND METHODS: We analyzed data on 247 patients with documented M0 CRPC from the SEARCH database. Patients were selected regardless of primary treatment modality and all had a negative bone scan after a castration resistant prostate cancer diagnosis. Cox models were used to test associations of time to a second imaging test with several demographic and clinical factors. RESULTS: During a median followup of 29.0 months (IQR 12.9-43.5) after a post-castration resistant prostate cancer bone scan was negative, 190 patients (77%) underwent a second imaging test. On univariable analysis patients with higher prostate specific antigen (HR 1.13, p = 0.016), shorter prostate specific antigen doubling time (HR 0.79, p < 0.001) and faster prostate specific antigen velocity (HR 1.01, p < 0.001) were more likely to undergo a second imaging test. Treatment center was also a significant predictor of a second imaging test (p = 0.010). No other factor was a significant predictor. Results were similar on multivariable analysis. It was estimated that approximately 20% of men with a prostate specific antigen doubling time of less than 3 months did not undergo an imaging test in the first year after a post-castration resistant prostate cancer negative bone scan. However, 50% of patients with prostate specific antigen doubling time 15 months or greater underwent a second imaging test in the first year. CONCLUSIONS: Clinicians use some known predictors of positive imaging tests to determine which patients with M0 castration resistant prostate cancer undergo a second imaging test . However, there may be under imaging in those at high risk and over imaging in those at low risk. Further studies are needed to identify risk factors for metastasis and form clear imaging guidelines in patients with M0 castration resistant prostate cancer.

Racial disparities in thoracic aortic surgery: Myth or reality?

OBJECTIVE: We examined the relationship between Black or White race and adverse outcomes in patients who underwent surgery of the ascending aorta, aortic root, or aortic arch at our center. METHODS: We analyzed 2335 consecutive patients who identified as Black (n = 217, 9.3%) or White (n = 2118, 90.7%) and underwent proximal aortic surgery. Patient zip codes were used to determine community socioeconomic (CSE) characteristics. The composite adverse outcome comprised mortality, persistent neurologic injury, and renal failure necessitating dialysis at discharge. We performed multivariable analysis, Kaplan-Meier analysis, and propensity score matching adjusted for CSE factors. RESULTS: Median follow-up time was 3.7 years. Compared with White patients, Black patients lived in areas characterized by a higher percentage living below poverty level, lower income, and lower education level (P < .0001). Black patients had higher rates of emergency presentation (P < .0001) and lower 5- and 10-year survival rates (P = .0002). Short-term outcomes were similar between groups, except for respiratory failure and length of stay (P < .0001), which were higher in the Black population. After propensity score matching adjusted for CSE factors, Black and White patients (n = 204 each) had similar short-term outcomes and 5- and 10-year survival rates (P = .30). Multivariable analysis stratified by race showed that CSE factors independently predicted adverse outcomes in Black but not White patients. CONCLUSIONS: This is among few studies that have analyzed the relationship between race and proximal aortic surgery. Although outcomes were similar between Black and White patients in our cohort after adjusting for CSE factors, unfavorable CSE factors predicted adverse outcomes in Black but not White patients. More patient-specific studies are needed.

Unplanned readmissions, community socioeconomic factors, and their effects on long-term survival after complex thoracic aortic surgery.

OBJECTIVE: We evaluated community socioeconomic factors in patients who had unplanned readmission after undergoing proximal aortic surgery (ascending aorta, aortic root, or arch). METHODS: Unplanned readmissions for any reason within 60 days of the index procedure were reviewed by race, acuity at presentation, and gender. We also evaluated 3 community socioeconomic factors: poverty, household income, and education. Kaplan-Meier survival curves were used to assess long-term survival differences by group (race, acuity, and gender). RESULTS: Among 2339 patients who underwent proximal aortic surgery during the 20-year study period and were discharged alive, our team identified 146 (6.2%) unplanned readmissions. Compared with White patients, Black patients lived in areas characterized by more widespread poverty (20.8% vs 11.1%; P = .0003), lower income ($42,776 vs $65,193; P = .0007), and fewer residents with a high school diploma (73.7% vs 90.1%; P < .0001). Compared with patients whose index operation was elective, patients who had urgent or emergency index procedures lived in areas with lower income ($54,425 vs $64,846; P = .01) and fewer residents with a high school diploma (81.1% vs 89.2%; P = .005). Community socioeconomic factors did not differ by gender. Four- and 6-year survival estimates were 63.1% and 63.1% for Black patients versus 89.1% and 83.0% for White patients (P = .0009). No significant differences by acuity or gender were found. CONCLUSIONS: Among readmitted patients, Black patients and patients who had emergency surgery had less favorable community socioeconomic factors and poorer long-term survival. Earlier and more frequent follow-up in these patients should be considered. Developing off-campus clinics and specific postdischarge measures targeting these patients is important.

Contemporary Midterm Outcomes After Primary Repair of Chronic Type A Aortic Dissection.

BACKGROUND: Without surgical repair, acute type A aortic dissection (TAAD) is usually fatal. However, some patients survive without an early operation and progress to the chronic phase. Contemporary outcomes of primary surgical repair of chronic TAAD are unclear, so we evaluated them at our single-practice service. METHODS: During 1990 to 2021, 205 patients underwent repair of TAAD in the chronic phase (>60 days after onset). The 2 relevant DeBakey classifications were nearly equally represented: type I, 52% (n = 107), and type II, 48% (n = 98). The median interval between dissection onset and repair was 7 months (interquartile range, 3-25 months). Kaplan-Meier and competing-risk analyses provided time-dependent outcomes. RESULTS: At the time of intervention, most patients (40%) had chronic symptoms. Type I patients were younger than type II patients; however, comorbidities were similar. Most patients (n = 183 [87%]) underwent hemiarch or total arch repair, although total arch replacement was more common in type I dissection (P < .001). There were 15 operative deaths (7%), and 7 strokes (3%) persisted to the time of death or discharge. No patient had persistent paraplegia. Median follow-up was 5 years (interquartile range, 2-11 years). The 5-year reoperation-free survival was 61% (95% CI, 54%-68%), and the incidence of reoperation was 3% (95% CI, 0.4%-5%). Patients with type I and type II dissection did not differ significantly in survival (P = .2). CONCLUSIONS: Durable repair can be achieved with reasonable operative risk. Treatment is individualized and is associated with low rates of persistent neurologic complications. Despite differing operative approaches by DeBakey type, early and late outcomes were similar.

Independent associations with early mortality after open repair of Crawford extent IV thoracoabdominal aortic aneurysms.

OBJECTIVE: We aimed to identify outcomes and factors that independently associate with early mortality after open repair of Crawford extent IV thoracoabdominal aortic aneurysms, defined as aneurysms confined to the segment below the diaphragm. METHODS: This retrospective analysis included 721 extent IV thoracoabdominal aortic aneurysm repairs performed in our institution from 1986 to 2021. Indications for repair were aneurysm without dissection in 627 cases (87.0%) and aortic dissection in 94 cases (13.0%). Overall, 466 patients (64.6%) were symptomatic preoperatively; 124 (17.2%) procedures were performed in patients with acute presentation, including 58 (8.0%) ruptured aneurysms. RESULTS: Operative death occurred after 49 (6.8%) repairs. Persistent renal failure necessitating dialysis occurred after 43 (6.0%) repairs. Binary logistic regression modeling revealed that previous extent II thoracoabdominal aortic aneurysm repair, chronic kidney disease, previous myocardial infarction, urgent or emergency repair, and longer crossclamp times during surgery were independently associated with operative mortality. Among early survivors (n = 672), competing risk analysis revealed that cumulative incidence of mortality and reintervention rates at 10 years were 74.8% (95% confidence interval, 71.4%-78.5%) and 3.3% (95% confidence interval, 2.2%-5.1%), respectively. CONCLUSIONS: Although patient comorbidities contributed to operative mortality, factors associated with the repair, such as urgent or emergency status, the duration of aortic crossclamping, and certain types of complex reoperation, also played prominent roles. Patients who survive the operation can expect a durable repair that usually is free from late reintervention. Expanding our collective knowledge regarding patients who undergo open repair of extent IV thoracoabdominal aortic aneurysms will enable clinicians to establish best practices and improve patient outcomes.

The intersection of community socioeconomic factors with gender on outcomes after thoracic aortic surgery.

OBJECTIVE: We evaluated the relationship among community socioeconomic factors (poverty, income, and education), gender, and outcomes in patients who underwent ascending aortic, root, and arch surgery. METHODS: For 2634 consecutive patients, we associated patients' ZIP codes with community socioeconomic factors. The composite adverse outcome comprised death, persistent neurological injury, and renal failure necessitating dialysis at discharge. Multivariable analysis and Kaplan-Meier survival curves were used. Men and women from the full cohort and from the elective patients were propensity matched. RESULTS: Median follow-up was 3.6 years (interquartile range, 1.2-9.3). Men lived in areas characterized by less poverty (P = .03), higher household income (P = .01), and more education (P = .02) than women; likewise, in the elective cohort, all community socioeconomic factors favored men (P ≤ .009). Female gender predicted composite adverse outcome (P = .006). In the propensity-matched women and men (820 pairs), the composite adverse outcome rates were 14.2% and 11%, respectively (P = .06). In 583 propensity-matched pairs of elective patients, men had less composite adverse outcome (P = .02), operative mortality (P = .04), and renal (P = .02) and respiratory failure (P = .0006). The 5- and 10-year survivals for these men and women were 74.2% versus 71.4% and 50.2% versus 48.2%, respectively (P = .06). All community socioeconomic factors in both propensity-matched groups nonsignificantly favored men. CONCLUSIONS: This study is among the first to examine the association among community socioeconomic factors, gender, and outcomes in patients who undergo proximal aortic surgery. Female gender predicted a composite adverse outcome. In the elective patients, most adverse outcomes were significantly less in men. In the propensity-matched patients, all community socioeconomic factors favored men, although not significantly. Larger studies with patient-level socioeconomic information are needed.

Midterm outcomes of aortic root surgery in patients with Marfan syndrome: A prospective, multicenter, comparative study.

OBJECTIVE: The objective of this study was to compare midterm outcomes of aortic valve-replacing root replacement (AVR) and aortic valve-sparing root replacement (AVS) operations in patients with Marfan syndrome. METHODS: Patients who met strict Ghent diagnostic criteria for Marfan syndrome and who underwent either AVR or AVS between March 1, 2005 and December 31, 2010 were enrolled in a 3-year follow-up prospective, multicenter, international registry study; the study was subsequently amended to include 20-year follow-up. Enrollees were followed clinically and echocardiographically. RESULTS: Of the 316 patients enrolled, 77 underwent AVR and 239 underwent AVS; 214 gave reconsent for 20-year follow-up. The median clinical follow-up time for surviving patients was 64 months (interquartile range, 42-66 months). Survival rates for the AVR and AVS groups were similar at 88.2% ± 4.4% and 95.0% ± 1.5%, respectively (P = .1). Propensity score-adjusted competing risk modeling showed associations between AVS and higher cumulative incidences of major adverse valve-related events, valve-related morbidity, combined structural valve deterioration and nonstructural valve dysfunction, and aortic regurgitation ≥2+ (all P < .01). No differences were found for reintervention (P = .7), bleeding (P = .2), embolism (P = .3), or valve-related mortality (P = .8). CONCLUSIONS: Five years postoperatively, major adverse valve-related events and valve-related morbidity were more frequent after AVS than after AVR procedures, primarily because of more frequent aortic valve dysfunction. No between-group differences were found in rates of survival, valve-related mortality, reintervention on the aortic valve, or bleeding. We plan to follow this homogenous cohort for 20 years after aortic root replacement.

Effect of sarcopenia on survival and spinal cord deficit outcomes after thoracoabdominal aortic aneurysm repair in patients 60 years of age and older.

OBJECTIVE: Sarcopenia (core muscle loss) has been used as a surrogate marker of frailty. We investigated whether sarcopenia would adversely affect survival after thoracoabdominal aortic aneurysm repair. METHODS: We retrospectively reviewed prospectively collected data from patients aged 60 years or older who underwent thoracoabdominal aortic aneurysm repairs from 2006 to 2016. Imaging was reviewed by 2 radiologists blinded to clinical outcomes. The total psoas index was derived from total psoas muscle cross-sectional area (cm2) at the mid-L4 level, normalized for height (m2). Patients were divided by sex-specific total psoas index values into sarcopenia (lower third) and nonsarcopenia (upper two-thirds) groups. Multivariable modeling identified operative mortality and spinal cord injury predictors. Unadjusted and adjusted survival curves were analyzed. RESULTS: Of 392 patients identified, those with sarcopenia (n = 131) were older than nonsarcopenic patients (n = 261) (70.0 years vs 68.0 years; P = .02) and more frequently presented with aortic rupture or required urgent/emergency operations. Operative mortality was comparable (sarcopenia 13.7% vs nonsarcopenia 10.0%; P = .3); sarcopenia was not associated with operative mortality in the multivariable model (odds ratio, 1.40; 95% confidence interval, 0.73-2.77; P = .3). Sarcopenic patients experienced more frequent delayed (13.0% vs 4.6%; P = .005) and persistent (10.7% vs 3.4%; P = .008) paraplegia. Sarcopenia independently predicted delayed paraplegia (odds ratio, 3.17; 95% confidence interval, 1.42-7.08; P = .005) and persistent paraplegia (odds ratio, 3.29; 95% confidence interval, 1.33-8.13; P = .01) in the multivariable model. Adjusted for preoperative/operative covariates, midterm survival was similar for sarcopenic and nonsarcopenic patients (P = .3). CONCLUSIONS: Sarcopenia did not influence early mortality or midterm survival after thoracoabdominal aortic aneurysm repair but was associated with greater risk for delayed and persistent paraplegia.

Aortic arch repair: lessons learned over three decades at Baylor College of Medicine.

The treatment of complex aortic arch disease continues to be among the most demanding cardiovascular operations, with a considerable risk of death and stroke. Since January 1990, our single-practice service has performed over 3000 repairs of the aortic arch. Our aim was to describe the progression of our technical approach to open aortic arch repair. Our center's surgical technique has evolved considerably over the last three decades. When it comes to initial arterial cannulation, we have shifted away from femoral artery cannulation to innominate and axillary artery cannulation. During difficult repairs, this transition has made it easier to use antegrade cerebral perfusion rather than retrograde cerebral perfusion, which was commonly used in the early days. Brain protection tactics during open aortic arch procedures have evolved from profound (≤14 °C) hypothermia during circulatory arrest to moderate (22-24 °C) hypothermia. Aortic arch repair is performed through a median sternotomy and may treat acute aortic dissection, chronic aortic dissection, or degenerative aneurysm. Reoperative repair - that necessitating redo sternotomy - is common in patients undergoing aortic arch repair. The majority of repairs will include varying portions of the ascending aorta and may involve the aortic valve or the aortic root. In some patients, repair may extend into the proximal descending thoracic aorta; this includes elephant trunk, frozen elephant trunk, and antegrade hybrid approaches.

Early and late outcomes of surgical repair of mycotic aortic aneurysms: A 30-year experience.

OBJECTIVE: Mycotic aortic aneurysm and its associated complications are often catastrophic. In this study, we examined the early and late outcomes of surgical repair of mycotic aortic aneurysm at our center over the last 3 decades. METHODS: We retrospectively reviewed our prospectively maintained aortic surgery database with supplemental adjudication of medical records. Aortic infection was confirmed through clinical, radiological, intraoperative, pathological, and treatment evidence. RESULTS: Seventy-five patients (median age, 68 years; interquartile range, 62-74) who underwent surgical repair of a mycotic aortic aneurysm between 1992 and 2021 were included. Almost all patients (n = 72; 96%) presented with symptoms, including 26 patients (35%) with rupture, and many underwent urgent or emergency repair (n = 64; 85%). Sixty-one patients underwent open repair, and 14 patients underwent hybrid or endovascular repair. Infection-specific adjunct techniques included rifampin-soaked grafts (n = 16), omental pedicle flaps (n = 21), and antibiotic irrigation catheters (n = 8). There were 15 early deaths (20%), including 10 of the 26 patients (38%) who presented with rupture; however, persistent stroke, paraplegia or paraparesis, and renal failure necessitating dialysis were uncommon (each <5%). Almost all early survivors (52/60; 87%) were discharged with long-term antibiotic therapy. Estimated survival at 2, 6, and 10 years was 55.7% ± 5.8%, 39.0% ± 5.7%, and 26.9% ± 5.5%, respectively. CONCLUSIONS: A substantial proportion of patients with mycotic aortic aneurysm present with rupture and generally require urgent or emergency repair. Operative mortality and complications are common, especially for patients who present with rupture, and late survival is poor.

Sex Differences in Ascending Aortic and Arch Surgery: A Propensity-Matched Comparison of 1153 Pairs.

BACKGROUND: We investigated the relationship of sex with clinical outcomes after proximal aortic (ascending and arch) operations, and whether sex-specific preoperative factors are associated with mortality. METHODS: Of 3745 patients who underwent elective, urgent, and emergency proximal aortic operations over a 30-year period, 1153 pairs of men and women were propensity-matched, and their early and long-term outcomes were compared. Kaplan-Meier survival analysis was used to estimate late survival. RESULTS: Women and men had similar operative mortality (9.1% vs 8.8%, P = .8), stroke (5.7% vs 5.6%, P = .9), and renal failure rates (7.0% vs 6.6%, P = .7). Thirty-day mortality was 7.5% vs 5.6% (P = .06), respectively. Results were less favorable for women than for men regarding respiratory failure (34.3% vs 29.2%, P=0.008) and intensive care unit length of stay (9.11 ± 11.9 vs 7.87 ± 12.48 days; P = .023). Long-term survival was not significantly different between women and men: 66.3% (95% confidence interval [CI] 62.8%-69.5%) vs 67.1% (95% CI 63.6%-70.4%) at 5 years, and 45.9% (95% CI 41.76%-50.0%) vs 46.2% (95% CI 41.7%-50.6%) at 10 years (P = .4). Preoperative factors including diabetes, prior stroke, prior renal insufficiency, and peripheral vascular disease were associated with operative mortality in men, whereas chronic obstructive pulmonary disease was the main risk factor in women. CONCLUSIONS: No differences were seen between the sexes in life-changing adverse outcomes after ascending aortic and arch procedures, although specific preoperative variables were associated with specific adverse events. Recognizing differences in preoperative risk factors for mortality between the sexes may facilitate targeted preoperative assessment, preparation, and counseling.

Propensity score analysis in patients with and without previous isolated coronary artery bypass grafting who require proximal aortic and arch surgery.

OBJECTIVE: The risk posed by previous isolated coronary artery bypass grafting (CABG) in patients who require proximal aortic or aortic arch surgery is unclear. We compared outcomes of ascending aortic and arch procedures in patients with and without previous CABG. METHODS: Using propensity scores, we created 2 matched groups of patients who underwent proximal aortic surgery, including total arch repairs, at our institution: 126 patients who underwent isolated CABG before the index operation and 126 without previous CABG. Forty-four percent of aortic operations were emergency procedures. Eighty-six patients had a patent previous left internal mammary graft. We compared outcomes between the 2 groups and calculated Kaplan-Meier survival curves. RESULTS: The following outcomes were recorded for the patients with previous isolated CABG versus no CABG: operative mortality, 15.9% versus 11.1% (P = .3); 30-day mortality, 13.5% versus 7.1% (P = .1); persistent stroke, 6.3% versus 4.8% (P = .6); and renal failure necessitating hemodialysis at discharge, 7.9% versus 4.0% (P = .2). Previous CABG did not independently predict any adverse outcome, even though patients who underwent previous CABG more frequently needed intra-aortic balloon support (P < .01). The P value for the overall intergroup difference in long-term survival was .06. CONCLUSIONS: This is one of the largest studies yet reported to examine the impact of previous isolated CABG on proximal aortic or arch surgery outcomes. Although these results may be specific to aortic centers of excellence, in this complicated patient cohort, previous isolated CABG did not independently predict any adverse outcome. These results could serve as a benchmark for assessing future endovascular therapies.

A 23-year experience with the reversed elephant trunk technique for staged repair of extensive thoracic aortic aneurysm.

OBJECTIVE: The reversed elephant trunk technique permits staged repair of extensive thoracic aortic aneurysm in patients whose distal (ie, descending thoracic and thoracoabdominal) aorta is symptomatic or disproportionately large compared with their proximal aorta (ie, ascending aorta and transverse aortic arch). We present our 23-year experience with the reversed elephant trunk approach. METHODS: Between 1994 and 2017, 94 patients (median age 62 [46-69] years) underwent stage 1 reversed elephant trunk repair of the distal aorta. Fifty-three patients (56%) had aortic dissection, and 31 patients (33%) had heritable thoracic aortic disease. Eighty-eight operations (94%) were Crawford extent I or II thoracoabdominal aortic repairs. Twenty-seven patients (29%) underwent subsequent stage 2 repair of the proximal aorta; 14 patients (52%) required redo median sternotomy. The median time between the stage 1 and 2 operations was 18.8 (4.8-69.3) months. RESULTS: The operative mortality was 10% (9/94) for stage 1 repairs and 4% (1/27) for stage 2 repairs; 1 patient with heritable thoracic aortic disease died after stage 1 repair (1/31, 3%), and 1 patient died after stage 2 repair (1/13, 8%). Two patients (2%) had ruptures after stage 1 repair; 1 resulted in death, and 1 precipitated emergency stage 2 repair. In total, 36 patients (38%) who survived stage 1 repair died before stage 2 reversed elephant trunk completion repair could be performed. CONCLUSIONS: Managing extensive aortic aneurysm with the 2-stage reversed elephant trunk technique yields acceptable short-term outcomes. This technique is useful for the reversed elephant trunk in patients who require distal aortic repair before proximal repair and is particularly effective in patients with heritable thoracic aortic disease. The low number of patients returning for completion repair is concerning. Rigorous surveillance is needed.

Endovascular therapy for patients with heritable thoracic aortic disease.

BACKGROUND: Patients with genetic or heritable aortic conditions and thoracic aortic aneurysm syndrome often develop cardiovascular abnormalities originating at the aortic root and affecting the entire thoracoabdominal aorta. Although thoracic endovascular aortic repair (TEVAR) is usually avoided in these patients, TEVAR may be worthwhile for those at high risk for surgical complications and in certain emergency circumstances. We explored indications for TEVAR in patients with suspected or confirmed genetic or heritable aortic conditions and investigated early and mid-term outcomes. METHODS: Our institutional aortic surgery database was queried for patients with suspected or confirmed Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, Turner syndrome, neurofibromatosis, or familial aortic aneurysm and dissection who underwent TEVAR between February 1, 2002 and October 31, 2020. We extracted operative details and in-hospital, follow-up, and survival data. RESULTS: Thirty-seven patients who underwent 40 endovascular interventions met the inclusion criteria; 25 previously underwent ascending aorta or aortic root surgery, and 21 previously underwent open thoracoabdominal surgery. Postoperative complications included respiratory failure (24.3%), cardiac complications (16.2%), renal failure (13.5%), tracheostomy (8.1%), and spinal cord ischemia (paraplegia/paraparesis) (8.1%). Follow-up ranged from 1.3 to 8.5 years (median: 3.6 years), with 15 deaths overall (three early/in-hospital). Thirteen patients (35.1%) had 22 repeat interventions (open and endovascular) post-TEVAR; five had the endograft removed. CONCLUSIONS: Despite consensus that thoracic aneurysms in patients with genetic or heritable aortic conditions should be treated with conventional open surgery, the outcomes from our study suggest that TEVAR might be suitable in emergency settings or for patients in this population who are not candidates for open surgery, who are at high risk for reintervention, or who have a previously implanted Dacron graft. Nonetheless, lifelong surveillance is important for these patients after TEVAR to monitor for new dissection at distal or proximal landing zones, as repeat interventions are frequent.

Staged Repair of Extensive Aneurysms of the Thoracic Aorta by Using the Elephant Trunk Technique.

BACKGROUND: Staged open repair of extensive aortic aneurysms by using the elephant trunk (ET) technique has facilitated the treatment of aortic aneurysms that affect the entire thoracic aorta. We describe our nearly 3-decade experience with classic ET repairs. METHODS: From 1990 through 2021, we performed 363 stage 1 ET repairs to replace the transverse aortic arch in patients with a median age of 65 years (interquartile range, 56-71 years). Fifty-six patients (15.4%) presented with acute symptoms, and 182 (50.1%) underwent redo sternotomy. After a median interval of 3.2 months (interquartile range, 2.0-7.3 months), 203 patients (55.9%) underwent stage 2 ET completion; of these, 16 (7.9%) had acute symptoms. Stage 2 repairs comprised 162 (80.6%) extent I or II thoracoabdominal aortic replacements. We examined postoperative outcomes, including operative death, adverse event (a composite end point), survival, and repair failure. RESULTS: Operative mortality was 12.4% (45 of 363) after stage 1 and 10.3% (21 of 203) after stage 2. The rates of adverse event were 18.5% (67 of 363) for stage 1 and 18.4% (38 of 203) for stage 2. Acute symptoms independently predicted operative death and adverse events for both stage 1 and stage 2 repairs; additional predictors for stage 2 repairs were older age and extent II repair. Survival was significantly worse for patients who did not receive their stage 2 completion repair than for those who did (P < .001). CONCLUSIONS: Treating extensive aortic aneurysms by using the ET technique for staged repair is associated with substantial morbidity and mortality. Patients who present with acute symptoms are at greater risk of operative death and adverse events. Diligent surveillance is needed between stages.

Differential presentation in acuity and outcomes based on socioeconomic status in patients who undergo thoracoabdominal aortic aneurysm repair.

OBJECTIVES: Socioeconomic differences can lead to differences in how patients present with surgical conditions. We attempted to determine whether socioeconomic status (SES) affects survival outcomes after thoracoabdominal aortic aneurysm (TAAA) repair. METHODS: We retrospectively reviewed prospectively collected data from 981 TAAA repairs performed on domestic (noninternational) patients between 2006 and 2016. We excluded patients <18 years old (n = 3), those with no available US home address (n = 114), those not within the race and ethnicity categories assessed (n = 30), and those lost to follow-up (n = 6), leaving 832 repairs for analysis. We derived patient SES by using US Census Bureau data to estimate median household income according to patient home address. Patients were grouped into 3 SES groups: high (n = 283), middle (n = 274), and low (n = 275). Multivariable logistic regression modeling was used to identify predictors of operative mortality. Kaplan-Meier curves and Cox proportional hazards regression were used to analyze the association between SES and survival. RESULTS: Operative mortality occurred in 9% (n = 76) of patients. Patients of low SES had greater rates of acute symptoms, dissection, and urgent or emergency TAAA repair. However, lower SES was not an independent predictor of operative death. Kaplan-Meier analysis and Cox proportional hazards modeling did not show a significant difference in mid-term survival by SES. CONCLUSIONS: In our TAAA series from a single, high-volume practice, SES differences did not appear to influence operative mortality rates. In addition, SES was not associated with a difference in mid-term survival. Efforts to understand and ameliorate the greater acuity of presentation in patients of low SES appear worthwhile.

Early Gastrointestinal Complications After Open Thoracoabdominal Aortic Aneurysm Repair.

BACKGROUND: The present study was done to examine the incidence, predictors, and impact of early gastrointestinal (GI) complications after open thoracoabdominal aortic aneurysm repair. METHODS: We retrospectively analyzed data from 3587 open thoracoabdominal aortic aneurysm repairs performed at our center from 1986 to 2019. We used univariate analyses and multivariable logistic regression to identify risk factors associated with GI complications, including bleeding, ischemia, obstruction, and acute pancreatitis. Adverse event was defined as operative death or persistent stroke, paraplegia, paraparesis, or renal failure necessitating dialysis. RESULTS: Gastrointestinal complications developed after 213 repairs (5.9%). Gastrointestinal complications less often developed after extent I repair than after repairs that involved infrarenal abdominal aortic segments (ie, extent II to IV repairs; P = .003). Patients who had GI complications more often underwent endarterectomy, stenting, or bypass of visceral arteries (51.2% vs 42.2%; P = .01). Use of selective visceral perfusion did not differ between groups. Patients who had GI complications had higher rates of operative mortality (34.3% vs 6.6%) and adverse events (44.1% vs 13.2%) and had longer hospitalization (29 vs 11 days; P < .001 for all). Independent predictors of GI complications included incidental splenectomy, rupture, non-extent I repair, older age, and longer aortic cross-clamp time. Short-term, midterm, and long-term survival were poorer for patients who had GI complications (P < .001). CONCLUSIONS: Although uncommon, early GI complications after open thoracoabdominal aortic aneurysm repair are associated with significant early and late morbidity and mortality. Development of perioperative strategies to mitigate these complications is warranted.

Open Repair of Acute Type A Intramural Hematoma in 3 Patients.

Acute aortic syndrome encompasses classic aortic dissection and less common aortic phenomena, including intramural hematoma (IMH), a hemorrhage within the aortic media that occurs without a discrete intimal tear. We reviewed our experience with treating acute type A IMH to better understand this acute aortic syndrome. A review of our clinical database identified 1,902 proximal aortic repairs that were performed from January 2006 through December 2018; of these, 266 were for acute aortic syndrome, including 3 (1.1%) for acute type A IMH. Operative technique varied considerably. All IMH repairs involved hemiarch or total arch replacement. In all 3 patients, the IMH extended distally into the descending thoracic aorta. There were no operative deaths or major adverse events (stroke, paraplegia, paraparesis, or renal failure necessitating dialysis) that persisted to hospital discharge. Length of hospitalization ranged from 5 to 20 days. All 3 patients were alive at follow-up (range, 2-6 yr) and needed no aortic reintervention after their index or staged repairs. In our experience, repair of acute type A IMH was infrequent and could be either simple or complex. Despite our limited experience with this disease, we found that it can be repaired successfully in urgent and emergency cases. Following treatment guidelines for aortic dissection appears to be a reasonable strategy for treating IMH.

Spinal cord deficit after 1114 extent II open thoracoabdominal aortic aneurysm repairs.

OBJECTIVE: Crawford extent II repairs are the most extensive thoracoabdominal aortic aneurysm operations and pose the greatest risk of postoperative spinal cord deficit. We sought to examine spinal cord deficit after open extent II thoracoabdominal aortic aneurysm repair to identify predictors of the most serious type: persistent paraplegia or paraparesis. METHODS: We included 1114 extent II thoracoabdominal aortic aneurysm repairs performed from 1991 to 2017. Intercostal/lumbar artery reattachment (n = 959, 86.1%) and cerebrospinal fluid drainage (n = 698, 62.7%) were used to mitigate the risk of postoperative spinal cord deficit. We used univariate and multivariable analyses to examine spinal cord deficit and identify predictors of persistent paraplegia or paraparesis, defined as paraplegia or paraparesis present at the time of early death or hospital discharge. RESULTS: Spinal cord deficit developed after 151 (13.6%) repairs: 86 (7.7%) cases of persistent paraplegia or paraparesis (51 paraplegia; 35 paraparesis) and 65 (6.1%) cases of transient paraplegia or paraparesis. Patients with spinal cord deficit were older (median 68 vs 65 years, P < .001) and had more rupture (6.6% vs 2.2%, P = .002) and urgent/emergency repair (25.2% vs 16.9%, P = .01) than those without. Persistent paraplegia or paraparesis developed immediately in 47 patients (4.2%) and was delayed in 39 patients (3.5%). Urgent/emergency repair (relative risk ratio, 2.31; P = .002), coronary artery disease (relative risk ratio, 1.80, P = .01), and chronic symptoms (relative risk ratio, 1.76, P = .02) independently predicted persistent paraplegia or paraparesis. Reattaching intercostal/lumbar arteries (relative risk ratio, 0.38, P < .001) and heritable disease (relative risk ratio, 0.36, P = .01) were protective. Early and late survival were poorer in those with persistent paraplegia or paraparesis than in those without. CONCLUSIONS: Spinal cord deficit after extent II thoracoabdominal aortic aneurysm repairs remains concerning; survival is worse in patients with persistent paraplegia or paraparesis. The complexity of spinal cord deficit and persistent paraplegia or paraparesis warrant further study.