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Pediatric Pulmonology Dec 2022Pulmonary agenesis is a complete absence of the pulmonary parenchyma, airways, and vasculature unilaterally or bilaterally. Although bilateral cases are lethal, the...
INTRODUCTION
Pulmonary agenesis is a complete absence of the pulmonary parenchyma, airways, and vasculature unilaterally or bilaterally. Although bilateral cases are lethal, the outcome of unilateral cases remains not well described. We performed a comprehensive literature review to assess the clinical features of pulmonary agenesis.
METHODS
Four database sources were searched on October 10, 2021 and two cases were included from our institution. Studies related to the clinical impact of comorbidity and intervention on the survival outcome in pulmonary agenesis were included for full-text review.
RESULTS
We identified 259 patients-with right-sided (59%), left-sided (34%), and bilateral agenesis (7%)-among 195 articles and our two cases. Additional anomalies included cardiovascular (40%), skeletal (30%), gastrointestinal (20%), tracheal (20%: all stenoses), and genitourinary (14%) anomalies. Fifty-seven (24%) individuals in unilateral pulmonary agenesis had isolated disease. Outcomes related to survival were not uniformly reported, but the 2-year overall survival rate of unilateral agenesis was 62% and no subsequent death was reported until 13 years of age. The right-sided agenesis was more frequently associated with tracheal stenosis (27% vs. 11%, p = 0.003) than the left-sided disease. A multivariable analysis indicated that tracheal stenosis (hazard ratio [HR]: 2.2, 95% confidence interval [CI]: 1.3-4.1, p = 0.003) and gastrointestinal anomalies (HR: 2.0, 95% CI: 1.1-3.3, p = 0.010) were prognostic factors for mortality.
CONCLUSIONS
The poor prognostic factors were tracheal stenosis, right agenesis, and gastrointestinal anomalies. Treatment for these comorbidities is a key point for improving the survival of unilateral pulmonary agenesis.
Topics: Humans; Tracheal Stenosis; Lung Diseases; Abnormalities, Multiple; Lung
PubMed: 36069476
DOI: 10.1002/ppul.26135 -
Ultrasound in Obstetrics & Gynecology :... Jul 2023A favorable postnatal prognosis in cases of pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS-IVS) is generally equated with the possibility of... (Review)
Review
OBJECTIVE
A favorable postnatal prognosis in cases of pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS-IVS) is generally equated with the possibility of achieving biventricular (BV) repair. Identification of fetuses that will have postnatal univentricular (UV) circulation is key for prenatal counseling, optimization of perinatal care and decision-making regarding fetal therapy. We aimed to evaluate the accuracy of published models for predicting postnatal circulation in PA/CS-IVS using a large internationally derived validation cohort.
METHODS
This was a systematic review of published uni- and multiparametric models for the prediction of postnatal circulation based on echocardiographic findings at between 20 and 28 weeks of gestation. Models were externally validated using data from the International Fetal Cardiac Intervention Registry. Sensitivity, specificity, predictive values, area under the receiver-operating-characteristics curves (AUCs) and proportion of cases with true vs predicted outcome were calculated.
RESULTS
Eleven published studies that reported prognostic parameters of postnatal circulation were identified. Models varied widely in terms of the main outcome (UV (n = 3), non-BV (n = 3), BV (n = 3), right-ventricle-dependent coronary circulation (n = 1) or tricuspid valve size at birth (n = 1)) and in terms of the included predictors (single parameters only (n = 6), multiparametric score (n = 4) or both (n = 1)), and were developed on small sample sizes (range, 15-38). Nine models were validated externally given the availability of the required parameters in the validation cohort. Tricuspid valve diameter Z-score, tricuspid regurgitation, ratios between right and left cardiac structures and the presence of ventriculocoronary connections (VCC) were the most commonly evaluated parameters. Multiparametric models including up to four variables (ratios between right and left structures, right ventricular inflow duration, presence of VCC and tricuspid regurgitation) had the best performance (AUC, 0.80-0.89). Overall, the risk of UV outcome was underestimated and that of BV outcome was overestimated by most models.
CONCLUSIONS
Current prenatal models for the prediction of postnatal outcome in PA/CS-IVS are heterogeneous. Multiparametric models for predicting UV and non-BV circulation perform well in identifying BV patients but have low sensitivity, underestimating the rate of fetuses that will ultimately have UV circulation. Until better discrimination can be achieved, fetal interventions may need to be limited to only those cases in which non-BV postnatal circulation is certain. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Pregnancy; Infant, Newborn; Female; Humans; Pulmonary Atresia; Ventricular Septum; Constriction, Pathologic; Tricuspid Valve Insufficiency; Retrospective Studies
PubMed: 36776132
DOI: 10.1002/uog.26176 -
Trends in Cardiovascular Medicine Apr 2023This systematic review and meta-analysis aim to provide a comprehensive analysis of the literature directly comparing the outcomes of surgical aortic valve replacement... (Review)
Review
This systematic review and meta-analysis aim to provide a comprehensive analysis of the literature directly comparing the outcomes of surgical aortic valve replacement (SAVR) and TAVR in patients with BAV stenosis. Medline, PubMed, and Scopus were systematically searched for articles published between 2000 and 2023, 1862 studies were screened, and 6 retrospective studies met the inclusion criteria. We included 6550 patients in the final analyses: 3,292 and 3,258 in the SAVR and TAVR groups, respectively. Both groups have similar rates of in-hospital mortality (odds ratio (OR) 1.11; 95% CI 0.59-2.10; p = 0.75) and stroke (OR 1.25; 95% CI 0.85-1.86; p = 0.26. Patients who underwent SAVR experienced lower rates of permanent pacemaker implantation (OR 0.54; 95% CI 0.35-0.83; p = 0.005) and paravalvular leak (OR 0.47; 95% CI 0.26-0.86; p = 0.02). On the other hand, patients who underwent TAVR displayed lower rates of acute kidney injury (OR 1.81; 95% CI 1.15-2.84; p = 0.010), major bleeding (OR 3.76; 95% CI 2.18-6.49; p < 0.00001), and pulmonary complications (OR 7.68; 95% CI 1.21-48.84; p = 0.03). Despite the early mortality data suggesting that TAVR may be a reasonable strategy for patients with bicuspid AS with low to intermediate surgical risk, the increased risk of PPI and PVL is concerning. A prospective, randomized, controlled trial reporting long-term outcomes with pre-defined subgroup analyses based on BAV morphology is paramount. In the interim, caution should be exercised in the widespread adoption of TAVR in lower surgical-risk patients.
PubMed: 37121526
DOI: 10.1016/j.tcm.2023.04.004 -
Cardiovascular Diagnosis and Therapy Oct 2019Fibrosing mediastinitis (FM) is a very rare disease, often caused by histoplasmosis capsulatum, tuberculosis, sarcoidosis, autoimmunity and other diseases, such as IgG... (Review)
Review
Fibrosing mediastinitis (FM) is a very rare disease, often caused by histoplasmosis capsulatum, tuberculosis, sarcoidosis, autoimmunity and other diseases, such as IgG 4-related diseases. Fibrous structures in the mediastinum compress the pulmonary artery, pulmonary vein, superior vena cava, esophagus, trachea and cardiac vessels, leading to clinical symptoms. Drug therapeutic modality for pulmonary vein stenosis (PVS) caused by FM is palliative in essence and with limited efficacy, whereas surgical treatment causes high mortality. In recent years, catheter-based treatment to FM-caused PVS has emerged as a promising therapeutic modality, however, the safety and effectiveness of this modality remain unclear. Therefore, a systematic review on the safety and efficacy of the catheter-based treatment for PVS caused by FM was performed, in the hope to shed lights on the alternative therapeutic strategy to this fatal disease.
PubMed: 31737523
DOI: 10.21037/cdt.2019.09.14 -
Catheterization and Cardiovascular... Mar 2016The present study was performed to investigate the relationship between chronic obstructive pulmonary disease (COPD) and transcatheter aortic valve implantation (TAVI). (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The present study was performed to investigate the relationship between chronic obstructive pulmonary disease (COPD) and transcatheter aortic valve implantation (TAVI).
BACKGROUND
Controversies regarding the relationship between COPD and TAVI have intensified.
METHODS
A literature review of the PubMed online database was performed, and articles published between January 1, 2002 and March 20, 2015 were analyzed. Random-effect and fixed-effect models were used, depending on the between-study heterogeneity.
RESULTS
A total of 28 studies, involving 51,530 patients, were identified in our review. The burden of COPD ranged from 12.5% to 43.4%, and COPD negatively impacted both short-term and long-term all-cause survival (30 days: odds ratio [OR], 1.43, 95% CI, 1.14-1.79; >2 years: hazard ratio [HR], 1.34, 95% CI, 1.12-1.61). COPD was also associated with increased short-term and mid-term cardiac-cause mortality (30 days: OR, 1.29, 95% CI, 1.02-1.64; 1 year: HR: 1.09, 1.02-1.17). Moreover, COPD (OR, 1.97, 95% CI, 1.29-3.0) predicted post-TAVI acute kidney disease. Importantly, chronic kidney disease (CKD) (HR, 1.2, 95% CI, 1.1-1.32) and the distance of the 6 minute walk test (6MWT) (HR, 1.16, 1.06-1.27) predicted TAVI futility in patients with COPD.
CONCLUSION
COPD is common among patients undergoing TAVI, and COPD impacts both short- and long-term survival. COPD patients, who had a lower BMI, shorter distance of 6MWT and CKD, were at higher risk for TAVI futility.
Topics: Acute Kidney Injury; Aged, 80 and over; Aortic Valve Stenosis; Body Mass Index; Cardiac Catheterization; Chi-Square Distribution; Comorbidity; Exercise Test; Exercise Tolerance; Female; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Logistic Models; Lung; Male; Multivariate Analysis; Odds Ratio; Prevalence; Pulmonary Disease, Chronic Obstructive; Renal Insufficiency, Chronic; Risk Factors; Severity of Illness Index; Time Factors; Treatment Outcome; Walking
PubMed: 26856312
DOI: 10.1002/ccd.26443 -
Current Problems in Cardiology Jan 2023Transcutaneous aortic valve implantation (TAVI) has transformed the management of aortic stenosis (AS) and is increasingly being used for patients with symptomatic,... (Meta-Analysis)
Meta-Analysis Review
Transcutaneous aortic valve implantation (TAVI) has transformed the management of aortic stenosis (AS) and is increasingly being used for patients with symptomatic, severe aortic stenosis who are ineligible or at high risk for conventional cardiac surgery. PUBMED, Google Scholar, and SCOPUS databases were searched to identify studies reporting heart failure hospitalization after TAVI. Major factors evaluated for HF hospitalization were age, comorbidities such as hypertension, atrial fibrillation (AF), chronic pulmonary disease including COPD, chronic kidney disease, baseline LVEF before the procedure, NYHA symptom class, and society of thoracic surgeons (STS) score. Hazard ratio (HR) with a 95% confidence interval were computed using random-effects models. A total of eight studies were included comprising 77,745 patients who underwent TAVI for severe aortic stenosis. The presence of diabetes mellitus (HR: 1.39, 95% CI [1.17, 1.66], chronic kidney disease (CKD) (HR: 1.39, 95% CI [1.31, 1.48], atrial fibrillation (HR: 1.69, 95% CI [1.42, 2.01], chronic pulmonary disease (HR: 1.33, 95% CI [1.12, 1.58], and a high STS score (HR: 1.07, 95% CI [1.03, 1.11] were positive predictors of 1-year HF hospitalization after TAVI. Patients with diabetes mellitus, AF, CKD, chronic pulmonary disease, and a high STS score are at an increased risk of heart failure hospitalization at 1-year of TAVI, whereas increasing age, hypertension, LVEF <50%, and NYHA class III/IV symptoms did not predict HF hospitalization. Careful follow-up after TAVI in high-risk patients, with closer surveillance for HF particularly, is key to preventing HF hospitalizations and death.
Topics: Humans; Aortic Valve; Transcatheter Aortic Valve Replacement; Atrial Fibrillation; Patient Readmission; Risk Factors; Aortic Valve Stenosis; Heart Failure; Lung Diseases; Renal Insufficiency, Chronic; Hypertension; Treatment Outcome
PubMed: 36191693
DOI: 10.1016/j.cpcardiol.2022.101428 -
Circulation. Cardiovascular... Oct 2015Endovenous recanalization of iliofemoral stenosis or occlusion with angioplasty and stent placement has been increasingly used to maintain long-term venous patency in... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Endovenous recanalization of iliofemoral stenosis or occlusion with angioplasty and stent placement has been increasingly used to maintain long-term venous patency in patients with iliofemoral venous outflow obstruction. The purpose of this systematic review and meta-analysis was to determine safety and effectiveness of venous stent placement in patients with iliofemoral venous outflow obstruction.
METHODS AND RESULTS
We searched MEDLINE and EMBASE for studies evaluating safety or effectiveness of stent placement in patients with iliofemoral venous outflow obstruction. Data were extracted by disease pathogenesis: nonthrombotic, acute thrombotic, or chronic post-thrombotic. Main outcomes included technical success, periprocedural complications, symptom relief at final follow-up, and primary/secondary patency through 5 years. A total of 37 studies reporting 45 treatment effects (nonthrombotic, 8; acute thrombotic, 19; and chronic post-thrombotic, 18) from 2869 patients (nonthrombotic, 1122; acute thrombotic, 629; and chronic post-thrombotic, 1118) were included. Technical success rates were comparable among groups, ranging from 94% to 96%. Complication rates ranged from 0.3% to 1.1% among groups for major bleeding, from 0.2% to 0.9% for pulmonary embolism, from 0.1% to 0.7% for periprocedural mortality, and from 1.0% to 6.8% for early thrombosis. Patient symptom relief data were reported inconsistently. At 1 year, primary and secondary patency were 96% and 99% for nonthrombotic, 87% and 89% for acute thrombotic, and 79% and 94% for chronic post-thrombotic.
CONCLUSIONS
Stent placement for iliofemoral venous outflow obstruction results in high technical success and acceptable complication rates regardless of cause of obstruction.
Topics: Animals; Blood Vessel Prosthesis Implantation; Femoral Vein; Humans; Iliac Vein; Postoperative Complications; Stents; Survival Analysis; Treatment Outcome; Vascular Patency; Venous Thrombosis
PubMed: 26438686
DOI: 10.1161/CIRCINTERVENTIONS.115.002772 -
World Journal of Cardiology Feb 2023Pulmonary vein stenosis (PVS) is an uncommon but known cause of morbidity and mortality in adults and children and can be managed with percutaneous re-vascularization...
BACKGROUND
Pulmonary vein stenosis (PVS) is an uncommon but known cause of morbidity and mortality in adults and children and can be managed with percutaneous re-vascularization strategies of pulmonary vein balloon angioplasty (PBA) or pulmonary vein stent implantation (PSI).
AIM
To study the safety and efficacy outcomes of PBA PSI in all patient categories with PVS.
METHODS
We performed a literature search of all studies comparing outcomes of patients evaluated by PBA PSI for PVS. We selected all published studies comparing PBA PSI for PVS with reported outcomes of restenosis and procedure-related complications in all patient categories. In adults, PVS following atrial fibrillation ablation and in children PVS related to congenital etiology or post-procedural PVS following total or partial anomalous pulmonary venous return repair were included. The patient-centered outcomes were risk of restenosis requiring re-intervention and procedural-related complications. The meta-analysis was performed by computing odds ratios (ORs) using the random effects model based on underlying statistical heterogeneity.
RESULTS
Eight observational studies treating 768 severe PVS in 487 patients met our inclusion criteria. The age range of patients was 6 months to 70 years and 67% were males. The primary outcome of the re-stenosis requiring re-intervention occurred in 196 of 325 veins in the PBA group and 111 of 443 veins in the PSI group. Compared to PSI, PBA was associated with a significantly increased risk of re-stenosis (OR 2.91, 95%CI: 1.15-7.37, = 0.025, = 79.2%). Secondary outcomes of the procedure-related complications occurred in 7 of 122 patients in the PBA group and 6 of 69 in the PSI group. There were no statistically significant differences in the safety outcomes between the two groups (OR: 0.94, 95%CI: 0.23-3.76, = 0.929), = 0.0%).
CONCLUSION
Across all patient categories with PVS, PSI is associated with reduced risk of re-intervention and is as safe as PBA and should be considered first-line therapy for PVS.
PubMed: 36911751
DOI: 10.4330/wjc.v15.i2.64 -
Annals of Palliative Medicine Oct 2021This study analyzes and compares the efficacy of using catheter ablation (CA) and traditional drug treatments for atrial fibrillation (AF). Through a systematic review... (Meta-Analysis)
Meta-Analysis
BACKGROUND
This study analyzes and compares the efficacy of using catheter ablation (CA) and traditional drug treatments for atrial fibrillation (AF). Through a systematic review and meta-analysis, it seeks to provide a theoretical basis for using clinical CA for patients with AF.
METHODS
We searched through articles detailing randomly controlled trials (RCTs) that assessed the surgical effect of CA on the treatment of AF. These articles were published before January 31, 2000 in various English databases, including PubMed, Embase, Medline, Ovid, Springer, and Web of Sciences. The Cochrane Handbook for Systematic Reviews of Intervention 5.0.2 was adopted for the bias risk assessment, and Review Manager 5.3 software was used for the meta-analysis of the articles.
RESULTS
A total of 2,098 patients drawn from 13 articles were included in the study. For patients in the experimental group (Exp. group), the meta-analysis showed an increase in the effects of clinical treatment [mean deviation (MD) =3.91; 95% confidence interval (CI), 3.15-4.85; Z=12.36; P<0.00001], an improvement in daily life function (MD =1.45; 95% CI, 1.03-1.87; Z=6.82; P<0.00001), a decrease in body weakness (MD =-2.84; 95% CI, -3.24 to -2.45; Z=14.16; P <0.00001), and an increase in quality of life score (MD =14.15; 95% CI, 7.24-21.05; Z=4.01; P<0.0001). The Exp. group also experienced a reduction in postoperative pain level (MD =-2.5; 95% CI, -3.11 to -1.89; Z=8.04; P<0.00001), reoccurrence of symptomatic AF (OR =0.27; 95% CI, 0.11-0.67; Z=2.82; P=0.005), rehospitalization (MD =0.15; 95% CI, 0.07-0.31; Z=5.11; P<0.00001), other arrhythmia (MD =0.33; 95% CI, 0.18-0.6; Z=3.62; P=0.0003), and pulmonary vein stenosis (PVS) (MD =0.32; 95% CI, 0.14-0.72; Z=2.74; P=0.006). However, in contrast to patients in the control group (Ctrl group), the 'bleeding' mentioned above showed no statistical difference.
DISCUSSION
CA has a good postoperative clinical effect on AF patients, reducing incidences of pain, adverse reactions, and rehospitalization. For this reason, CA is a suitable treatment for AF patients who do not effectively respond to drug therapy.
Topics: Atrial Fibrillation; Catheter Ablation; Humans; Risk Assessment; Treatment Outcome
PubMed: 34763501
DOI: 10.21037/apm-21-2313 -
Cureus Jan 2024Chronic thromboembolic pulmonary hypertension (CTEPH) is underdiagnosed and has recently surfaced as one of the leading triggers of severe pulmonary hypertension. This... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is underdiagnosed and has recently surfaced as one of the leading triggers of severe pulmonary hypertension. This disease process is described by structural changes of pulmonary arteries such as fibrous stenosis, complete obliteration, or the presence of a resistant intraluminal thrombus, resulting in increased pulmonary resistance and eventually progressing to right-sided heart failure. Hence, this study aims to describe the current treatments for CTEPH and their efficacy in hemodynamic improvement and prevention of recurring thromboembolic episodes in patients. This systematic review promptly follows the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. On February 13, 2022, our team searched through the following databases: PubMed, ProQuest, and ScienceDirect. The following keywords were used across all databases: CTEPH AND Pulmonary Endarterectomy (PEA), CTEPH AND Balloon Pulmonary Angioplasty (BPA), and CTEPH AND Medical Therapy OR Anticoagulation therapy. Twenty-nine thousand eighty-nine articles on current management techniques (PEA, Balloon angioplasty, anticoagulants) were selected, analyzed, and reviewed with each other. We found 19 articles concerning PEA, 15 concerning BPA, and six regarding anticoagulants. Most papers showed high success rates and promising evidence of PEA and anticoagulants as a post-operative regimen. BPA was the least preferred but is still reputable in patients unfit for invasive techniques. CTEPH is a condition presenting with either fibrous stenosis, complete obliteration of the artery, or a clogged thrombus. Recent studies have shown three techniques that physicians have used to treat CTEPH: balloon-pulmonary angioplasty, PEA, and medical management with anticoagulants. PEA followed by anticoagulants is preferred to balloon pulmonary angioplasties. CTEPH is an ongoing topic in research; as it continues to be researched, we hope to see more management techniques available.
PubMed: 38435894
DOI: 10.7759/cureus.53336