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1.
Zhonghua Er Ke Za Zhi ; 58(1): 25-29, 2020 Jan 02.
Artigo em Chinês | MEDLINE | ID: mdl-31905472

RESUMO

Objective: To improve the diagnosis and therapy of childhood pulmonary thromboembolism (PTE) by analyzing the clinical features of this rare condition. Methods: A total of 8 pediatric patients (4 males, 4 females) with PTE diagnosed in the Children's Hospital of Zhejiang University School of Medicine from March, 2014 to March, 2019 were enrolled. The clinical manifestation, laboratory results, imaging findings, diagnosis and treatment were summarized. Results: Among these 8 cases, aged from 9 hours to 14 years and 10 months. Fever was found in 4 cases, cough aggravation in 4, short of breath in 3, chest pain in 2, abdominal and back pain in one, hemoptysis in 2, cyanosis in 1, and edema of lower extremities in 2. Physical examination found decreased breath sound in 2 cases, phlegm rale in 3, and pleural friction rub in one. Pleural effusion was found in 5 cases by ultrasound. Plasma D-dimer increased in 6 cases (0.66-9.96 mg/L) and hypersensitive C-reactive protein elevated in 5 cases (10.78-78.00 mg/L). Chest enhanced CT showed pulmonary artery or venous filling defects, including pulmonary artery embolism in 7 cases and pulmonary vein embolism in one. The primary disease of these patients included Mycoplasma Pneumoniae pneumonia in 4 cases, nephritis in 2 and postoperative congenital heart disease in 2. Apart from one case who withdrew the treatment and was discharged, the other 7 patients received anticoagulant treatment had good outcome. Conclusions: For children with Mycoplasma pneumoniae pneumonia, immune disorders, long-term hormone therapy, cardiovascular invasive operation or other high-risk factors, PTE should be considered when fever, cough aggravation, short of breath, chest and back pain with pleural effusion are present. Chest enhanced CT scan should be performed as soon as possible, and anticoagulation should be started once the diagnosis is confirmed.


Assuntos
Pneumonia por Mycoplasma/complicações , Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico , Adolescente , Dor no Peito/diagnóstico por imagem , Criança , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Humanos , Masculino , Derrame Pleural , Pneumonia por Mycoplasma/diagnóstico , Embolia Pulmonar/complicações , Tomografia Computadorizada por Raios X , Resultado do Tratamento
2.
Zhonghua Yi Xue Za Zhi ; 100(1): 47-50, 2020 Jan 07.
Artigo em Chinês | MEDLINE | ID: mdl-31914558

RESUMO

Objective: To evaluate the clinical value of modified computed tomography angiography(CTA) in detecting bronchial artery-pulmonary artery fistula(BPF). Methods: Retrospective analysis was performed on 246 patients with hemoptysis admitted to the First Affiliated Hospital of Wenzhou Medical University from July 2017 to December 2018, who underwent modified CTA and DSA examination at the same time. CT was performed with Toshiba Aquilion one 320 row 640-slice spiral CT scanner. All modified CTA images were read blindly by two radiologists above the attending doctors. The sensitivity, specificity and accuracy of the modified CTA in diagnosing BPF were calculated with the DSA results as the reference,and the consistency of the two tests was analyzed. Results: DSA detected 186 cases of positive and 60 cases of negative, modified CTA detected 160 cases of positive and 86 cases of negative. The sensitivity,specificity and accuracy of modified CTA for BPF diagnosis was 85.5%(159/186),98.3%(59/60), 88.6%(218/246) respectively, and they were with high consistency with DSA examination results (kappa=0.73,P<0.01). Conclusion: Modified CTA has high diagnostic specificity for BPF,which can be used as the preferred method for non-invasive screening of suspected BPF patients.


Assuntos
Angiografia por Tomografia Computadorizada , Fístula , Artérias Brônquicas , Humanos , Artéria Pulmonar , Estudos Retrospectivos
3.
Zhonghua Wai Ke Za Zhi ; 57(12): 939-943, 2019 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-31826600

RESUMO

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(Q(R))), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model. Results: There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(Q(R))) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death. Conclusions: The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.


Assuntos
Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Cuidados Paliativos/métodos , Artéria Pulmonar/cirurgia , Procedimentos Cirúrgicos Vasculares/mortalidade , Feminino , Seguimentos , Ventrículos do Coração/anormalidades , Humanos , Lactente , Recém-Nascido , Masculino , Circulação Pulmonar , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodos
4.
Zhonghua Xin Xue Guan Bing Za Zhi ; 47(12): 993-999, 2019 Dec 24.
Artigo em Chinês | MEDLINE | ID: mdl-31877596

RESUMO

Objective: To investigate whether platelet-derived growth factor-BB (PDGF-BB) can regulate phenotypic transformation of pulmonary artery smooth muscle cells (PASMCs) via SIRT3 affecting glycolytic pathway. Methods: The PASMCs were isolated from Sprague Dawley rats. PASMCs were divided into 3 groups by using 2-deoxyglucose (2-DG), an inhibitor of the glycolytic pathway: normal control group, PDGF-BB group(30 ng/ml) and PDGF-BB (30 ng/ml)+2-DG (10 mmol/L) group. In lentivirus-mediated overexpression assay, cells were divided into control group, PDGF-BB group(30 ng/ml), PDGF-BB+deacetylase sirtuin-3 (SIRT3) overexpression group and PDGF-BB+empty vector group. The expression levels of phenotype related index such as α-smooth muscle actin (α-SMA), smooth muscle myosin heavy chain (SM-MHC), calponin, vimentin were detected by qRT-PCR and Western blot. Meanwhile, the expression of α-SMA was detected by cellular immunofluorescence staining. EDU staining was used to detect the proliferation of PASMCs. The expression of SIRT3 was detected by Western blot. The expressions of glucose transporter 1 and aerobic glycolytic enzymes were detected by qRT-PCR and Western blot in lentivirus-mediated overexpression assay. Results: (1) PDGF-BB affects PASMCs phenotypic transformation through glycolytic pathway: compared with normal control group, PDGF-BB significantly decreased the expressions of contractile phenotype markers such as α-SMA, SM-MHC, calponin mRNA and protein (all P<0.05), but it increased the expressions of the synthetic phenotype marker vimentin mRNA and protein (both P<0.05). Cellular immunofluorescence assay showed that PDGF-BB significantly decreased the number of α-SMA positive cells, while 2-DG reversed the process. (2) PDGF-BB promoted cell proliferation through glycolytic pathway: the proliferation of PASMCs was significantly higher in PDGF-BB group than in control group (P<0.05), and which could be significantly reduced by 2-DG (P<0.05). (3) PDGF-BB inhibited the expression of SIRT3 protein in PASMCs: the expression of SIRT3 protein in PDGF-BB group was lower than that in control group (P<0.05). (4) PDGF-BB affected glycolytic pathway through SIRT3:compared with the control group, PDGF-BB significantly increased the expression levels of glucose transporter 1 (Glut1), hexokinase 2 (HK2) and 6-phosphfructo-2-kinase 3 (PFKFB3) mRNA (all P<0.05), which was reserved by over-expression of SIRT3. There were no significant difference in mRNA expression levels between PDGF-BB group and PDGF-BB+empty vector group (P>0.05).Compared with the control group, PDGF-BB significantly increased the expression levels of Glut1, HK2 and PFKFB3 protein(all P<0.05), which was reserved by over-expression of SIRT3. There were no significant differences in protein expression levels between PDGF-BB group and PDGF-BB+empty vector group (all P>0.05). Conclusion: PDGF-BB regulates phenotypic transformation of PASMCs via SIRT3 affecting glycolytic pathway.


Assuntos
Becaplermina , Miócitos de Músculo Liso , Animais , Proliferação de Células , Células Cultivadas , Fenótipo , Proteínas Proto-Oncogênicas c-sis , Artéria Pulmonar , Ratos , Ratos Sprague-Dawley , Sirtuínas
5.
Zhonghua Fu Chan Ke Za Zhi ; 54(10): 660-665, 2019 Oct 25.
Artigo em Chinês | MEDLINE | ID: mdl-31648441

RESUMO

Objective: To analyze the pregnancy outcomes of fetal tetralogy of Fallot and to explore its prenatal diagnosis and treatment procedures. Methods: The clinical data of 63 cases of fetal tetralogy of Fallot (62 cases were singleton and 1 case was one of twin) were collected retrospectively from November, 2013 to November, 2017 in Beijing Obstetrics and Gynecology Hospital. Results: (1) Totally, 63 cases out of 46 352 pregnancies were diagnosed fetal tetralogy of Fallot by fetal ultrasonic cardiogram with about 0.136%(63/46 352) occurrence rate, and the mean gestational age was (23±3) weeks. And 50 cases (79%, 50/63) terminated pregnancy by induced labour. (2) Totally, 57 cases (90%,57/63) accepted genetic diagnosis.Eight cases (13%, 8/63) existed chromosome abnormality including 21-trimosy in 6 cases, 18-trisomy in 1 case and 22q11.2 microdeletion syndrome in 1 case; and these 8 cases were determined before 28 gestational weeks. (3) And 13 cases (21%, 13/63) of no fetal genetic abnormality selected to continue pregnancy. Twelve cases underwent full term delivery (5 cases were cesarean section delivery and 7 cases were vaginal delivery). Twelve newborns underwent surgical radical operation on heart malformation and got recovery. One case underwent preterm cesarean section at 35 gestational weeks for one of twin, and the newborn with tetralogy of Fallot was dead. The other the newborns survived and were followed up for tetralogy of Fallot surgery from 1 month to 3 years old after birth and recovered. Conclusions: Fetal tetralogy of Fallot mainly is diagnosed by ultrasonic cardiogram in the second trimester. The gestational age of diagnosis may be as early as 15 gestational weeks. Fetal tetralogy of Fallot with no genetic abnormality could underwent radical heart malformation operation after birth. It is necessary to undergo genetic testing on fetal tetralogy of Fallot and prenatal multidisciplinary counseling as well.


Assuntos
Resultado da Gravidez , Diagnóstico Pré-Natal/métodos , Tetralogia de Fallot/diagnóstico por imagem , Ultrassonografia Pré-Natal , Aorta/diagnóstico por imagem , Aorta/patologia , Cesárea , Feminino , Feto , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Gravidez , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Tetralogia de Fallot/diagnóstico
6.
Kyobu Geka ; 72(10): 829-833, 2019 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-31582704

RESUMO

There are 2 surgical approaches to secure and treat interlobar pulmonary artery in severe insufficient interlobar fissure of the lung. One is an interlobar approach which is called "fissure first technique". This approach is standard, but sometimes difficult to secure interlobar artery safely. So, lung parenchyma injury and accidental bleeding from the pulmonary artery become troublesome problems. On the other hand, hilar approach without contacting to interlobar fissure which called "hilar first technique" is very useful to avoid lung parenchyma injuries. There are some knack and pitfalls in every lobe of the lung. The key of this approach is to be familiar with the anatomical position of pulmonary artery running by confirming preoperative 3-dimensional computed tomography( 3D-CT). In the final step of this technique cutting the interlobar line exactly is more important to prevent atelectasis and congestion of residual lung. It is very important for thoracic surgeons to know the details of both approaches in the surgical treatment of severe insufficient interlobar fissure of the lung.


Assuntos
Pulmão , Artéria Pulmonar , Pneumonectomia , Tomografia Computadorizada por Raios X
7.
Cancer Radiother ; 23(8): 926-929, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31611052

RESUMO

Stereotactic body radiation therapy is still controversial for inoperable patients with central lung lesion. We report the case of a 59-year-old woman with previous history of head and neck squamous cell carcinoma who was treated by lung stereotactic body irradiation for an inoperable lymph node in station 10R. One year after, a fibroscopy showed a necrosis of the right main bronchus mucosae and the CT showed a radio-induced aneurysm protruding into the right inferior lobular bronchus. The patient eventually died a few hours later with a massive haemoptysis. This case highlights the potential toxicity of central lung stereotactic body radiation therapy and raises the question of its legitimacy.


Assuntos
Aneurisma/etiologia , Carcinoma Pulmonar de Células não Pequenas/radioterapia , Neoplasias Pulmonares/radioterapia , Irradiação Linfática/efeitos adversos , Artéria Pulmonar/efeitos da radiação , Radiocirurgia/efeitos adversos , Aneurisma/diagnóstico por imagem , Carcinoma Pulmonar de Células não Pequenas/patologia , Carcinoma de Células Escamosas/terapia , Fracionamento da Dose de Radiação , Feminino , Neoplasias de Cabeça e Pescoço/terapia , Humanos , Neoplasias Pulmonares/patologia , Irradiação Linfática/métodos , Pessoa de Meia-Idade , Neoplasias Primárias Desconhecidas/terapia , Artéria Pulmonar/diagnóstico por imagem , Radiocirurgia/métodos
8.
Nihon Hoshasen Gijutsu Gakkai Zasshi ; 75(10): 1165-1172, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31631110

RESUMO

There are many variations in branching and running of pulmonary artery (PA) and pulmonary vein (PV). It is desirable to separate as a surgical simulation of lung cancer and important to grasp before video-assisted thoracic surgery (VATS) to perform quick and safe. Therefore, the purpose of this study was to evaluate objective and subjective image quality (contrast attenuation, separation ability, and vascular visualization) of PA and PV of splitbolus single-phase protocol (SBSPP) in preoperative three-dimensional computed tomography angiography (3DCTA). CT value of PA was 410.2±71.0 Hounsfield unit (HU), PV was 245.1±24.8 HU, difference between CT value of PA and CT value of PV was 164.5±60.9 HU. Subjective image quality of PA and PV could be visualized until more than the segmental branch level. SBSPP can obtain sufficient CT value for separate visualization of PA and PV, and before VATS useful PA and PV 3D-CTA imaging.


Assuntos
Neoplasias Pulmonares , Cirurgia Torácica Vídeoassistida , Tomografia Computadorizada por Raios X , Meios de Contraste/administração & dosagem , Humanos , Imagem Tridimensional , Neoplasias Pulmonares/diagnóstico por imagem , Artéria Pulmonar , Cirurgia Torácica Vídeoassistida/métodos
9.
Zhonghua Jie He He Hu Xi Za Zhi ; 42(10): 755-759, 2019 Oct 12.
Artigo em Chinês | MEDLINE | ID: mdl-31594109

RESUMO

Objective: To analyze the clinical characteristics of pulmonary embolism patients from different altitudes in plateau areas. Methods: A retrospective cross-sectional study was used to analyze the patients with acute pulmonary embolism diagnosed definitely by pulmonary angiography or pulmonary artery CT angiography admitted to Tibet Autonomous Region People's Hospital from August 2014 to December 2018. The subjects were divided into 3 groups according to the altitude of long-term residence before onset, i.e. low-altitude group (group 1, 2 700 m ≤ altitude ≤3 700 m, n=44), medium-altitude group (group 2, 3 700 m

Assuntos
Altitude , Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Dor no Peito/epidemiologia , China/epidemiologia , Angiografia por Tomografia Computadorizada , Estudos Transversais , Dispneia/epidemiologia , Humanos , Hipóxia , Incidência , Embolia Pulmonar/epidemiologia , Estudos Retrospectivos
11.
Life Sci ; 238: 116974, 2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31639399

RESUMO

AIM: Analyze the effects of voluntary running during the development of pulmonary arterial hypertension (PAH) induced by monocrotaline (MCT) on the right ventricle (RV) structure, RV myocyte contractility and intracellular Ca2+ transient in rats with MCT-induced PAH. MAIN METHODS: Male Wistar rats were housed sedentary or with free access to a running wheel after MCT or saline injection for until HF or median end-point day of HF in sedentary animals (24 days). Echocardiographic examination and exercise tolerance test were carried out at specific time points of the experimental period. After euthanasia, the heart was dissected, weighed and processed for either histological or single myocyte contractility and intracellular Ca2+ transient analyzes. KEY FINDINGS: Voluntary running delayed the onset of HF (29 days) and the increase in pulmonary artery resistance, and improved exercise tolerance. In the median end-point day of HF, exercise retarded RV adverse remodeling (i.e. increase in extracellular matrix and collagen content). At this stage, exercise also delayed impairments in cell contractile function (i.e. amplitude and times to peak and to half relaxation) and intracellular calcium cycling (i.e. amplitude and times to peak and to half decay) in RV single myocytes. SIGNIFICANCE: Along with HF onset delay and physical effort tolerance enhancement, voluntary running during the development of PAH postpones pulmonary artery resistance increases, RV adverse remodeling and myocyte contractility and intracellular calcium cycling deterioration in rats. Therefore, self-paced intermittent exercise of high intensity may contribute positively to the health and survival of individuals with PAH.


Assuntos
Insuficiência Cardíaca/prevenção & controle , Hipertensão Pulmonar/complicações , Hipertrofia Ventricular Direita/prevenção & controle , Contração Muscular , Miócitos Cardíacos/patologia , Condicionamento Físico Animal , Artéria Pulmonar/patologia , Remodelação Ventricular , Animais , Cálcio , Modelos Animais de Doenças , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/patologia , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/patologia , Masculino , Ratos , Ratos Wistar , Corrida
12.
Medicine (Baltimore) ; 98(38): e17212, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31567975

RESUMO

RATIONALE: Anomalous Origin of Left Coronary Arteries from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland (BWG), is a rare form of coronary artery anomaly that is usually discovered in the first few months of life. Only rarely can patients with this anomaly reach adulthood without symptoms. PATIENT CONCERNS: A 28-year-old female was witnessed suddenly collapse with a seizure-like episode by her colleagues at work. DIAGNOSIS AND INTERVENTION: Routine cardiopulmonary resuscitation was performed by emergency medical service technologists. The patient was unable to be revived. Postmortem examination revealed the patient had ALCAPA with a focal chronic ischemic injury of the left ventricle. Moreover, a high take-off of the right coronary artery was also discovered. OUTCOMES: The patient passed away due to ALCAPA. The mechanism of death was cardiac arrhythmia being triggered by myocardial ischemic changes. LESSONS: In the rare cases where ALCAPA manifests in an asymptomatic adult, the mortality rate is very high. This case demonstrates the importance of awareness of such patients living under the tremendous risk of sudden cardiac death.


Assuntos
Anomalias dos Vasos Coronários/patologia , Artéria Pulmonar/anormalidades , Adulto , Arritmias Cardíacas/etiologia , Doenças Assintomáticas , Autopsia , Morte Súbita Cardíaca , Feminino , Humanos
13.
Medicine (Baltimore) ; 98(40): e17369, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31577738

RESUMO

This study aimed to analyze the correlation between the efficiency coefficient of right ventricular-pulmonary artery coupling (ηvv) and the prognosis of patients with pulmonary arterial hypertension (PAH).A total of 64 patients who underwent right heart catheterization (RHC) were enrolled and divided into PAH and control groups depending on the RHC results. Pressure and volumetric methods were adopted to analyze the results of RHC and cardiac magnetic resonance imaging examination. The ηvv of patients in 2 groups were calculated, and the relationship between ηvv calculated by the 2 methods and the 2-year prognosis of patients with PAH was evaluated.The hemodynamic index and right ventricular-pulmonary artery coupling parameter of patients with PAH were significantly higher than those in the control group (P < .05). The right ventricular volume parameter in the PAH group was significantly different from that in the control group (P < .05). For patients with PAH, the end-systolic elastance/effective arterial elastance (Ees/Ea) calculated by the volumetric method was significantly related to the prognosis of patients (odds ratio = 0.192, 95% confidence interval: 0.042-0.868, P = .032). When Ees/Ea <0.67 was calculated by the volumetric method, the adverse prognosis of patients with PAH increased significantly (P < .05).The Ees/Ea calculated by the volumetric method may be better an independent factor for the prognosis of patients with PAH.


Assuntos
Hemodinâmica/fisiologia , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Idoso , Cateterismo Cardíaco , Criança , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Contração Miocárdica/fisiologia , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Volume Sistólico , Adulto Jovem
14.
Medicine (Baltimore) ; 98(40): e17449, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31577770

RESUMO

BACKGROUND: Pulmonary artery sling (PAS) is rare, often with tracheal stenosis. And the postoperative mortality is high. For now, there is no consensus on the tracheoplasty for the patients with PAS and tracheal stenosis. METHODS: Studies involving surgical repair of PAS and tracheal stenosis with and without tracheoplasty were identified by searching the PubMed, EMBASE, and the Cochrane Library databases until June 5, 2019. The assessed variables included ventilation time, early and late mortality, and symptom at follow-up. A random-effect/fixed-effect model was used to summarize the estimates of the mean difference (MD)/risk ratio (RR) with 95% confidence interval (CI). RESULTS: This study will be submitted to a peer-reviewed journal for publication. CONCLUSION: This study will assess the safety and efficacy of tracheoplasty for patients with PAS and tracheal stenosis, and provide more evidence-based guidance in clinical practice. PROSPERO REGISTRATION NUMBER: CRD42019139788.


Assuntos
Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/anormalidades , Procedimentos Cirúrgicos Reconstrutivos , Estenose Traqueal/complicações , Estenose Traqueal/cirurgia , Humanos
16.
ABC., imagem cardiovasc ; 32(4): 318-30, out.-dez. 2019. ilus
Artigo em Português | LILACS | ID: biblio-1024061

RESUMO

A doença pulmonar hipertensiva pode ser definida como um conjunto de alterações fisiopatológicas pulmonares que resultam em uma patologia grave, progressiva e com alta morbimortalidade. O ecocardiograma transtorácico é um método de imagem de fácil acesso e essencial para avaliação desta doença, principalmente na faixa pediátrica, na qual há limitações para realização frequente e de rotina do cateterismo direito. Nesta revisão, abordaremos as principais técnicas ecocardiográficas para o diagnóstico e a avaliação hemodinâmica da hipertensão pulmonar na população pediátrica. O diagnóstico precoce e o adequado estadiamento no acompanhamento das intervenções clínicas são fundamentais para escolha assertiva da abordagem terapêutica e, consequentemente, melhora do desfecho clínico


Assuntos
Humanos , Masculino , Feminino , Pediatria , Ecocardiografia/métodos , Criança , Hipertensão Pulmonar/mortalidade , Artéria Pulmonar , Valva Tricúspide , Veia Cava Inferior , Cateterismo Cardíaco , Ecocardiografia Doppler/métodos , Disfunção Ventricular Direita , Disfunção Ventricular Esquerda , Átrios do Coração , Ventrículos do Coração
17.
Rev Port Cir Cardiotorac Vasc ; 26(2): 147-149, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31476817

RESUMO

We report the case of a 44 year-old patient with complex ACHD, admitted with acute decompensated heart failure (ADHF) in hemodynamic profile B. He had a single ventricle with pulmonary atresia, previously submitted to three modified Blalock-Taussig shunts (mBTs) at the age of 2, 12 and 19 years old. Despite conventional treatment with diuretics, ß-blockers (BB) and isosorbide dinitrate the patient progressed to profile C and the transthoracic echocardiogram disclosed a reduced systolic function. Likewise, levosimendan was commenced and an appropriate decongestion and a marked reduction in the NT-proBNP were seen. Treatment with angiotensin-converting-enzyme inhibitor, BB, ivabradine and mineralocorticoid receptor was optimized. The patient was discharged home after 26 days in NYHA class III and referred for heart transplant after right heart catheterization. To our knowledge, this is the first report of successful levosimendan's use in ADHF in a mBTs long-term survivor.


Assuntos
Fármacos Cardiovasculares/uso terapêutico , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/tratamento farmacológico , Ventrículos do Coração/anormalidades , Simendana/uso terapêutico , Adulto , Procedimento de Blalock-Taussig , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/cirurgia , Humanos , Masculino , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Resultado do Tratamento
18.
Isr Med Assoc J ; 21(8): 528-531, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31474014

RESUMO

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, distinct pulmonary vascular disease, which is caused by chronic obstruction of major pulmonary arteries. CTEPH can be cured by pulmonary endarterectomy (PEA). PEA for CTEPH is a challenging procedure, and patient selection and the perioperative management are complex, requiring significant experience. OBJECTIVES: To describe the establishment of a national CTEPH-PEA center in Israel and present results of surgery. METHODS: In this study, we reviewed the outcomes of PEA in a national referral, multi-disciplinary center for CTEPH-PEA. The center was established by collaborating with a high-volume center in Europe. A multidisciplinary team from our hospital (pulmonary hypertension specialist, cardiac surgeon, cardiac anesthesiologist and cardiac surgery intensivist was trained under the guidance of an experienced team from the European center. RESULTS: A total of 38 PEA procedures were performed between 2008 and 2018. We included 28 cases in this analysis for which long-term follow-up data were available. There were two hospital deaths (7%). At follow-up, median New York Heart Association (NYHA) class improved from III to I (P < 0.0001), median systolic pulmonary pressure decreased from 64 mmHg to 26 mmHg (P < 0.0001), and significant improvements were seen in right ventricular function and exercise capacity. CONCLUSIONS: A national center for performance of a rare and complex surgical procedure can be successfully established by collaboration with a high-volume center and by training a dedicated multidisciplinary team.


Assuntos
Endarterectomia/métodos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/cirurgia , Embolia Pulmonar/complicações , Embolia Pulmonar/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Feminino , Humanos , Israel , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/cirurgia , Encaminhamento e Consulta , Fatores de Risco , Resultado do Tratamento , Adulto Jovem
19.
Nat Commun ; 10(1): 4143, 2019 09 12.
Artigo em Inglês | MEDLINE | ID: mdl-31515519

RESUMO

In pulmonary hypertension vascular remodeling leads to narrowing of distal pulmonary arterioles and increased pulmonary vascular resistance. Vascular remodeling is promoted by the survival and proliferation of pulmonary arterial vascular cells in a DNA-damaging, hostile microenvironment. Here we report that levels of Eyes Absent 3 (EYA3) are elevated in pulmonary arterial smooth muscle cells from patients with pulmonary arterial hypertension and that EYA3 tyrosine phosphatase activity promotes the survival of these cells under DNA-damaging conditions. Transgenic mice harboring an inactivating mutation in the EYA3 tyrosine phosphatase domain are significantly protected from vascular remodeling. Pharmacological inhibition of the EYA3 tyrosine phosphatase activity substantially reverses vascular remodeling in a rat model of angio-obliterative pulmonary hypertension. Together these observations establish EYA3 as a disease-modifying target whose function in the pathophysiology of pulmonary arterial hypertension can be targeted by available inhibitors.


Assuntos
Proteínas de Ligação a DNA/metabolismo , /fisiopatologia , Remodelação Vascular , Animais , Apoptose/efeitos dos fármacos , Benzobromarona/análogos & derivados , Benzobromarona/farmacologia , Cardiomegalia/complicações , Cardiomegalia/patologia , Cardiomegalia/fisiopatologia , Proliferação de Células/efeitos dos fármacos , Sobrevivência Celular/efeitos dos fármacos , Dano ao DNA , Reparo do DNA/efeitos dos fármacos , Proteínas de Ligação a DNA/antagonistas & inibidores , Ventrículos do Coração/efeitos dos fármacos , Ventrículos do Coração/patologia , Hipóxia/complicações , Hipóxia/fisiopatologia , Masculino , Camundongos Transgênicos , Miócitos de Músculo Liso/efeitos dos fármacos , Miócitos de Músculo Liso/metabolismo , Artéria Pulmonar/patologia , Ratos Sprague-Dawley , Remodelação Vascular/efeitos dos fármacos
20.
Zhonghua Er Ke Za Zhi ; 57(9): 705-709, 2019 Sep 02.
Artigo em Chinês | MEDLINE | ID: mdl-31530357

RESUMO

Objective: To characterize the clinical features and outcomes of scimitar syndrome (SS) to aid the understanding of this syndrome. Methods: This retrospective study included 6 children who were diagnosed with SS at the pediatric cardiovascular center of Beijing Anzhen Hospital from January 2012 to September 2018. SS was diagnosed by echocardiography and confirmed by cardiac computed tomography angiography(CTA) or surgery. All data were collected to analyze the clinical and imaging characteristics and prognosis. Results: Among the 6 SS children (aged 2 months to 15 years; 5 males) weighed 5.6-17.1 kg. Three cases were infant type, the clinical manifestations were recurrent respiratory tract infection with growth retardation, including 2 cases with severe pulmonary hypertension, while 3 cases with adult type, were asymptomatic. Cardiac CTA imaging showed that the right single or all pulmonary veins descended through the diaphragm and converged into the inferior vena cava. One case was isolated infracardiac partial anomalous pulmonary venous connection (PAPVC) without other malformations. The remaining 5 cases complicated with atrial septal defect, different vascular and trachea malformations as well as spinal malformations. Vascular malformations included pulmonary veins stenosis, abnormal origin of pulmonary artery branches, collateral branches of systemic artery supplying local lung tissue, and persistent left superior vena cava. The treatment varied according to the specific location of anomalous pulmonary venous connection, the degree of pulmonary hypertension and the severity of clinical symptoms. Four cases underwent one-stage radical surgery, one case accepted intervention to occlude the collateral artery which was supplying the right lower lung and received stage Ⅱ radical surgery half a year later, and the remaining one case died from pulmonary hypertension crisis preoperation. Conclusions: Isolated SS can easily miss diagnosis due to mild clinical symptoms. Patients with complicated malformations can benefit from combination therapy. SS associated with severe pulmonary hypertension can lead to early death. Therefore, early diagnosis and appropriate treatment can improve the prognosis of patients.


Assuntos
Anormalidades Múltiplas/diagnóstico , Síndrome de Cimitarra/diagnóstico , Adolescente , Criança , Pré-Escolar , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Comunicação Interatrial/complicações , Humanos , Lactente , Masculino , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Infecções Respiratórias/complicações , Estudos Retrospectivos , Síndrome de Cimitarra/etiologia , Síndrome de Cimitarra/mortalidade , Síndrome de Cimitarra/terapia
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