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Journal de la Faculté de Médecine d'Oran ; 6(2): 819-824, 2023. figures
Article in French | AIM | ID: biblio-1415186


La double discordance est une cardiopathie congénitale complexe extrêmement rare, elle se caractérise par une discordance auriculo-ventriculaire suivie d'une autre discordance ventriculo-artérielle. Son diagnostic est posé généralement à l'âge adulte par des examens d'imagerie. Son pronostic dépend essentiellement des autres malformations congénitales cardiaques associées, des arythmies et des troubles de la conduction, ainsi que de la fonction systolique du ventricule droit en position systémique. Nous rapportons le cas d'un patient âgé de 23 ans, porteur d'une cardiopathie congénitale complexe cyanogène. Il s'agit d'une double discordance associée à une large communication interventriculaire sous aortique avec un shunt inversé et une hypoplasie de l'artère pulmonaire. Ce diagnostic a été confirmé par l'imagerie multimodale. Non opéré, le patient a vu son stade fonctionnel s'aggraver rapidement avec une dyspnée qui est passée d'un stade I à un stade III-IV de la New York Heart Association (NYHA), et apparition de signes hypoxiques et congestifs. A travers cette observation, nous avons précisé les caractéristiques anatomocliniques de cette cardiopathie congénitale complexe cyanogène, son exploration, ses options thérapeutiques, ainsi que le rôle péjoratif de son association avec d'autres malformations congénitales.

Double discordance is an extremely rare complex congenital heart disease; It is characterized by atrioventricular discordance followed by another ventriculo-arterial discordance. Its diagnosis is usually made in adulthood by imaging examinations. Its prognosis depends mainly on other associated congenital heart defects, arrhythmias and disorders of the conduction, as well as systolic function of the right ventricle in the systemic position. We report the case of a 23-year-old patient with a complex cyanogen heart disease.It is a double discordance associated to an interventricular communication with reverse shunt and an hypoplasia of the pulmonary artery.This Diagnosis was confirmed in multimodal imaging. Non-operated, the patient saw his functional status worsen rapidly with dyspnea which went from stage I to stage III-IV of the New York Heart Association (NYHA), and appearance of hypoxic and congestive signs. Through this observation, we have clarified the anatomoclinical characteristics of this complex congenital cyanogens heart disease, the necessary explorations and the different therapeutic options, as well as the pejorative role of its association with other congenital malformations.

Humans , Female , Pulmonary Artery , Therapeutics , Transposition of Great Vessels , Heart Defects, Congenital , Arrhythmias, Cardiac , Congenital Abnormalities
Acta Physiologica Sinica ; (6): 503-511, 2023.
Article in Chinese | WPRIM | ID: wpr-1007765


In this study, we investigated the effects of Panax notoginseng saponins (PNS) on pulmonary vascular remodeling and ADAM10/Notch3 pathway in pulmonary arterial hypertension (PAH). PAH rat model was established, and male Sprague Dawley (SD) rats were randomly divided into control group, monocrotaline (MCT) group and MCT+PNS group, with 10 rats in each group. Rats in the control group were intraperitoneally injected with equal volume of normal saline. Rats in the MCT group was injected intraperitoneally with 60 mg/kg MCT on the first day, and then with the same volume of normal saline every day. Rats in the MCT+PNS group was injected intraperitoneally with 60 mg/kg MCT on the first day, and then with 50 mg/kg PNS every day. The modeling time of each group lasted for 21 days. After the model was established, the mean pulmonary artery pressure (mPAP) was measured by right heart catheterization technique, the right ventricular hypertrophy index (RVHI) was calculated, the microscopic morphology and changes of pulmonary vascular wall were observed by HE and Masson staining, and the expressions of ADAM10, Notch3, Hes-1, P27, PCNA, Caspase-3 proteins and mRNA in pulmonary vascular tissue of rats were detected by Western blot and qPCR. The expression and localization of Notch3 and α-SMA were detected by immunofluorescence staining. The protein expression of ADAM10 was detected by immunohistochemical staining. The results showed that compared with the control group, mPAP, RVHI, pulmonary vessels and collagen fibers in the MCT group were significantly increased, the expressions of ADAM10, Notch3, Hes-1, and PCNA protein and mRNA were significantly increased, while the expressions of P27 and Caspase-3 protein and mRNA were decreased significantly. Compared with the MCT group, mPAP and RVHI were significantly decreased, pulmonary vessels were significantly improved and collagen fibers were significantly reduced, the expressions of protein and mRNA of ADAM10, Notch3, Hes-1, and PCNA were decreased in MCT+PNS group, but the expressions of protein and mRNA of P27 and Caspase-3 were increased slightly. The results of immunofluorescence showed that Notch3 and α-SMA staining could overlap, which proved that Notch3 was expressed in smooth muscle cells. The expression of Notch3 in the MCT group was increased significantly compared with that in the control group, while PNS intervention decreased the expression of Notch3. Immunohistochemical staining showed that compared with the control group, the amount of ADAM10 in the MCT group was increased significantly, and the expression of ADAM10 in the MCT+PNS group was decreased compared with the MCT group. These results indicate that PNS can improve the PAH induced by MCT in rats by inhibiting ADAM10/Notch3 signaling pathway.

Animals , Male , Rats , Caspase 3/metabolism , Collagen , Disease Models, Animal , Hypertension, Pulmonary/drug therapy , Monocrotaline/adverse effects , Panax notoginseng/chemistry , Proliferating Cell Nuclear Antigen/pharmacology , Pulmonary Arterial Hypertension , Pulmonary Artery/metabolism , Rats, Sprague-Dawley , Receptor, Notch3/genetics , RNA, Messenger , Saline Solution , Signal Transduction , Saponins/pharmacology
Acta Physiologica Sinica ; (6): 497-502, 2023.
Article in Chinese | WPRIM | ID: wpr-1007764


In this study, we used a rat model of pulmonary arterial hypertension (PAH) induced by monocrotaline (MCT) to investigate the role and mechanism of angiotensin (Ang)-(1-7) in regulating pulmonary artery diastolic function. Three weeks after subcutaneous injection of MCT or normal saline, the right ventricular systolic pressure (RVSP) and right ventricular hypertrophy index (RVHI) of rats were detected using a right heart catheter. Vascular endothelium-dependent relaxation was evaluated by acetylcholine (ACh)-induced vasodilation. The relaxation function of vascular smooth muscle was evaluated by sodium nitroprusside (SNP)-induced vasodilation. Human pulmonary artery endothelial cells (HPAECs) were incubated with Ang-(1-7) to measure nitric oxide (NO) release levels. The results showed that compared with control rats, RVSP and RVHI were significantly increased in the MCT-PAH rats, and both ACh or SNP-induced vasodilation were worsened. Incubation of pulmonary artery of MCT-PAH rats with Ang-(1-7) (1 × 10-9-1 × 10-4 mol/L) caused significant vaso-relaxation. Pre-incubation of Ang-(1-7) in the pulmonary artery of MCT-PAH rats significantly improved ACh-induced endothelium-dependent relaxation, but had no significant effect on SNP-induced endothelium-independent relaxation. In addition, Ang-(1-7) treatment significantly increased NO levels in HPAECs. The Mas receptor antagonist A-779 inhibited the effects of Ang-(1-7) on endothelium-dependent relaxation and NO release from endothelial cells. The above results demonstrate that Ang-(1-7) promotes the release of NO from endothelial cells by activating Mas receptor, thereby improving the endothelium-dependent relaxation function of PAH pulmonary arteries.

Rats , Humans , Animals , Vasodilation , Pulmonary Arterial Hypertension , Monocrotaline/toxicity , Rats, Sprague-Dawley , Hypertension, Pulmonary/chemically induced , Endothelial Cells , Pulmonary Artery , Endothelium , Acetylcholine/pharmacology , Nitroprusside/pharmacology
Chinese Journal of Pediatrics ; (12): 141-145, 2023.
Article in Chinese | WPRIM | ID: wpr-970253


Objective: To summarize the clinical characteristics of bronchial-pulmonary artery fistula and evaluate the effect of interventional closure of bronchial-pulmonary artery fistula. Methods: A retrospective case study was conducted. Fifteen children with hemoptysis who were diagnosed with bronchial-pulmonary artery fistula in Beijing Children's Hospital, Capital Medical University from January 2018 to March 2022 were selected. Their clinical symptoms and chest-enhanced CT findings were recorded. The children who failed to improve after anti-infection and hemostasis treatment were treated with transcatheter embolization through microparticles under digital subtraction angiography (DSA). The efficacy and post-operation recurrence were evaluated. Results: There were 15 children, including 9 males and 6 females, aged 9.8 (3.7, 12.1) years, weighing 35 (16, 55) kg. There was hemoptysis of varying degrees before surgery. Only 2 children had decreased hemoglobin. Chest enhanced CT showed that their bronchial arteries were thickened and tortuous, including 11 cases of single vessel disease and 4 cases of multivessel disease; 11 children had varying degrees of pneumonia and 4 children had atelectasis. Except for one case effectively treated with medical therapy, the remaining 14 cases were all treated with transcatheter interventional closure with embolic microparticles, among whom 12 had their fistula completely blocked with a single operation and the other 2 children underwent multiple operations because of too many fistulas. One child had extensive bronchial-pulmonary artery fistula which failed to be blocked completely even after multiple operations. Among the remaining 13 children, only 2 patients whose fistula was considered to be completely closed had recurrence presenting with hemoptysis at 3 months and 2 years after the operation, and no hemoptysis was found after the second closure. All children were discharged without chest pain, spinal cord paraplegia, or other serious complications. Fourteen children were followed up for 1.4 (0.9,2.9) years, among whom one still has intermittent mild hemoptysis due to incomplete closure and the rest had a satisfactory outcome. Conclusions: Hemoptysis is the first symptom of bronchial-pulmonary artery fistula. For children with failed medical treatment, transcatheter closure with an embolic pellet is effective, safe and feasible, with a low recurrence rate.

Child , Female , Male , Humans , Pulmonary Artery , Retrospective Studies , Chest Pain , Hemoptysis/therapy , Hospitals, Pediatric
Chinese Journal of Contemporary Pediatrics ; (12): 407-414, 2023.
Article in Chinese | WPRIM | ID: wpr-981971


OBJECTIVES@#To study the effect of platelet-derived growth factor-BB (PDGF-BB) on pulmonary vascular remodeling in neonatal rats with hypoxic pulmonary hypertension (HPH).@*METHODS@#A total of 128 neonatal rats were randomly divided into four groups: PDGF-BB+HPH, HPH, PDGF-BB+normal oxygen, and normal oxygen (n=32 each). The rats in the PDGF-BB+HPH and PDGF-BB+normal oxygen groups were given an injection of 13 μL 6×1010 PFU/mL adenovirus with PDGF-BB genevia the caudal vein. After 24 hours of adenovirus transfection, the rats in the HPH and PDGF-BB+HPH groups were used to establish a neonatal rat model of HPH. Right ventricular systolic pressure (RVSP) was measured on days 3, 7, 14, and 21 of hypoxia. Hematoxylin-eosin staining was used to observe pulmonary vascular morphological changes under an optical microscope, and vascular remodeling parameters (MA% and MT%) were also measured. Immunohistochemistry was used to measure the expression levels of PDGF-BB and proliferating cell nuclear antigen (PCNA) in lung tissue.@*RESULTS@#The rats in the PDGF-BB+HPH and HPH groups had a significantly higher RVSP than those of the same age in the normal oxygen group at each time point (P<0.05). The rats in the PDGF-BB+HPH group showed vascular remodeling on day 3 of hypoxia, while those in the HPH showed vascular remodeling on day 7 of hypoxia. On day 3 of hypoxia, the PDGF-BB+HPH group had significantly higher MA% and MT% than the HPH, PDGF-BB+normal oxygen, and normal oxygen groups (P<0.05). On days 7, 14, and 21 of hypoxia, the PDGF-BB+HPH and HPH groups had significantly higher MA% and MT% than the PDGF-BB+normal oxygen and normal oxygen groups (P<0.05). The PDGF-BB+HPH and HPH groups had significantly higher expression levels of PDGF-BB and PCNA than the normal oxygen group at all time points (P<0.05). On days 3, 7, and 14 of hypoxia, the PDGF-BB+HPH group had significantly higher expression levels of PDGF-BB and PCNA than the HPH group (P<0.05), while the PDGF-BB+normal oxygen group had significantly higher expression levels of PDGF-BB and PCNA than the normal oxygen group (P<0.05).@*CONCLUSIONS@#Exogenous administration of PDGF-BB in neonatal rats with HPH may upregulate the expression of PCNA, promote pulmonary vascular remodeling, and increase pulmonary artery pressure.

Rats , Animals , Hypertension, Pulmonary , Becaplermin , Animals, Newborn , Proliferating Cell Nuclear Antigen , Vascular Remodeling , Pulmonary Artery/metabolism , Hypoxia , Oxygen , Cell Proliferation , Myocytes, Smooth Muscle/metabolism
Chinese Medical Journal ; (24): 1198-1206, 2023.
Article in English | WPRIM | ID: wpr-980888


BACKGROUND@#Right ventricular (RV)-arterial uncoupling is a powerful independent predictor of prognosis in heart failure with preserved ejection fraction (HFpEF). Coronary artery disease (CAD) can contribute to the pathophysiological characteristics of HFpEF. This study aimed to evaluate the prognostic value of RV-arterial uncoupling in acute HFpEF patients with CAD.@*METHODS@#This prospective study included 250 consecutive acute HFpEF patients with CAD. Patients were divided into RV-arterial uncoupling and coupling groups by the optimal cutoff value, based on a receiver operating characteristic curve of tricuspid annular plane systolic excursion to pulmonary artery systolic pressure (TAPSE/PASP). The primary endpoint was a composite of all-cause death, recurrent ischemic events, and HF hospitalizations.@*RESULTS@#TAPSE/PASP ≤0.43 provided good accuracy in identifying patients with RV-arterial uncoupling (area under the curve, 0.731; sensitivity, 61.4%; and specificity, 76.6%). Of the 250 patients, 150 and 100 patients could be grouped into the RV-arterial coupling (TAPSE/PASP >0.43) and uncoupling (TAPSE/PASP ≤0.43) groups, respectively. Revascularization strategies were slightly different between groups; the RV-arterial uncoupling group had a lower rate of complete revascularization (37.0% [37/100] vs . 52.7% [79/150], P <0.001) and a higher rate of no revascularization (18.0% [18/100] vs . 4.7% [7/150], P <0.001) compared to the RV-arterial coupling group. The cohort with TAPSE/PASP ≤0.43 had a significantly worse prognosis than the cohort with TAPSE/PASP >0.43. Multivariate Cox analysis showed TAPSE/PASP ≤0.43 as an independent associated factor for the primary endpoint, all-cause death, and recurrent HF hospitalization (hazard ratios [HR]: 2.21, 95% confidence interval [CI]: 1.44-3.39, P <0.001; HR: 3.32, 95% CI: 1.30-8.47, P = 0.012; and HR: 1.93, 95% CI: 1.10-3.37, P = 0.021, respectively), but not for recurrent ischemic events (HR: 1.48, 95% CI: 0.75-2.90, P = 0.257).@*CONCLUSION@#RV-arterial uncoupling, based on TAPSE/PASP, is independently associated with adverse outcomes in acute HFpEF patients with CAD.

Humans , Prognosis , Prospective Studies , Stroke Volume/physiology , Echocardiography, Doppler/adverse effects , Coronary Artery Disease/complications , Heart Failure , Pulmonary Artery/diagnostic imaging , Ventricular Function, Right/physiology , Ventricular Dysfunction, Right
Journal of Zhejiang University. Medical sciences ; (6): 750-757, 2023.
Article in English | WPRIM | ID: wpr-971092


Phenotypic transformation of pulmonary artery smooth muscle cells (PASMCs) is a key factor in pulmonary vascular remodeling. Inhibiting or reversing phenotypic transformation can inhibit pulmonary vascular remodeling and control the progression of hypoxic pulmonary hypertension. Recent studies have shown that hypoxia causes intracellular peroxide metabolism to induce oxidative stress, induces multi-pathway signal transduction, including those related to autophagy, endoplasmic reticulum stress and mitochondrial dysfunction, and also induces non-coding RNA regulation of cell marker protein expression, resulting in PASMCs phenotypic transformation. This article reviews recent research progress on mechanisms of hypoxia-induced phenotypic transformation of PASMCs, which may be helpful for finding targets to inhibit phenotypic transformation and to improve pulmonary vascular remodeling diseases such as hypoxia-induced pulmonary hypertension.

Humans , Pulmonary Artery , Hypertension, Pulmonary , Vascular Remodeling/genetics , Hypoxia/genetics , Myocytes, Smooth Muscle , Cell Proliferation/physiology , Cells, Cultured , Cell Hypoxia/genetics
Chinese Journal of Cardiology ; (12): 481-489, 2023.
Article in Chinese | WPRIM | ID: wpr-984679


Objectives: To analyze the reasons of missed diagnosis or misdiagnosis on anomalous origin of left coronary artery from pulmonary artery (ALCAPA) by echocardiography. Methods: This is a retrospective study. Patients with ALCAPA who underwent surgical treatment in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2008 to December 2021 were included. According to the results of preoperative echocardiography and surgical diagnosis, the patients were divided into confirmed group or missed diagnosis/misdiagnosis group. The results of preoperative echocardiography were collected, and the specific echocardiographic signs were analyzed. According to the experience of the doctors, the echocardiographic signs were divided into four types, namely clear displayed, vague/doubtful displayed, no display and no notice, and the display rate of each sign was calculated (display rate=number of clearly displayed cases/total number of cases×100%). By referring the surgical data, we analyzed and recorded the pathological anatomy and pathophysiological characteristics of the patients, and the rate of missed diagnosis/misdiagnosis of echocardiography in patients with different characteristics was compared. Results: A total of 21 patients were enrolled, including 11 males, aged 1.8 (0.8, 12.3) years (range 1 month to 47 years). Except for one patient with anomalous origin of left anterior descending artery, the others were all originated from the main left coronary artery (LCA). There were 13 cases of ALCAPA in infant and children, and 8 cases of adult ALCAPA. There were 15 cases in the confirmed group (diagnostic accuracy was 71.4% (15/21)), and 6 cases in the missed diagnosis/misdiagnosis group (three cases were misdiagnosed as primary endocardial fibroelastosis, two cases were misdiagnosed as coronary-pulmonary artery fistula; and one case was missed diagnosis). The working years of the physicians in the confirmed group were longer than those in the missed diagnosis/misdiagnosed group ((12.8±5.6) years vs. (8.3±4.7) years, P=0.045). In infants with ALCAPA, the detection rate of LCA-pulmonary shunt (8/10 vs. 0, P=0.035) and coronary collateral circulation (7/10 vs. 0, P=0.042) in confirmed group was higher than that in missed diagnosis/misdiagnosed group. In adult ALCAPA patients, the detection rate of LCA-pulmonary artery shunt was higher in confirmed group than that in missed diagnosis/misdiagnosed group (4/5 vs. 0, P=0.021). The missed diagnosis/misdiagnosis rate of adult type was higher than that of infant type (3/8 vs. 3/13, P=0.410). The rate of missed diagnosis/misdiagnosis was higher in patients with abnormal origin of branches than that of abnormal origin of main trunk (1/1 vs. 5/21, P=0.028). The rate of missed diagnosis/misdiagnosis in patients with LCA running between the main and pulmonary arteries was higher than that distant from the main pulmonary artery septum (4/7 vs. 2/14, P=0.064). The rate of missed diagnosis/misdiagnosis in patients with severe pulmonary hypertension was higher than that in patients without severe pulmonary hypertension (2/3 vs. 4/18, P=0.184). The reasons with an echocardiography missed diagnosis/misdiagnosis rate of≥50% included that (1) the proximal segment of LCA ran between the main and pulmonary arteries; (2) abnormal opening of LCA at the right posterior part of the pulmonary artery; (3) abnormal origin of LCA branches; (4) complicated with severe pulmonary hypertension. Conclusions: Echocardiography physicians' knowledge of ALCAPA and diagnostic vigilance are critical to the accuracy of diagnosis. Attention should be paid to the pediatric cases with no obvious precipitating factors of left ventricular enlargement, regardless of whether the left ventricular function is normal or not, the origin of coronary artery should be routinely explored.

Male , Adult , Infant , Child , Humans , Bland White Garland Syndrome/diagnostic imaging , Pulmonary Artery/diagnostic imaging , Retrospective Studies , Missed Diagnosis , Hypertension, Pulmonary , Echocardiography , Coronary Vessel Anomalies/diagnostic imaging
Rev. chil. cardiol ; 41(3): 186-189, dic. 2022. ilus
Article in Spanish | LILACS | ID: biblio-1423691


Se presentan dos casos clínicos de "criscross" de ramas pulmonares, una forma infrecuente de malformación del origen de estas arterias desde lados opuestos del tronco pulmonar y cuyos trayectos se cruzan en su camino hacia sus respectivos pulmones. De manera aislada es una condición benigna, sin consecuencias hemodinámicas y de buen pronóstico.

Two clinical cases of "crisscrossed" pulmonary arteries are presented. This is an unusual malformation in which arteries from opposite sides of the pulmonary trunk cross along their course towards their respective lungs. Usually, it is a benign condition when found as an isolated malformation, with no hemodynamic consequences and good prognosis.

Humans , Female , Infant, Newborn , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Heart Defects, Congenital/diagnostic imaging , Echocardiography, Doppler
Rev. chil. enferm. respir ; 38(4): 246-252, dic. 2022. ilus, tab
Article in Spanish | LILACS | ID: biblio-1441386


Las malformaciones arteriovenosas pulmonares (MAVP) consisten en comunicaciones directas entre el sistema arterial y el sistema venoso pulmonar, sin paso de la sangre por el lecho capilar, produciéndose un cortocircuito de derecha a izquierda extracardíaco, pueden ser congénitas o adquiridas. Algunos casos pueden ser asintomáticos, en cambio en otros pueden ocasionar diversas manifestaciones clínicas y se pueden asociar a complicaciones severas. En niños se observa una baja incidencia y son más frecuentes las formas congénitas. La MAVP se debe sospechar por las manifestaciones clínicas y las imágenes de la radiografía de tórax (RxTx) y su confirmación se realiza mediante una AngioTomografía Computada (TC) de tórax. La embolización endovascular es actualmente el tratamiento de elección, con excelentes resultados, aunque requiere de un seguimiento posterior y de un operador experimentado. Reportamos el caso de una niña que ingresó con clínica muy sugerente, incluyendo: disnea, acropaquia, cianosis periférica, e hipoxemia refractaria. Sin embargo, inicialmente el cuadro clínico fue confundido con una crisis asmática. La Angio-TC de tórax confirmó el diagnóstico y el tratamiento mediante embolización endovascular resultó exitoso.

Pulmonary arteriovenous malformations (PAVM) are communications between the arterial and the pulmonary venous system, without passage of blood through the capillary bed, causing a left to right extracardiac shunt. Some cases may be asymptomatic, while others may cause various clinical manifestations and may be associated with severe complications. In children a low incidence is observed, and congenital forms are more frequent. PAVM should be suspected by clinical manifestations and chest x-ray imaging and confirmed by chest Computed Tomography Angiography (CTA). Endovascular embolization is currently the treatment of choice, with excellent results, although it requires subsequent follow-up. We report a patient who was admitted with a very suggestive clinical history, including: dyspnea, clubbing, peripheral cyanosis, and severe hypoxemia, refractory to oxygen therapy. However, initially the clinical picture was confounded with an asthmatic crisis. CTA confirmed the diagnosis and treatment by endovascular embolization was successful.

Humans , Female , Child , Arteriovenous Malformations/therapy , Arteriovenous Malformations/diagnostic imaging , Pulmonary Artery/diagnostic imaging , Pulmonary Veins/diagnostic imaging , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Radiography, Thoracic , Embolization, Therapeutic , Computed Tomography Angiography , Oxygen Saturation , Hypoxia
Arch. argent. pediatr ; 120(3): e133-e136, junio 2022. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1368481


El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

Humans , Male , Infant , Tracheal Diseases , Tracheal Stenosis/surgery , Tracheal Stenosis/diagnosis , Bronchial Diseases/surgery , Vascular Malformations/complications , Vascular Ring/complications , Heart Defects, Congenital/complications , Pulmonary Artery/abnormalities , Trachea/abnormalities , Bronchi/abnormalities , Bronchi/surgery , Constriction, Pathologic
São Paulo med. j ; 140(3): 505-508, May-June 2022. tab, graf
Article in English | LILACS | ID: biblio-1377380


ABSTRACT BACKGROUND: Severe pulmonary arterial hypertension (PAH) is a contraindication for heart transplantation (HT). It has been correlated with increased early and late mortality, mainly associated with right ventricular failure. Ventricular assistance devices (VADs) can promote reduction of intracardiac pressures and consequent reduction of PAH over the medium and long terms, thus enabling future candidature for HT. The diminution of early pulmonary pressure within this scenario remains unclear. OBJECTIVE: To evaluate the reduction of PAH and correlate data from right catheterization with the earliness of this reduction. DESIGN AND SETTING: Cross-sectional study in a general hospital in São Paulo, Brazil. METHODS: This was a retrospective analysis on the medical records of patients undergoing VAD implantation in a single hospital. Patients for whom VAD had been indicated as a bridge to candidature for HT due to their condition of constant PAH were selected. RESULTS: Four patients with VADs had constantly severe PAH. Their mean pulmonary artery systolic pressure (PASP) before VAD implantation was 66 mmHg. Over the 30-day period after the procedure, all the patients evolved with a drop in PASP to below 60 mmHg. Their new average was 36 mmHg, which was a drop of close to 50% from baseline values. The one-year survival of this sample was 100%. CONCLUSION: VAD implantation can reduce PAH levels. Early reduction occurred in all patients. Thus, use of VAD is an important bridge tool for enabling candidature for HT among patients with constantly severe PAH.

Humans , Pulmonary Arterial Hypertension/surgery , Hypertension, Pulmonary/surgery , Pulmonary Artery , Brazil , Cross-Sectional Studies , Retrospective Studies
Journal of Southern Medical University ; (12): 718-723, 2022.
Article in Chinese | WPRIM | ID: wpr-936368


OBJECTIVE@#To explore the therapeutic mechanism of tanshinone IIA in the treatment of pulmonary arterial hypertension (PAH) in rats.@*METHODS@#A total of 100 male SD rats were randomized into 5 groups (n=20), and except for those in the control group with saline injection, all the rats were injected with monocrotaline (MCT) on the back of the neck to establish models of pulmonary hypertension. Two weeks after the injection, the rat models received intraperitoneal injections of tanshinone IIA (10 mg/kg), phosphatidylinositol 3 kinase (PI3K) inhibitor (1 mg/kg), both tanshinone IIA and PI3K inhibitor, or saline (model group) on a daily basis. After 2 weeks of treatment, HE staining and α-SMA immunofluorescence staining were used to evaluate the morphology of the pulmonary vessels of the rats. The phosphorylation levels of PI3K, protein kinase B (PKB/Akt) and endothelial nitric oxide synthase (eNOS) in the lung tissue were determined with Western blotting; the levels of eNOS and NO were measured using enzyme-linked immunosorbent assay (ELISA).@*RESULTS@#The results of HE staining and α-SMA immunofluorescence staining showed that tanshinone IIA effectively inhibited MCT-induced pulmonary artery intimamedia thickening and muscularization of the pulmonary arterioles (P < 0.01). The results of Western blotting showed that treatment with tanshinone IIA significantly increased the phosphorylation levels of PI3K, Akt and eNOS proteins in the lung tissue of PAH rats; ELISA results showed that the levels of eNOS and NO were significantly decreased in the rat models after tanshinone IIA treatment (P < 0.01).@*CONCLUSION@#Treatment with tanshinone IIA can improve MCT-induced pulmonary hypertension in rats through the PI3K/Akt-eNOS signaling pathway.

Animals , Male , Rats , Abietanes , Hypertension, Pulmonary/drug therapy , Monocrotaline/toxicity , Nitric Oxide Synthase Type III/therapeutic use , Phosphatidylinositol 3-Kinase/pharmacology , Phosphatidylinositol 3-Kinases/metabolism , Proto-Oncogene Proteins c-akt/metabolism , Pulmonary Artery , Rats, Sprague-Dawley , Signal Transduction
Chinese Journal of Pediatrics ; (12): 20-24, 2022.
Article in Chinese | WPRIM | ID: wpr-935633


Objective: To evaluate the effectiveness and safety of Pul-Stent as the treatment of postoperative branch pulmonary artery stenosis in children with congenital heart disease. Methods: This was a retrospective study. Thirty-three patients who underwent Pul-Stent implantation in Shanghai Children's Medical Center due to postoperative residual pulmonary artery stenosis from August 2014 to June 2015 were included. The immediate curative effect, follow-up and complications of Pul-Stent implantation were assessed. Comparisons between groups were performed with unpaired Student t test. Results: Pul-Stent implantation of 33 patients (19 males and 14 females) were performed successfully. Thirty-one patients underwent percutaneous stenting, and 2 patients underwent hybrid stenting. A total of 35 Pul-Stents were implanted (19 of model small, 15 of model medium and one of model large), 23 stents were planted in the proximal left pulmonary artery and 12 stents were in the proximal right pulmonary artery. The initial diameter of dilation balloon ranged from 6 to 16 mm, and the long sheath of percutaneous implantation ranged from 8 to 10 F in 29 patients (29/31, 94%). After stenting, the diameter of the narrowest segment of pulmonary artery increased from (4.0±1.7) mm to (9.1±2.1) mm in all patients (t=-21.60, P<0.001). The pressure gradient at the stenosis in 26 patients after biventricular correction decreased from (30.5±12.3) mmHg (1 mmHg=0.133 kPa) to (9.9±9.6) mmHg (t=12.92, P<0.001), and the right ventricular to aortic pressure ratio decreased from 0.57±0.14 to 0.44±0.12 (t=7.44, P<0.001). The pressure of the superior vena cava after stenting in 5 patients after cavopulmonary anastomosis decreased from (17.0±1.9) mmHg to (14.0±0.7) mmHg (t=2.86, P=0.046). Two patients died during reoperation for repairing other cardiac malformations. The remaining 31 patients were clinically stable during the follow-up period of (5.3±1.6) years, and one stent fracture was found on chest X-ray. Cardiac catheterization reexaminations in 16 patients showed that restenosis was found in one stent, while stent position and patency were satisfactory in the remaining stents. Nine children underwent post-dilation without stent fracture, displacement or aneurysm formation. Cardiac tomography showed no stent stenosis, fracture observed, or significant change in diameter of the stent in 8 patients. The inner diameter and pulmonary blood perfusion could not be accurately evaluated due to artifacts by cardiac magnetic resonance imaging in 4 patients. Conclusions: Pul-Stent has good compliance and adequate radial strength, and can dilate further over time to accommodate for somatic growth. It performs safely and effectively in treating post-operative branch pulmonary artery stenosis in children.

Child , Female , Humans , Male , China , Follow-Up Studies , Heart Defects, Congenital/surgery , Pulmonary Artery/surgery , Retrospective Studies , Stenosis, Pulmonary Artery/surgery , Stents , Treatment Outcome , Vena Cava, Superior
Chinese Journal of Cardiology ; (12): 49-54, 2022.
Article in Chinese | WPRIM | ID: wpr-935102


Objective: To explore the efficacy and safety of percutaneous transluminal pulmonary angioplasty (PTPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: This prospective single arm study included 19 CTEPH patients (7 male, age(56.3±12.5)years) admitted to Wuhan Asia Heart Hospital from January 2017 to June 2019 and received PTPA interventional therapy. Baseline data, including age, sex, WHO functional class, 6-minute walk distance (6MWD), NT-proBNP, right heart catheterization values, were collected. Patients received single or repeated PTPA. Number of dilated vessels from each patient was analyzed, patients were followed up for 24 weeks and right heart catheterization was repeated at 24 weeks post initial PTPA. All-cause death, perioperative complications, and reperfusion pulmonary edema were reported. WHO functional class, 6MWD, NT-proBNP, right heart catheterization values were compared between baseline and at 24 weeks follow up. Results: Nineteen CTEPH patients received a total of 56 PTPA treatments. The pulmonary artery pressure (mPAP) decreased from (40.11±7.55) mmHg (1 mmHg=0.133 kPa) to (27.53±4.75) mmHg (P<0.001), and the total pulmonary resistance (TPR) decreased from (13.00±3.56) Wood U to (5.48±1.56) Wood U (P<0.001), cardiac output increased from (3.19±0.63) L/min to (5.23±0.94) L/minutes (P<0.01) at 24 weeks post PTPA. The WHO functional class improved significantly (P<0.001), 6MWD increased from (307.08±129.51) m to (428.00±112.64) m (P=0.002), the NT-proBNP decreased at 24 weeks post PTPA (P=0.002). During the follow-up period, there was no death; hemoptysis occurred in 4 patients during the operation, none of which resulted in serious adverse clinical consequences. One patient developed reperfusion pulmonary edema and recovered after treatment. Conclusion: PTPA treatment is safe and can significantly improve the hemodynamics and WHO functional class of patients with CTEPH.

Adult , Aged , Humans , Male , Middle Aged , Angioplasty , Angioplasty, Balloon , Chronic Disease , Hypertension, Pulmonary/surgery , Prospective Studies , Pulmonary Artery/surgery , Pulmonary Embolism , Treatment Outcome
China Journal of Chinese Materia Medica ; (24): 1024-1030, 2022.
Article in Chinese | WPRIM | ID: wpr-928022


This study investigated the effect of salidroside on phenotypic transformation of rat pulmonary artery smooth muscle cells(PASMCs) induced by hypoxia. Rat pulmonary arteries were isolated by tissue digestion and PASMCs were cultured. The OD values of cells treated with salidroside at different concentrations for 48 hours were measured by cell counting kit-8(CCK-8) to determine the appropriate concentration range of salidroside. The cells were divided into a normal(normoxia) group, a model(hypoxia) group, and three hypoxia + salidroside groups(40, 60, and 80 μg·mL~(-1)). Quantitative real-time PCR(qRT-PCR) was used to detect the mRNA expression of cell contractile markers in each group, such as α-smooth muscle actin(α-SMA), smooth muscle 22(SM22), and calcium-binding protein(calponin), and synthetic marker vimentin. The expression levels of cell phenotypic markers and proliferating cell nuclear antigen(PCNA) were detected by Western blot. The proliferation of cells in each group was detected by the 5-ethynyl-2'-deoxyuridine(EdU) assay. Cell migration was measured by Transwell assay. As revealed by results, compared with the normal group, the model group showed decreased mRNA and protein expression of contractile phenotypic markers of PASMCs and increased mRNA and protein expression of synthetic markers. Compared with the conditions in the model group, salidroside could down-regulate the mRNA and protein expression of synthetic markers in PASMCs and up-regulated the mRNA and protein expression of contractile phenotypic markers. Compared with the normal group, the model group showed potentiated proliferation and migration. Compared with the model group, the hypoxia + salidroside groups showed blunted proliferation and migration of cells after phenotypic transformation. The results suggest that salidroside can inhibit the expression of synthetic markers in PASMCs and promote the expression of contractile markers to inhibit the hypoxia-induced phenotypic transformation of PASMCs. The mechanism of salidroside in inhibiting the proliferation and migration of PASMCs is related to the inhibition of the phenotypic transformation of PASMCs.

Animals , Rats , Cell Proliferation , Cells, Cultured , Glucosides , Hypoxia , Myocytes, Smooth Muscle , Phenols , Pulmonary Artery
ABC., imagem cardiovasc ; 35(1): eabc242, 2022. ilus, tab
Article in Portuguese | LILACS | ID: biblio-1371030


Fundamento: O tempo de aceleração na artéria pulmonar (TAP) pode ser utilizado para avaliação da hipertensão pulmonar na análise da função diastólica do ventrículo esquerdo. Objetivo: Avaliar se existe correlação entre o valor do tempo de aceleração na artéria pulmonar e parâmetros da função diastólica do ventrículo esquerdo em indivíduos com função sistólica do ventrículo esquerdo preservada e de acordo com sexo, idade e fatores de risco cardiovasculares. Métodos: Estudo observacional, transversal. Foram selecionados 119 pacientes (59 mulheres; 49,6%). Os indivíduos foram submetidos ao ecocardiograma transtorácico incluindo os valores de tempo de aceleração na artéria pulmonar; ondas E e A e relação E/A ao Doppler espectral do influxo mitral; ondas e' septal, e' lateral e relação E/e' ao Doppler tecidual do anel mitral; pressão sistólica na artéria pulmonar e volume atrial esquerdo. Resultados: No sexo feminino, foi encontrada correlação positiva (coeficiente de correlação de Spearman) entre o valor do tempo de aceleração na artéria pulmonar e e' lateral (coeficiente de correlação de Spearman de 0,47; p=0,002), relação E/A (coeficiente de correlação de Spearman de 0,32; p=0,04) e e' septal (coeficiente de correlação de Spearman de 0,36; p=0,023) e uma correlação negativa entre o valor do tempo de aceleração na artéria pulmonar e pressão sistólica na artéria pulmonar (coeficiente de correlação de Spearman de -0,43; p=0,034). No sexo masculino, não foi encontrada correlação significativa. Foram encontrados menores valores de tempo de aceleração na artéria pulmonar em mulheres com hipertensão arterial sistêmica quando comparadas a mulheres sem hipertensão arterial sistêmica (0,13±0,03 segundos versus 0,16±0,03 segundos; p = 0,015). Conclusão: O presente estudo mostrou correlação significativa dos valores do TAP com alguns parâmetros da função diastólica do ventrículo esquerdo apenas no sexo feminino, sendo que mulheres hipertensas apresentaram menores valores de TAP. (AU)

Background: Pulmonary artery acceleration time (PAAT) can be used as a parameter in the evaluation of pulmonary hypertension and aids left ventricular diastolic function (LVDF) analyses. Objective: To assess whether there is a correlation between PAAT and LVDF parameters in individuals with a preserved left ventricular systolic function and by sex, age, and cardiovascular risk factors. Method: Observational cross-sectional study. One hundred nineteen patients were selected (59 women [49.6%]). The subjects underwent transthoracic echocardiography including measurements of PAAT, E and A waves and E/A ratio, e' septal and e' lateral waves and E/e' ratio, pulmonary artery systolic pressure (PASP), and left atrial volume. Results: In female patients, a positive correlation (Spearman's correlation coefficient ­ Spearman correlation coefficient [SCC]) was found between the PAAT value and the lateral e' (SCC, 0.47; p = 0.002), with the E/A ratio (SCC, 0.32; p = 0.04), and with septal e' (SCC, 0.36; p = 0.023), and a negative correlation between PAAT and PASP (SCC, -0.43; p = 0.034). In men, no correlation was found between PAAT. and any parameters. Lower PAAT values were found in women with systemic arterial hypertension (hypertension) than in women without hypertension (0.13 ± 0.03 s versus 0.16 ± 0.03 s; p = 0.015). Conclusion: The present study showed a significant correlation between PAAT and some LVDF parameters in female patients only. Hypertension was correlated with lower PAAT values in women. (AU)

Humans , Male , Female , Adult , Middle Aged , Aged , Pulmonary Artery/physiology , Heart Ventricles/abnormalities , Hypertension, Pulmonary/diagnosis , Tobacco Use Disorder/complications , Coronary Artery Disease/complications , Echocardiography/methods , Statistics, Nonparametric , Echocardiography, Doppler, Pulsed/methods , Dyslipidemias/complications , Heart Disease Risk Factors