Coronary flow capacity and survival prediction after revascularization: physiological basis and clinical implications.

BACKGROUND AND AIMS: Coronary flow capacity (CFC) is associated with an observed 10-year survival probability for individual patients before and after actual revascularization for comparison to virtual hypothetical ideal complete revascularization. METHODS: Stress myocardial perfusion (mL/min/g) and coronary flow reserve (CFR) per pixel were quantified in 6979 coronary artery disease (CAD) subjects using Rb-82 positron emission tomography (PET) for CFC maps of artery-specific size-severity abnormalities expressed as percent left ventricle with prospective follow-up to define survival probability per-decade as fraction of 1.0. RESULTS: Severely reduced CFC in 6979 subjects predicted low survival probability that improved by 42% after revascularization compared with no revascularization for comparable severity (P = .0015). For 283 pre-and-post-procedure PET pairs, severely reduced regional CFC-associated survival probability improved heterogeneously after revascularization (P < .001), more so after bypass surgery than percutaneous coronary interventions (P < .001) but normalized in only 5.7%; non-severe baseline CFC or survival probability did not improve compared with severe CFC (P = .00001). Observed CFC-associated survival probability after actual revascularization was lower than virtual ideal hypothetical complete post-revascularization survival probability due to residual CAD or failed revascularization (P < .001) unrelated to gender or microvascular dysfunction. Severely reduced CFC in 2552 post-revascularization subjects associated with low survival probability also improved after repeat revascularization compared with no repeat procedures (P = .025). CONCLUSIONS: Severely reduced CFC and associated observed survival probability improved after first and repeat revascularization compared with no revascularization for comparable CFC severity. Non-severe CFC showed no benefit. Discordance between observed actual and virtual hypothetical post-revascularization survival probability revealed residual CAD or failed revascularization.

ARISE: First-In-Human Evaluation of a Novel Stent Graft to Treat Ascending Aortic Dissection.

BACKGROUND: Operative mortality for type A aortic dissection is still 10-20% at centers of excellence. Additionally, 10-20% are not considered as viable candidates for open surgical repair and not offered life-saving emergency surgery. ARISE is a multicenter investigation evaluating the novel GORE® Ascending Stent Graft (ASG; Flagstaff, AZ). OBJECTIVE: The purpose of this study is to assess early feasibility of using these investigational devices to treat ascending aortic dissection. METHODS: This a prospective, multicenter, non-randomized, single-arm study that enrolls patients at high surgical risk with appropriate anatomical requirements based on computed tomography imaging at 7 of 9 US sites. Devices are delivered transfemorally under fluoroscopic guidance. Primary endpoint is all-cause mortality at 30 days. Secondary endpoints include major adverse cardiovascular and cerebrovascular events (MACCE) at 30 days, 6 months, and 12 months. RESULTS: Nineteen patients were enrolled with a mean age of 75.7 years (range 47-91) and 11 (57.9%) were female. Ten (52.6%) had DeBakey type I disease, and the rest were type II. Sixteen (84.2%) of the patients were acute. Patients were treated with safe access, (7/19 (36.8%) percutaneous, 10/19 (52.6%) transfemoral, 2/19 (10.5%) iliac conduit), delivery, and deployment completed in all cases. Median procedure time was 154 mins (range 52-392) and median contrast used was 111 mL (range 75-200). MACCE at 30 days occurred in 5 patients including mortality 3/19 (15.8%), disabling stroke in 1/19 (5.3%), and myocardial infarction in 1/19 (5.3%). CONCLUSION: Results from the ARISE early feasibility study of a specific ascending stent graft device to treat ascending aortic dissection are promising.

Emergency and Compassionate Use of a Novel Ascending Endograft for Ascending and Arch Aortic Pathology.

PURPOSE: Patients with complicated ascending aortic pathology, including patients with acute type A aortic dissection may be at extreme risk for open repair. Thoracic endovascular aortic repair (TEVAR), infrequently used for the ascending aorta, may be considered an alternative in this setting. We describe early results for emergency and compassionate (E&C) use of a novel endograft, specifically designed for use to treat pathology of the ascending aorta. MATERIALS AND METHODS: This case series evaluated 19 patients (mean age, 68.84±13.12 years; 57.9% female) treated with ascending TEVAR for acute and chronic acute (4), subacute (1), or chronic (1) aortic dissection or pseudoaneurysm (13). Six of the 19 patients (31.5%) were treated under compassionate use and 13 patients (68.4%) were treated under the emergency use exemption. Ten patients (52.6%) received additional devices to extend treatment into the arch and descending aorta. RESULTS: Device delivery was achieved in all patients (100%). Thirty-day mortality and stroke occurred in 3 patients (15.8%) and in 1 patient (5.3%), respectively. In 1 patient (5.3%), with an Unanticipated Adverse Device Event, the aorta ruptured when the endograft eroded into the adventitial portion of dissection site at the posterior aspect of the ascending wall. Devices were explanted in 2 patients (10.5%), 353 and 610 days after the index procedure, respectively. Six patients had endoleaks (31.6%), including type I (n=2, 10.5%), type II endoleaks (n=3, 15.8%), and indeterminate endoleak (n=1, 5.3%). CONCLUSIONS: Delivery and deployment of a novel ascending thoracic stent graft with or without an additional branched arch extension is feasible in patients with complex anatomy and pathology, including acute aortic dissection and pseudoaneurysm. Additional experience with this novel device will further refine the patient population most suitable for endovascular ascending aortic repair for these pathologies. CLINICAL IMPACT: This study describes a novel stent graft specifically designed for treatment of ascending aortic pathology, including acute type A dissection. The patients described in this series constituted a group outside the formal US FDA sponsored clinical trial, and were those accepted as part of an emergency and compassionate use basis.

Timing of Intervention for Aortic Intramural Hematoma.

Intramural hematoma (IMH) is one of the acute aortic syndromes along with acute aortic dissection and penetrating aortic ulcer. The three conditions can occur alone or in combination with overlapping presentation. Medical, open surgical, and endovascular treatment is tailored depending on clinical presentation, timing, and location within the aorta. Among patients who present with acute IMH affecting the ascending aorta (Type A), urgent open surgical repair is considered the primary line of treatment in patients who are suitable candidates and unstable. The management of IMH in the descending aorta and aortic arch (Type B) is similar to that applied to treat acute dissections in the same segment. Medical treatment with sequential imaging is recommended in patients with uncomplicated course, and endovascular repair is indicated in patients with rupture, persistent pain, end-organ ischemia, or rapid aortic enlargement. This review discusses the ideal timing for treatment of IMH.

Sex Representation Among Principal Investigators in Cardiac Surgery Clinical Trials in the United States: The Glass Ceiling and Room for Improvement.

OBJECTIVE: The aim of this study was to determine the sex representation among principal investigators (PIs) in US cardiac surgery clinical trials. SUMMARY BACKGROUND DATA: Being a principal investigator in a US clinical trial confers national recognition among peers. Sex representation among principal investigators (PIs) in US cardiac surgery clinical trials has not been evaluated. METHODS: We evaluated 124 US cardiac surgery trials registered on Clin-icalTrials.gov from 2014 to 2019. Sixty trials included PIs (n = 266) from 128 institutions that had a combined total of 1040 adult cardiac surgeons. We examined sex representation among junior-level (instructor or assistant professor) and senior-level (associate, full, or Emeritus professor) PIs by calculating the participation-to-prevalence ratio (PPR), whereby a PPR range of 0.8 to 1.2 reflects equitable representation. RESULTS: The pool representation percentage was 6.1% (63/1040) for women and 93.9% (977/1040) for men. A total of 266 PI positions were assigned to adult cardiac surgeons: 6 (9.5%; PPR = 0.37) from the female pool and 260 (26.6%; PPR = 1.04) from the male pool ( P = 0.004). The percentage of PIs with studies funded by industry was 9.5% ofthe female pool (PPR = 0.39) and 25.0% of the male pool (PPR = 1.04) ( P = 0.009). No National Institutes of Health-funded or other funded trials had female PIs. An overall trend was observed toward disproportionally more men than women among PIs, especially at the senior level ( P = 0.027). CONCLUSIONS: Equitable opportunities for PI positions are available for junior-level but not senior-level cardiothoracic surgeons. These results suggest a need for active engagement and promotion of equal opportunities in cardiac surgery.

Surgical and endovascular treatment of late postcoarctation repair aortic aneurysms: Results from an international multicenter study.

OBJECTIVE: The formation of postaortic coarctation aneurysms (pCoAA) is well-described in the literature and carries a significant risk of rupture and death. Treatment strategies include open surgical, hybrid, and endovascular repair, depending on the clinical presentation, risk assessment, and anatomy. The aim of this study was to report the early and midterm results of open surgical and endovascular repair of pCoAA. METHODS: This is an international multicenter retrospective study including patients who underwent open surgical or endovascular repair for pCoAA between 2000 and 2021 at 14 highly specialized academic cardiovascular centers. The preoperative, intraoperative, and postoperative data were recorded and analyzed. RESULTS: A total of 74 patients (46 male; median age, 44 years; interquartile range [IQR], 35-53 years) underwent pCoAA repair. All patients had previously undergone surgical repair of aortic coarctation at a median age of 11 years for the index procedure (IQR, 7-17 years). The most common first surgical correction was synthetic patch aortoplasty in 48 patients, followed by graft interposition in 11. The median pCoAA diameter was 54 mm (IQR, 44-63 mm). The median time from the aortic coarctation repair to the pCoAA diagnosis was 33 years (IQR, 25-40 years). A total of 33 patients had symptoms at presentation, including thoracic or back pain in 8 patients. Open surgical repair was performed in 28 patients, including four frozen elephant trunk procedures and one Bentall. The remaining 46 patients underwent endovascular repair of the pCoAA. Two in-hospital deaths were observed (one frozen elephant trunk and one endovascular). After a median follow-up of 50 months (IQR, 14-127 months), there were a total of seven reinterventions. CONCLUSIONS: This international multicenter study demonstrates that patients with pCoAA can be safely treated with either open surgical or endovascular interventions. Because the median time between the coarctation repair and the aneurysm formation was more than 30 years, life-long surveillance of these patients is warranted.

In-hospital outcomes and long-term survival of women of childbearing age with aortic dissection.

OBJECTIVE: In the present study, we defined the outcomes and effects of pregnancy in a cohort of women of childbearing age with acute aortic dissection (AAD). METHODS: We reviewed our database of AAD to identify all eligible female patients. Women aged <45 years were included. Data on pregnancy timing with respect to the occurrence of dissection, the demographic data, dissection extent, dissection treatment, dissection-related outcomes, overall maternal and fetal mortality, and genetic testing results were analyzed. RESULTS: A total of 62 women aged <45 years had presented to us with AAD from 1999 to 2017. Of the 62 women, 37 (60%) had had a history of pregnancy at AAD. Of these 37 patients, 10 (27%) had had a peripartum aortic dissection, defined as dissection during pregnancy or within 12 months postpartum. Of the 10 AADs, 5 were type A and 5 were type B. Three patients had presented with AAD during pregnancy (one in the second and two in the third trimester). Five patients (50%) had developed AAD in the immediate postpartum period (within 3 months) and two (20%) in the late postpartum period. For the immediate postpartum AADs (<3 months), four of the five patients delivered via cesarean section. Of these 10 peripartum AADs, 3 (30%) had occurred in patients with known Marfan syndrome. In-hospital mortality for those with peripartum AAD was 10% (1 of 10). Fetal mortality was 20% (2 of 10). CONCLUSIONS: The frequency of aortic dissection in women of childbearing age at our institution was low. However, pregnancy might increase the risk of those young women genetically predisposed to dissection events. From these data, this risk appears to be greatest in the immediate postpartum period, even for those who undergo cesarean section. Close clinical and radiographic surveillance is required for all women with suspected aortopathy, especially in the third trimester and early postpartum period.

Validation of scoring system predicting permanent pacemaker implantation after transcatheter aortic valve replacement.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) is being increasingly performed in patients with severe aortic stenosis. Despite newer generation valves, atrioventricular (AV) conduction disturbance is a common complication, necessitating permanent pacemaker (PPM) implantation in about 10% of patients. Hence, it is imperative to improve periprocedural risk stratification to predict PPM implantation after TAVR. The objective of this study was to externally validate a novel risk-stratification model derived from the National Inpatient Sample (NIS) database that predicts risk of PPM from TAVR. METHODS: Components of the score included pre-TAVR left and right bundle branch block, sinus bradycardia, second-degree AV block, and transfemoral approach. The scoring system was applied to 917 patients undergoing TAVR at our institution from November 2011 to February 2017. We assessed its predictive accuracy by looking at two components: discrimination using the C-statistic and calibration using the Hosmer-Lemeshow goodness of fit test. RESULTS: Ninety patients (9.8%) required PPM. The scoring system showed good discrimination with C-statistic score of 0.6743 (95% CI: 0.618-0.729). Higher scores suggested increased PPM risk, that is, 7.3% with score ⩽3, 19.23% with score 4-6, and 37.50% with score ≥7. Patients requiring PPM were older (81.4 versus 78.7 years, P = .002). Length of stay and in-hospital mortality was significantly higher in PPM group. CONCLUSIONS: The NIS database derived PPM risk prediction model was successfully validated in our database with acceptable discriminative and gradation capacity. It is a simple but valuable tool for patient counseling pre-TAVR and in identifying high-risk patients.

Rates, predictors, and outcomes of early readmissions after tricuspid valve surgery.

BACKGROUND: The data on readmissions following tricuspid valve repair/replacement (TVR) are scarce. We examined rates, predictors, causes, and outcomes of readmissions after TVR, using the National Readmission Database. METHODS: The International Classification of Diseases-9th version was used to identify the patients who underwent isolated TVR or concomitant aortic, mitral, and coronary bypass surgeries. Rates, causes, and outcomes were assessed using the analysis of variance and the χ2 test, and predictors of readmissions were evaluated using multivariate analysis. RESULTS: A total of 8254 patients who underwent TVR during 2013 to 2014 were included, of whom 1994 (24.16%) were isolated, and 6260 (75.84%) were performed concomitantly with other heart valve or coronary bypass surgery. A total of 1720 (20.84%) patients were readmitted within 30 days. The readmission rates were 448 (22.46%) after isolated TVR and similar after concomitant TVR (TVR + aortic valve replacement, TVR + mitral valve repair, TVR + coronary artery bypass graft, and TVR + multiple) (P = .194); whereas 1305 (20.11%) and 414 (23.45%) were after tricuspid valve repair and replacement (P = .080), respectively. The independent predictors of readmission were acute kidney injury during index visit and Charlson comorbidity index of more than 2. Mean time to readmission and median length of stay during readmission were 13.02 (±7.93) and 5 (interquartile range: 3-9) days, respectively. Total mortality during rehospitalization was 105 (6.1%), a very high (26.86%) number of patients were discharged to skilled facilities after readmission. CONCLUSIONS: One out of five patients were readmitted within 30 days after the TVR, associated with 6.1% mortality during rehospitalization, and very high need for skilled facility placement.

Time of day does not influence outcomes in acute type A aortic dissection: Results from the IRAD.

BACKGROUND: Type A acute aortic dissection (TAAAD) represents a surgical emergency requiring intervention regardless of time of day. Whether such a "evening effect" exists regarding outcomes for TAAAD has not been previously studied using a large registry data. METHODS: Patients with TAAAD were identified from the International Registry of Acute Aortic Dissections (1996-2019). Outcomes were compared between patients undergoing operative repair during the daytime (D), defined as 8 am-5 pm, versus the evening (N), defined as 5 pm-8 am. RESULTS: Four thousand one-hundrd and ninety-seven surgically treated patients with TAAAD were identified, with 1824 patients undergoing daytime surgery (43.5%) and 2373 patients undergoing evening surgery (56.5%). Daytime patients were more likely to have undergone prior cardiac surgery (13.2% vs. 9.5%; p < .001) and have had a prior aortic dissection (4.8% vs. 3.4%; p = .04). Evening patients were more likely to have been transferred from a referring hospital (70.8% vs. 75.0%; p = .003). Daytime patients were more likely to undergo aortic valve sparing root procedures (23.3% vs. 19.2%; p = .035); however, total arch replacement was performed with equal frequency (19.4% vs. 18.8%; p = .751). In-hospital mortality (D: 17.3% vs. N. 16.2%; p = .325) was similar between both groups. Subgroup analysis examining the effect of weekend presentation revealed no significant mortality difference. CONCLUSIONS: A majority of TAAAD patients underwent surgical repair at night. There were higher rates of postoperative tamponade in evening patients; however, mortality was similar. The expertise of cardiac-dedicated operative and critical care teams regardless of time of day as well as training paradigms may explain similar mortality outcomes in this high risk population.

Clinically Important Misclassification of Aortic Valve Stenosis Severity Using Non-Invasive Techniques: Simultaneous Echocardiography and Cardiac Catheterization During Transcatheter Aortic Valve Implantation in Awake Patients.

BACKGROUND: Suitability for transcatheter aortic valve (AV) implantation (TAVI) is determined by using transthoracic echocardiography (TTE), although left-sided cardiac catheterization (LCC) provides directly measured pressure data. TAVI in awake patients permits simultaneous comparison of TTE and LCC under physiologically relevant left ventricular loading conditions. We hypothesized that clinically important discrepancies between TTE and LCC would be identified. METHODS AND RESULTS: TAVI was performed in 108 awake patients undergoing intra-procedural TTE and LCC between January 1, 2016 and December 31, 2016, based upon pre-procedure TTE data. Intra-procedural assessments simultaneously were performed before and after prosthesis implantation. Based upon mean trans-AV systolic ejection pressure gradient (MSEPG), AS was graded as: mild (<20 mm Hg; grade 1), moderate (20 - <40 mm Hg; grade 2), or severe (≥40 mm Hg; grade 3). In 79 of the 108 (73.1%) patients, intra-procedural TTE and LCC assessments were concordant. In 2 of the 108 (1.9%) patients, TTE overestimated AS severity by ≥1 grade. In 27 of the 108 (25.0%) patients, TTE underestimated AS severity by ≥1 grade. In total, AS severity reclassification occurred in 29 (26.9%) patients. Overall, TTE underestimated MSEPG by 8.9 ± 1.2 mm Hg (TTE MSEPG versus LCC MSEPG; P < .001). CONCLUSION: Current TTE criteria appear to frequently and importantly underestimate AS severity. Because decision-making regarding TAVI often exclusively is based upon TTE data, these findings suggest either a continued role for LCC in the diagnostic assessment of AS in patients who do not meet standard TTE criteria or lowering TTE cutoffs for TAVI.

Genetic Variants in LRP1 and ULK4 Are Associated with Acute Aortic Dissections.

Acute aortic dissections are a preventable cause of sudden death if individuals at risk are identified and surgically repaired in a non-emergency setting. Although mutations in single genes can be used to identify at-risk individuals, the majority of dissection case subjects do not have evidence of a single gene disorder, but rather have the other major risk factor for dissections, hypertension. Initial genome-wide association studies (GWASs) identified SNPs at the FBN1 locus associated with both thoracic aortic aneurysms and dissections. Here, we used the Illumina HumanExome array to genotype 753 individuals of European descent presenting specifically with non-familial, sporadic thoracic aortic dissection (STAD) and compared them to the genotypes of 2,259 control subjects from the Atherosclerosis Risk in Communities (ARIC) study matched for age, gender, and, for the majority of cases, hypertension. SNPs in FBN1, LRP1, and ULK4 were identified to be significantly associated with STAD, and these results were replicated in two independent cohorts. Combining the data from all cohorts confirmed an inverse association between LRP1 rs11172113 and STAD (p = 2.74 × 10(-8); OR = 0.82, 95% CI = 0.76-0.89) and a direct association between ULK4 rs2272007 and STAD (p = 1.15 × 10(-9); OR = 1.35, 95% CI = 1.23-1.49). Genomic copy-number variation analysis independently confirmed that ULK4 deletions were significantly associated with development of thoracic aortic disease. These results indicate that genetic variations in LRP1 and ULK4 contribute to risk for presenting with an acute aortic dissection.

The role of ascending aortic size in outcomes of patients with uncomplicated acute type B aortic dissection.

OBJECTIVE: Recent studies demonstrate that uncomplicated acute type B aortic dissection (uATBAD) patients with enlarged descending thoracic aortic diameters are at high risk for development of complications. This study aimed to determine the association of maximum ascending aortic diameter and area and outcomes in patients with uATBAD. METHODS: All patients admitted with uATBAD from June 2000 to January 2015 were reviewed, and those with available imaging were included. All measurements were obtained by a specialized cardiovascular radiologist, including the maximum ascending aortic diameter and area. Outcomes, including the need for intervention and mortality, were tracked over time. Data were analyzed by stratified Kaplan-Meier and multiple Cox regression analyses using SAS 9.4 software (SAS Institute, Cary, NC). RESULTS: During the study period, 298 patients with uATBAD were admitted, with 238 having available computed tomography and 131 having computed tomography angiography imaging and adequate follow-up available for analysis. The cohort had an average age of 60.96 ± 13.4 years (60% male, 53% white). Ascending aortic area >12.1 cm2 and ascending aortic diameter >40.8 mm were associated with subsequent arch and proximal progression necessitating open ascending aortic repair (P < .027 and P < .033, respectively). Ascending diameter >40.8 mm predicted lower intervention-free survival (P = .01). However, it failed to predict overall survival (P = .12). Ascending aortic area >12.1 cm2 predicted lower intervention-free survival (P = .005). However, this was not predictive of mortality (P = .08). Maximum aortic diameter along the length of the aorta >44 mm persisted as a risk factor for mortality (P < .001). Neither maximum ascending aortic diameter >40.8 mm (hazard ratio [HR], 1.09; 95% confidence interval [CI], 0.42-2.83; P = .85) nor area >12.1 cm2 (HR, 0.992; 95% CI, 0.38-2.61; P = .99) significantly predicted mortality when controlling for maximum aortic diameter along the length of the aorta >44 mm (HR, 7.34; 95% CI, 2.3-23.41; P < .001), diabetes mellitus (HR, 6.4; 95% CI, 2.17-18.93; P < .001), age (HR, 1.06/y; 95% CI, 1.03-1.10; P < .001), history of stroke (HR, 5.03; 95% CI, 1.52-16.63; P = .008), and syncope on admission (HR, 21.11; 95% CI, 2.3-193.84; P = .007). Ascending aortic diameter >40.8 mm (HR, 2.01; 95% CI, 1.03-3.95; P = .04) and maximum ascending aortic area >12.1 cm2 (HR, 1.988; 95% CI, 1.02-3.87; P = .04) on admission persisted as predictors of decreased intervention-free survival after controlling for maximum aortic diameter along the length of the aorta >44 mm (HR, 3.142; 95% CI, 1.47-6.83; P < .004), syncope on admission (HR, 26.3; 95% CI, 2.81-246; P < .004), and pleural effusion on admission (HR, 3.02; 95% CI, 1.58-5.77; P < .001). CONCLUSIONS: uATBAD patients with ascending aortic area >12.1 cm2 or maximum ascending aortic diameter >40.8 mm are at high risk for development of subsequent arch and proximal progression and may require closer follow-up or earlier intervention. Ascending aortic size (diameter and area) is predictive of decreased intervention-free survival in patients with uATBAD.

Imaging characteristics of acute type A aortic dissection and candidacy for repair with ascending aortic endografts.

BACKGROUND: Acute type A aortic dissection (ATAD) remains associated with substantial short-term mortality, and despite increasing rates of surgical repair, as many as 10% to 20% of patients do not undergo surgery because of comorbidities and dissection-related complications. For patients unable to undergo open repair, previous attempts at endovascular treatment of ATAD used devices originally designed for deployment in the descending thoracic aorta. Industry has begun to support early investigational devices meant specifically for placement within the ascending aorta. We evaluated relevant aortic parameters to examine which patients may be candidates for repair with an ascending aortic endograft. METHODS: We reviewed 100 consecutive patients in our institutional ATAD database with contrast-enhanced computed tomography imaging on an Aquarius iNtuition workstation (TeraRecon, San Mateo, Calif), using curved planar reformatting (vessel tracking) and orthogonal views for measurements. We compared relevant aortic measurements against proposed criteria for future ascending endografts, including various landing zone diameters and intimal tear distances from the distal coronary ostium. RESULTS: Of the 100 patients examined, 39% had proximal intimal tears located outside the tubular ascending aorta. In all, 30% were excluded on the basis of either the presence of a prosthetic aortic valve or significant aortic insufficiency, and 6% were excluded on the basis of the presence of patent coronary artery bypass grafts from the ascending aorta. Many patients had multiple exclusion criteria, and based on various proposed criteria, overall candidacy ranged from 2% to 23%. If a maximum landing zone diameter of 42 mm and intimal tears as little as 20 mm distal to the distalmost coronary were considered treatable, only 8% of patients would have been candidates compared with 20% candidacy if aortic diameters up to 46 mm and intimal tears as little as 10 mm distal to the distalmost coronary were considered treatable. The most frequent single cause for exclusion was inadequacy of the proximal landing zone. Iliofemoral vascular access was also assessed and deemed adequate in >90% of cases. CONCLUSIONS: A minority of patients suffering ATAD would currently qualify for ascending aortic endografting on the basis of anatomic criteria alone. Future device designs should take into account these common anatomic exclusion criteria so that more versatile devices may be developed and commercially available to treat a larger number of patients.

Clinical history and management recommendations of the smooth muscle dysfunction syndrome due to ACTA2 arginine 179 alterations.

PURPOSE: Smooth muscle dysfunction syndrome (SMDS) due to heterozygous ACTA2 arginine 179 alterations is characterized by patent ductus arteriosus, vasculopathy (aneurysm and occlusive lesions), pulmonary arterial hypertension, and other complications in smooth muscle-dependent organs. We sought to define the clinical history of SMDS to develop recommendations for evaluation and management. METHODS: Medical records of 33 patients with SMDS (median age 12 years) were abstracted and analyzed. RESULTS: All patients had congenital mydriasis and related pupillary abnormalities at birth and presented in infancy with a patent ductus arteriosus or aortopulmonary window. Patients had cerebrovascular disease characterized by small vessel disease (hyperintense periventricular white matter lesions; 95%), intracranial artery stenosis (77%), ischemic strokes (27%), and seizures (18%). Twelve (36%) patients had thoracic aortic aneurysm repair or dissection at median age of 14 years and aortic disease was fully penetrant by the age of 25 years. Three (9%) patients had axillary artery aneurysms complicated by thromboembolic episodes. Nine patients died between the ages of 0.5 and 32 years due to aortic, pulmonary, or stroke complications, or unknown causes. CONCLUSION: Based on these data, recommendations are provided for the surveillance and management of SMDS to help prevent early-onset life-threatening complications.

New type A dissection after acute type B aortic dissection.

OBJECTIVE: Aortic dissection is a dynamic process that can progress both proximal and distal to the initial entry tear. We sought to determine associations for development of proximal progression or new type A aortic dissection (NTAD) after acute type B dissection (ATBD) and its effect on survival of the patient. METHODS: We reviewed all cases of acute aortic dissection that we managed from 1999 to 2014. Univariate and bivariate analyses were performed to identify correlates of NTAD. Multivariable regression and proportional hazards regression analysis was done to determine the effect of dissection progression on long-term survival. RESULTS: Among 477 cases of ATBD managed, 19 (4.0%) patients developed NTAD during a median follow-up of 4.1 (interquartile range, 1.4-7.7) years. Median time from diagnosis of ATBD to NTAD was 124 (interquartile range, 23-1201) days. Baseline predictors for development of NTAD at initial ATBD admission included bicuspid aortic valve (P = .006) and age <60 years (P = .012). Although not statistically significant, point estimates indicate that thoracic endovascular aortic repair was twice as frequent in NTAD cases as in non-NTAD cases. Overall 5-year survival was 70.2%. Patients who had repair of NTAD appear to have longer survival, although this effect is on the margin of statistical significance (P = .051). After risk factor and correlates of NTAD adjustment, this effect was no longer apparent (P = .089). CONCLUSIONS: The natural history of ATBD is such that there is a persistent risk of NTAD, with the highest risk in the first 6 months. Factors associated with NTAD include bicuspid aortic valve and young age. Thoracic endovascular aortic repair did not have a large effect on risk. Timely diagnosis and repair of NTAD are associated with good survival rates. Lifelong surveillance is warranted in all cases of descending thoracic aortic dissection regardless of initial treatment modality.

Outcomes and readmissions after transcatheter and surgical aortic valve replacement in patients with cirrhosis: A propensity matched analysis.

BACKGROUND: The data on the comparative outcomes and readmissions after transcatheter aortic valve replacement (TAVR) and surgical aortic valve replacement (SAVR) in patients with cirrhosis are limited. We compared mortality, complications, discharge disposition, 30-day readmission rates, length of stay, and cost of hospitalization in cirrhotic patients undergoing TAVR and SAVR. METHODS: The National Inpatient Sample (NIS) and the National Readmission Database (NRD) were used for the study. The International Classification of Diseases-9th version was used to define cohorts of patients undergoing TAVR and SAVR. Patients undergoing concomitant other valve or coronary bypass surgery were excluded. Propensity-score matching was used to compare the outcomes between the groups. RESULTS: From 2012 to 2014, a total of 126 and 157 patients with cirrhosis underwent TAVR and SAVR, respectively. Of the 283 patients, 16 (5.7%) died during the same hospitalization. We found 345 patients with cirrhosis who had undergone an aortic valve replacement (156 with TAVR, and 189 with SAVR) in the 2013 and 2014 NRD. On propensity matching, there were no significant differences between the in-hospital mortality, readmissions, hospitalization costs, and discharges to home within the TAVR and SAVR groups. However, post-procedure length of stay (6.3 vs. 10.2 days; P < 0.001) and blood transfusion rates (22% vs. 58%; P < 0.001) were significantly lower in TAVR patients. CONCLUSIONS: Cirrhotic patients undergoing TAVR has high, but similar mortality and 30-day readmission rates when compared to SAVR; however, has shorter length of stay and lower blood transfusion rates.

LOX Mutations Predispose to Thoracic Aortic Aneurysms and Dissections.

RATIONALE: Mutations in several genes have been identified that are responsible for 25% of families with familial thoracic aortic aneurysms and dissections. However, the causative gene remains unknown in 75% of families. OBJECTIVES: To identify the causative mutation in families with autosomal dominant inheritance of thoracic aortic aneurysms and dissections. METHODS AND RESULTS: Exome sequencing was used to identify the mutation responsible for a large family with thoracic aortic aneurysms and dissections. A heterozygous rare variant, c.839G>T (p.Ser280Arg), was identified in LOX, encoding a lysyl oxidase, that segregated with disease in the family. Sanger and exome sequencing was used to investigate mutations in LOX in an additional 410 probands from unrelated families. Additional LOX rare variants that segregated with disease in families were identified, including c.125G>A (p.Trp42*), c.604G>T (p.Gly202*), c.743C>T (p.Thr248Ile), c.800A>C (p.Gln267Pro), and c.1044T>A (p.Ser348Arg). The altered amino acids cause haploinsufficiency for LOX or are located at a highly conserved LOX catalytic domain, which is relatively invariant in the population. Expression of the LOX variants p.Ser280Arg and p.Ser348Arg resulted in significantly lower lysyl oxidase activity when compared with the wild-type protein. Individuals with LOX variants had fusiform enlargement of the root and ascending thoracic aorta, leading to ascending aortic dissections. CONCLUSIONS: These data, along with previous studies showing that the deficiency of LOX in mice or inhibition of lysyl oxidases in turkeys and rats causes aortic dissections, support the conclusion that rare genetic variants in LOX predispose to thoracic aortic disease.

Management strategies in acute type B aortic intramural hematoma.

PURPOSE OF REVIEW: Intramural hematoma (IMH) is one of the acute aortic syndromes. IMH was originally noted as an 'aortic dissection without intimal tear.' It had been speculated that IMHs were caused by ruptured vasa vasorum in the aortic media and distinguished from classical aortic dissections. However, the majority of IMHs are now detected with an intimal defect using high-resolution computed tomography, which implies that IMHs may be a subset of aortic dissections with very limited flow in the false lumen. Management of IMHs had been controversial, but guidelines published in the early 2010 s recommend treating IMHs as classical aortic dissections: surgical repairs were indicated for type A IMHs; complicated acute type B IMHs were treated with endovascular or open repairs and uncomplicated type B IMHs were to be managed with antiimpulse therapy and close surveillance. To date, based on the favorable long-term outcomes of preemptive thoracic endovascular aortic repair (TEVAR) for uncomplicated type B aortic dissections, early TEVAR for uncomplicated type B IMHs has been attempted. In this review, we describe the pathophysiology and management strategies for acute IMHs. RECENT FINDINGS: IMHs have intimal tears or disruptions in 70-80%. SUMMARY: Type B IMH should be managed with antiimpulse therapy, unless the patient has persistent pain or large ulcer-like projection.

Early readmissions after transcatheter and surgical aortic valve replacement.

OBJECTIVES: We aimed to determine and compare the prevalence, and predictors of readmissions after the transcatheter aortic valve replacement (TAVR) and surgical aortic valve replacement (SAVR). BACKGROUND: There are limited data on the readmission rates after TAVR in comparison with SAVR. METHODS: We analyzed the data from 2013 National Readmission Database. Propensity-matched pairs were used to analyze differences in readmission rates between TAVR and SAVR for patients aged ≥65. RESULTS: A total of 24,020 (TAVR-transfemoral 3,469, TAVR-transapical 1,433, SAVR 19,118) patients were included. The readmission rates were not statistically different for all propensity-matched TAVR and SAVR patients (17.2% vs. 20.6%, P = 0.28). However, in subgroup analysis, transapical TAVR had the highest readmission rate (22.8% vs. 16.5% vs. 16.0%, P < 0.001, respectively) and readmission leading to death (7.1% vs. 5.3% vs. 3.9%, P = 0.022, respectively) when compared with transfemoral TAVR and SAVR. In all the groups, two-thirds of readmissions were due to noncardiac causes. Congestive heart failure (CHF) and arrhythmia were the most frequent cardiac etiologies. The independent predictors of readmission were female sex, CHF, and chronic obstructive pulmonary disease. Patients who received care in teaching hospitals had lower probability of readmission. CONCLUSIONS: One of six patients were readmitted within 30 days after the aortic valve replacement. On propensity score analysis, there were no significant differences between the early readmission rates between TAVR and SAVR groups. However, the patients undergoing transapical TAVR were at higher risk for readmission, and subsequent deaths when compared with transfemoral TAVR and SAVR. © 2017 Wiley Periodicals, Inc.