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Annals of Medicine and Surgery (2012) Feb 2023Unilateral absence of pulmonary artery (UAPA) is a very rare condition, with an estimated prevalence of 1 in 200,000 population, which is commonly associated with...
UNLABELLED
Unilateral absence of pulmonary artery (UAPA) is a very rare condition, with an estimated prevalence of 1 in 200,000 population, which is commonly associated with various cardiovascular anomalies or can occur in an isolated manner. Isolated cases survive to adulthood and remain asymptomatic, but they may frequently experience hemoptysis, repeated infections, or symptoms like dyspnea and chest pain. Due to the rarity of the disorder and its ambiguous appearance, diagnosis can be very challenging.
CASE PRESENTATION
We present a case of a 28-year-old male who visited our center with the diagnosis of ventricular septal defect with Eisenmenger syndrome elsewhere for further evaluation and was found to have right-sided UAPA with ipsilateral pulmonary hypoplasia and some associated cardiac anomalies.
CLINICAL DISCUSSION
Discussions are held regarding typical chest radiograph findings, diagnostic methods, and possible therapies.
CONCLUSION
Physicians should be aware of UAPA, which might go undiagnosed for several years despite regular medical care and can show up later in life, causing chronic respiratory symptoms along with Eisenmenger syndrome and ventricular septal defect like in our case.
PubMed: 36845809
DOI: 10.1097/MS9.0000000000000213 -
Endoscopic Ultrasound 2020There are limited data on multistage-based training programs focused on EUS. We aimed to explore an effective training system for diagnosing pancreaticobiliary diseases...
BACKGROUND AND OBJECTIVE
There are limited data on multistage-based training programs focused on EUS. We aimed to explore an effective training system for diagnosing pancreaticobiliary diseases with EUS.
MATERIALS AND METHODS
Nine advanced endoscopy trainees (AETs) with less EUS experience from nine institutions were recruited. The training system consisted of multiple stages and multi-teaching methods, including biliopancreatic standard scanning, anatomy and imaging knowledge, simulator, hands-on operations, error correction, and case analysis over a 12-month training period. Grading for technical and cognitive skills was assessed using The EUS Skills Assessment Tool.
RESULTS
After training, the overall scores for radial (4.16 ± 0.21 vs. 1.46 ± 0.16, P < 0.01) and linear (4.43 ± 0.20 vs. 1.63 ± 0.23, P < 0.01) scanning were significantly improved. The aortopulmonary window/mediastinum station can be learned more easily by AETs compared with other stations (P = 0023). The scanning of the descending part of the duodenum seemed to improve the slowest after training (P = 0.0072), indicating that the descending part of the duodenum can be more difficult and should be the focus of training. Every teaching method heightened EUS competence, especially case analysis and hands-on operations. AETs achieved equivalent EUS competence after training despite their initial experience. Through a poststudy questionnaire, it was found that all AETs strongly agreed they were satisfied with the training system, and their confidence was greatly enhanced when EUS was performed independently.
CONCLUSIONS
The current multistage and multi-methods training system showed efficient performance in the cognitive and technical competence of EUS. Descending part of duodenum scanning was difficult for beginners and should be the focus of training.
PubMed: 32913147
DOI: 10.4103/eus.eus_47_20 -
Thoracic Cancer Jul 2020This study reports the outcomes of a single institutional experience treating non-small cell lung cancer (NSCLC) involving the pulmonary hilum with low-dose stereotactic...
Low-dose hilar and mediastinal stereotactic body radiation therapy for non-small cell lung cancer: Analysis of outcomes in patients receiving one or multiple courses of treatment.
BACKGROUND
This study reports the outcomes of a single institutional experience treating non-small cell lung cancer (NSCLC) involving the pulmonary hilum with low-dose stereotactic body radiation therapy (SBRT). The authors also present a series of repeat hilar SBRT.
METHODS
Inclusion criteria required treatment with SBRT for NSCLC involving regional lymph nodes of the: (i) hilum, (ii) mediastinum, (iii) aortopulmonary window (station 5), or (iv) mainstem bronchus. At least one clinical follow-up with imaging was required, unless the patient had a prior documented death from cancer.
RESULTS
A total of 32 patients with 44 treatments were included, and 37 treatments targeted the hilum directly, with seven concerning the mediastinum, AP window, or mainstem bronchus. Median dose was 28 Gy in four fractions with once-weekly fractionation. At a median clinical follow-up of 23 months, local control was 64%. Median overall survival was 24 months, and median progression-free survival was 15 months. A total of 48% of treatments resulted in complete radiographic response on last imaging follow-up, and no cases of grade ≥ 3 toxicity were reported. For repeat SBRT (after prior hilar SBRT), local control was 92%. Median overall survival was 20 months, and median progression-free survival was 19 months. Complete radiographic response was noted after 58% of treatments, with 0 instances of progressive response and no reported side effects.
CONCLUSIONS
Low-dose hilar SBRT was efficacious and well-tolerated, with impressive overall survival and no grade ≥ 3 toxicity. Repeat treatments with SBRT were feasible and effective, demonstrating overall survival, local control, and toxicity comparable to primary treatments.
KEY POINTS
Significant findings of the study Low-dose hilar SBRT was efficacious and well-tolerated Repeated treatments with SBRT demonstrated encouraging results, comparable to primary treatments What this study adds This study contributes to the small body of literature concerning hilar SBRT Repeat hilar SBRT was safe and feasible Toxicity was minimal with low-dose SBRT Once-weekly fractionation may have contributed to low rate of side effects.
Topics: Adenocarcinoma of Lung; Aged; Carcinoma, Non-Small-Cell Lung; Carcinoma, Squamous Cell; Female; Follow-Up Studies; Humans; Lung Neoplasms; Male; Mediastinal Neoplasms; Prognosis; Radiosurgery; Retreatment; Retrospective Studies; Survival Rate
PubMed: 32469465
DOI: 10.1111/1759-7714.13501 -
The Journal of Thoracic and... Jun 2019
Topics: Child; Humans; Risk Factors; Truncus Arteriosus; Truncus Arteriosus, Persistent; Ventricular Outflow Obstruction
PubMed: 30846262
DOI: 10.1016/j.jtcvs.2019.01.047 -
The Journal of Thoracic and... Feb 2021To determine the outcomes of patients with a quadricuspid truncal valve (TV) and durability of TV repair.
OBJECTIVE
To determine the outcomes of patients with a quadricuspid truncal valve (TV) and durability of TV repair.
METHOD
We reviewed 56 patients with truncus arteriosus and a quadricuspid TV who underwent complete repair between 1979 and 2018.
RESULTS
TV insufficiency was present in 39 patients (mild, n = 22; moderate, n = 14; and severe, n = 3). Fourteen patients had concomitant TV surgery. Early mortality in patients who had concomitant TV surgery was 14% (2 out of 14 patients) and overall survival was 77.1% ± 11.7% at 15 years. Freedom from TV reoperation was 30.3% ± 14.6% at 15 years. Early mortality in patients who did not undergo concomitant TV surgery was 9.5% (4 out of 42 patients) and overall survival was 74.9% ± 6.9% at 15 years. Progression of TV insufficiency requiring TV surgery occurred in 16.7% (7 out of 42 patients). Freedom from TV reoperation was 77.1% ± 7.8% at 15 years. The most common method of repair was tricuspidization of the TV. Freedom from TV reoperation was 64.3% ± 21.0% at 10 years after tricuspidization and 0% at 6 years after other types of TV surgery. Overall follow-up was 97.6% (41 out of 42 patients) complete for survivors with median follow-up of 16.6 years. At last follow-up there was no TV insufficiency in 16 patients, mild insufficiency in 24 patients, and moderate insufficiency in 1 patient.
CONCLUSIONS
More than one-third of patients with a quadricuspid TV require TV surgery. Tricuspidization of the quadricuspid TV appears to be a durable repair option with good long-term outcomes.
Topics: Cardiac Surgical Procedures; Humans; Infant; Infant, Newborn; Reoperation; Treatment Outcome; Truncus Arteriosus, Persistent
PubMed: 32122575
DOI: 10.1016/j.jtcvs.2020.01.039 -
Indian Journal of Thoracic and... Jul 2022We report a case of a 1-year-old patient with aortopulmonary window, anomalous right coronary artery arising from the pulmonary artery, and a large perimembranous...
We report a case of a 1-year-old patient with aortopulmonary window, anomalous right coronary artery arising from the pulmonary artery, and a large perimembranous ventricular septal defect. Computed tomography was successful in diagnosing the coronary anomaly and the case was managed surgically with an intrapulmonary baffle, rerouting the right coronary ostium to the aortic root and a fenestrated patch closure of the ventricular septal defect. Pre-operative diagnosis and thorough planning were essential for the successful outcome of this case.
PubMed: 35756565
DOI: 10.1007/s12055-022-01334-1 -
Annals of Pediatric Cardiology 2022Acquired aortopulmonary fistula (APF) in the setting of repaired congenital heart disease is extremely rare but potentially fatal, so timely diagnosis and treatment are...
Acquired aortopulmonary fistula (APF) in the setting of repaired congenital heart disease is extremely rare but potentially fatal, so timely diagnosis and treatment are critical. We present a case of an 8-year-old female with a history of complex Taussig-Bing anomaly, who underwent an arterial switch procedure with LeCompte maneuver and ventricular septal defect closure early in life. The patient developed neopulmonary stenosis and branch pulmonary artery (PA) stenosis, for which she underwent patch augmentation and balloon dilatation of the left PA. The patient presented with a fistula between the ascending aorta and the left branch PA, confirmed by echocardiography and cardiac catheterization. She underwent repair of the APF with a homograft patch reconstruction of the ascending aorta.
PubMed: 36246743
DOI: 10.4103/apc.apc_251_20 -
The Journal of Thoracic and... Oct 2014Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE)...
Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance.
OBJECTIVE
Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation.
METHODS
Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge.
RESULTS
Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.1±4.2 vs 5.6±4.5 days, respectively; P=.57).
CONCLUSIONS
Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.
Topics: Biomarkers; Collateral Circulation; Coronary Angiography; Female; Heart Septal Defects; Humans; Incidence; Intercellular Adhesion Molecule-1; Interleukin-6; Male; Postoperative Complications; Pulmonary Atresia; Pulmonary Edema; Receptor for Advanced Glycation End Products; Receptors, Immunologic; Reperfusion Injury; Respiration, Artificial; Tetralogy of Fallot
PubMed: 24534681
DOI: 10.1016/j.jtcvs.2014.01.017 -
Frontiers in Cardiovascular Medicine 2022Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare, complex, and heterogeneous congenital...
A Novel 3D Visualized Operative Procedure in the Single-Stage Complete Repair With Unifocalization of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries.
OBJECTIVES
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare, complex, and heterogeneous congenital heart disease. As one of the effective treatments, the midline unifocalization strategy still remains complicated and challenging due to the diverse forms of MAPCAs and pulmonary arteries. The purpose of this study is to summarize our experience of a novel three-dimensional (3D) visualized operative procedure in the single-stage complete repair with unifocalization and to clarify the benefits it may bring to us.
METHODS
We described our experience of the 3D visualized operative procedure such as 3D printing, virtual reality (VR), and mixed reality (MR) technology in patients with PA/VSD/MAPCAs who underwent a single-stage complete repair with unifocalization. The data from the patients who underwent this procedure (3D group) and those who underwent the conventional procedure (conventional group) were compared.
RESULTS
The conventional and 3D groups included 11 patients from September 2011 to December 2017 and 9 from January 2018 to March 2021, respectively. The baseline characteristics such as age, body weight, preoperative saturation, the anatomy of the pulmonary arteries and MAPCAs, the Nakata index, and TNPAI had no statistical significance. All 9 patients in the 3D group were operated only through a median sternotomy, while 8 cases (72.7%) in the conventional group needed another posterolateral thoracotomy ( = 0.001). In the 3D group, the CPB time was shorter (93.2 ± 63.8 vs. 145.1 ± 68.4 min, = 0.099), and the median pre-CPB time per MAPCAs was significantly shorter [25.7 (14.0, 46.3) vs. 65 (41.3, 75.0) min, = 0.031]. There was no early death in the 3D group, while there were 3 in the conventional group (0 vs. 27.3%, = 0.218).
CONCLUSION
The novel 3D visualized operative procedure may help improve the performance of the single-stage complete repair with the midline unifocalization of PA/VSD/MAPCAs and help shorten the dissecting time of the MAPCAs. It may promote the routine and successful application of this strategy in more centers.
PubMed: 35548444
DOI: 10.3389/fcvm.2022.836200 -
Iranian Journal of Neurology Jan 2018Myasthenia gravis (MG) is an autoimmune disease affecting acetylcholine postsynaptic receptor of voluntary muscles. Thymectomy is done in these patients and is a...
Myasthenia gravis (MG) is an autoimmune disease affecting acetylcholine postsynaptic receptor of voluntary muscles. Thymectomy is done in these patients and is a mainstay in the treatment of MG; however, the long-term result of surgery is still controversial. This study dealt with the investigation of the results of thymectomy in treatment, recovery and control of the symptoms of these patients. This study was performed through a retrospective method in patients suffering from MG who underwent trans-sternal thymectomy between 2011 and 2016. We conducted thymectomy, excision of mediastinal mass and contents of tissues between the right and left phrenic nerves for all patients. Then, the effect of various variables including age, sex, time interval between onset of disease and surgery, thymus pathology and the dosage of drug on clinical response after surgery was determined using various statistical tests. 47 patients including 26 men and 21 women with the mean age of 33.0 ± 4.6 years have been investigated. The mean age of patients was 36.2 and 29.7 in men and women respectively (P = 0.041). Spiral chest computed tomography (CT) scan was present in 47 patients demonstrating mediastinal mass in 40 (85.1%) patients. Also, our pathological results showed thymic cells in aortopulmonary window contents of 4 patients. According to the results, the younger age of patients at the time of surgery, shorter time between diagnosis and thymectomy, being a woman and non-thymoma pathology were along with better clinical outcomes after thymectomy. Our study shows better clinical results of thymectomy in patients with normal chest CT scan and normal or atrophic thymus in pathologic reports. Generally, it seems that performing thymectomy in a shorter time interval after diagnosis of MG is beneficial. Moreover, in MG patients who do not suffer from thymoma, it is along with positive results.
PubMed: 30186552
DOI: No ID Found