الملخص
Objective: To analyze the classification, diagnosis and treatment status of patients with pulmonary hypertension (PH) in Yunnan province. Methods: This was a retrospective study. Hospitalized patients with PH at Yan'an Affiliated Hospital of Kunming Medical University from January 2012 to December 2019 were enrolled. The clinical data of enrolled patients, including demographic data, comorbidities, targeted drug therapy, echocardiography and right heart catheterization results, were obtained through the electronic medical record system. The composition ratio of PH, diagnosis and treatment were analyzed. Results: A total of 13 590 patients with PH were enrolled, accounting for 3.09% (13 590/440 056) of the total number of hospitalizations during the same period. The composition of PH was predominantly pulmonary arterial hypertension (PAH) (55.50% (7 542/13 590)), followed by pulmonary hypertension (PH) caused by left heart disease (24.16% (3 284/13 590)). Among them, PAH could be subdivided into four types: idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disease, PAH associated with portal hypertension, and PAH associated with congenital heart disease (CHD-PAH), with CHD-PAH as the predominating type (98.09% (7 398/7 542). Patients with PAH were predominantly adolescents. In hospitalized patients with PH, from 2012 to 2019, the proportion of children and adolescents showed a decreasing trend from year to year, and the proportion of middle-aged and older adults showed a significant increasing trend, and the proportion of female patients showed a gradual decreasing trend, and the proportion of patients with comorbid hypertension, diabetes mellitus, coronary artery disease, arrhythmia, and pneumonia showed an increasing trend. A total of 1 034 patients (7.61% (1 034/13 590)) underwent right heart catheterization. The concordance rate between echocardiographic and right heart catheterization findings was (86.98% (875/1 006)). A total of 2 574 (18.94%) of PH patients were treated with PAH targeted drugs, of which 58.16% (1 497/2 574) were treated with monotherapy. Among the PH patients treated with PAH targeted drugs, the majority of patients were PAH patients (86.44% (2 225/2 574)), and 83.53% (2 150/2 574) patients treated with PAH targeted drugs were CHD-PAH. Conclusions: Hospitalized PH patients in our center between 2012 and 2019 are predominantly CHD-PAH, and the proportion of patients receiving right heart catheterization and targeted drug therapy is relatively low. The percentage of middle-aged and elderly PH patients shows an increasing trend from year to year, as well as the percentage of those with concomitant comorbidities.
الموضوعات
Child , Aged , Adolescent , Middle Aged , Humans , Female , Hypertension, Pulmonary/therapy , Retrospective Studies , China/epidemiology , Familial Primary Pulmonary Hypertension , Pulmonary Arterial Hypertension/complications , Heart Defects, Congenitalالملخص
Objective: To analyze the classification, diagnosis and treatment status of patients with pulmonary hypertension (PH) in Yunnan province. Methods: This was a retrospective study. Hospitalized patients with PH at Yan'an Affiliated Hospital of Kunming Medical University from January 2012 to December 2019 were enrolled. The clinical data of enrolled patients, including demographic data, comorbidities, targeted drug therapy, echocardiography and right heart catheterization results, were obtained through the electronic medical record system. The composition ratio of PH, diagnosis and treatment were analyzed. Results: A total of 13 590 patients with PH were enrolled, accounting for 3.09% (13 590/440 056) of the total number of hospitalizations during the same period. The composition of PH was predominantly pulmonary arterial hypertension (PAH) (55.50% (7 542/13 590)), followed by pulmonary hypertension (PH) caused by left heart disease (24.16% (3 284/13 590)). Among them, PAH could be subdivided into four types: idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disease, PAH associated with portal hypertension, and PAH associated with congenital heart disease (CHD-PAH), with CHD-PAH as the predominating type (98.09% (7 398/7 542). Patients with PAH were predominantly adolescents. In hospitalized patients with PH, from 2012 to 2019, the proportion of children and adolescents showed a decreasing trend from year to year, and the proportion of middle-aged and older adults showed a significant increasing trend, and the proportion of female patients showed a gradual decreasing trend, and the proportion of patients with comorbid hypertension, diabetes mellitus, coronary artery disease, arrhythmia, and pneumonia showed an increasing trend. A total of 1 034 patients (7.61% (1 034/13 590)) underwent right heart catheterization. The concordance rate between echocardiographic and right heart catheterization findings was (86.98% (875/1 006)). A total of 2 574 (18.94%) of PH patients were treated with PAH targeted drugs, of which 58.16% (1 497/2 574) were treated with monotherapy. Among the PH patients treated with PAH targeted drugs, the majority of patients were PAH patients (86.44% (2 225/2 574)), and 83.53% (2 150/2 574) patients treated with PAH targeted drugs were CHD-PAH. Conclusions: Hospitalized PH patients in our center between 2012 and 2019 are predominantly CHD-PAH, and the proportion of patients receiving right heart catheterization and targeted drug therapy is relatively low. The percentage of middle-aged and elderly PH patients shows an increasing trend from year to year, as well as the percentage of those with concomitant comorbidities.
الموضوعات
Child , Aged , Adolescent , Middle Aged , Humans , Female , Hypertension, Pulmonary/therapy , Retrospective Studies , China/epidemiology , Familial Primary Pulmonary Hypertension , Pulmonary Arterial Hypertension/complications , Heart Defects, Congenitalالملخص
Objective: To explore the short-term efficacy of fenestrated atrial septal defect (ASD) occulders in the treatment of pulmonary arterial hypertension (PAH). Methods: Thirty-six healthy dogs were divided into the balloon atrial septostomy (BAS)+fenestrated ASD occulders group (n=12), BAS group (n=12) and non-septostomy group (n=12). PAH was induced by intra-atrial injection of dehydrogenized monocrotaline (1.5 mg/kg) in all dogs. Animals in the BAS+fenestrated ASD occulders group underwent atrial septal puncture and fenestrated ASD occulders implantation. Animals in the BAS group underwent balloon atrial septostomy. The non-septostomy group received no surgical intervention. The hemodynamic indexes and blood N-terminal pro-B-type natriuretic peptide (NT-proBNP) of dogs were measured before modeling, 2 months after modeling, 1, 3, and 6 months after surgery, respectively. Echocardiography was performed to observe the patency of the shunt and atrial septostomy of the dogs in the BAS+fenestrated ASD occulders group and BAS group at 1, 3, and 6 months after surgery. Three dogs were sacrificed in each group at 1, 3, and 6 months after surgery, respectively. Atrial septal tissue and fenestrated ASD occulders were removed to observe the patency and endothelialization of the device. Lung tissues were obtained for hematoxylin-eosin (HE) staining to observe the inflammatory cells infiltration and the thickening and narrowing of the pulmonary arterials. Results: Among 36 dogs, 2 dogs died within 24 hours after modeling, and 34 dogs were assigned to BAS+fenestrated ASD occulders group (n=12), BAS group (n=11), and non-septostomy group (n=11). Compared with BAS group, the average right atrial pressure (mRAP) and NT-proBNP of dogs in the BAS+fenestrated ASD occulders group were significantly reduced at 3 months after surgery (P<0.05), and the cardiac output (CO) was significantly increased at 6 months after surgery, arterial oxygen saturation (SaO2) was also significantly reduced (P<0.05). Compared with non-septostomy group, dogs in the BAS+fenestrated ASD occulders group had significantly lower mRAP and NT-proBNP at 1, 3, and 6 months after surgery (P<0.05), and higher CO and lower SaO2 at 6 months after surgery (P<0.05). Compared with the non-septostomy group, the dogs in the BAS group had significantly lower mRAP and NT-proBNP at 1 month after surgery (P<0.05), and there was no significant difference on mRAP and NT-proBNP at 3 and 6 months after surgery (P>0.05). Echocardiography showed that there was a minimal right-to-left shunt in the atrial septum in the BAS group at 1 month after the surgery, and the ostomy was closed in all the dogs in the BAS group at 3 months after the surgery. There was still a clear right-to-left shunt in the dogs of BAS+fenestrated ASD occulders group. The shunt was well formed and satisfactory endothelialization was observed at 1, 3 and 6 months after surgery. The results of HE staining showed that the pulmonary arterials were significantly thickened, stenosis and collapse occurred in the non-septostomy group. Pulmonary microvascular stenosis and inflammatory cell infiltration in the pulmonary arterials were observed in the non-septostomy group. Pulmonary arterial histological results were comparable between BAS+fenestrated ASD occulders group and non-septostomy group at 6 months after surgery . Conclusions: The fenestrated ASD occulder has the advantage of maintaining the open fistula hole for a longer time compared with simple balloon dilation. The fenestrated ASD occulder can improve cardiac function, and it is safe and feasible to treat PAH in this animal model.
الموضوعات
Animals , Dogs , Atrial Septum/surgery , Cardiac Catheterization/methods , Familial Primary Pulmonary Hypertension , Heart Septal Defects, Atrial/surgery , Hypertension, Pulmonary , Pulmonary Arterial Hypertensionالملخص
Objective: To explore the impact of composite clinical worsening (cCW) events and its components on the prognosis of patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH). Methods: This is a retrospective study. Patients who were diagnosed with CHD-PAH in Beijing Anzhen Hospital between January 2007 and July 2018, were included, and their baseline clinical data including demographic, clinical manifestations and New York Heart Association (NYHA) classification were collected retrospectively. All-cause deaths and clinical worsening events were recorded, which included syncope, PAH related hospitalization, NYHA classification deterioration and ≥ 2 PAH related clinical symptoms (dyspnea, hemoptysis, edema, chest pain, palpitations, cyanosis) appearance/progress. Three kinds of cCW events were defined: cCW1 (included PAH related hospitalization, NYHA classification deterioration), cCW2 (increased syncope on the basis of cCW1) and cCW3 (increased ≥ 2 PAH related clinical symptoms appearance/progress on the basis of cCW2). The Kaplan-Meier survival curve was used to analyze the long-term survival of the included patients. Univariate and multivariate Cox regression models were used to evaluate the impact of cCW events and their components on the risk of all-cause mortality. Results: A total of 525 patients with CHD-PAH were included in this study. The median age at diagnosis was 20.7 (11.2, 30.3) years. There were 43.8% children (<18 years), and 68.8% female patients. There were 431 patients (82.1%) with NYHA classification II. A total of 180 patients had PAH symptoms at diagnosis. The median follow-up time was 4.5 (2.6, 6.7) years. Forty-seven patients (9.0%) died during the follow-up period. Survival rates at 1, 5 and 10 years after diagnosis of PAH were 98.0%, 89.9% and 84.4%, respectively. Cox multivariate analysis showed that NYHA classification deterioration (HR=3.901, 95%CI 1.863-8.169, P<0.001), ≥2 PAH symptoms appearance/progress (HR=4.458, 95%CI 1.870-10.625, P<0.001), PAH-related hospitalization (HR=4.058, 95%CI 1.851-8.896, P<0.001) and syncope (HR=11.313, 95%CI 4.860-26.332, P<0.001) were independent predictors of increased risk of death. All 3 kinds of cCW events were significantly associated with the significantly increased risk of death, and cCW2 was highly predictive to increased risk of death (HR=15.476, 95%CI 4.346-37.576, P<0.001). Conclusions: The overall long-term prognosis of CHD-PAH patients in this study is relatively good. cCW events and its components (NYHA classification deterioration, ≥2 PAH symptoms occurrence/worsening, PAH-related hospitalization and syncope) have adverse influence on all-cause death in this patient cohort.
الموضوعات
Child , Female , Humans , Male , Familial Primary Pulmonary Hypertension/complications , Heart Defects, Congenital/complications , Hypertension, Pulmonary/complications , Pulmonary Arterial Hypertension , Retrospective Studiesالملخص
Objective: To explore the risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension (IPAH). Methods: Forty-four patients (age<18 years) with IPAH admitted to the Department of Pediatric Cardiology, Beijing Anzhen Hospital between May 2011 and October 2021 were retrospectively included. Patients were devided into syncope group and non-syncope group. Clinical manifestation and hemodynamic parameters including echocardiography, blood tests, right heart catheterization and acute pulmonary vascular expansion test were compared between two groups. Comparisons between groups were performed with unpaired Student t test, or Mann-Whitney U test or chi-square test. Logistic regression was used to calculate the odds ratio (OR) for parameters with statistically significant differences between groups and analyze the statistical correlation. Results: Among the 44 patients, 16 were males, the onset age was (7.2±3.9) years. Twenty-four (55%) children presented with 1 to 11 times of episodes of syncope, and 18 cases of whom induced by physical activity. Syncope group had a larger proportion of New York Heart Association (NYHA) heart function class Ⅲ-Ⅳ (67% (16/24) vs. 25% (5/20), χ2=7.59, P=0.006), higher brain natriuretic peptide (BNP) value ((251±39) vs. (61±40) pg/L, t=-2.18, P=0.035), higher pulmonary-to-aorta diameter ratio (1.6±0.4 vs. 1.4±0.2, t=-2.25, P=0.030) and larger pulmonary vascular resistance index ((22±11) vs. (16±7) WU/m2, t=-2.13, P=0.039) compared with non-syncope group. The proportion of patent foramen ovale (4% (1/24) vs. 45% (9/20), χ2=10.36, P=0.001), left ventricular ejection fraction (LVEF) ((68±5)% vs. (72±8)%, t=2.23, P=0.031) and the positive rate of acute pulmonary vascular expansion test (8% (2/24) vs. 35% (7/20), χ2=4.77, P=0.029) of syncope group were significantly lower than those of non-syncope group. Multiple Logistic regression analysis showed that NYHA heart function Ⅲ-Ⅳ (OR=6.787, 95%CI 1.445-31.880), pulmonary vascular resistance index (OR=1.247, 95%CI 1.020-1.525) and BNP (OR=1.036, 95%CI 1.007-1.066) were independent risk factors for syncope. The patent foramen ovale (OR=0.010, 95%CI 0.000-0.586) was a protective factor for syncope. Conclusions: NYHA cardiac function grade, pulmonary vascular resistance index and BNP are independent risk factors for syncope. Patent foramen ovale is a protective factor for syncope. Exercise is the main inducement of syncope in children with IPAH.
الموضوعات
Adolescent , Child , Child, Preschool , Female , Humans , Male , Familial Primary Pulmonary Hypertension , Foramen Ovale, Patent , Retrospective Studies , Risk Factors , Stroke Volume , Syncope/etiology , Ventricular Function, Leftالملخص
Objective: To investigate the gene expression characteristics of peripheral blood mononuclear cells from patients with high altitude pulmonary hypertension (HAPH) in Naxi residents living in Lijiang, Yunnan, and to explore the underlying pathogenesis and value for potential drug selection. Methods: This is a case-control study. Six patients with HPAH (HPAH group) and 4 normal subjects (control group) were selected from the Naxi residents who originally lived in Lijiang, Yunnan Province. The general clinical data of the two groups were collected, and the related indexes of pulmonary artery pressure were collected. Peripheral blood mononuclear cells of the subjects were collected for RNA sequencing. The differences on gene expression, regulatory network of transcription factors and drug similarity between the two groups were compared. The results were compared with the public data of idiopathic pulmonary arterial hypertension (IPAH). Biological processes and signal pathways were analyzed and compared between HPAH and IPAH patients. Results: The age of 6 patients with HAPH was (68.1±8.3) years old, and there were 2 males (2/6). The age of 4 subjects in the control group was (62.3±10.9) years old, and there were 2 males (2/4). Tricuspid regurgitation velocity, tricuspid pressure gradient and pulmonary systolic pressure in HAPH group were significantly higher than those in control group (all P<0.05). The results of RNA sequencing showed that compared with the control group, 174 genes were significantly upregulated and 169 genes were downregulated in peripheral blood mononuclear cells of HAPH group. These differentially expressed genes were associated with 220 biological processes, 52 molecular functions and 23 cell components. A total of 21 biological processes and 2 signal pathways differed between HPAH and IPAH groups, most of which were related to inflammation and immune response. ZNF384, SP1 and STAT3 were selected as highly correlated transcription factors by transcription factor prediction analysis. Trichostatin A and vorinostat were screened out as potential drugs for the treatment of HAPH by drug similarity analysis. Conclusions: There are significant differences in gene expression in peripheral blood monocytes between HAPH patients and normal population, and inflammation and immune dysfunction are the main pathogenic factors. Trichostatin A and Vorinostat are potential drugs for the treatment of HAPH.
الموضوعات
Aged , Humans , Male , Middle Aged , Altitude , Altitude Sickness/genetics , Case-Control Studies , China , Familial Primary Pulmonary Hypertension/genetics , Hydroxamic Acids/therapeutic use , Hypertension, Pulmonary/genetics , Inflammation , Leukocytes, Mononuclear/pathology , Transcription Factors , Transcriptome/genetics , Vorinostat/therapeutic useالملخص
The accompanying images demonstrate giant pulmonary artery aneurysms in a patient with idiopathic pulmonary arterial hypertension (Image 1). In addition to the main pulmonary artery, both the left and right pulmonary arteries are aneurysmal and are compressing the lung parenchyma (Image 2).
Las imágenes adjuntas muestran aneurismas gigantes de la arteria pulmonar en un paciente con hipertensión arterial pulmonar idiopática (Imagen 1). Además de la arteria pulmonar principal, tanto la arteria pulmonar izquierda como la derecha son aneurismáticas y están comprimiendo el parénquima pulmonar (Imagen 2).
الموضوعات
Humans , Pulmonary Artery , Radiology , Aneurysm , Familial Primary Pulmonary Hypertension , Parenchymal Tissue , Heart Defects, Congenitalالملخص
SUMMARY OBJECTIVE: Oxidative stress plays a pivotal role in the pathogenesis of pulmonary arterial hypertension. 8-Hydroxy-2'-deoxyguanosine is a sensitive biomarker that reflects the degree of oxidative damage to DNA. We investigated whether serum 8-Hydroxy-2'-deoxyguanosine is a clinically useful biomarker for the severity of pulmonary arterial hypertension. METHODS: We measured serum 8-Hydroxy-2'-deoxyguanosine levels in 25 patients (age 37±13 years, 68% women) diagnosed with idiopathic pulmonary arterial hypertension, familial pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease. The severity of pulmonary arterial hypertension was evaluated by six-min walking distance, World Health Organization functional class, and serum brain natriuretic peptide levels. Age and gender-matched 22 healthy subjects served as the control group. RESULTS: The comparison of 8-Hydroxy-2'-deoxyguanosine levels between patients and controls was not statistically different [(19.86±9.79) versus (18.80±3.94) ng/mL, p=0.622)]. However, there was a significant negative correlation between 8-Hydroxy-2'-deoxyguanosine levels and six-min walking distance (r= −0.614, p=0.001). Additionally, serum 8-Hydroxy-2'-deoxyguanosine levels in patients with functional class III-IV were significantly higher than those with functional class I-II (functional class III-IV 32.31±10.63 ng/mL versus functional class I-II 16.74±6.81 ng/mL, respectively, p=0.003). CONCLUSION: The 8-Hydroxy-2'-deoxyguanosine levels were significantly correlated with exercise capacity (six-min walking distance) and symptomatic status (functional class), both of which show the severity of pulmonary arterial hypertension in patients.
الموضوعات
Humans , Male , Adult , Young Adult , Pulmonary Arterial Hypertension , Hypertension , Oxidative Stress , Familial Primary Pulmonary Hypertension , Middle Agedالملخص
ABSTRACT BACKGROUND: The mechanism of exercise limitation in idiopathic pulmonary arterial hypertension (IPAH) is not fully understood. The role of hemodynamic alterations is well recognized, but mechanical, ventilatory and gasometric factors may also contribute to reduction of exercise capacity in these individuals. OBJECTIVE: To investigate whether there is an association between ventilatory pattern and stress Doppler echocardiography (SDE) variables in IPAH patients. DESIGN AND SETTING: Single-center prospective study conducted in a Brazilian university hospital. METHODS: We included 14 stable IPAH patients and 14 age and sex-matched controls. Volumetric capnography (VCap), spirometry, six-minute walk test and SDE were performed on both the patients and the control subjects. Arterial blood gases were collected only from the patients. The IPAH patients and control subjects were compared with regard to the abovementioned variables. RESULTS: The mean age of the patients was 38.4 years, and 78.6% were women. The patients showed hypocapnia, and in spirometry 42.9% presented forced vital capacity (FVC) below the lower limit of normality. In VCap, IPAH patients had higher respiratory rates (RR) and lower elimination of CO2 in each breath. There was a significant correlation between reduced FVC and the magnitude of increases in tricuspid regurgitation velocity (TRV). In IPAH patients, VCap showed similar tidal volumes and a higher RR, which at least partially explained the hypocapnia. CONCLUSIONS: The patients with IPAH showed hypocapnia, probably related to their higher respiratory rate with preserved tidal volumes; FVC was reduced and this reduction was positively correlated with cardiac output.
الموضوعات
Humans , Female , Adult , Pulmonary Arterial Hypertension , Cross-Sectional Studies , Prospective Studies , Echocardiography, Stress , Exercise Test , Familial Primary Pulmonary Hypertension , Lung/diagnostic imagingالملخص
A proteinose alveolar pulmonar (PAP) é rara e caracterizada por preenchimento alveolar com material lipoproteináceo. A proteinose alveolar é caracterizada por um alvéolo com material eosinofílico, acelular, finamente granular, com fendas de colesterol. Este relato de caso descreve um paciente do sexo masculino, 2 anos, portador de hipogamaglobulinemia. O paciente foi internado com quadro compatível com Stevens- Johnson após uso de amoxicilina e clavulanato para quadro de otite média aguda. Posteriormente, foi encaminhado à unidade de terapia intensiva devido à piora respiratória clínica e radiológica. Biópsia pulmonar: proteinose alveolar com alvéolos distendidos por material proteináceo, eosinofílico e grumoso com infiltrado linfo- histiocitário local. A proteinose alveolar pulmonar é rara e o diagnóstico correto deve ser realizado para que seja realizado tratamento adequado e acompanhamento da evolução. Deve-se atentar para complicações, especialmente infecções oportunistas.
Pulmonary alveolar proteinosis (PAP) is rare and it is characterized by alveolar filling with lipoproteinaceous material. Alveolar proteinosis is characterized by an alveolus with eosinophilic, acellular, finely granular material, with cholesterol cracks. This case report describes a 2-yearold male patient with hypogammaglobulinemia. The patient was hospitalized with Stevens-Johnson-compatible condition after use of amoxicillin and clavulanate for acute otitis media. Subsequently, he was referred to the intensive care unit due to worsening clinical and radiological breathing. Lung biopsy: alveolar proteinosis with alveoli distended by proteinaceous, eosinophilic and lumpy material with local lymphohistiocytic infiltrate. Pulmonary alveolar proteinosis is rare and the correct diagnosis must be made in order to carry out an appropriate treatment and follow-up of the evolution. Attention should be paid to complications, especially opportunistic infections.
الموضوعات
Male , Child, Preschool , Pulmonary Alveolar Proteinosis , Respiratory Distress Syndrome, Newborn , Opportunistic Infections , Dyspnea , Tachypnea , Familial Primary Pulmonary Hypertension , Amoxicillinالموضوعات
Humans , Female , Middle Aged , Pulmonary Artery/diagnostic imaging , Coronary Vessels/diagnostic imaging , Dilatation, Pathologic/diagnostic imaging , Familial Primary Pulmonary Hypertension/physiopathology , Pulmonary Artery/pathology , Follow-Up Studies , Coronary Vessels/pathology , Computed Tomography Angiographyالملخص
OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.
الموضوعات
Extracorporeal Membrane Oxygenation/methods , Lung Diseases/therapy , Postoperative Complications , Pulmonary Emphysema/therapy , Pulmonary Emphysema/epidemiology , Pulmonary Fibrosis/therapy , Pulmonary Fibrosis/epidemiology , Time Factors , Brazil/epidemiology , Bronchiectasis/therapy , Bronchiectasis/epidemiology , Prospective Studies , Retrospective Studies , Lung Transplantation/methods , Treatment Outcome , Familial Primary Pulmonary Hypertension/therapy , Familial Primary Pulmonary Hypertension/epidemiology , Intensive Care Units , Length of Stay , Lung Diseases/epidemiologyالملخص
Objective: To explore the risk factors for death in patients with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) and the clinical characteristics of different subtypes in patients with PAH-CHD. Methods: It was a retrospective study. A total of 507 PAH-CHD patients, who were hospitalized in the Department of Pediatric Cardiology of Beijing Anzhen Hospital between September 2005 and May 2019, were included. Patients were divided into 4 subgroups: (1) Eisenmenger syndrome(ES) group. (2) PAH associated with prevalent systemic-to-pulmonary shunts(SP) group. (3) PAH associated with small defects(SD) group. (4) PAH after defect correction(CD) group. According to the complexity of cardiac malformation, patients were divided into simple-medium complex malformation group and complex malformation group. According to the location of shunts, patients were divided into pre-tricuspid group, post-tricuspid group, and mixed group or complex deformity group. Baseline clinical data of patients were collected from the electronic medical record system. Demographic data (age, gender, etc.), percutaneous oxygen saturation(SPO(2)), New York Heart Association(NYHA) cardiac function classification, 6 minutes walking distance(6MWD), and B type natriuretic peptide(BNP), systolic pulmonary arterial pressure(sPAP) estimated by echocardiography and mean pulmonary artery pressure (mPAP), mean right atrial pressure(mRAP), cardiac index(CI), and calculated pulmonary vascular resistance (PVR) estimated by right heart catheterization were compared among various groups. The results of regular follow-up of all enrolled patients were collected, including the status of monotherapy or combination of PAH-targeted drugs during the follow-up period, cardiac-related adverse events(hemopysis, syncope, edema, arrhythmia, etc.) and primary endpoint event(all-cause death) were obtained and analyzed. Risk factors for all-cause death were analyzed using univariate and multivariate Cox regression analysis model. Results: The median age at diagnosis was 23.1(13.9,32.1) years, 345 cases(68.0%) were female. Two hunderds and thirty-five cases(46.4%) were diagnosed with ES; 193 cases(38.1%) were diagnosed with CD, 47 cases (9.3%) were diagnosed with SD. Among them, 32 cases(6.3%) were in the SP group. All 507 patients underwent echocardiography examination, there were significant differences in sPAP among different clinical subgroups(P<0.001). A total of 289 patients(57.0%) received right heart catheterization examination, the results showed that the ES group had the highest mPAP and PVR and the lowest mRAP(all P<0.001), the CD group had the highest mRAP and CI(both P<0.001). The 6MWD in the ES group was significantly shorter than that in the SP, SD, and CD groups(all P<0.001). The proportion of patients with NYHA class Ⅲ/Ⅳ was higher in SD group than in SP group(P<0.001), which was similar between SD, ES and CD groups (P values were 0.077 and 0.072, respectively). At admission, the proportion of patients with NYHA class Ⅰ/Ⅱwas the highest in SP group(96.9% (31/32) ), followed by CD group (85.5%(165/193)) and the ES group(85.1%(200/235)), and the SD group(75.0%(35/47)). The BNP level at admission was also higher in SD group than in SP, ES and CD groups(P<0.001). Of the 507 patients, 379(74.8%) patients received PAH-targeted drug therapy at the last follow-up, and the treatment plan was mainly monotherapy(75.7%(287/379)). The median follow-up time was 3.6(2.0, 5.6) years and 37(7.3%) patients died, including 13 in the CD group, 17 in the ES group, and 7 in the SD group. No deaths occurred in the SP group. Right heart failure was the most common cause of death(11(29.7%)), followed by severe hemoptysis dyspnea(7(18.9%)), sudden cardiac death(6(16.2%)), and pulmonary hypertensive crisis(4(10.8%)). Kaplan-Meier curve showed that survival rates of end-point-free events at 1, 3, 5 and 10 years after diagnosis of PAH were 98.0%, 95.4%, 89.9%, and 84.4%, respectively; there were statistically significant differences in survival among the subgroups(P=0.026); there was no significant difference in the survival rate between the ES group and the CD group(P=0.918), and both were higher than the SD group(P values were 0.011 and 0.013, respectively). Univariate Cox regression analysis showed that NYHA class Ⅲ/Ⅳ and BNP>100 ng/L at admission were the risk factors for all-cause death in patients with PAH-CHD(HR=6.452, 95%CI 3.378-12.346, P<0.001, and HR=2.481, 95%CI 1.225-5.025, P=0.012). Multivariate Cox regression analysis showed that NYHA class Ⅲ/Ⅳ was an independent risk factor for all-cause death in patients with PAH-CHD(HR=4.998, 95%CI 1.246-20.055, P=0.023). Conclusions: PAH-CHD patients with different clinical subtypes have different clinical symptoms, cardiac functional class, hemodynamic characteristics, and mid to long-term survival rates. SP patients have the best prognosis, outcome of ES and CD patients is similar, and SD patients have the worst prognosis. NYHA class Ⅲ/Ⅳ is an independent risk factor for all-cause death in patients with PAH-CHD.
الموضوعات
Adolescent , Adult , Female , Humans , Young Adult , Familial Primary Pulmonary Hypertension , Heart Defects, Congenital , Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Retrospective Studies , Risk Factorsالملخص
Objetivo: Realizar um estudo de caso de um jovem portador de tuberculose pulmonar (TP) e construir um plano de assistência de enfermagem, visando a melhoria da qualidade de vida do paciente. Métodos: Estudo de caso com abordagem qualitativa de um paciente portador de TP, hipertensão arterial pulmonar, desnutrição e alcoolismo, no Hospital Universitário Oswaldo Cruz (HUOC) em Recife-PE, no período de março a junho de 2016. A coleta de dados foi realizada mediante anamnese, exame físico, análise do prontuário, pesquisa literária e histórica de enfermagem. Resultados: Identificamos 13 diagnósticos de enfermagem: padrão respiratório ineficaz, nutrição/fadiga, manutenção ineficaz da saúde e do lar, distúrbios da identidade/autoestima, risco de solidão/suicídio e angustia espiritual. Conclusão: A enfermagem tem especial importância nas ações de controle da TP realizando a supervisão do tratamento, evitando o abandono e a tuberculose resistente, garantindo a adesão e um tratamento bem-sucedido
Objetivo: Realizar un estudio de caso de un paciente joven con tuberculosis pulmonar (TP) y construir un plan de atención de enfermería con el fin de mejorar la calidad de vida del paciente. Métodos: Un estudio de caso con enfoque cualitativo de un paciente con enfermedad de Parkinson, la hipertensión arterial pulmonar, la desnutrición y el alcoholismo, el Hospital Universitario Oswaldo Cruz (HUOC) en Recife, de marzo a junio de 2016. La recolección de datos fue realizado por la historia clínica, examen físico, análisis de la historia clínica, la investigación literaria y la historia de enfermería. Resultados: Se identificaron 13 diagnósticos de enfermería: Modelo ineficaz la respiración, la nutrición/fatiga, mantenimiento ineficaz de la salud y el hogar, trastornos de identidad/autoestima, la soledad de riesgo/suicidio y la angustia espiritual. Conclusión: La enfermería tiene especial importancia en las acciones de control TP mediante la supervisión del tratamiento, evitar el abandono y la tuberculosis resistente, asegurando la adherencia y un tratamiento exitoso
Objective: Performing a case study of a young patient having pulmonary tuberculosis disease (TB) and design a nursing care plan aiming to improve the patient life quality. Methods: Case study with a qualitative approach of a patient having TB, pulmonary arterial hypertension, malnutrition state and also alcoholism. The study was done at the Oswaldo Cruz University Hospital (HUOC) in Recife, Brazil, from March to June 2016. Data collection was performed through anamnesis, physical examination, medical records analysis, literary research and nursing history. Results: We were able to identify 13 nursing diagnoses, as follows: ineffective respiratory pattern, nutrition/fatigue, ineffective maintenance of health and home, identity/self-esteem disorders, solitude/ suicide risk and spiritual distress. Conclusion: The nursing professionals have especial importance regarding the actions of TB control, supervising the treatment, avoiding abandonment and resistant tuberculosis, as well as, guaranteeing adherence and a successful treatment
الموضوعات
Humans , Male , Adult , Nursing Care , Treatment Adherence and Compliance , Tuberculosis, Pulmonary/complications , Tuberculosis, Pulmonary/nursing , Alcoholism/nursing , Familial Primary Pulmonary Hypertension/nursing , Malnutrition/nursingالملخص
Pathogenic variants of bone morphogenic protein receptor type 2 gene (BMPR2) are related to the majority of cases of heritable pulmonary arterial hypertension (PAH). Over 400 pathogenic variants have been identified. However, clinical characterization of PAH is still incomplete. We present a case of heritable PAH in a Korean family showing serious clinical presentation with high penetrance. Genetic sequencing revealed a known heterozygous BMPR2 pathogenic variant, c.418+5G>A, at a splice site of intron 3. Serious clinical presentation with high penetrance suggested that the interplay of other factors with pathologic variants might be in genotype-phenotype correlation. Further studies are needed to clarify these issues for the development of personalized medicine approaches for PAH.
الموضوعات
Humans , Familial Primary Pulmonary Hypertension , Genetic Association Studies , Hypertension , Hypertension, Pulmonary , Introns , Penetrance , Precision Medicine , Pulmonary Arteryالملخص
<p><b>BACKGROUND</b>The clinical significance of acute vasoreactivity testing (AVT) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains unclear. We analyzed changes in hemodynamics and oxygenation dynamics indices after AVT in patients with CTEPH using patients with pulmonary arterial hypertension (PAH) as controls.</p><p><b>METHODS</b>We analyzed retrospectively the results of AVT in 80 patients with PAH and 175 patients with CTEPH registered in the research database of Beijing Chao-Yang Hospital between October 2005 and August 2014. Demographic variables, cardiopulmonary indicators, and laboratory findings were compared in these two subgroups. A long-term follow-up was conducted in patients with CTEPH. Between-group comparisons were performed using the independent-sample t-test or the rank sum test, within-group comparisons were conducted using the paired t-test or the Wilcoxon signed-rank test, and count data were analyzed using the Chi-squared test. Survival was estimated using the Kaplan-Meier method and log-rank test.</p><p><b>RESULTS</b>The rates of positive response to AVT were similar in the CTEPH (25/175, 14.3%) and PAH (9/80, 11.3%) groups (P > 0.05). Factors significantly associated a positive response to AVT in the CTEPH group were level of N-terminal pro-brain natriuretic peptide (≤1131.000 ng/L), mean pulmonary arterial pressure (mPAP, ≤44.500 mmHg), pulmonary vascular resistance (PVR, ≤846.500 dyn·s-1·m-5), cardiac output (CO, ≥3.475 L/min), and mixed venous oxygen partial pressure (PvO2, ≥35.150 mmHg). Inhalation of iloprost resulted in similar changes in mean blood pressure, mPAP, PVR, systemic vascular resistance, CO, arterial oxygen saturation (SaO2), mixed venous oxygen saturation, partial pressure of oxygen in arterial blood (PaO2), PvO2, and intrapulmonary shunt (Qs/Qt) in the PAH and CTEPH groups (all P > 0.05). The survival time in patients with CTEPH with a negative response to AVT was somewhat shorter than that in AVT-responders although the difference was not statistically significant (χ2 =3.613, P = 0.057). The survival time of patients with CTEPH who received calcium channel blockers (CCBs) was longer than that in the group with only basic treatment and not shorter than that of patients who receiving targeted drugs or underwent pulmonary endarterectomy (PEA) although there was no significant difference between the four different treatment regimens (χ2 =3.069, P = 0.381).</p><p><b>CONCLUSIONS</b>The rates of positive response to AVT were similar in the CTEPH and PAH groups, and iloprost inhalation induced similar changes in hemodynamics and oxygenation dynamics indices. A positive response to AVT in the CTEPH group was significantly correlated with milder disease and better survival. Patients with CTEPH who cannot undergo PEA or receive targeted therapy but have a positive response to AVT might benefit from CCB treatment.</p>
الموضوعات
Adult , Aged , Female , Humans , Male , Middle Aged , Administration, Inhalation , Arterial Pressure , Atrial Natriuretic Factor , Metabolism , Calcium Channel Blockers , Therapeutic Uses , Endarterectomy , Familial Primary Pulmonary Hypertension , Drug Therapy , Hemodynamics , Hypertension, Pulmonary , Drug Therapy , Iloprost , Therapeutic Uses , Protein Precursors , Metabolism , Retrospective Studies , Software , Vasodilator Agents , Therapeutic Usesالملخص
A hipertensão arterial pulmonar (HAP) é uma doença complexa, caracterizada por disfunção endotelial, que resulta em remodelamento vascular pulmonar e elevação da pressão arterial pulmonar, com consequente insuficiência cardíaca direita. O speckle tracking bidimensional (2D-STE) é uma das mais recentes ferramentas da ecocardiografia, o qual tem sido empregado para avaliação mais precoce da função ventricular e do efeito da HAP sobre a função dos ventrículos esquerdo e direito. O objetivo deste estudo foi avaliar a deformação (St e StR) miocárdica radial do VE em modelo experimental de suínos com HAP induzida e tratados com angiotensina-(1-7), a fim de verificar as possibilidades desse novo fármaco nas respostas clínica e hemodinâmica, pois apresenta efeitos anti-inflamatório e vasodilatador, bem como ações antiproliferativas no sistema cardiovascular. Neste estudo, foi possível observar que os animais tratados com Ang-(1-7) apresentaram St e StR radiais maiores que o grupo placebo aos 60 dias de experimento, demonstrando uma melhora na função sistólica do miocárdio pelo aumento da deformação miocárdica (16,06±7,50 - placebo; 25,14±14,91 - Ang-(1-7)) e StR (1,28±0,51 - placebo; 1,51±0,58 - Ang-(1-7)). Essa melhora na função sistólica pode ser atribuída aos efeitos do fármaco, que reduziram também o desenvolvimento da hipertensão pulmonar. Diante dos resultados, acredita-se que a Ang-(1-7) possa ser um medicamento promissor para tratamento da HAP.(AU)
Pulmonary arterial hypertension (PAH) is a complex disease characterized by endothelial dysfunction resulting in pulmonary vascular remodeling, increased pulmonary arterial pressure, with subsequent right heart failure. The two-dimensional speckle tracking (2D-STE) is one of the newer tools used for early assessment of ventricular function. The aim of this study was to evaluate the radial myocardial deformation (St and StR) of LV in experimental pigs with induced PAH and treated with Ang- (1-7), checking the possibilities of this new drug in clinical and hemodynamic response, since it has anti-inflammatory effects and antiproliferative actions on the cardiovascular system. In this study we observed that animals treated with Ang (1-7) had radial St and StR higher than the placebo group at 60 days of the experiment demonstrating an improvement in systolic function of the myocardium by increased myocardial deformation (16.06±7.50 - placebo; 25.14±14.91 - Ang (1-7)) and StR (1.28±0.51 - placebo; 1.51±0.58 - Ang (1-7)). This improvement in systolic function can be attributed to drug effects while also reducing the development of pulmonary hypertension. Based on the results it is believed that Ang (1-7) may be a promising drug for the treatment of PAH.(AU)
الموضوعات
Animals , Angiotensins/therapeutic use , Familial Primary Pulmonary Hypertension/veterinary , Swine , Ventricular Dysfunction/veterinary , Echocardiography/veterinaryالملخص
Introducción: la hipertensión arterial pulmonar es una enfermedad grave y progresiva. Si la enfermedad no es tratada, la sobrecarga de presión del ventrículo derecho lleva a dilatación, hipertrofia, falla cardíaca en estado final entre dos a tres años y a muerte. El sildenafilo, un inhibidor de la Fosfodiesterasa tipo 5, es una alternativa para su tratamiento actualmente en uso clínico. Esta evaluación de tecnología se desarrolló para informar la toma de decisiones en el marco de la actualización integral del Plan Obligatorio de Salud para Colombia. Objetivo: examinar la efectividad y seguridad comparativas del sildenafilo para el tratamiento de pacientes con hipertensión arterial pulmonar. Metodología: se realizó una búsqueda sistemática en MEDLINE, EMBASE, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects y LILACS. La tamización de referencias se realizó por dos revisores de forma independiente y la selección de estudios fue hecha por un revisor, aplicando los criterios de elegibilidad predefinidos en el protocolo de la evaluación. La calidad de las revisiones sistemáticas se valoró con la herramienta AMSTAR. Se realizó una síntesis narrativa de las estimaciones del efecto para las comparaciones y desenlaces de interés. Resultados: los hallazgos de efectividad y seguridad de la presente evaluación se basan en cuatro revisiones sistemáticas, dos de calidad alta y dos de calidad media, y cuatro ensayos controlados aleatorizados cabeza a cabeza, en general de buena calidad, para un total aproximado de 658 pacientes. Se identificó evidencia de los efectos del sildenafilo en monoterapia comparado con bosentán, y en terapia combinada más bosentán o epoprostenol, para una variedad de desenlaces incluyendo el tiempo al primer evento de morbilidad/mortalidad, capacidad de ejercicio, mortalidad, hospitalización, cambio en la clase funcional (OMS), empeoramiento de la hipertensión, trasplante de pulmón, disnea, parámetros hemodinámicos, calidad de vida, eventos adversos globales, serios y específicos. La evidencia disponible corresponde a adultos con hipertensión arterial pulmonar, de clase funcional (OMS) I, II, III y IV. También se presentan los eventos adversos reportados en la etapa post-clínica con el uso del sildenafilo. Conclusiones: la evidencia identificada en esta evaluación de tecnología, muestra efectos mixtos en la efectividad y seguridad del sildenafilo para el tratamiento de adultos con hipertensión arterial pulmonar: en cuanto a la monoterapia con sildenafilo, los resultados de efectividad demuestran que este medicamento es similar a sus comparadores; respecto a su seguridad, hay incertidumbre. En terapia combinada, algunos hallazgos de efectividad muestran superioridad del sildenafilo frente a sus comparadores, otros datos indican que esta tecnología es similar respecto a sus alternativas y para algunos desenlaces existe incertidumbre. La seguridad comparada del sildenafilo en terapia combinada puede ser mayor, similar, menor o incierta. A juicio de los expertos clínicos y representantes de los pacientes, el sildenafilo en monoterapia y en terapia combinada, tiene una relación favorable entre los beneficios y riesgos, esto sugiere que los efectos deseables con el uso de esta tecnología superan a los efectos indeseables.(AU)
الموضوعات
Humans , Familial Primary Pulmonary Hypertension/drug therapy , Technology Assessment, Biomedical , Reproducibility of Results , Treatment Outcome , Colombia , Sildenafil Citrate/administration & dosageالملخص
O avanço no tratamento das cardiopatias tem permitido um progressivo número de mulheres com doenças cardíacas congênitas alcançarem a idade reprodutiva e a gravidez. Assim, o conhecimento sobre as modificações fisiológicas do ciclo gravídico-puerperal e sua interface com as lesões cardiacas estruturais é fundamental para a adequada conduta em portadoras de cardiopatia congênita durante a gestação. Neste artigo, discutiremos os fatores de prognóstico materno-fetal, as principais complicações, os cuidados obstétricos durante a gestação, parto e puerpério e o planejamento familiar da mulher com cardiopatia congênita.
Advances in the treatment of congenital heart defects has led to a gradually increase in the number of women with congenital heart defects reaching reproductive age and pregnancy. Thus, a knowledge of the physiological changes of the pregnant-puerperal cycle, and their interface with structural heart defects, is essential for adequate conduct in patients with congenital heart disease during pregnancy. In this article, we discusses thefactors of maternal-fetal prognosis, the main complications, the obstetric care provided during pregnancy, labor and puerperium, and family planning for women with congenital heart defects.
الموضوعات
Humans , Female , Adult , Heart Defects, Congenital/mortality , Heart Defects, Congenital/therapy , Prenatal Care/methods , Risk Factors , Pregnancy , Parturition , Fetal Development , Heredity , Familial Primary Pulmonary Hypertension , Maternal Mortality , Women , Fontan Procedure/methodsالملخص
BACKGROUND/AIMS: Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it has not been studied. The purpose of this study was to evaluate survival outcomes of Korean patients with IPAH treated with warfarin. METHODS: We performed a retrospective cohort study of patients diagnosed previously with pulmonary arterial hypertension (PAH) at the Asan Medical Center in Korea, between January 1994 and February 2013. We excluded patients with associated PAH, patients who did not undergo right heart catheterization (RHC), and patients with a positive vasoreactivity test. Patients in the study cohort were classified into a "warfarin group" and a "non-warfarin group," according to the treatment they received during the first year after diagnosis. RESULTS: We identified 31 patients with IPAH and a negative vasoreactivity test on RHC. Median patient age was 36.0 years, and 23 patients (74.2%) were female. The median time from the onset of symptoms to diagnosis was 19.0 months, and the most common presenting symptom was dyspnea. Survival rates of the patients at 1, 3, 5, and 10 years were 90.2%, 79.5%, 62.7%, and 34.8%, respectively. The mean survival period was 12.0 years in the warfarin group and 6.1 years in the non-warfarin group. Warfarin treatment had significant survival benefits in patients with IPAH (p = 0.023). CONCLUSIONS: Warfarin treatment substantially improved survival outcomes in Korean cases of IPAH.