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Diagnostics (Basel, Switzerland) Oct 2023Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left... (Review)
Review
Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left ventricle. The aim of our paper is to describe the morphological and imaging features of DOLV and to assess the prevalence of the associated malformations and their surgical outcomes. METHODS From 2011 to 2022, we retrospectively reviewed the electronic case records of patients diagnosed with DOLV at the Bambino Gesu Children's Hospital. A systematic search was developed in MEDLINE, Web of Science, and EMBASE databases to identify reports assessing the morphology and outcomes of DOLV between 1975 and 2023. RESULTS: Over a median follow-up of 9.9 years (IQR 7.8-11.7 y), four cases of DOLV were identified at our institution. Two patients were diagnosed with (S,D,D) DOLV subaortic VSD and pulmonary stenosis (PS): one patient had (S,D,D) DOLV with doubly committed VSD and hypoplastic right ventricle, and another patient had (S,D,L) DOLV with subaortic VSD and PS (malposition type). Pulmonary stenosis was the most commonly associated lesion (75%). LITERATURE REVIEW: After systematic evaluation, a total of 12 reports fulfilled the eligibility criteria and were included in our analysis. PS or right ventricular outflow tract obstruction was the most commonly associated lesion (69%, 95% CI 62-76%). The most common locations of VSD were subaortic (pooled prevalence: 75%, 95% CI 68-81), subpulmonary (15%, 95% CI 10-21), and doubly committed (7%, 95% CI 4-12). The position of the great arteries showed that d-transposition of the aorta was present in 128 cases (59% 95% CI 42-74), and l-transposition was present in 77 cases (35%, 95% CI 29-43).
PubMed: 37891996
DOI: 10.3390/diagnostics13203175 -
European Journal of Vascular and... Feb 2023Inferior vena cava (IVC) stenting may provide benefit to patients with symptomatic obstruction; however, there are no devices currently licensed for use in the IVC and... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Inferior vena cava (IVC) stenting may provide benefit to patients with symptomatic obstruction; however, there are no devices currently licensed for use in the IVC and systematic reviews on the topic are lacking. The aim of this study was to carry out a systematic review of the literature and meta-analysis to investigate the safety and efficacy of IVC stenting in all adult patient groups.
DATA SOURCES
The Medline and Embase databases were searched for studies reporting outcomes for safety and effectiveness of IVC stenting for any indication in series of 10 or more patients.
REVIEW METHODS
A systematic review of the literature was carried out according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
RESULTS
Thirty-three studies were included describing 1 575 patients. Indications for stenting were malignant IVC syndrome (229 patients), thrombotic disease (807 patients), Budd-Chiari syndrome (501 patients), and IVC stenosis post liver transplantation (47 patients). The male:female ratio was 2:1 and the median age ranged from 30 to 61 years. The studies included were not suitable for formal meta-analysis as 30/33 were single centre retrospective studies with no control groups and there was considerable inconsistency in outcome reporting. There was significant risk of bias in 94% of studies. Median reported technical success was 100% (range 78 - 100%), primary patency was 75% (38 - 98%), and secondary patency was 91.5% (77 - 100%). Major complications were pulmonary embolism (three cases), stent migration (12 cases), and major bleeding (15 cases), and there were three deaths in the immediate post-operative period. Most studies reported improvement in clinical symptoms but formal reporting tools were not used consistently.
CONCLUSION
The evidence base for IVC stenting consists of predominantly single centre, retrospective, observational studies that have a high risk of bias. Nonetheless the procedure appears safe with few major adverse events, and studies that reported clinical outcomes demonstrate improvement in symptoms and quality of life. Randomised controlled trials and prospective registry based studies with larger patient numbers and standardised outcome are required to improve the evidence base for this procedure.
Topics: Adult; Humans; Male; Female; Middle Aged; Vena Cava, Inferior; Retrospective Studies; Quality of Life; Treatment Outcome; Budd-Chiari Syndrome; Stents
PubMed: 36334902
DOI: 10.1016/j.ejvs.2022.11.006 -
Brazilian Journal of Cardiovascular... May 2023Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal... (Review)
Review
INTRODUCTION
Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome.
METHODS
Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal.
RESULTS
Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality.
CONCLUSION
The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.
Topics: Humans; Heart Defects, Congenital; Pulmonary Atresia; Aortic Valve Stenosis; Pulmonary Valve Stenosis; Risk Assessment
PubMed: 36592074
DOI: 10.21470/1678-9741-2022-0273 -
Pediatric Cardiology Oct 2020We performed this meta-analysis to assess the safety and efficacy of tracheoplasty for patients with pulmonary artery sling (PAS) and tracheal stenosis. Published... (Meta-Analysis)
Meta-Analysis
We performed this meta-analysis to assess the safety and efficacy of tracheoplasty for patients with pulmonary artery sling (PAS) and tracheal stenosis. Published studies that included surgical treatment of PAS and tracheal stenosis with and without tracheoplasty were identified by searching the PubMed, EMBASE, and Cochrane Library databases until May 2020. The outcomes assessed included postoperative ventilation time, early and late mortality, and follow-up respiratory symptoms. The mean difference (MD)/risk ratio (RR) with 95% confidence intervals (CI) was estimated with a random-effects/fixed-effects model. Subgroup analysis was performed stratified by percentage of patients with tracheal rings. A total of eight studies comprising 219 patients with PAS accompanied by tracheal stenosis were included. The pooled estimates of postoperative ventilation time (MD 17.68, 95% CI 6.38 to 28.98, p < 0.01) and early mortality (RR 3.93, 95% CI 1.55 to 9.95, p < 0.01) favored the repair-only group. Late mortality (RR 1.33, 95% CI 0.48 to 3.68, p = 0.58) and respiratory symptoms (RR 1.51, 95% CI 0.50 to 4.57, p = 0.47) at follow-up showed no significant differences between the groups with repair-only and repair with tracheoplasty. The same results were found in subgroup analyses. For the surgical treatment of PAS with tracheal stenosis, repair without tracheoplasty appears to result in shorter postoperative ventilation time and lower early mortality, with no increase in late mortality or respiratory symptoms at follow-up, compared with concomitant tracheoplasty.
Topics: Child; Child, Preschool; Female; Heart Defects, Congenital; Humans; Infant; Male; Odds Ratio; Pulmonary Artery; Plastic Surgery Procedures; Respiration, Artificial; Trachea; Tracheal Stenosis; Treatment Outcome
PubMed: 32494877
DOI: 10.1007/s00246-020-02386-z -
Intractable & Rare Diseases Research Aug 2023Ortner's syndrome (OS), also called cardiovocal syndrome, is a rare condition hallmarked by left recurrent laryngeal nerve palsy due to underlying cardiopulmonary... (Review)
Review
Ortner's syndrome (OS), also called cardiovocal syndrome, is a rare condition hallmarked by left recurrent laryngeal nerve palsy due to underlying cardiopulmonary disease. The purpose of this review is to systemically analyze the existing literature for cases of OS to outline typical presentation, methods of diagnosis, and management of these patients. Case reports, case series, and cohort studies describing OS between 1955 and 2021 were identified. Individual manuscripts were reviewed for clinical features, presentation, and management. A total of 117 patient cases were gathered from 92 published articles. Common symptoms included hoarseness, dyspnea, cough, and dysphagia. The most common associated comorbidity was aortic aneurysm (41%), followed by pulmonary hypertension (35%), mitral stenosis (17%), and hypertension (12%). Among those who were managed surgical intervention, 85.4% reported improvement in their hoarseness. While historically OS was associated with mitral stenosis, in recent decades, aortic aneurysms and dilation of the pulmonary artery from pulmonary hypertension have emerged as primary etiologies of OS. Therefore, OS should be considered in any patient presenting with hoarseness and history of cardiopulmonary disease. Surgical intervention in appropriate candidates resolves OS in most cases.
PubMed: 37662622
DOI: 10.5582/irdr.2023.01047 -
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 -
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