Utility of Hook Sign in the Diagnosis of Median Arcuate Ligament Syndrome.

BACKGROUND: Median arcuate ligament syndrome (MALS) is a clinical syndrome caused by compression of the celiac artery by the median arcuate ligament that often manifests with nonspecific abdominal pain. Identification of this syndrome is often dependent on imaging of compression and upward bending of the celiac artery by lateral computed tomography angiography, the so-called "hook sign." The purpose of this study was to assess the relationship of radiologic characteristics of the celiac artery to clinically relevant MALS. METHODS: An institutional review board-approved retrospective chart review from 2,000 to 2,021 of 293 patients at a tertiary academic center diagnosed with celiac artery compression (CAC) was performed. Patient demographics and symptoms of 69 patients who were diagnosed with symptomatic MALS were compared to 224 patients without MALS (but with CAC) per electronic medical record review. Computed tomography angiography images were reviewed and the fold angle (FA) was measured. The presence of a hook sign (defined as a visual FA < 135°), as well as stenosis (defined as >50% of luminal narrowing on imaging) were recorded. Wilcoxon rank-sum test and Chi-squared test were used for comparative analysis. Logistic model was run to relate the presence of MALS with comorbidities and radiographic findings. RESULTS: Imaging was available in 59 patients (25 males, 34 females) and 157 patients (60 males, 97 females) with and without MALS, respectively. Patients with MALS were more likely to have a more severe FA (120.7 ± 33.6 vs. 134.8 ± 27.9, P = 0.002). Males with MALS were also more likely to have a more severe FA compared with males without MALS (111.1 ± 33.7 vs. 130.4 ± 30.4, P = 0.015). In patients with body mass index (BMI) >25, MALS patients also had narrower FA compared with patients without MALS (112.6 ± 30.5 vs. 131.7 ± 30.3, P = 0.001). The FA was negatively correlated with BMI in patients with CAC. The hook sign and stenosis were associated with diagnosis of MALS (59.3% vs. 28.7%, P < 0.001, and 75.7% vs. 45.2%, P < 0.001, respectively). In logistic regression, pain, stenosis, and a narrow FA were statistically significant predictors of the presence of MALS. CONCLUSIONS: The upward deflection of the celiac artery in patients with MALS is more severe compared with patients without MALS. Consistent with prior literature, this bending of the celiac artery is negatively correlated with BMI in patients with and without MALS. When demographic variables and comorbidities are considered, a narrow FA is a statistically significant predictor of MALS. Regardless of MALS diagnosis, a hook sign was associated with narrower FA. While demographics and imaging findings may inform MALS diagnosis, clinicians should not rely on a visual assessment of a hook sign but should quantitatively measure the anatomic bending angle of the celiac artery to assist with the diagnosis and understand the outcomes.

Evidence Accumulates: Patients with Ascending Aneurysms Are Strongly Protected from Atherosclerotic Disease.

Ascending thoracic aortic aneurysms may be fatal upon rupture or dissection and remain a leading cause of death in the developed world. Understanding the pathophysiology of the development of ascending thoracic aortic aneurysms may help reduce the morbidity and mortality of this disease. In this review, we will discuss our current understanding of the protective relationship between ascending thoracic aortic aneurysms and the development of atherosclerosis, including decreased carotid intima-media thickness, low-density lipoprotein levels, coronary and aortic calcification, and incidence of myocardial infarction. We also propose several possible mechanisms driving this relationship, including matrix metalloproteinase proteins and transforming growth factor-ß.

Institution representation in publications reporting mitral valve repair durability: A scoping review.

BACKGROUND: Mitral valve repair durability currently plays a key role in operative decision making and in defining optimal surgical practice. However, mitral valve durability outcomes measures are not captured by national registries and limited to centers that publish their outcomes. In this study, we aim to describe the scope of institutions represented by reports describing durability outcomes after mitral valve repair within the contemporary literature. METHODS AND RESULTS: A scoping review of the literature was performed to extract abstracts potentially reporting mitral valve operation outcomes published between 2000-2019. 370 full text articles reporting mitral valve durability outcomes by either reoperation rate or rate of recurrent mitral regurgitation met criteria for analysis. Study characteristics including case volume, country and institution of origin, and surgeon volume were extracted and used to calculate the proportion of total cases in the top 3, 5, and 10 represented countries and institutions by the sum of reported mitral valve repairs described. The top 5 of 21 countries represented 78.9% of the mitral valve repair cases described. The top 3 most represented institutions described 20,120 (37.3%) of all mitral valve repairs in 58 (33.9%) single-center studies. CONCLUSION: Published mitral valve repair durability data must be interpreted with caution when used to derive policies and practice recommendations that govern the cardiovascular community at large.

Socioeconomic disparities in surveillance and follow-up of patients with thoracic aortic aneurysm.

BACKGROUND: Thoracic aortic aneurysm (TAA) is a significant risk factor for aortic dissection and rupture. Guidelines recommend referral of patients to a cardiovascular specialist for periodic surveillance imaging with surgical intervention determined primarily by aneurysm size. We investigated the association between socioeconomic status (SES) and surveillance practices in patients with ascending aortic aneurysms. METHODS: We retrospectively reviewed records of 465 consecutive patients diagnosed between 2013 and 2016 with ascending aortic aneurysm ≥4 cm on computed tomography scans. Primary outcomes were clinical follow-up with a cardiovascular specialist and aortic surveillance imaging within 2 years following index scan. We stratified patients into quartiles using the area deprivation index (ADI), a validated percentile measure of 17 variables characterizing SES at the census block group level. Competing risks analysis was used to determine interquartile differences in risk of death before follow up with a cardiovascular specialist. RESULTS: Lower SES was associated with significantly lower rates of surveillance imaging and referral to a cardiovascular specialist. On competing risks regression, the ADI quartile with lowest SES had lower hazard of follow-up with a cardiologist or cardiac surgeon before death (hazard ratio: 0.46 [0.34, 0.62], p < .001). Though there were no differences in aneurysm size at time of surgical repair, patients in the lowest socioeconomic quartile were more frequently symptomatic at surgery than other quartiles (92% vs. 23%-38%, p < .001). CONCLUSION: Patients with lower SES receive less timely follow-up imaging and specialist referral for TAAs, resulting in surgical intervention only when alarming symptoms are already present.

Long-term risk of arch complications in Loeys Dietz syndrome patients undergoing proximal ascending aortic replacement.

PURPOSE: Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder. In LDS patients with normal arch morphology, whether the arch should be prophylactically replaced at the time of proximal aortic replacement remains unknown. We evaluated the risk of long-term arch complications in genetically confirmed LDS patients who underwent proximal ascending aortic replacement. METHODS: We retrospectively reviewed the records of patients with LDS who have been followed at our institution between 1994 and 2020. Patients were only included if whole exome genetic testing confirmed a mutation in an LDS-causing gene (TGFBR1, TGFBR2, SMAD3, TGFB2, or TGFB3). Mutations were categorized as pathogenic, benign, or of unknown significance. We collected demographic information, aortic dimensions, comorbidities, mortality, and operative course from patients' charts. Descriptive statistics and freedom from reoperation plots were generated. RESULTS: Of the 18 patients with a mutation in an LDS-causing gene, 15 had known pathogenic variants, two had mutations of unknown significance, and one had a benign genetic variant. For the 15 patients with confirmed pathogenic variants of LDS the median follow-up duration was 5 years (interquartile range [IQR]: 4-8). Eleven patients underwent ascending aortic replacements (AAR) ± aortic valve replacement. Two patients required an additional operation; one required arch and staged elephant trunk for a dissection 18 years post-AAR and the other patient required an isolated descending aortic replacement for dissection 5 years post-AAR. Among patients who underwent surgery, the median ascending aortic diameter at intervention was 5.0 cm (IQR: 4.3-5.3). There was no surgical or late follow-up mortality observed for any of the 18 patients in the study. CONCLUSION: LDS patients who underwent proximal aortic replacement appeared to have low long-term risk of arch complications. While our study is somewhat limited by its sample size and follow-up duration, it suggests that routine prophylactic total arch replacement may not be warranted in LDS patients with nonaneurysmal aortic arches.

Center-level CABG and valve operative outcomes and volume-outcome relationships in New York State.

BACKGROUND: We analyzed center-level outcome correlations between valve surgery and coronary artery bypass graft (CABG) in New York (NY) State and how volume-outcome effect differ between case types. METHODS: We used the 2014-2016 NY cardiac surgery outcomes report. Center-level observed to-expected (observed-to-expected ratio [O/E]) ratio for operative mortality provided risk-adjusted operative outcomes for isolated CABG and valve operations. Correlation coefficient characterized the concordance in center-level outcomes in CABG and valve. Discordant outcomes were defined as having O/E ratio greater than 2 in one operation type with O/E ratio ≤1 in another. Linearized slope of volume-outcome effect in case types offered insights into centers with discordant performances between procedures. RESULTS: Among 37 NY centers, annual center volumes were 220 ± 120 cases for CABG and 190 ± 178 cases for valve operations. Modest center-level correlation between CABG and valve O/E ratio was shown (R2 = 0.31). Two centers had discordant performance between valve and CABG (O/E ≤ 1 for CABG while O/E > 2 for valve procedures). No centers had CABG O/E ratio greater than 2 while valve O/E ratio ≤1. Linearized slope describing volume-outcome effects showed stronger effect in valve operations compared to CABG: O/E ratio declined 0.1 units per 100 CABG volume increase, while O/E ratio declined 0.33 units per 100 valve volume increase. CONCLUSION: In NY hospitals, favorable valve outcomes may indicate good CABG outcomes but good CABG outcomes may not ensure valve outcomes. Outcome variation in valve operation could be related to stronger volume-outcome effect in valve operations relative to CABG. Valve operations may benefit from regionalization.

The relationship between cardiac surgeon experience and average patient risk profile: CA and NY statewide analysis.

BACKGROUND: It is unknown how high and low-risk cases are distributed among cardiac surgeons of different experience levels. The purpose of this study was to determine if high and low-risk coronary artery bypass grafting (CABG) cases are distributed among surgeons in such a way that would optimize outcomes in light of recent studies that show mid-career surgeons may obtain better patient outcomes on more complex cases. METHODS: We performed a cross-sectional study using aggregated New York (NY) and California (CA) statewide surgeon-level outcome data, including 336 cardiac surgeons who performed 43,604 CABGs. The surgeon observed and expected mortality rates (OMR and EMR) were collected and the number of years-in-practice was determined by searching for surgeon training history on online registries. Loess and linear regression models were used to characterize the relationship between surgeon EMR and surgeon years-in-practice. RESULTS: The median number of surgeon years-in-practice was 20 (interquartile range [IQR] 11-28) with a median annual case volume of 46 (IQR 19, 70.25). The median surgeon observed to expected mortality (O:E) ratio was 0.87 (IQR 0.19-1.4). Median EMR for CA surgeons was 2.42% and 1.44% for NY surgeons. Linear regression models showed EMR was similar across years in practice. Regression models also showed surgeon O:E ratios were similar across years-in-practice. CONCLUSION: High and low-risk CABG cases are relatively equally distributed among surgeons of differing experience levels. This equal distribution of high and low-risk cases does not reflect a triaging of more complex cases to more experienced surgeons, which prior research shows may optimize patient outcomes.

The impact of trainees' working hour regulations on outcome in CABG and valve surgery in the State of New York.

BACKGROUND AND AIM: In 2011, the Accreditation Council for Graduate Medical Education (ACGME) restricted the first-year residents' duty-hour to less than 16-hour shifts, decreased the maximum shift duration for senior residents, and increased minimum time off after on-call duties. Whether these changes may have impacted the outcomes in cardiac surgery remains unclear. METHODS: We performed a difference-in-difference analysis of the New York State Cardiac Surgery Reporting System data in 2004-2006 (before the duty-hour policies change) and 2014-2016 (after the change). We evaluated differences in 30-day risk-adjusted mortality rates (RAMR) in coronary artery bypass grafting (CABG) and valve surgeries, stratifying data by hospital type: teaching hospitals (TH) versus nonteaching hospitals (NTH). NTH served as the control not affected by the duty-hour policies. RESULTS: (1) The overall surgical volume for CABG surgery has decreased over time (37,645-24,991), while the volume for valve surgery remained similar (20,969-21,532); (2) TH had better short-term outcomes for CABG procedures during 2014-2016 (median RAMR: 1.01% vs. 1.55% in TH vs. NTH, respectively; p = .025) as well as for valve procedures during both 2004-2006 (5.16% vs. 7.49%, p = .020) and 2014-2016 (2.59% vs. 4.09%, p = .033); (3) at difference-in-difference analysis, trainees' duty-hour regulations were not associated with worsening short-term outcomes in both CABG (p = .296) and valve (p = .651) procedures performed in TH. CONCLUSION: The introduction of the 2011 trainees' duty-hour regulations was not associated with worse short-term outcomes for CABG and valve surgery performed in the State of NY by TH.

A robotic-assisted approach to sliding plasty, neochordoplasty, and annuloplasty in a technically complex mitral valve repair.

In experienced hands, complex mitral valve repair can be safely and effectively performed in a totally endoscopic, robotic-assisted manner. We present a technically complex case of a 76-year-old man with severe, symptomatic mitral regurgitation due to Barlow's disease, moderate-to-severe tricuspid regurgitation, and atrial fibrillation.

Prevalence and Characteristics of Patients with Median Arcuate Ligament Syndrome in a Cohort Diagnosed with Celiac Artery Compression.

BACKGROUND: Median arcuate ligament syndrome (MALS) is a frequent differential diagnosis in patients with postprandial abdominal symptoms, but diagnosis remains challenging. The aim of this study was to identify characteristics of patients who had MALS compared with non-MALS patients among a cohort of patients diagnosed with celiac artery compression (CAC). STUDY DESIGN: An IRB-approved retrospective chart review (2000 to 2021) of patients at our institution with a discharge diagnosis of CAC was performed. Medical record review for clinical symptoms and findings consistent with MALS was performed. RESULTS: Two hundred ninety-three patients with a diagnosis of CAC were identified; 59.7% were women, and average age was 63.9 ± 20.2 years. Sixty-nine (23.5%) patients with CAC had MALS. There were no significant differences in sex or race between MALS and non-MALS patients, but MALS patients were younger (55.7 vs 68.1, p < 0.001). There was no significant difference in gastrointestinal comorbidities between the 2 groups. Patients with MALS were less likely to have diabetes (12.5% vs 26.9%), renal disease (4.6% vs 8.2%), hypertension (41.5% vs 70.3%), mesenteric atherosclerotic disease (14% vs 61.9%), and peripheral artery disease (15.0% vs 39.7%). CONCLUSIONS: We demonstrate a novel observation that MALS patients tend to have fewer atherosclerotic characteristics than non-MALS patients with CAC. Patients in our study with MALS were more likely to be younger, women, and presenting with epigastric pain. MALS patients had a significantly lower incidence of diabetes, hypertension, renal disease, mesenteric artery disease, and peripheral arterial disease compared with the non-MALS group. An important clinically relevant feature of MALS patients may be their lack of atherosclerotic phenotype compared with non- MALS patients with CAC.

Growth rate of ascending thoracic aortic aneurysms in a non-referral-based population.

BACKGROUND: Prior studies on ascending thoracic aortic aneurysm (ATAA) growth rates have reported approximately 1 mm of growth per year but these studies are based on referral-based study populations which are biased towards the highest risk patients who may not represent the true natural history of aortic aneurysm disease. We aimed to characterize the growth rate of ATAAs in a non-referral-based population, using a large institutional database of computed tomography (CT) scans. METHODS: We queried the 21,325 CT scans performed at our institution between 2013 and 2016 on patients ages 50-85 years old for radiologic diagnosis of aortic aneurysm or dilatation. 560 patients were identified to have aortic dilatation > 4 cm, of which 207 had follow-up scan intervals > 6 months. This comprised our non-referral-based study population. Linearized annual aneurysm growth rates were calculated by dividing the change in aortic size by the time interval between CT scans. RESULTS: The median time interval between scans was 2.7 years (interquartile range [IQR] 1.5-4.2) for the 207 patients included in the study. The median initial aneurysm size was 4.3 cm (IQR 4.1-4.5). 38.2% (n = 79) of patients did not experience aortic dilatation. The median growth rate was 0.13 mm/year (IQR - 0.24 to 0.49). Of patients in the top quartile of growth rates, 26.9% of patients were female whereas 12.9% of patients were female in the bottom three quartiles of growth rates. CONCLUSION: While some patients' ATAAs may grow at previously published rates of around 1 mm/year, this is not the predominant pattern in a non-referral-based population and may over-estimate the overall growth rate of ATAAs.

Widening volume and persistent outcome disparity in valve operations: New York statewide analysis, 2005-2016.

OBJECTIVES: Volume concentration of complex noncardiac operations to high-volume centers has been observed, but whether this is also occurring in cardiac surgery is unknown. We examined the relationship between volume concentration and mortality rates for valve surgery and coronary artery bypass grafting (CABG) between 2005 and 2016 in New York State. METHODS: We analyzed publicly available, hospital-level case volume and risk-adjusted mortality rates (RAMRs) from 2005 to 2016 for isolated CABG and isolated or concomitant valve operations performed in New York. We identified hospitals in the top- and bottom-volume quartiles for each procedure type and compared changes in percent market share and outcomes. Bivariate and univariate longitudinal analysis was used to evaluate the statistical significance of the temporal trend. RESULTS: Among 36 centers, percent market share of the top-volume quartile increased for valve cases from 54.4% to 59.4%, whereas CABG share increased from 41.4% to 44.3%. No significant changes were noted in market share for the bottom quartile. The top-volume quartile demonstrated significant trends in improving outcomes over the study period for both valve procedures (RAMR: -0.261%/year, P < .001) and CABG (RAMR: -0.071%/year, P = .018). No significant trends were noted in the bottom quartile for either procedure. CONCLUSIONS: In New York, over the last decade, highest-volume hospitals increased their market share for valve operations while maintaining lower mortality rates than lowest-volume hospitals. Valve volume is regionalizing in the setting of a persistent outcome gap between the highest- and lowest-volume hospitals, suggesting that volume-based referrals for specialized cardiac procedures may improve surgical mortality.

Patterns of Surveillance Imaging for Incidentally Detected Ascending Aortic Aneurysms.

BACKGROUND: Ascending aortic aneurysms (AsAA) remain a silent killer for which timely intervention and surveillance intervals are critical. Despite this, little is known about the follow-up care patients receive after incidental detection of an AsAA. We examined the pattern of surveillance and follow-up care for these high-risk patients. METHODS: We identified patients at our institution with incidentally detected AsAAs (≥37 mm) between 2013 and 2016. We collected information on patients' aneurysms and clinical follow-up. Logistic regression models related aneurysm size and demographics to whether patients received follow-up imaging or referral. RESULTS: From 2013 to 2016, 261 patients were identified to have incidentally detected AsAAs among the 21,336 computed tomography scans performed at our institution. The median aneurysm size was 4.2 cm (interquartile range, 4 to 4.4). Only 18 (6.9%) of the identified patients were referred to a cardiac surgeon for evaluation, and only 37.9% of the identified patients had a follow-up chest computed tomography scan within 1 year of detection; 34% had an echocardiogram. The median follow-up duration for the study was 5 years. Logistic regression models showed that aneurysm size and family history were significant predictors of whether a patient was referred to a cardiac surgeon (odds ratio 10.34; 95% confidence interval, 2.3 to 47.9), but not whether the patients received follow-up imaging. CONCLUSIONS: Among 261 patients with incidentally detected AsAAs, only a third received any follow-up imaging within 1 year after detection, with very low clinical penetrance for expert referral. Surveillance of this high-risk patient population appears insufficient and may require standardization.

Impact of secondary mitral regurgitation on survival in atrial and ventricular dysfunction.

BACKGROUND: Natural history of atrial and ventricular secondary mitral regurgitation (SMR) is poorly understood. We compared the impact of the degree of SMR on survival between atrial and ventricular dysfunction. METHODS: We conducted a retrospective cohort study of patients who underwent echocardiography in a healthcare network between 2013-2018. We compared the survival of patients with atrial and ventricular dysfunction, using propensity scores developed from differences in patient demographics and comorbidities within SMR severity strata (none, mild, moderate or severe). We fitted Cox proportional hazards models to estimate the risk-adjusted hazards of death across different severities of SMR between patients with atrial and ventricular dysfunction. RESULTS: Of 11,987 patients included (median age 69 years [IQR 58-80]; 46% women), 6,254 (52%) had isolated atrial dysfunction, and 5,733 (48%) had ventricular dysfunction. 3,522 patients were matched from each arm using coarsened exact matching. Hazard of death in atrial dysfunction without SMR was comparable to ventricular dysfunction without SMR (HR 1.1, 95% CI 0.9-1.3). Using ventricular dysfunction without SMR as reference, hazards of death remained higher in ventricular dysfunction than in atrial dysfunction across increasing severities of SMR: mild SMR (HR 2.1, 95% CI 1.8-2.4 in ventricular dysfunction versus HR 1.7, 95%CI 1.5-2.0 in atrial dysfunction) and moderate/severe SMR (HR 2.8, 95%CI 2.4-3.4 versus HR 2.4, 95%CI 2.0-2.9). CONCLUSIONS: SMR across all severities were associated with better survival in atrial dysfunction than in ventricular dysfunction, though the magnitude of the diminishing survival were similar between atrial and ventricular dysfunction in increasing severity of SMRs.

Cavitron ultrasonic surgical aspirator for mitral annular decalcification.

Mitral valve surgery can be especially difficult in patients with mitral annular calcification. Not only is it associated with higher morbidity and mortality, but there is no consensus strategy for the treatment of mitral annular calcification, which may render some patients inoperable. We describe a case of severe mitral annular calcification successfully treated with the cavitron ultrasonic surgical aspirator.   We demonstrate the proper technique for and the efficacy of using this instrument for decalcification of the mitral annulus. The video tutorial includes the patient presentation, preoperative and postoperative imaging, and the proper surgical technique for cavitron ultrasonic surgical aspirator-assisted mitral annular decalcification. With the proper technique, the cavitron ultrasonic surgical aspirator may mitigate the risk of surgical complications associated with severe calcification of the mitral annulus.

Risk reduction and pharmacological strategies to prevent progression of aortic aneurysms.

INTRODUCTION: While size thresholds exist to determine when aortic aneurysms warrant surgical intervention, there is no consensus on how best to treat this disease before aneurysms reach the threshold for intervention. Since a landmark study in 1994 first suggested ß-blockers may be useful in preventing aortic aneurysm growth, there has been a surge in research investigating different pharmacologic therapies for aortic aneurysms - with very mixed results. AREAS COVERED: We have reviewed the existing literature on medical therapies used for thoracic and abdominal aortic aneurysms in humans. These include ß-blockers, angiotensin II receptor blockers, and angiotensin-converting enzyme inhibitors as well as miscellaneous drugs such as tetracyclines, macrolides, statins, and anti-platelet medications. EXPERT OPINION: While multiple classes of drugs have been explored for risk reduction in aneurysm disease, with few exceptions results have been disappointing with an abundance of contradictory findings. The vast majority of studies have been done in patients with abdominal aortic aneurysms or thoracic aortic aneurysm patients with Marfan Syndrome. There exists a striking gap in the literature when it comes to pharmacologic management of non-Marfan Syndrome patients with thoracic aortic aneurysms. Given the differences in pathogenesis, this is an important future direction for aortic aneurysm research.

Sex Differences in Inflammation During Venous Remodeling of Arteriovenous Fistulae.

Vascular disorders frequently have differing clinical presentations among women and men. Sex differences exist in vascular access for hemodialysis; women have reduced rates of arteriovenous fistula (AVF) maturation as well as fistula utilization compared with men. Inflammation is increasingly implicated in both clinical studies and animal models as a potent mechanism driving AVF maturation, especially in vessel dilation and wall thickening, that allows venous remodeling to the fistula environment to support hemodialysis. Sex differences have long been recognized in arterial remodeling and diseases, with men having increased cardiovascular events compared with pre-menopausal women. Many of these arterial diseases are driven by inflammation that is similar to the inflammation during AVF maturation. Improved understanding of sex differences in inflammation during vascular remodeling may suggest sex-specific vascular therapies to improve AVF success.

Robotic, totally endoscopic excision of mitral valve papillary fibroelastoma.

Papillary fibroelastoma is a rare, benign tumor that affects males more frequently  than females and that tends to be diagnosed during the fifth or sixth decade of life. It tends to arise on cardiac valves, with the aortic valve being the most frequent location followed by the mitral valve, the tricuspid valve, and the pulmonary valve. We present the case of a robotic-assisted, totally endoscopic excision of a mitral valve papillary fibroelastoma.