The Influence of Biological Sex on Presentation and Outcomes of Acute Myocarditis: A Systematic Review and Meta-Analysis.

There is growing evidence of sex-related differences in the epidemiology and pathophysiology of cardiovascular diseases. This is the first systematic review and meta-analysis that aimed to highlight the sex-specific differences in the clinical features and outcomes of acute myocarditis. Electronic searches were performed on Scopus, Embase, and PubMed from inception up to June 2023 to identify studies comparing the clinical features and outcomes of acute myocarditis in males and females. Both qualitative and quantitative summaries were conducted. In this systematic review and meta-analysis of 11 studies involving 34,791 patients presenting with acute myocarditis. Male patients, who comprised 69.8% of the entire pooled population, presented at a markedly younger age (mean difference: -8.99 years; 95% CI: -13.60, -4.38; p=0.0001). They also had significantly lower rates of hypertension, diabetes mellitus, and coronary artery disease compared to female patients (p<0.01). Male patients were more likely to present with ST elevation (RR: 2.57 [1.38, 4.79]; p=0.003) and higher C-reactive protein levels (RR: 3.04 [2.75, 3.34]; p<0.00001) compared to female patients. This review underscores the crucial sex-specific evaluation in acute myocarditis, necessitating tailored approaches in assessment and diagnostic evaluation, and emphasizing the need for additional research in this domain.

Incidence Rates of Penn Classes and Class-Specific Mortality in Acute Type A Aortic Dissection Patients: An Epidemiologic Systematic Review and Meta-Analysis.

Acute type A aortic dissection (ATAAD) is a life-threatening emergency that is associated with a high morbidity and mortality rate. One of the complications is end-organ ischemia, a known predictor of mortality. The primary aims of this meta-analysis were to summarize the findings of observational studies investigating the utility of the Penn classification system and to analyze the incidence rates and mortality patterns within each class. The electronic databases PubMed, MEDLINE, and Embase were searched through to April 2023. These were filtered by multiple reviewers to give 10 studies that met the inclusion criteria. The extracted data included patient characteristics, and primary outcomes were the incidence rates of different Penn classes, along with the corresponding mortality for each class. Out of 1,512 studies identified during the initial search, 10 studies, including 4,494 patients, met the inclusion criteria. The pooled incidence of Penn A was highest at 0.55 (95% CI 0.52, 0.58), followed by Penn B at 0.21 (95% CI 0.17, 0.25), and finally Penn C at 0.14 (95% CI 0.11, 0.17). Patients with Penn BC were found to be at the highest risk of death, as their early mortality rates were 0.36 (95% CI 0.31, 0.41). Within those populations, the subtype with the highest individual mortality was Penn C at 0.21 (95% CI 0.15, 0.27), followed by Penn B at 0.19 (95% CI 0.15, 0.23) and Penn A at 0.07 (95% CI 0.05, 0.10). Among patients presenting with ATAAD, class A was most frequently observed, followed by classes B, C, and BC. These findings indicate an incremental increase in mortality rates with the progression of Penn classification.

Sex-related differences in Cushing's disease: a systematic review and meta-analysis.

BACKGROUND AND OBJECTIVES: Cushing's disease is a rare endocrine disorder. This review aimed to examine sex-specific differences in Cushing's disease. DESIGN AND SETTINGS: A meta-analysis was performed on published articles discussing the gender impact of Cushing's disease. METHODS: A systematic search was conducted to identify studies from Medline, Embase, CENTRAL and Scopus. Nine studies enrolling 1047 patients diagnosed with Cushing's disease were included in this meta-analysis. RESULTS: Male patients presented at a younger age (MD [mean difference]=-5.43; 95% CI [-5.78, -5.08]; P<.00001) than females. Male patients had a significantly higher prevalence of osteoporosis (RR [risk ratio]=1.75; 95% CI [1.36, 225]; P<.0001) and hypokalemia (RR=1.66; 95% CI [1.27, 2.16]; P=.0002). In addition, males had significantly higher rates of negative magnetic resonance imaging (RR=1.53; 95% CI [1.18, 2.0]; P=.002). No sex difference was observed in the prevalence of diabetes (RR=0.92; 95% CI [0.70, 1.22]); P=.57) and dyslipidemia (RR=1.33; 95% CI [0.88, 2.0]; P=.17). CONCLUSION: Cushing's disease has a worse clinical presentation in males and more diagnostic difficulties compared to females.

Local versus General Anaesthesia for Transcatheter Aortic Valve Implantation (TAVI): A Systematic Review, Meta-Analysis, and Trial Sequential Analysis of Randomised and Propensity-Score Matched Studies.

Transcatheter aortic valve implantation (TAVI) is a common practice for severe aortic stenosis, but the choice between general (GA) and local anesthesia (LA) remains uncertain. We conducted a comprehensive literature review until April 2023, comparing the safety and efficacy of LA versus GA in TAVI procedures. Our findings indicate significant advantages of LA, including lower 30-day mortality rates (RR: 0.69; 95% CI [0.58, 0.82]; p < 0.001), shorter in-hospital stays (mean difference: -0.91 days; 95% CI [-1.63, -0.20]; p = 0.01), reduced bleeding/transfusion incidents (RR: 0.64; 95% CI [0.48, 0.85]; p < 0.01), and fewer respiratory complications (RR: 0.56; 95% CI [0.42, 0.76], p<0.01). Other operative outcomes were comparable. Our findings reinforce prior evidence, presenting a compelling case for LA's safety and efficacy. While patient preferences and clinical nuances must be considered, our study propels the discourse towards a more informed anaesthesia approach for TAVI procedures.

Transcatheter edge-to-edge repair in mitral regurgitation: A comparison of device systems and recommendations for tailored device selection. A systematic review and meta-analysis.

BACKGROUND: Mitral valve transcatheter edge-to-edge repair (M-TEER) is a minimally invasive method for the treatment of mitral regurgitation (MR) in patients with prohibitive surgical risks. The traditionally used device, MitraClip, showed both safety and effectiveness in M-TEER. PASCAL is a newer device that has emerged as another feasible option to be used in this procedure. METHODS: We searched for observational studies that compared PASCAL to MitraClip devices in M-TEER. The electronic databases searched for relevant studies were PubMed/MEDLINE, Scopus, and Embase. The primary outcomes were technical success and the grade of MR at follow-up. Secondary outcomes included all-cause mortality, bleeding, device success and reintervention. RESULTS: Technical success (PASCAL: 96.5% vs MitraClip: 97.6%, p = 0.24) and MR ≤ 2 at 30-day follow-up (PASCAL: 89.4vs MitraClip 89.9%, p = 0.51) were comparable between both groups. Both devices showed similar outcomes including all-cause mortality (RR: 0.68 [0.34, 1.38]; P = 0.28), major bleeding (RR: 1.87 [0.68, 5.10]; P = 0.22) and reintervention (RR: 1.02 [0.33, 3.16]; P = 0.97). Device success was more frequent with PASCAL device (PASCAL: 86% vs MitraClip 68.5%; P = 0.44), however, the results did not reach statistical significance. CONCLUSION: Clinical outcomes of PASCAL were comparable to those of MitraClip with no significant difference in safety and effectiveness. The choice between MitraClip and PASCAL devices should be guided by various factors, including mitral valve anatomy, etiology of regurgitation, and device-specific characteristics.

Gender-Based Outcome Discrepancies in Patients Who Underwent Alcohol Septal Ablation or Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy: A Systematic Review and Meta-Analysis.

Clinical evidence and emerging studies suggest that the clinical heterogeneity observed in hypertrophic cardiomyopathy could be because of gender-based differences. We aimed to explore the gender-related differences pertaining to the treatment outcomes after alcohol septal ablation (ASA) and septal myectomy (SM). We searched PUBMED/MEDLINE, EMBASE, and SCOPUS to identify studies that report gender-stratified comparison of outcomes. The primary outcome of interest was short-term (within 30 days) mortality. A total of 15 studies totaling 31,907 patients (47% men and 53% women) were included. Women were found to be significantly older at the time of intervention (ASA: mean difference [MD] 7.55 years; SM: MD 4.41). In the ASA and SM treatment arms, women had a significantly higher risk of short-term all-cause mortality (ASA: risk ratio 0.48, 95% confidence interval 0.32 to 0.71, p = 0.0003; SM: risk ratio 0.63, 95% confidence interval 0.44 to 0.90, p = 0.01), more frequent permanent pacemaker implantation (ASA; p = 0.002, SM: p = 0.05), and longer in-hospital stay (ASA: MD 1.00 days, SM: MD 0.69). Among those who underwent ASA, women had a significantly higher rate of atrioventricular block. In conclusion, regardless of ASA or SM, women consistently presented at an older age and exhibited a higher risk-increased mortality rate, a greater incidence of atrioventricular block, and a higher likelihood of permanent pacemaker requirement-and longer hospital stay among women than men. This strongly emphasizes the need for a gender-specific approach to optimize care and improve treatment outcomes in hypertrophic cardiomyopathy.

Preoperative Imaging Signs of Cerebral Malperfusion in Acute Type A Aortic Dissection: Influence on Outcomes and Prognostic Implications-A 20-Year Experience.

BACKGROUND: Acute type A aortic dissection (ATAAD) continues to be a subject of active research due to its high mortality rates and associated complications. Cerebral malperfusion in ATAAD can have a devastating impact on patients' neurological function and overall quality of life. We aimed to explore the risk profile and prognosis in ATAAD patients presenting with preoperative imaging signs of cerebral malperfusion (PSCM). METHODS: We obtained patient data from our Aortic Dissection Registry, which included 480 consecutive ATAAD cases who underwent surgical repair between 2001 and 2021. Primary endpoint outcomes included the in-hospital and 30-day mortality, postoperative new neurological deficit, mechanical ventilation hours, and intensive care unit (ICU) length of stay. RESULTS: Of the total cohort, 82 patients (17.1%) had PSCM. Both groups had similar distributions in terms of age, sex, and body mass index. The patients in the PSCM group presented with a higher logistic EuroSCORE (47, IQR [31, 64] vs. 24, IQR [15, 39]; p < 0.001) and a higher portion of patients with a previous cardiac surgery (7.3% vs. 2.0%; p = 0.020). Intraoperatively, the bypass, cardioplegia, and aortic cross-clamp times were similar between both groups. However, the patients in the PSCM group received significantly more intraoperative packed red blood cells, fresh frozen plasma, and platelets transfusions (p < 0.05). Following the surgery, the patients who presented with PSCM had markedly longer ventilation hours (108.5 h, IQR [44, 277] vs. 43 h, IQR [16, 158], p < 0.001) and a significantly longer ICU length of stay (7 days, IQR [4, 13] vs. 5 days, IQR [2, 11]; p = 0.013). Additionally, the patients with PSCM had significantly higher rates of postoperative new neurological deficits (35.4% vs. 19.4%; p = 0.002). In the Cox regression analysis, PSCM was associated with significantly poorer long-term survival (hazard ratio (HR) 1.75, 95%CI [1.20-2.53], p = 0.003). Surprisingly, hypertension was shown as a protective factor against long-term mortality (HR: 0.59, 95%CI [0.43-0.82], p = 0.001). CONCLUSION: PSCM in ATAAD patients is linked to worse postoperative outcomes and poorer long-term survival, emphasizing the need for early recognition and tailored management.

Comparative Efficacy of Oral Apixaban and Subcutaneous Low Molecular Weight Heparins in the Treatment of Cancer-Associated Thromboembolism: A Meta-Analysis.

Cancer patients' risk of developing venous thromboembolism (VTE) is four to seven times higher than the general population. Cancer-associated VTE (CA-VTE), is a leading cause of morbidity and mortality in cancer patients. Low Molecular Weight Heparin (LMWH) has historically been the mainstay treatment of CA-VTE; however, complications such as bleeding and recurrent VTE make it challenging to manage these patients. Recent randomized controlled trials (RCTs) have proven that direct oral anticoagulants (DOACs) are as efficacious as LMWHs in treating CA-VTE. We conducted a systematic review and meta-analysis to ascertain the efficacy and safety of LMWH and Apixaban for the treatment of CA-VTE. A systematic review was conducted using Medline, Embase, and Scopus, databases for all cohort studies, case-control studies, and RCTs in English comparing cancer patients undergoing treatment with Apixaban or LMWH to treat CA-VTE from inception-May 2023. The Review Manager program, version 5.4.1, was used for statistical analysis and the Mantel-Haenszel fixed-effects models to calculate the risk ratio (RR) and 95% confidence intervals (CIs) and the inverse variance approach to get the weighted mean difference for the continuous outcomes. Q-test for heterogeneity was used to examine statistical heterogeneity and an I2 statistics value >50% was defined as significant heterogeneity. A total of four studies were included, and the total number of patients was 1,632 across all studies. The Apixaban group was associated with a statistically significant increase in minor bleeding (RR 1.57; 95% CI (1.12, 2.21); p=0.009; I2=0%), but not for major and total bleeding. The Apixaban group showed a statistically significant lower risk of recurrent VTE when compared to the LMWH group (RR: 0.61; 95% CI (0.41, 0.92); p=0.02; I2 = 7%), and there was no statistically significant difference in terms of mortality between the two groups (RR: 0.89; 95% CI (0.73, 1.09); I2=0). Our findings suggest that Apixaban may be a favorable anticoagulant option for managing cancer-associated thromboembolism, as it demonstrated a lower risk of recurrent VTE. The risk of bleeding with DOAC in gastrointestinal cancers warrants further investigation.

Safety and efficacy of P2X3 receptor antagonist for the treatment of refractory or unexplained chronic cough: A systematic review and meta-analysis of 11 randomized controlled trials.

BACKGROUND AND OBJECTIVES: Chronic refractory cough is a challenging condition that requires a thorough evaluation and management approach. P2X3 receptors that are ATP-dependent play an important part in nerve fiber sensitization and pathological pain pathways. We conducted this systematic review and meta-analysis to determine the long-term safety and efficacy of P2X3 receptor antagonist drugs in chronic cough. METHODS: We systematically searched PubMed, Scopus, Web of Science, and Embase to identify all relevant published studies through January 15, 2023 that assessed P2X3 antagonists in chronic cough. The protocol was registered in the PROSPERO database with ID: CRD42023422408. Efficacy outcomes were awake (daytime) cough frequency, night cough frequency, 24-h cough frequency, Cough Severity Diary, and total Leicester Cough Questionnaire score. We used the random-effect model to pool the data using RStudio and CMA software. RESULTS: A total of 11 randomized controlled trials comprising 1350 patients receiving a p2x3 antagonist compared to the placebo group were included in this meta-analysis. A significant decrease in 24-h cough frequency (MD = -4.99, 95% CI [-7.15 to -2.82], P < 0.01), awake (daytime) cough frequency (MD = -7.18, 95% CI [-9.98 to 4.37], P < 0.01), and total Leicester Cough Questionnaire score (MD = 1.74, 95% CI [1.02 to 2.46], P < 0.01) exhibited between the P2X3 antagonist and placebo groups. The frequency of the night cough showed an insignificant difference between the two groups. According to the safety, drug-related adverse events, dysgeusia, hypogeusia, and ageusia significantly increased between the P2X3 antagonist and placebo groups. CONCLUSION: P2X3 receptor antagonists are promising drugs for treating chronic cough by significantly reducing the frequency, severity, and quality. Some potential side effects may include drug-related adverse events such as hypogeusia, ageusia, and dysgeusia.

Early Versus Delayed Thoracic Endovascular Aortic Repair for Blunt Traumatic Aortic Injury: A Systematic Review and Meta-Analysis.

Blunt aortic injury is the second most prevalent cause of patient fatalities post-trauma, closely following head injuries as the leading cause. In recent years, thoracic endovascular aortic repair (TEVAR) has evidently improved survival rates and reduced complications in patients suffering from blunt traumatic aortic injury (BTAI) in comparison to open surgery and non-operative management. It is difficult to characterize the appropriate criteria for the timing of TEVAR, whether early or delayed for BTAI, considering the discrepancies related to timing. Electronic databases, including PubMed, Scopus, the Cochrane Central Register of Controlled Trials (CENTRAL), and Embase, were searched through April 2023. The primary outcomes were short-term mortality and hospital and intensive care unit (ICU) stays. Time to TEVAR, acute respiratory distress syndrome, sepsis, deep vein thrombosis, delayed stroke, and renal failure were also evaluated. We included a total of seven studies, comprising 4177 patients who met the inclusion criteria. Short-term mortality was significantly higher in the early TEVAR group (RR: 1.86; 95% confidence interval (CI); (1.26-2.74); p<0.001; I2=33%). In contrast, the ICU length of stay was significantly shorter in the early group (mean difference: -2.82 days; 95% CI; (-4.09 - -1.56); p<0.0001; I2=55%). There was no significant difference between both groups in the presenting profile or postoperative complications. Patients undergoing delayed TEVAR had markedly lower mortality rates but a longer ICU stay. The need for future studies with more robust designs is imperative to investigate the factors influencing the timing of repair and the associated outcomes.

Elective Thoracic Aortic Aneurysm Surgery: A Tertiary Center Experience.

Background A thoracic aortic aneurysm (TAA) is a diseased expansion of the thoracic aorta. There is morbidity associated with a dilated aorta, as well as significant mortality. Open thoracic surgery is the fundamental management for proximal lesions, offering definitive treatment with excellent results. This study aimed to summarize preoperative data and operative outcomes of patients who underwent TAA repair at our institution. Methods Data were retrospectively collected from 234 patients that underwent elective open thoracic surgery at University Hospital Southampton for TAA disease, between 2015 and 2019. Demographics, clinical factors, surgical details, as well as outcome measures, were gathered. Results There were 166 males and 68 females, with an overall mean age of 66 years. The breakdown of operations comprised 105 aortic roots, 171 ascending aorta, 20 aortic arch, and 12 descending aorta cases. The mean follow-up was 370 days. 30-day mortality was 5.13%. Mortality was associated with female gender, aortic root surgery, and prosthetic valves. Mean aortic diameters at the time of surgery for the non-genetic aortopathy and genetic aortopathy groups were respectively 4.93cm and 4.63cm in the aortic root, 5.56cm and 4.88cm in the ascending aorta, 5.08cm and 3.87cm in the aortic arch, and 6.63cm and 5.50cm in the descending aorta. Conclusion Several factors are associated with complications and morbidity, which should be considered when discussing the risks of intervention with patients. There were no neuroprotective strategies that altered post-operative neurological function. Current practice in our unit fits in with current international guidance.

Impact of patient demographics and intraoperative characteristics on abdominal aortic aneurysm sac following endovascular repair.

BACKGROUND: Endovascular aortic repair (EVAR) has become the preferred treatment for abdominal aortic aneurysm (AAA). Its main aim is to seal the perfusion of the aneurysmal sac and, thus, induce sac regression and subsequent aortic remodelling. Aneurysmal sac regression has been linked to the short- and long-term clinical outcomes post-EVAR. It has also been shown to be influenced by endograft device choice, with several of these available commercially. This review summarises and discusses current evidence on the influence of pre- and intraoperative factors on sac regression. Additionally, this review aims to highlight the device-specific variations in sac regression to provide an overall holistic approach to treating AAAs with EVAR. METHODS: A comprehensive literature search was conducted using multiple electronic databases to identify and extract relevant data. RESULTS: Female sex, >70 mm original sac diameters, higher pre-procedural fibrinogen levels, smoking and low intra-aneurysmal pressure were found to positively impact sac regression. Whereas renal impairment, ischemic heart disease, high intra-aneurysmal pressure and aneurysm neck thrombus negatively influenced sac regression. Patent lumbar arteries, age, statins and hypercholesterolaemia displayed conflicting evidence regarding sac regression. Regarding the EVAR endografts compared, newer generation devices such as the Anaconda mainly showed the most optimal results. CONCLUSION: Sac regression following EVAR in AAA is an important prognostic factor for morbidity and mortality. Nevertheless, several pre- and intraoperative factors can have an influence on sac regression. Therefore, it is necessary to take them into account when assessing AAA patients for EVAR to optimise outcomes. The choice of EVAR stent-graft can also affect sac regression, with evidence suggesting that the Fenestrated Anaconda is associated with the most favourable results.

Correlative effect between sac regression and clinical outcomes following endovascular repair in abdominal aortic aneurysm: fact or myth?

INTRODUCTION: Endovascular aneurysm repair (EVAR) has rapidly become the preferred management of abdominal aortic aneurysm (AAA). Sac regression status post-EVAR has been linked to clinical outcomes as well as the choice of EVAR device. The aim of this narrative review is to investigate the relationship between sac regression and clinical outcomes post-EVAR in AAA. Another aim is to compare sac regression achieved with the main EVAR devices. AREAS COVERED: We carried out a comprehensive literature search on multiple electronic databases. Sac regression was usually defined as a decrease in the sac diameter (>10 mm) over follow-up. This revealed that individuals who had sac regression post-EVAR had significantly lower mortality, and higher event-free survival rates. Further, lower rates of endoleak and reintervention were observed in patients with regressing aneurysm sacs. Sac regression patients also had significantly lower odds of rupture compared to counterparts with stable or expanded sacs. The choice of EVAR device was also shown to impact regression, with the Fenestrated Anaconda showing favorable results. EXPERT OPINION: Sac regression post-EVAR in AAA is an important prognostic factor as it translates to improved mortality and morbidity. Therefore, this relationship must be seriously taken into consideration during follow-up.

Endovascular Solutions for Abdominal Aortic Aneurysms: Fenestrated, Branched and Custom-Made Devices.

BACKGROUND: Abdominal aortic aneurysm (AAA) has a prevalence of 4.8%. AAA rupture is associated with significant mortality, thus surgical intervention is generally required once the aneurysm diameter exceeds 5.5 cm. Endovascular aneurysm repair (EVAR) is the predominant repair modality for AAA. However, in patients with complex aortic anatomy, fenestrated or branched EVAR is a superior repair option vs standard EVAR. Fenestrated and branched endoprostheses can be off-the-shelf or custom-made, which offers a more individualised approach. AIM: To summarise and evaluate the clinical outcomes achieved by fenestrated EVAR (FEVAR) and branched EVAR (BEVAR), and to explore the role of custom-made endoprostheses in contemporary AAA management. METHODS: A literature search using Ovid Medline and Google Scholar was conducted to identify literature pertaining to the use and outcomes of fenestrated, branched, fenestrated-branched and custom-made endoprostheses for AAA repair. RESULTS: FEVAR is an effective repair modality for patients with AAA that offers similar early survival, improved early morbidity but higher rates of reintervention in comparison to open surgical repair (OSR). Compared with standard EVAR, FEVAR is associated with similar in-hospital mortality yet higher rates of morbidity, especially regarding renal outcomes. BEVAR outcomes are rarely reported exclusively in the context of AAA repair. When reported, BEVAR is an acceptable alternative to EVAR in the treatment of complex aortic aneurysms and has similar reported complication issues to FEVAR. Custom-made grafts are a good alternative treatment option for complex aneurysms where hostile aneurysm anatomy precludes the use of conventional EVAR and sufficient time is available for the manufacturing of such devices. CONCLUSION: FEVAR offers a very effective treatment for patients with complex aortic anatomy and has been well-characterised over the past decade. RCTs and longer-term studies are desirable for unbiased comparison of non-standard EVAR modalities.

Chronic Cavitary Pulmonary Histoplasmosis in an Immunocompetent Patient.

Histoplasma capsulatum is a fungal organism that causes systemic histoplasmosis. It is commonly asymptomatic in healthy immunocompetent individuals. The clinical symptoms of chronic cavitary histoplasmosis are typically seen in the immunodeficient population, particularly in smokers with pre-existing structural lung disease. We report a case of chronic cavitary histoplasmosis in an immunocompetent patient from an endemic area without pre-existing structural lung pathology. She presented complaining of right hypochondrial pain and had no history of respiratory symptoms nor history suggestive of immunosuppression, tuberculosis, or recent travel. CT scan revealed a cavitary lung lesion and a hilar mediastinal mass. Biopsies obtained by bronchoscopy revealed signs of necrosis, granulomas, and the presence of fungal organisms consistent with histoplasmosis. Histoplasma antibodies by complement fixation for yeast antibodies test were positive establishing the diagnosis of chronic cavitary pulmonary histoplasmosis (CCPH). She was then started on itraconazole with good tolerance. On follow-up three months later, a chest CT done along with measurement of inflammatory markers and liver enzymes demonstrated complete clinical recovery. This case emphasizes the importance of expanding our current understanding of the clinical presentation and manifestations of histoplasmosis beyond the conventional assumption that severe disease only affects immunocompromised individuals.

Rituximab-Induced Immune Dysregulation Leading to Organizing Pneumonia, Bronchiectasis, and Pulmonary Fibrosis.

We present a case of rituximab-induced organizing pneumonia (OP) along with bronchiectasis and pulmonary fibrosis, in a patient with a history of granulomatosis with polyangiitis (GPA), on long-term maintenance therapy with rituximab. T-cell dysregulation and B-cell depletion associated with the chronic use of rituximab often lead to a profound immunosuppressed state with hypogammaglobulinemia and unbalanced T-cell response. This acquired immunodeficient state with severe immune dysregulation predisposed this patient to recurrent pulmonary infection and ultimately led to bronchiectasis and pulmonary fibrosis.

Transseptal vs Transapical Transcatheter Mitral Valve-in-Valve and Valve-in-Ring Implantation: A Systematic Review and Meta-Analysis.

Transcatheter mitral valve replacement has become a useful alternative for patients with failed mitral prosthesis or annuloplasty rings who are deemed high risk for redo surgery. We aimed to compare the clinical outcomes following transseptal (TS) and transapical (TA) approaches in transcatheter mitral valve-in-valve and valve-in-ring implantation (TMViV/R). Electronic databases PubMed, MEDLINE, and Embase were searched through November 2022. Both clinical trials and observational studies comparing patients undergoing TS and TA TMViV/R were eligible for inclusion. Primary outcomes were 30-day and 1-year mortality. Postoperative stroke, left ventricle outlet tract (LVOT) obstruction, mitral valve pressure gradient (MVPG), bleeding, and length of hospital stay were also evaluated. Seven observational studies were included comparing patients undergoing TS (n = 1875) and TA (n = 1120) TMViV/R. The TS group had significantly lower 30-day mortality (OR: 0.66; 95% confidence interval [CI] [0.47, 0.94]; P = 0.02, I²â€¯= 0%) and lower one-year mortality risk group (HR: 0.79; 95% CI [0.63, 0.99]; P = 0.04, I²â€¯= 0%) compared to the TA group. The TS group had consistent shorter in-hospital stay (MD = -3.79; 95% CI [-5.23, -2.34] days; P < 0.0001, I²â€¯= 75%). Postoperative stroke, bleeding and LVOT obstruction tended to be lower in the TS but the results did not reach statistical significance. Postoperative MVPG was similar between both groups. The TS approach has lower early mortality, lower 1-year death hazard, shorter in-hospital stay, and a trend toward lower complication rates when compared to TA TMViV/R. Further controlled trials may support the evidence and provide long-term outcomes.

Interleukin-1 Receptor Antagonist Gene Polymorphisms in Egyptian Children and Adolescents With Primary Immune Thrombocytopenia: Association With Disease Susceptibility, Response to Therapy, and Outcome.

Immune thrombocytopenia (ITP) is one of the most common hematologic disorders with poorly predictable clinical course and outcome. We studied the distribution of interleukin 1 receptor antagonist (IL-1Ra) gene polymorphism (intron-2) among children and adolescents with ITP and correlated IL-1Ra gene polymorphism to disease susceptibility, response to therapy, and outcome. Sixty children with ITP (mean age: 9.2±4.5 y) and 100 healthy controls (mean age: 8.83±4.05 y) were enrolled. The frequencies of the allele A2 and genotype A1A2 were significantly higher in patients compared with controls ( P <0.0001, P =0.0008, respectively). Allele A2 conferred 3.1 times increased relative risk for disease development. Allele A2 and genotypes A1A2 and A2A2 were significantly more frequent among remitted patients ( P =0.028 and 0.024, respectively). There was no significant difference between different genotypes and alleles regarding bleeding score ( P >0.05). Patients with polymorphic allele A2 (A1A2/A2A2) showed significantly better response to steroids than those with homozygous wild allele A1 ( P =0.028). IL-1Ra polymorphism might contribute to the susceptibility to ITP in Egyptian children. The presence of A2 polymorphic allele of IL-1Ra gene was found to be associated with better disease outcome and response to steroids than those with homozygous wild allele.

Driving Technology for Thoracic Endovascular Aortic Repair: An International Analysis of Single-versus Double-Branch RELAY Outcomes.

BACKGROUND: The introduction of endovascular aortic arch repair (EAR) represents a paradigm shift in the management of complex arch pathologies usually treated with open surgical repair (OSR). This shift in treatment of aortic arch disease has also been accompanied with a rise in commercial interest in EAR resulting in the development of several endografts. However, the RELAY™ Branched by Terumo Aortic can be considered the prime endograft choice. In this international original study, a retrospective comparative analysis of international prospective outcomes data on the single- and double-branch RELAY stent grafts is provided. METHODS: International data on EAR procedures carried out from January 2019 to January 2022 using the single- and double-branch RELAY endograft configurations were collected prospectively. Follow-up data were collected at 30 days, 6 months, 12 months, and 24 months postoperatively. Retrospective descriptive analysis, logistic regression, and Kaplan-Meier analysis were performed. RESULTS: A total of 125 patients were included, out of which 17 (13.6%) received the single-branch RELAY and 108 the double-branch device. In the single-branch group, no mortalities, strokes, or reinterventions were recoded over 24 months of follow-up. The rates of these outcomes were higher in the other group; however, the differences did not reach significance. Target vessel patency was maintained at 100% throughout the study period with the single-branched RELAY. Overall, our Kaplan-Meier analyses proved that both configurations of RELAY Branched yield excellent short- and mid-term survival and freedom from reintervention. CONCLUSIONS: Our series has clearly demonstrated that EAR using RELAY Branched is a highly efficacious strategy that yields very favorable results which can be considered superior to other EAR devices as well as OSR. Nevertheless, it seems that clinical outcomes with the single-branch RELAY are more optimal than with the double-branch device.