Role of Endovascular Aortic Repair in the Treatment of Infected Aortic Aneurysms Complicated by Aortoenteric or Aortobronchial Fistulae.

BACKGROUND: The aim of this study was to compare outcomes and identify factors related to increased mortality of open surgical and endovascular aortic repair (EVAR) of primary mycotic aortic aneurysms complicated by aortoenteric fistula (AEF) or aortobronchial fistula (ABF). METHODS: Patients with primary mycotic aortic aneurysms complicated by an AEF or ABF treated by open surgery or endovascular repair between January 1993 and January 2014 were retrospectively reviewed. Outcomes were compared between the open surgery and endovascular groups, and a Cox's proportional hazard model was used to determine factors associated with mortality. RESULTS: A total of 29 patients included 14 received open surgery and 15 received endovascular repair. Positive initial bacterial blood culture results included Salmonella spp., oxacillin-resistant Staphylococcus aureus, and Klebsiella pneumoniae. Mortality within 1 month of surgery was higher in the open surgery than in the endovascular group (43 vs. 7%, respectively, p = 0.035). Shock, additional surgery to repair gastrointestinal (GI) or airway pathology, and aneurysm rupture were associated with a higher risk of death. Compared with patients without resection surgery, the adjusted hazard ratio of death within 4 years in patients with resection for GI/bronchial disease was 0.25. Survival within 6 months was better in the endovascular group (p = 0.016). CONCLUSION: The results of this study showed that EVAR/thoracic EVAR (TEVAR) is feasible for the management of infected aortic aneurysms complicated by an AEF or ABF, and results in good short-term outcomes. However, EVAR/TEVAR did not benefit long-term survival compared with open surgery.

Outcomes of percutaneous coronary intervention in patients with rheumatoid arthritis and systemic lupus erythematosus: an 11-year nationwide cohort study.

OBJECTIVES: Patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) have an increased risk of developing coronary atherosclerosis. However, the impact of RA and SLE on the outcomes in patients undergoing percutaneous coronary intervention (PCI) remains largely underdetermined. METHODS: Using the National Health Insurance Research Database of Taiwan, we identified 171 547 adult patients who underwent first-time PCI between 2000 and 2010. Among these patients, 525 had established RA, and 211 had SLE. The ORs of inhospital mortality and HRs of overall mortality and adverse cardiac outcomes after PCI (ie, ischaemic events, repeat revascularisation and major adverse cardiac events (MACE)) in relation to RA and SLE were estimated. RESULTS: After adjustment for potential confounders, including patient characteristics and procedural variables, RA (OR=1.73, 95% CI 1.11 to 2.68) and SLE (OR=3.81, 95% CI 2.02 to 7.16) were independent predictors of inhospital mortality. In addition, RA was independently associated with overall mortality (HR=1.55, 95% CI 1.35 to 1.79), ischaemic events (HR=1.18, 95% CI 1.01 to 1.39) and MACE (HR=1.20, 95% CI 1.07 to 1.34) during long-term follow-up, whereas SLE was independently associated with overall mortality (HR=2.20, 95% CI 1.74 to 2.78), repeat revascularisation (HR=1.27, 95% CI 1.02 to 1.58) and MACE (HR=1.47, 95% CI 1.24 to 1.75). Compared with patients without autoimmune diseases, patients with more recent SLE-related hospitalisations prior to PCI were at higher risk of inhospital mortality (p for trend <0.0001). CONCLUSIONS: This study recognises the inherent risks associated with RA and SLE in patients undergoing PCI and highlights the necessity to improve the caring and secondary prevention strategies for these high-risk patients.

Deep brain stimulation for Parkinson's disease using frameless technology.

Historically deep brain stimulation (DBS) for Parkinson's disease (PD) has been performed by frame-based stereotaxy. However, recently the option of frameless stereotaxy has become available. This avoids the potential discomfort the patient may experience because of the frame fixed to the head. This study compared clinical outcomes of DBS performed using frame-based and frameless procedures for PD patients. Twelve patients underwent DBS operations; from these patients, six underwent frame-based and six underwent frameless DBS operations, and assessed 6 months later. Operation time, subthalamic electrode contact length, microelectrode recording (MER) tracts, and unified PD rating scale scores were evaluated and the scores were compared. This small study found no differences between frameless or frame based DBS, and concludes that framless system maybe an acceptable alternative.

Recombinant human thrombomodulin suppresses experimental abdominal aortic aneurysms induced by calcium chloride in mice.

OBJECTIVE: To investigate whether recombinant thrombomodulin containing all the extracellular domains (rTMD123) has therapeutic potential against aneurysm development. SUMMARY BACKGROUND DATA: The pathogenesis of abdominal aortic aneurysm (AAA) is characterized by chronic inflammation and proteolytic degradation of extracellular matrix. Thrombomodulin, a transmembrane glycoprotein, exerts anti-inflammatory activities such as inhibition of cytokine production and sequestration of proinflammatory high-mobility group box 1 (HMGB1) to prevent it from engaging the receptor for advanced glycation end product (RAGE) that may sustain inflammation and tissue damage. METHODS: The in vivo effects of treatment and posttreatment with rTMD123 on aortic dilatation were measured using the CaCl2-induced AAA model in mice. RESULTS: Characterization of the CaCl2-induced model revealed that HMGB1 and RAGE, both localized mainly to macrophages, were persistently upregulated during a 28-day period of AAA development. In vitro, rTMD123-HMGB1 interaction prevented HMGB1 binding to macrophages, thereby prohibiting activation of HMGB1-RAGE signaling in macrophages. In vivo, short-term treatment with rTMD123 upon AAA induction suppressed the levels of proinflammatory cytokines, HMGB1, and RAGE in the aortic tissue; reduced the infiltrating macrophage number; and finally attenuated matrix metalloproteinase production, extracellular matrix destruction, and AAA formation without disturbing vascular calcification. Consistently, posttreatment with rTMD123 seven days after AAA induction alleviated vascular inflammation and retarded AAA progression. CONCLUSIONS: These data suggest that rTMD123 confers protection against AAA development. The mechanism of action may be associated with reduction of proinflammatory mediators, blockade of macrophage recruitment, and suppression of HMGB1-RAGE signaling involved in aneurysm formation and downstream macrophage activation.

Coarctation-induced degenerative abdominal aortic aneurysm in a porcine model.

OBJECTIVE: Hemodynamic stress participates in the initiation and progression of aneurysmal degeneration. Coarctation increases flow-mediated stress on the aortic wall. We tested the hypothesis that prolonged coarctation of an infrarenal abdominal aorta (AA) segment leads to abdominal aortic aneurysm (AAA) formation in mini pigs. METHODS: An asymmetric, funnel-shaped flow path was created by constricting the infrarenal AA segment of Taiwanese Lanyu mini pigs (age, 7-10 months; male and female) wrapped with an 8-mm-wide expanded polytetrafluoroethylene Teflon strip for 4 weeks (4w), 8 weeks (8w), and 12 weeks (12w) (seven pigs per group). This mimics the tortuous aneurysm neck in human AAA, which increases downstream flow-mediated stress. Significant flow disturbance resulting from moderate coarctation was indicated by a pulsatility index reduced to one third the inherent levels. Sham control pigs received Teflon wrapping without coarctation. RESULTS: Aneurysm characterized by progressive medial degeneration occurred at the terminal AA after 12w coarctation. The outer dimension enlargement of the distal AA exceeded 50% compared with that of the proximal AA at 4w, 8w, and 12w postcoarctation (sham, 1.0; 4w, 1.7 ± 0.08; 8w, 1.5 ± 0.09; 12w, 1.7 ± 0.01). Lumen ratio of the distal-to-suprarenal AA increased time dependently, with 12w postcoarctation exhibiting significant increase (sham, 1.0 ± 0.05; 4w, 1.1 ± 0.11; 8w, 1.4 ± 0.20; 12w, 1.5 ± 0.09). In the distal AA, elastic lamellae exhibited fragmentation at 4w and more pronounced fragmentation with decreased density at 8w and 12w postcoarctation. Medial collagen density exhibited the trend to increase at 4w and 8w but was reversed at 12w postcoarctation. Smooth muscle exhibited disarray and nuclear density decrease at 8w and 12w postcoarctation (sham, 6966 ± 888/mm; 4w, 5747 ± 1340/mm; 8w, 4153 ± 323/mm; 12w, 4083 ± 465/mm). Gelatin zymography revealed that matrix metalloproteinase-9 activity markedly increased at 4w postcoarctation. CONCLUSIONS: Prolonged moderate coarctation caused regional hemodynamic stress and thereby induced degenerative AAA in the terminal AA.

Mycotic aneurysms in the abdominal aorta and iliac arteries: CT-based grading and correlation with surgical outcomes.

BACKGROUND: Computed tomography (CT) is the modality of choice in the diagnosis of mycotic aneurysms. The present study aimed to classify the CT findings of mycotic aneurysms, and to assess their predictive value based on the correlation of a CT-based grading system with prognostic factors and outcomes. METHODS: Over the past 21 years, 40 consecutive patients underwent open surgery for mycotic aneurysms of the abdominal aorta and iliac arteries in our hospital. The CT appearances of mycotic aneurysms were categorized into four grades: grade 1, periarterial changes without destruction of the arterial wall; grade 2, presence of saccular outpouching; grade 3, extensive retroperitoneal infection; and grade 4, massive perianeurysmal hemorrhage. Clinical data were recorded for analysis. RESULTS: The surgical mortality and overall aneurysm-related mortality rates were 17.5 and 25%, respectively. The poor prognostic predictors were shock, rupture, and concomitant gastrointestinal procedures. The increasing proportions of shock and rupture status corresponded to mycotic aneurysms of higher grades in the CT-based grading. In addition, one patient in grades 1 and 2, versus five in grades 3 and 4 (P = 0.02), required concomitant gastrointestinal procedures. The CT-based grading exhibited a strong association with surgical mortality (Cramer's V coefficient = 0.65; P = 0.002) and a relatively strong association with overall aneurysm-related mortality (Cramer's V coefficient = 0.53; P = 0.01). CONCLUSIONS: For patients surgically treated for abdominal mycotic aneurysms, the CT-based grading is correlated with clinical severity, surgical complexity, and outcomes, and thus it may serve as a simple scale for risk classification.

Functional preservation of deep brain stimulation electrodes after brain shift induced by traumatic subdural haematoma - case report.

A Parkinson's disease patient with deep brain stimulation (DBS) implantation experienced an acute subdural haematoma (SDH) after a fall. The DBS electrodes and brain parenchyma were shifted. Fortunately, the patient recovered after craniectomy and removal of SDH, and the DBS was re-activated with the same parameters. Patients with DBS implants who suffer a traumatic brain injury do not necessarily incur permanent implant failure; there is every chance that the DBS may continue to work as reported here.

Surgical Treatment of Pseudoaneurysm of Innominate Artery Infected with Burkholderia Pseudomallei.

UNLABELLED: The management of melioidosis, caused by Burkholderia pseudomallei, presenting as an infected pseudoaneurysm requires radical debridement and prolonged antibiotics because the pathogen is resistant to host immunity. An extra-anatomical bypass might be a better treatment choice than in situ graft interposition or other methods.We report on a 76-year-oldman with an infected pseudoaneurysmlocated in the innominate artery and a method of extra-anatomical bypass that has not yet been reported in the literature. The patient recovered well without recurrence of infection after the surgical procedure. KEY WORDS: Burkholderia pseudomallei; Extra-anatomical bypass; Innominate artery; Melioidosis; Pseudoaneurysm.

The feasibility of endovascular aortic repair strategy in treating infected aortic aneurysms.

BACKGROUND: Open surgical treatment for an infected aortic aneurysm has a high rate of surgical morbidity and mortality and does not guarantee eradication of the infected nidus. The use of endovascular aortic repair (EVAR) might simplify the procedure and provide a good alternative for this critical condition, but this remains to be proved. This study assessed the efficacy and outcome of EVAR with an adjunctive antibiotic treatment strategy. METHODS: We focused on the experiences and results of the management of infected aortic aneurysms with positive blood cultures. We drew the blood for culture study, immediately prescribed broad-spectrum antibiotics, performed EVAR procedures, and followed this with sensitive antibiotics and adjunctive procedures. RESULTS: Twelve consecutive patients (mean age, 70 years) were included in this EVAR strategy. Three patients had thoracic, two thoracoabdominal, and the remaining seven had infected abdominal aneurysms. Ten Salmonella, one Staphylococcus, and one Streptococcus spp were identified. There was no hospital death. Three patients underwent computed tomography (CT)-guided drainage, and one underwent open laparotomy debridement. Mean follow-up was 24 months. One late death occurred but was unrelated to reinfection. All patients seemed well, with no evidence of EVAR graft infection at a mean follow-up of 23.6 months. CONCLUSIONS: This small multi-institutional study summarizing the experiences of patients with an infected aortic aneurysm managed by EVAR and an aggressive antibiotic strategy revealed that this EVAR strategy might be a suitable approach to treating this disease. These favorable results may be typical for Salmonella infection, which was present in most of the patients. Further experience is needed to assess whether this therapeutic strategy works equally well in aneurysm infection caused by other organisms.

Intramyocardial peptide nanofiber injection improves postinfarction ventricular remodeling and efficacy of bone marrow cell therapy in pigs.

BACKGROUND: Growing evidence suggests that intramyocardial biomaterial injection improves cardiac functions after myocardial infarction (MI) in rodents. Cell therapy is another promising approach to treat MI, although poor retention of transplanted cells is a major challenge. In this study, we hypothesized that intramyocardial injection of self-assembling peptide nanofibers (NFs) thickens the infarcted myocardium and increases transplanted autologous bone marrow mononuclear cell (MNC) retention to attenuate cardiac remodeling and dysfunction in a pig MI model. METHODS AND RESULTS: A total of 40 mature minipigs were divided into 5 groups: sham, MI+normal saline, MI+NFs, MI+MNCs, and MI+MNCs/NFs. MI was induced by coronary occlusion followed by intramyocardial injection of 2 mL normal saline or 1% NFs with or without 1×10(8) isolated autologous MNCs. NF injection significantly improved diastolic function and reduced ventricular remodeling 28 days after treatment. Injection of MNCs alone ameliorated systolic function only, whereas injection of MNCs with NFs significantly improved both systolic and diastolic functions as indicated by +dP/dt and -dP/dt (1214.5±91.9 and -1109.7±91.2 mm Hg/s in MI+NS, 1693.7±84.7 and -1809.6±264.3 mm Hg/s in MI+MNCs/NFs, respectively), increased transplanted cell retention (29.3±4.5 cells/mm(2) in MI+MNCs and 229.4±41.4 cells/mm(2) in MI+MNCs/NFs) and promoted capillary density in the peri-infarct area. CONCLUSIONS: We demonstrated that NF injection alone prevents ventricular remodeling, whereas cell implantation with NFs improves cell retention and cardiac functions after MI in pigs. This unprecedented combined treatment in a large animal model has therapeutic effects, which can be translated to clinical applications in the foreseeable future.

Cell transplantation for myocardial injury: a preliminary comparative study.

BACKGROUND AIMS: Cell transplantation may restore viable muscle after myocardial infarction. Because many studies have focused on one cell type, we compared the characteristics of skeletal myoblasts (SKM), bone marrow stromal/stem cells (BMSC) and smooth muscle cells (SMC) and their effects on cardiac function after myocardial injury. METHODS: In vitro cell characteristics, including proliferation, hypoxic survival and vascular endothelial cell growth factor (VEGF) expression, of SKM, BMSC and SMC were compared. An in vivo left anterior descending artery ligation rat model was used, and cells were implanted into the infarct (n = 16 per cell type). Cell survival was determined by PKH26 staining and real-time polymerase chain reaction (PCR). Cardiac function, tissue VEGF and stem cell factor (SCF) expression and vasculogenesis were evaluated. RESULTS: Although cell morphologies were distinct, in vitro proliferation was similar. In vitro studies showed that SKM had the highest hypoxic survival, whereas BMSC had the lowest hypoxic survival but the highest VEGF expression. After implantation, SKM showed the highest overall survival and in vivo SCF expression, and both SMC and SKM expressed the highest VEGF levels. Vasculogenesis was significantly (P < 0.001) improved after transplantation of each cell type. Overall, BMSC and SKM promoted the greatest improvement in cardiac function. CONCLUSIONS: SKM, BMSC and SMC expressed VEGF and SCF and promoted vasculogenesis. Although BMSC showed the greatest regenerative potential relative to cell survival and growth factor expression, the greatest improvement in cardiac function was observed with BMSC and SKM.

The efficacy of aortic stent grafts in the management of mycotic abdominal aortic aneurysm-institute case management with systemic literature comparison.

BACKGROUND: Conventional surgery (CS) for treatment of mycotic aortic aneurysm has rather high surgical morbidity and mortality rates. The use of endovascular aortic repair (EVAR) might simplify the procedure and provide a good alternative for this critical condition, but this remains to be proved. We analyzed all mycotic abdominal aortic aneurysm (AAA) cases treated by CS or EVAR in our institute and the reported cases treated by EVAR from the literature to determine the risk factors for aneurysm-related mortality and morbidity and to clarify the efficacy of the EVAR technique. METHODS AND RESULTS: All relevant literature reports of EVAR management of mycotic AAA and all cases treated in our institute, 41 cases, were included and analyzed. Of the 20 cases treated by EVAR, one had early mortality (1/20, 5%); of the remaining 21 cases that received CS, the early mortality rate was 4.8% (1/21). Patients in the CS group had a higher late mortality rate than those in the EVAR group (45% vs. 10.5%, p<0.05). However, the 24-month actual survival rate and actuarial aneurysm-related event-free rate were 83.9+/-8.6% and 78.3+/-9.7%, respectively, for the EVAR group and did not significantly differ from the CS group (70.4+/-10.2% and 80.1+/-8.9%). The significant predictors for aneurysm-related mortality and morbidity were age, Salmonella species infection, and leukocytosis, and possibly aortoenteric fistula and shock, but not the EVAR or CS procedures themselves. CONCLUSION: Compared with CS, EVAR might be an alternative strategy for managing mycotic AAAs.

The Effect of Age, Gender, and Job on Skin Conductance Response among Smartphone Users Who are Prohibited from Using Their Smartphone.

The smartphone is a widely used and rapidly growing phenomenon worldwide, and problematic smartphone use is common in our society. This study's objective was to examine the gender difference of baseline and post-intervention skin conductance response (SCR) among smartphone users and explore the relationships among problematic smartphone use level, anxiety level, and SCR changes by evaluating SCR, the Zung Self-Rating Anxiety Scale score, and the Chinese version of the Smartphone Addiction Inventory (SPAI) score in a one-group baseline and post-test design. Sixty participants were recruited from two communities, and data were collected from April to June 2017. There was a significant difference in terms of SCR changes between young males and old males and between young females and old females. Additionally, the SCR changes in young females were significantly greater than those in young males with twofold mean difference. This study provides strong evidence supporting the effectiveness of SCR measurement for assessing problematic smartphone use (PSU) anxiety when users are in a withdrawal-like state. The SCR measurement can help healthcare providers identify cases with risk factors of PSU for early intervention.

Health care-associated endocarditis caused by Staphylococcus aureus with reduced susceptibility to vancomycin.

Infective endocarditis (IE) caused by methicillin-resistant Staphylococcus aureus with reduced vancomycin susceptibility (SARV) has been reported worldwide. We report the successful treatment of a pediatric patient with SARV IE and characterization of the infecting strain. The MIC of vancomycin rose from 1.5 to 2 microg/ml, and the SARV was confirmed by population analysis.

Successful conservative management with positive end-expiratory pressure for massive haemothorax complicating pacemaker implantation.

Haemothorax resulting from injury to a great vessel is a potential complication during transvenous pacemaker implantation that can be caused by perforation by the electrode. If the amount of bleeding is massive, control needs thoracotomy. We report on a 70-year-old man who had a massive haemothorax following transvenous pacemaker implantation. This complication was controlled successfully by using positive end-expiratory pressure (PEEP). We conclude that this simple but reproducible experience may offer effective haemostasis for a massive haemothorax caused by transvenous catheter perforation.

Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury.

Chylopericardium after an intrapericardial procedure is rare, and satisfactory explanations of its possible causes are lacking.Herein, we present 4 cases of chylopericardium that developed after intrapericardial surgery, and we review the literature. Our literature review revealed 29 cases of chylopericardium that complicated intrapericardial operations, to which we added our 4 cases for analysis. The 33 surgical procedures involved repair for congenital heart disease (n=21), valve surgery (n=5), coronary artery bypass grafting (n=6), and other (n=1). Causes were verified in 7 patients: small lymphatic injury in 3 and high venous pressure or venous thrombosis in 4. Of the 26 patients with chylopericardium of unknown origin, 15 had congenital heart disease. Ten of these 15 had chromosomal abnormalities, especially trisomy 21 (Down syndrome); these patients typically had increased lymphatic permeability, which raised the likelihood of chylopericardium. Five revascularizations for coronary artery disease required harvesting of the left internal thoracic artery for reconstruction, incurring a risk of damage to the drainage site of the right efferent lymphatic trunk. In addition, all 26 patients with chylopericardium of unknown origin underwent dissection of the ascending aorta and the main pulmonary artery, near the right efferent lymphatic trunk. Inadvertent injury to the trunk during the dissection would have increased the risk of chylopericardium. Accordingly, even though the overall incidence of chylopericardium during intrapericardial procedures is low, we recommend a meticulous dissection of the ascending aorta from the main pulmonary artery.

Bilateral persistent sciatic arteries complicated with acute left lower limb ischemia.

Persistent sciatic artery (PSA) is a rare congenital malformation. In the early embryonic stage, the sciatic artery is the major blood supply for the lower limb bulb and is later replaced by the iliofemoral artery as the limb develops. Its failure to regress, sometimes associated with femoral arterial hypoplasia, and therefore becoming the dominant inflow to the lower extremity is called PSA. This anomaly is often associated with a higher rate of aneurysm formation or thromboembolic complications causing lower extremity ischemia. Here, we describe a 79-year-old male patient who presented with acute left lower extremity ischemia. He was treated initially with conventional embolectomy through inguinal and popliteal incisions. The bilateral PSA with thrombosed aneurysms was not identified at first on computed tomographic angiography. It was later diagnosed intraoperatively due to the discontinuity of the superficial femoral artery and popliteal artery found with embolectomy catheter, and was managed successfully with ePTFE graft bypass. Careful interpretation of the imaging study may be helpful in preoperative diagnosis.

Recombinant adeno-associated virus vector carrying the thrombomodulin lectin-like domain for the treatment of abdominal aortic aneurysm.

BACKGROUND AND AIMS: Thrombomodulin (TM), through its lectin-like domain (TMD1), sequesters proinflammatory high-mobility group box 1 (HMGB1) to prevent it from engaging the receptor for advanced glycation end product (RAGE) that sustains inflammation and tissue damage. Our previous study demonstrated that short-term treatment with recombinant TM containing all the extracellular domains (i.e., rTMD123) inhibits HMGB1-RAGE signaling and confers protection against CaCl2-induced AAA formation. In this study, we attempted to further optimize TM domains, as a potential therapeutic agent for AAA, using the recombinant adeno-associated virus (AAV) vector. METHODS: The therapeutic effects of recombinant TMD1 (rTMD1) and recombinant AAV vectors carrying the lectin-like domain of TM (rAAV-TMD1) were evaluated in the CaCl2-induced AAA model and angiotensin II-infused AAA model, respectively. RESULTS: In the CaCl2-induced model, treatment with rTMD1 suppressed the tissue levels of HMGB1 and RAGE, macrophage accumulation, elastin destruction and AAA formation, and the effects were comparable to a mole-equivalent dosage of rTMD123. In the angiotensin II-infused model, a single intravenous injection of rAAV-TMD1 (1011 genome copies), which resulted in a persistently high serum level of TMD1 for at least 12 weeks, effectively attenuated AAA formation with suppression of HMGB1 and RAGE levels and inhibition of proinflammatory cytokine production, macrophage accumulation, matrix metalloproteinase activities and oxidative stress in the aortic wall. CONCLUSIONS: These findings corroborate the therapeutic potential of the TM lectin-like domain in AAA. The attenuation of angiotensin II-infused AAA by one-time delivery of rAAV-TMD1 provides a proof-of-concept validation of its application as potential gene therapy for aneurysm development.

Correction of tetralogy of fallot with absent pulmonary valve syndrome in a young infant using a bicuspid equine pericardial tube.

Absent pulmonary valve syndrome (APVS) is an uncommon variant of tetralogy of Fallot (TOF), which manifests morphologically as vestigial pulmonary valve cusps at the right ventricle-pulmonary trunk junction. The aneurysmally dilated pulmonary arteries may compress the tracheobronchial tree and cause severe respiratory distress in the neonatal or infant stage. Early surgical correction in these patients is necessary despite the high operative mortality rate. A 1-day-old male neonate suffered from progressive shortness of breath after birth. Echocardiography confirmed the diagnosis of TOF with APVS. The marked dilatation of pulmonary arteries resulted in airway compression in addition to heart failure. Total surgical correction was performed at 40 days of age, using a homemade bicuspid equine pericardial tube for right ventricular outflow reconstruction. The short-term follow-up echocardiogram demonstrated good motility of the pericardial leaflet. However, patients receiving this type of valved conduit require meticulous long-term follow-up.

Toll-Like Receptor 4 Is Essential in the Development of Abdominal Aortic Aneurysm.

Toll-like receptor (TLR) family plays a key role in innate immunity and various inflammatory responses. TLR4, one of the well-characterized pattern-recognition receptors, can be activated by endogenous damage-associated molecular pattern molecules such as high mobility group box 1 (HMGB1) to sustain sterile inflammation. Evidence suggested that blockade of TLR4 signaling may confer protection against abdominal aortic aneurysm (AAA). Herein we aimed to obtain further insight into the mechanism by which TLR4 might promote aneurysm formation. Characterization of the CaCl2-induced AAA model in mice revealed that upregulation of TLR4 expression, localized predominantly to vascular smooth muscle cells (VSMCs), was followed by a late decline during a 28-day period of AAA development. In vitro, TLR4 expression was increased in VSMCs treated with HMGB1. Knockdown of TLR4 by siRNA attenuated HMGB1-enhanced production of proinflammatory cytokines, specifically interleukin-6 and monocyte chemoattractant protein-1 (MCP-1), and matrix-degrading matrix metalloproteinase (MMP)-2 from VSMCs. In vivo, two different strains of TLR4-deficient (C57BL/10ScNJ and C3H/HeJ) mice were resistant to CaCl2-induced AAA formation compared to their respective controls (C57BL/10ScSnJ and C3H/HeN). Knockout of TLR4 reduced interleukin-6 and MCP-1 levels and HMGB1 expression, attenuated macrophage accumulation, and eventually suppressed MMP production, elastin destruction and VSMC loss. Finally, human AAA exhibited higher TLR4 expression that was localized to VSMCs. These data suggest that TLR4 signaling contributes to AAA formation by promoting a proinflammatory status of VSMCs and by inducing proteinase release from VSMCs during aneurysm initiation and development.