The TNFΔARE Mouse as a Model of Intestinal Fibrosis.

Crohn disease (CD) is a highly morbid chronic inflammatory disease. Although many patients with CD also develop fibrostenosing complications, there are no medical therapies for intestinal fibrosis. This is due, in part, to a lack of high-fidelity biomimetic models to enhance understanding and drug development, which highlights the need for developing in vivo models of inflammatory bowel disease-related intestinal fibrosis. This study investigates whether the TNFΔARE mouse, a model of ileal inflammation, also develops intestinal fibrosis. Several clinically relevant outcomes were studied, including features of structural fibrosis, histologic fibrosis, and gene expression. These include the use of a new luminal casting technique, traditional histologic outcomes, use of second harmonic imaging, and quantitative PCR. These features were studied in aged TNFΔARE mice as well as in cohorts of numerous ages. At >24 weeks of age, TNFΔARE mice developed structural, histologic, and transcriptional changes of ileal fibrosis. Protein and RNA expression profiles showed changes as early as 6 weeks, coinciding with histologic changes as early as 14 to 15 weeks. Overt structural fibrosis was delayed until at least 16 weeks and was most developed after 24 weeks. This study found that the TNFΔARE mouse is a viable and highly tractable model of ileal fibrosis. This model and the techniques used herein can be leveraged for both mechanistic studies and therapeutic development for the treatment of intestinal fibrosis.

Chemokine Signatures of Pathogen-Specific T Cells II: Memory T Cells in Acute and Chronic Infection.

Pathogen-specific memory T cells (TM) contribute to enhanced immune protection under conditions of reinfection, and their effective recruitment into a recall response relies, in part, on cues imparted by chemokines that coordinate their spatiotemporal positioning. An integrated perspective, however, needs to consider TM as a potentially relevant chemokine source themselves. In this study, we employed a comprehensive transcriptional/translational profiling strategy to delineate the identities, expression patterns, and dynamic regulation of chemokines produced by murine pathogen-specific TM CD8+TM, and to a lesser extent CD4+TM, are a prodigious source for six select chemokines (CCL1/3/4/5, CCL9/10, and XCL1) that collectively constitute a prominent and largely invariant signature across acute and chronic infections. Notably, constitutive CCL5 expression by CD8+TM serves as a unique functional imprint of prior antigenic experience; induced CCL1 production identifies highly polyfunctional CD8+ and CD4+TM subsets; long-term CD8+TM maintenance is associated with a pronounced increase of XCL1 production capacity; chemokines dominate the earliest stages of the CD8+TM recall response because of expeditious synthesis/secretion kinetics (CCL3/4/5) and low activation thresholds (CCL1/3/4/5/XCL1); and TM chemokine profiles modulated by persisting viral Ags exhibit both discrete functional deficits and a notable surplus. Nevertheless, recall responses and partial virus control in chronic infection appear little affected by the absence of major TM chemokines. Although specific contributions of TM-derived chemokines to enhanced immune protection therefore remain to be elucidated in other experimental scenarios, the ready visualization of TM chemokine-expression patterns permits a detailed stratification of TM functionalities that may be correlated with differentiation status, protective capacities, and potential fates.

Chemokine Signatures of Pathogen-Specific T Cells I: Effector T Cells.

The choreography of complex immune responses, including the priming, differentiation, and modulation of specific effector T cell populations generated in the immediate wake of an acute pathogen challenge, is in part controlled by chemokines, a large family of mostly secreted molecules involved in chemotaxis and other patho/physiological processes. T cells are both responsive to various chemokine cues and a relevant source for certain chemokines themselves; yet, the actual range, regulation, and role of effector T cell-derived chemokines remains incompletely understood. In this study, using different in vivo mouse models of viral and bacterial infection as well as protective vaccination, we have defined the entire spectrum of chemokines produced by pathogen-specific CD8+ and CD4+T effector cells and delineated several unique properties pertaining to the temporospatial organization of chemokine expression patterns, synthesis and secretion kinetics, and cooperative regulation. Collectively, our results position the "T cell chemokine response" as a notably prominent, largely invariant, yet distinctive force at the forefront of pathogen-specific effector T cell activities and establish novel practical and conceptual approaches that may serve as a foundation for future investigations into the role of T cell-produced chemokines in infectious and other diseases.

Aging boosts antiviral CD8+T cell memory through improved engagement of diversified recall response determinants.

The determinants of protective CD8+ memory T cell (CD8+TM) immunity remain incompletely defined and may in fact constitute an evolving agency as aging CD8+TM progressively acquire enhanced rather than impaired recall capacities. Here, we show that old as compared to young antiviral CD8+TM more effectively harness disparate molecular processes (cytokine signaling, trafficking, effector functions, and co-stimulation/inhibition) that in concert confer greater secondary reactivity. The relative reliance on these pathways is contingent on the nature of the secondary challenge (greater for chronic than acute viral infections) and over time, aging CD8+TM re-establish a dependence on the same accessory signals required for effective priming of naïve CD8+T cells in the first place. Thus, our findings reveal a temporal regulation of complementary recall response determinants that is consistent with the recently proposed "rebound model" according to which aging CD8+TM properties are gradually aligned with those of naïve CD8+T cells; our identification of a broadly diversified collection of immunomodulatory targets may further provide a foundation for the potential therapeutic "tuning" of CD8+TM immunity.

Aging of Antiviral CD8+ Memory T Cells Fosters Increased Survival, Metabolic Adaptations, and Lymphoid Tissue Homing.

Aging of established antiviral T cell memory can foster a series of progressive adaptations that paradoxically improve rather than compromise protective CD8+ T cell immunity. We now provide evidence that this gradual evolution, the pace of which is contingent on the precise context of the primary response, also impinges on the molecular mechanisms that regulate CD8+ memory T cell (TM) homeostasis. Over time, CD8+ TM generated in the wake of an acute infection with the natural murine pathogen lymphocytic choriomeningitis virus become more resistant to apoptosis and acquire enhanced cytokine responsiveness without adjusting their homeostatic proliferation rates; concurrent metabolic adaptations promote increased CD8+ TM quiescence and fitness but also impart the reacquisition of a partial effector-like metabolic profile; and a gradual redistribution of aging CD8+ TM from blood and nonlymphoid tissues to lymphatic organs results in CD8+ TM accumulations in bone marrow, splenic white pulp, and, particularly, lymph nodes. Altogether, these data demonstrate how temporal alterations of fundamental homeostatic determinants converge to render aged CD8+ TM poised for greater recall responses.

Cardiac surgeons' concerns, perceptions, and responses during the COVID-19 pandemic.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has had an unprecedented impact on health care and cardiac surgery. We report cardiac surgeons' concerns, perceptions, and responses during the COVID-19 pandemic. METHODS: A detailed survey was sent to recruit participating adult cardiac surgery centers in North America. Data regarding cardiac surgeons' perceptions and changes in practice were analyzed. RESULTS: Our study comprises 67 institutions with diverse geographic distribution across North America. Nurses were most likely to be redeployed (88%), followed by advanced care practitioners (69%), trainees (28%), and surgeons (25%). Examining surgeon concerns in regard to COVID-19, they were most worried with exposing their family to COVID-19 (81%), followed by contracting COVID-19 (68%), running out of personal protective equipment (PPE) (28%), and hospital resources (28%). In terms of PPE conservation strategies among users of N95 respirators, nearly half were recycling via decontamination with ultraviolet light (49%), followed by sterilization with heat (13%) and at home or with other modalities (13%). Reuse of N95 respirators for 1 day (22%), 1 week (21%) or 1 month (6%) was reported. There were differences in adoption of methods to conserve N95 respirators based on institutional pandemic phase and COVID-19 burden, with higher COVID-19 burden institutions more likely to resort to PPE conservation strategies. CONCLUSIONS: The present study demonstrates the impact of COVID-19 on North American cardiac surgeons. Our study should stimulate further discussions to identify optimal solutions to improve workforce preparedness for subsequent surges, as well as facilitate the navigation of future healthcare crises.

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.

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.

Report of the Third Heart Surgery Forum Scientific Sessions: Zagreb, Croatia December 6-8, 2017: Conference Highlights.

The Heart Surgery Forum is an online community dedicated to topics related to all aspects of cardiothoracic surgery. It consists of an informative website (www.hsforum.com), a traditional indexed journal both in print and online, and an email-based "list-serv" for discussion of surgical cases and techniques. The email list-serv, "OpenHeart-L" (The Forum) is composed of surgeons and allied specialties (perfusion, anesthesia, nursing). Dr. Mark Levinson (USA) started The Forum originally in 1995. He also served as the first Editor-in-Chief of the print journal for many years. Coinciding with the popularity of The Forum, and the desire by many members to meet in person, the First Heart Surgery Forum Conference (#1 HSF) was held in Savudrija, Istria, Croatia in 2010. The overwhelming success of this meeting, in terms of attendance and scientific content, resulted in #2 HSF being held in Split, Croatia in 2014. Recently, the #3 HSF meeting was held in Zagreb, Croatia on December 6-8th, 2017. This report highlights the scientific events of this meeting, and more importantly aims to inspire greater involvement by the international cardiothoracic community. Substantial increasing attendance was seen at each subsequent meeting, not only in terms of the number of participants and lectures, but also in the number of countries represented.

Assigning clinical meaning to somatic and germ-line whole-exome sequencing data in a prospective cancer precision medicine study.

PURPOSE: Implementing cancer precision medicine in the clinic requires assessing the therapeutic relevance of genomic alterations. A main challenge is the systematic interpretation of whole-exome sequencing (WES) data for clinical care. METHODS: One hundred sixty-five adults with metastatic colorectal and lung adenocarcinomas were prospectively enrolled in the CanSeq study. WES was performed on DNA extracted from formalin-fixed paraffin-embedded tumor biopsy samples and matched blood samples. Somatic and germ-line alterations were ranked according to therapeutic or clinical relevance. Results were interpreted using an integrated somatic and germ-line framework and returned in accordance with patient preferences. RESULTS: At the time of this analysis, WES had been performed and results returned to the clinical team for 165 participants. Of 768 curated somatic alterations, only 31% were associated with clinical evidence and 69% with preclinical or inferential evidence. Of 806 curated germ-line variants, 5% were clinically relevant and 56% were classified as variants of unknown significance. The variant review and decision-making processes were effective when the process was changed from that of a Molecular Tumor Board to a protocol-based approach. CONCLUSION: The development of novel interpretive and decision-support tools that draw from scientific and clinical evidence will be crucial for the success of cancer precision medicine in WES studies.Genet Med advance online publication 26 January 2017.

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.

Minimally Invasive Versus Transcatheter and Surgical Aortic Valve Replacement: A Propensity Matched Study.

BACKGROUND: An increasing number of options exist for the treatment of severe symptomatic aortic stenosis. The study aim was to compare short-term outcomes in patients undergoing surgical aortic valve replacement (SAVR), minimally invasive aortic valve replacement (MIAVR), and transcatheter aortic valve replacement (TAVR). METHODS: A multi-institutional retrospective review of 2,571 patients undergoing SAVR (n = 842), MIAVR via right anterior thoracotomy (n = 699) and TAVR (n = 1,030) between 2011 and 2014 was conducted. TAVR patients were further stratified as either transfemoral (TF) or transapical (TA). Propensity matching was performed between MIAVR and SAVR (384 pairs), MIAVR and TA-TAVR (115 pairs), and MIAVR and TF-TAVR (247 pairs). RESULTS: Total numbers of AVR increased between 2011 and 2014. When stratified by procedure type, MIAVR and TF-TAVR accounted for most of the growth, while TA-TAVR and SAVR each experienced a decreased volume. Propensity matched comparisons of SAVR, TF-TAVR, and TA-TAVR versus MIAVR revealed no difference in 30-day mortality. TF-TAVR versus MIAVR revealed that MIAVR had a decreased rate of stroke (0.4% versus 3.6%, p = 0.02) and increased atrial fibrillation (AF; 19.4% versus 4%, p <0.01). When compared to SAVR, MIAVR had a lower incidence of AF (19% versus 32.6%, p <0.01). MIAVR exhibited decreased ventilation time (27.2 versus 134 h, p = 0.03) and intensive care unit time (63.7 versus 92.7 h, p = 0.02) compared to TA-TAVR. CONCLUSIONS: During recent years, MIAVR and TFTAVR have experienced significant growth in volume with near-comparable short-term outcomes, while SAVR and TA-TAVR volumes have declined. These results underscore the importance of surgeons adopting MIAVR and TF-TAVR techniques in order to offer patients optimal outcomes.

Outcomes of Patients With Acute Type B (DeBakey III) Aortic Dissection: A 13-Year, Single-Center Experience.

BACKGROUND: Aortic dissection remains the most common aortic catastrophe. In the endovascular era, the management of acute type B aortic dissection (ATBAD) is undergoing dramatic changes. The aim of this study is to evaluate the long-term outcomes of patients with ATBAD who were treated at our center over a 13-year period. METHODS AND RESULTS: We reviewed patients with ATBAD between 2001 and 2014, analyzing variables based on status (complicated [c] versus uncomplicated [u]) and treatment modalities. We defined cATBAD as rupture, expansion of diameter on imaging during the admission, persistent pain, or clinical malperfusion leading to a deficit in cerebral, spinal, visceral, renal, or peripheral vascular territories at presentation or during initial hospitalization. Postoperative outcomes were defined as deficits not present before the intervention. Outcomes were compared between the groups by use of Kaplan-Meier and descriptive statistics. We treated 442 patients with ATBAD. Of those 442, 60.6% had uATBAD and were treated medically, and 39.4% had cATBAD, of whom 39.0% were treated medically to 30.0% with open repair, 21.3% with thoracic endovascular aortic repair, and 9.7% with other open peripheral procedures. Intervention-free survival at 1 and 5 years was 84.8% and 62.7% for uATBAD, 61.8% and 44.0% for cATBAD-medical, 69.2% and 47.2% for cATBAD-open, and 68.0% and 42.5% for cATBAD-thoracic endovascular aortic repair, respectively (P=0.001). Overall survival was significantly related primarily to complicated presentation. CONCLUSIONS: In our experience, early and late outcomes of ATBAD were dependent on the presence of complications, with cATBAD faring worse. Although uATBAD was associated with favorable early survival, late complications still occurred, mandating radiographic surveillance and open or endovascular interventions. Prospective trials are required to better determine the optimal therapy for uATBAD.

Extracellular matrix remodeling in wound healing of critical size defects in the mitral valve leaflet.

The details of valvular leaflet healing following valvuloplasty and leaflet perforation from endocarditis are poorly understood. In this study, the synthesis and turnover of valvular extracellular matrix due to healing of a critical sized wound was investigated. Twenty-nine sheep were randomized to either CTRL (n = 11) or HOLE (n = 18), in which a 2.8-4.8 mm diameter hole was punched in the posterior mitral leaflet. After 12 weeks, posterior leaflets were harvested and histologically stained to localize extracellular matrix components. Immunohistochemistry was also performed to assess matrix components and markers of matrix turnover. A semi-quantitative grading scale was used to quantify differences between HOLE and CTRL. After 12 weeks, the hole diameter was reduced by 71.3 ± 1.4 % (p < 0.001). Areas of remodeling surrounding the hole contained more activated cells, greater expression of proteoglycans, and markers of matrix turnover (prolyl 4-hydroxylase, metalloproteases, and lysyl oxidase, each p ≤ 0.025), along with fibrin accumulation. Two distinct remodeling regions were evident surrounding the hole, one directly bordering the hole rich in versican and hyaluronan and a second adjacent region with abundant collagen and elastic fiber turnover. The remodeling also caused reduced delineation between valve layers (p = 0.002), more diffuse staining of matrix components and markers of matrix turnover (p < 0.001), and disruption of the collagenous fibrosa. In conclusion, acute valve injury elicited distinct, heterogeneous alterations in valvular matrix composition and structure, resulting in partial wound closure. Because these changes could also affect leaflet mechanics and valve function, it will be important to determine their impact on healing wounds.

Need for Limb Revascularization in Patients with Acute Aortic Dissection is Associated with Mesenteric Ischemia.

BACKGROUND: Acute aortic dissection (AAD) can cause limb ischemia due to branch vessel occlusion. A minority of patients have persistent ischemia after central aortic repair and require peripheral arterial revascularization. We investigated whether the need for limb revascularization is associated with adverse outcomes. METHODS: We reviewed our cases of AAD from 2000 to 2014 and identified patients with malperfusion syndromes (coronary, cerebral, spinal, visceral, renal, or peripheral ischemia). Patients with DeBakey I/II (Stanford type A) dissection had urgent open repair of the ascending aorta. Patients with DeBakey III (Stanford type B) dissection were initiated on anti-impulse medical therapy and had either open aortic repair or thoracic endovascular aortic repair for malperfusion syndromes. Patients with persistent lower limb ischemia after aortic repair usually had either extra-anatomic bypass grafting or iliac stenting. Some DeBakey III patients had peripheral revascularization without central aortic repair. We performed univariate and multivariate analysis to determine the effects of need for limb revascularization and clinical outcomes. RESULTS: We treated 1,015 AAD patients (501 [49.4%] DeBakey I/II and 514 [50.6%] DeBakey III) with a mean age of 59.7 ± 14.5 years (67.5% males). Aortic repair was performed in all DeBakey I/II patients and in 103 (20.0%) DeBakey III patients. Overall 30-day mortality was 11.3%. Lower limb ischemia was present in 104 (10.3%) patients and was more common in DeBakey I/II compared with DeBakey III dissections (65.4% vs. 34.6%; odds ratio [OR] 2.1, confidence interval [CI] 1.4-3.2; P = 0.001). Among the 40 patients who required limb revascularization, there was no difference in need for revascularization between DeBakey I/II and III patients. Patients requiring limb revascularization were more likely to have mesenteric ischemia compared with the rest of the cohort in both DeBakey I/II (P = 0.037) and DeBakey III dissections (P < 0.001) with worse 10-year survival (21.9 % vs. 59.2%, P < 0.001). When adjusted for other malperfusion syndromes, patients with limb revascularization had similar long-term survival compared to uncomplicated dissection patients (P = 0.960). CONCLUSIONS: Patients requiring lower limb revascularization after treatment for AAD are more likely to have mesenteric ischemia and worse survival. The need for limb revascularization is a marker for more extensive dissection and should prompt evaluation for visceral malperfusion.

Repair of extensive aortic aneurysms: a single-center experience using the elephant trunk technique over 20 years.

OBJECTIVES: We report the early and late outcomes after repair of extensive aortic aneurysms using the 2-stage elephant trunk (ET) technique. BACKGROUND: Management of aneurysm involving the entire aorta is a significant challenge. Given the anatomical complexity, the staged ET procedure was devised. A paucity of long-term data of outcomes of this approach exists. METHODS: A single-center retrospective analysis of a prospectively collected database of all patients undergoing repair for extensive aortic aneurysm was performed. RESULTS: Between 1991 and 2013, we repaired 3012 aneurysms of the ascending or thoracoabdominal aorta. Of these, we performed 503 operations in 348 patients using the ET technique. Mean age was 62.4 ± 14.3 years, and 156/346 (45.1%) operations were in women; 288 patients underwent first-stage ET with 157 receiving a complete second-stage repair. Index repair early mortality was 29/317 (9.1%). Completion stage early mortality was 17/186 = 9.1%. Stroke after first-stage ET repair was 10/297 (3.4%) and immediate neurologic deficit after the second-stage ET repair was 6/206 (2.9%). In the 131 patients who did not receive a second-stage repair, 17.8% died in the interval between 31 and 45 days. CONCLUSIONS: Extensive aortic aneurysm is a complex problem, but it can be managed safely with a 2-stage open procedure. Those patients who could not complete the completion repair fared poorly. Better predictors for early outcome need to be determined. The use of ET technique remains a valuable approach for repair of extensive aortic aneurysm.

A novel elastin gene mutation in a Vietnamese patient with cutis laxa.

We report a 3-year-old girl from Vietnam with severe congenital cutis laxa; no cardiovascular, pulmonary, neurologic, or visceral involvement; and no family history of cutis laxa. Mutational analysis of the elastin gene identified heterozygosity for a previously unreported de novo c.2184delT mutation in exon 30 not present in either parent.