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Journal of Thoracic Disease Sep 2019Extended thymectomy has been considered the goal of surgery for myasthenia gravis (MG) mainly due to the existence of ectopic thymic tissue. Recently, ectopic thymic... (Review)
Review
Extended thymectomy has been considered the goal of surgery for myasthenia gravis (MG) mainly due to the existence of ectopic thymic tissue. Recently, ectopic thymic tissue has attracted increasing attention in patients with MG following thymectomy. However, the specific role of ectopic thymic tissue in patients with MG is still under debate. A systematic search of the literature was performed on PubMed and Medline according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISM) statement. Studies evaluating the rate of ectopic thymic tissue in patients with MG with or without thymoma were included. Extraction was performed for all eligible studies and the rate of ectopic thymic tissue at common locations was calculated. Eighteen out of fifty-nine studies were eligible for inclusion, of which ten studies reported the common locations of ectopic thymic tissue in mediastinal fat. Of these ten studies, the presence of ectopic thymic tissue was investigated in different anatomical locations in 882 patients, of whom, 509 patients (58%) have at least one positive location with the most common ones being anterior mediastinal fat, pericardiophrenic angles, aortopulmonary window, cervical region (pretracheal fat) and lateral to phrenic nerves. On the other hand, nine studies analyzed the influence of the presence of ectopic thymic tissue on the clinical outcomes of MG patients. Of these, six found that the presence of ectopic thymic tissue in MG patients is a significant predictor of poor outcome after thymectomy, however, the other three did not find a significance. Altogether, ectopic thymic tissue is likely to present in more than a half of patients undergoing thymectomy for MG. Besides, MG patients who have ectopic thymic tissue after thymectomy do not seem to have as good outcome as those who have not.
PubMed: 31656680
DOI: 10.21037/jtd.2019.08.109 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Oct 2023This study aims to investigate whether the invasive staging of aortopulmonary window lymph nodes could be omitted in the presence of a suspected isolated metastasis in...
Prognostic significance of mediastinal standardized uptake value on positron emission tomography/computed tomography in patients with left upper lobe non-small cell lung cancer: Is invasive staging of aortopulmonary window lymph nodes necessary?
BACKGROUND
This study aims to investigate whether the invasive staging of aortopulmonary window lymph nodes could be omitted in the presence of a suspected isolated metastasis in the aortopulmonary window lymph node on positron emission tomography/computed tomography.
METHODS
Between January 2010 and January 2016, a total of 67 patients (54 males, 13 females; mean age: 59.9±8.7 years; range, 44 to 76 years) with metastatic left upper lobe tumors to aortopulmonary window lymph nodes were retrospectively analyzed. According to positron emission tomography/computed tomography findings in clinical staging, the patients were classified as positive (+) (n=33) and negative (-) (n=34) groups.
RESULTS
There was a statistically significant difference between the two groups in terms of sex distribution, lymph node diameter on computed tomography, maximum standardized uptake value of aortopulmonary window lymph nodes, and tumor diameter (p<0.001 for all). A trend toward significance was found to be in pT status, LN #6 metastases, and pathological stage between the two groups (p=0.067). The five-year overall survival rate for all patients was 42.4% and there was no significant difference between the groups (p=0.896). The maximum standardized uptake value of the aortopulmonary window lymph nodes was a poor prognostic factor for survival (area under the curve=0.533, 95% confidence interval: 0.407-0.675, p=0.648).
CONCLUSION
Invasive staging of aortopulmonary window lymph nodes can be omitted in patients with isolated suspected metastasis to aortopulmonary window lymph nodes in non-small cell lung cancer of the left upper lobe.
PubMed: 38075993
DOI: 10.5606/tgkdc.dergisi.2023.24627 -
The Journal of Thoracic and... May 2017Berry syndrome is a combination of distal aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, and interrupted... (Comparative Study)
Comparative Study
OBJECTIVES
Berry syndrome is a combination of distal aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, and interrupted aortic arch. We present here our current experience of primary repair of this syndrome with the goal of optimizing treatment for this rare condition.
METHODS
From January 2003 through December 2015, 16 infants with Berry syndrome underwent one-stage repair at Shanghai Children's Medical Center. Three different surgical correction techniques were used to repair the APW and aortic origin of the RPA, including intra-aortic baffle in 5, RPA detachment in 6, and RPA angioplasty with aortic cuff in 5 patients.
RESULTS
The median age at repair was 90.5 days (range, 8-170 days). The interrupted aortic arch morphology was type A in 14 and type B in 2 patients. The APW morphology was type IIa in 4, type IIb in 10, and type III in 2 patients. Hospital death occurred in 2 patients, and death at follow-up occurred in one other patient. Three patients who previously underwent RPA angioplasty with aortic cuff required reoperation for aortic or RPA stenosis. Freedom from reoperation was 84.8%, 75.4%, and 75.4%, respectively at 1, 5, and 10 years after surgery.
CONCLUSIONS
One-stage repair of Berry syndrome has achieved acceptable outcomes. Reoperations mainly are related to aortic or RPA stenosis, and the reoperation rate is higher when RPA arterioplasty is performed with an aortic cuff.
Topics: Abnormalities, Multiple; Angioplasty; Aorta, Thoracic; Aortography; Cardiac Surgical Procedures; China; Computed Tomography Angiography; Female; Heart Defects, Congenital; Hospital Mortality; Humans; Infant; Infant, Newborn; Magnetic Resonance Angiography; Male; Postoperative Complications; Progression-Free Survival; Pulmonary Artery; Reoperation; Retrospective Studies; Risk Factors; Time Factors; Vascular Surgical Procedures
PubMed: 28089641
DOI: 10.1016/j.jtcvs.2016.11.058 -
Pediatric Cardiology Oct 2018A comprehensive understanding of the native pulmonary blood supply is crucial in newborns with pulmonary atresia with ventricular septal defect and aortopulmonary...
Catheter, MRI and CT Imaging in Newborns with Pulmonary Atresia with Ventricular Septal Defect and Aortopulmonary Collaterals: Quantifying the Risks of Radiation Dose and Anaesthetic Time.
A comprehensive understanding of the native pulmonary blood supply is crucial in newborns with pulmonary atresia with ventricular septal defect and aortopulmonary collaterals (PA/VSD/MAPCA). We sought to describe the accuracy in terms of identifying native pulmonary arteries, radiation dose and anaesthetic time associated with multi-modality imaging in these patients, prior to their first therapeutic intervention. Furthermore, we wanted to evaluate the cumulative radiations dose and anaesthetic time over the study period. Patients with PA/VSD/MAPCA diagnosed at < 100 days between 2004 and 2014 were identified. Cumulative radiation dose and anaesthetic times were calculated, with imaging results compared with intraoperative findings. We then calculated the cumulative risks to date for all surviving children. Of 19 eligible patients, 2 had echocardiography only prior to first intervention. The remaining 17 patients underwent 13 MRIs, 4 CT scans and 13 cardiac catheterization procedures. The mean radiation dose was 169 mGy cm (47-461 mGy cm), and mean anaesthetic time was 111 min (33-185 min). 3 children had MRI only with no radiation exposure, and one child had CT only with no anaesthetic. Early cross-sectional imaging allowed for delayed catheterisation, but without significantly reducing radiation burden or anaesthetic time. The maximum cumulative radiation dose was 8022 mGy cm in a 6-year-old patient and 1263 min of anaesthetic at 5 years. There is the potential to generate very high radiation doses and anaesthetic times from diagnostic imaging alone in these patients. As survival continues to improve in many congenital heart defects, the important risks of serial diagnostic imaging must be considered when planning long-term management.
Topics: Adolescent; Anesthesia; Anesthetics; Cardiac Catheterization; Child; Child, Preschool; Collateral Circulation; Echocardiography; Female; Heart Septal Defects; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Multimodal Imaging; Pulmonary Artery; Pulmonary Atresia; Radiation Dosage; Retrospective Studies; Risk Assessment; Tomography, X-Ray Computed; United Kingdom
PubMed: 29744658
DOI: 10.1007/s00246-018-1895-7 -
The Journal of Thoracic and... Oct 2021We compared the risk of mortality and reintervention after common arterial trunk (CAT) repair for different surgical techniques, in particular the reconstruction of the...
OBJECTIVES
We compared the risk of mortality and reintervention after common arterial trunk (CAT) repair for different surgical techniques, in particular the reconstruction of the right ventricle outflow tract with left atrial appendage (LAA) without a monocusp.
METHODS
The study population comprised 125 patients with repaired CAT who were followed-up at our institution between 2000 and 2018. Statistical analysis included Cox proportional hazard models.
RESULTS
Median follow-up was 10.6 years. The 10-year survival rate was 88.2% (95% confidence interval [CI], 80.6-92.4) with the poorest outcome for CAT type IV (64.3%; 95% CI, 36.8-82.3; P < .01). In multivariable analysis, coronary anomalies (hazard ratio [HR], 11.63 [3.84-35.29], P < .001) and CAT with interrupted aortic arch (HR, 6.50 [2.10-20.16], P = .001) were substantial and independent risk factors for mortality. Initial repair with LAA was not associated with an increased risk of mortality (HR, 0.37 [0.11-1.24], P = .11). The median age at reintervention was 3.6 years [7.3 days-13.1 years]. At 10 years, freedom from reintervention was greater in the group with LAA repair compared with the valved conduit group, 73.3% (95% CI, 41.3-89.4) versus 17.2% (95% CI, 9.2-27.4) (P < .001), respectively. Using a valved conduit for repair (HR, 4.79 [2.45-9.39], P < .001), truncal valve insufficiency (HR, 2.92 [1.62-5.26], P < .001) and DiGeorge syndrome (HR, 2.01 [1.15-3.51], P = .01) were independent and clinically important risk factors for reintervention.
CONCLUSIONS
For the repair of CAT, the LAA technique for right ventricle outflow tract reconstruction was associated with comparable survival and greater freedom from reintervention than the use of a valved conduit.
Topics: Atrial Appendage; Cardiovascular Surgical Procedures; Child; Coronary Vessel Anomalies; France; Heart Ventricles; Humans; Infant, Newborn; Male; Mortality; Outcome and Process Assessment, Health Care; Postoperative Complications; Reoperation; Risk Adjustment; Risk Factors; Truncus Arteriosus, Persistent
PubMed: 33342576
DOI: 10.1016/j.jtcvs.2020.10.147 -
Anatomical Record (Hoboken, N.J. : 2007) Mar 2023This review examines and discusses the morphology and embryology of two main groups of conotruncal cardiac malformations: (a) transposition of the great arteries... (Review)
Review
This review examines and discusses the morphology and embryology of two main groups of conotruncal cardiac malformations: (a) transposition of the great arteries (complete transposition and incomplete/partial transposition namely double outlet right ventricle), and (b) aortic dextroposition defects (tetralogy of Fallot and Eisenmenger malformation). In both groups, persistent truncus arteriosus was included because maldevelopment of the neural crest cell supply to the outflow tract, contributing to the production of the persistent truncus arteriosus, is shared by both groups of malformations. The potentially important role of the proximal conal cushions in the rotatory sequence of the conotruncus is emphasized. Most importantly, this study emphasizes the differentiation between the double-outlet right ventricle, which is a partial or incomplete transposition of the great arteries, and the Eisenmenger malformation, which is an aortic dextroposition. Special emphasis is also given to the leftward shift of the conoventricular junction, which covers an important morphogenetic role in both aortic dextropositions and transposition defects as well as in normal development, and whose molecular genetic regulation seems to remain unclear at present. Emphasis is placed on the distinct and overlapping roles of Tbx1 and Pitx2 transcription factors in modulating the development of the cardiac outflow tract.
Topics: Humans; Transposition of Great Vessels; Truncus Arteriosus, Persistent; Heart Defects, Congenital; Arteries
PubMed: 36426596
DOI: 10.1002/ar.25129 -
European Journal of Cardio-thoracic... Feb 2022Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the...
OBJECTIVES
Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the correction of all components of the anomaly, using autologous arterial tissue. This aims to enhance results, availability worldwide, and importantly to avoid the need for repeated reoperations.
METHODS
From January 2019 to 4 January 2021, all patients with isolated CAT had repair of the defect using autologous arterial trunk tissue with direct right ventricle (RV) to pulmonary artery (PA) connection. Clinical outcomes, follow-up which included multi-slice computed tomography 3D segmentation and 4D cardiovascular magnetic resonance flow, are presented.
RESULTS
Twenty patients were included in the study (median age 4.5 months). There were 2 hospital deaths due to systemic infection and pulmonary hypertensive crisis, respectively. Following discharge all patients remained asymptomatic with no signs of heart failure and improved pattern of growth (median follow-up: 8 months). Early postoperative 3D segmentation showed a conical shaped neo-right ventricular outflow chamber connecting the body of the RV to the main PA through a valveless ostium, and normal crossing of PA and neo-aorta. 4D cardiovascular magnetic resonance pattern of flow showed normal rapid laminar flow through the atrioventricular valves followed by a vortex towards the outflow tracts. There was laminar flow through the neo-aorta and neo-PA with velocity not exceeding 2.5 m/s. The PA regurgitant fraction was 25 ± 5% and was limited to early diastole.
CONCLUSIONS
The initial results of utilizing the key concepts, using autologous arterial tissue for the repair of CAT, are encouraging, both clinically and by multimodality imaging.
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Pulmonary Artery; Reoperation; Truncus Arteriosus, Persistent
PubMed: 34347066
DOI: 10.1093/ejcts/ezab336 -
Journal of Cardiology Cases Jun 2022Aortopulmonary window is a rare congenital heart disease. It results from incomplete separation of the aorta and pulmonary artery at the conotruncal septum. It accounts...
Aortopulmonary window is a rare congenital heart disease. It results from incomplete separation of the aorta and pulmonary artery at the conotruncal septum. It accounts for 0.2%-0.6% of all congenital cardiac defects. Closure should be done in all cases. We present the case of a 12-month-old female child with a large aortopulmonary window, complicated by severe pulmonary hypertension. Cardiac catheterization was performed and showed pulmonary vascular resistance 3 Woods units. Ascending aortography showed a large aortopulmonary window measuring 5 mm. Balloon sizing of the defect showed stretched diameter of 8 mm. A multifunctional occluder device 12 × 10 mm (Konar, Lifetech, Shenzhen, China) was properly positioned across the defect under transesophageal echocardiography guidance. Pulmonary angiograms showed a well seated pulmonary disc with mild encroachment on pulmonary bifurcation. Follow up transthoracic echocardiography 24 hours after the procedure showed a well seated device with no residual flow, mean pulmonary artery pressure 25 mmHg, laminar flow into main pulmonary artery and pulmonary bifurcation. To the best of our knowledge this is the first successful closure to be reported for an aortopulmonary window with Konar multifunctional occluder. Having a double disc with a low profile and small sheath size may allow closure of larger defects in smaller children. < Transcatheter closure of large aortopulmonary window in small children with severe pulmonary hypertension is possible, yet technically demanding. Having a double disc with a low profile and small sheath size allow closure of larger defects in smaller children.>.
PubMed: 35685269
DOI: 10.1016/j.jccase.2021.12.014 -
The Journal of Thoracic and... Oct 2021
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Truncus Arteriosus; Truncus Arteriosus, Persistent
PubMed: 33334604
DOI: 10.1016/j.jtcvs.2020.11.023 -
Diagnostics (Basel, Switzerland) Feb 2022Multiple congenital anomaly syndromes pose a challenge to neonatologists, as many anomalies may indicate cryptogenic malformations or disorders. Aortopulmonary window...
Multiple congenital anomaly syndromes pose a challenge to neonatologists, as many anomalies may indicate cryptogenic malformations or disorders. Aortopulmonary window (APW) is a very rare congenital heart disease (CHD) and causes many difficulties in prenatal diagnostics. In this report, we describe a case of a female patient with multiple rare congenital malformations: aortopulmonary window, right thumb aplasia, facial nerve palsy and cleft lip and alveolus. None of the malformations were diagnosed prenatally. A long-term follow-up (40 years) is presented. The presence of certain defects (thumb aplasia) may indicate the need for a careful fetal examination extended by a fetal ECHO performed in a reference center of prenatal cardiology. The coexistence or syndromic character of the presented malformations should be verified in future if more such cases are described.
PubMed: 35328123
DOI: 10.3390/diagnostics12030569