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1.
J Transl Med ; 22(1): 502, 2024 May 26.
Artigo em Inglês | MEDLINE | ID: mdl-38797830

RESUMO

BACKGROUND: Inflammation and dysregulated immunity play vital roles in idiopathic pulmonary arterial hypertension (IPAH), while the mechanisms that initiate and promote these processes are unclear. METHODS: Transcriptomic data of lung tissues from IPAH patients and controls were obtained from the Gene Expression Omnibus database. Weighted gene co-expression network analysis (WGCNA), differential expression analysis, protein-protein interaction (PPI) and functional enrichment analysis were combined with a hemodynamically-related histopathological score to identify inflammation-associated hub genes in IPAH. The monocrotaline-induced rat model of pulmonary hypertension was utilized to confirm the expression pattern of these hub genes. Single-cell RNA-sequencing (scRNA-seq) data were used to identify the hub gene-expressing cell types and their intercellular interactions. RESULTS: Through an extensive bioinformatics analysis, CXCL9, CCL5, GZMA and GZMK were identified as hub genes that distinguished IPAH patients from controls. Among these genes, pulmonary expression levels of Cxcl9, Ccl5 and Gzma were elevated in monocrotaline-exposed rats. Further investigation revealed that only CCL5 and GZMA were highly expressed in T and NK cells, where CCL5 mediated T and NK cell interaction with endothelial cells, smooth muscle cells, and fibroblasts through multiple receptors. CONCLUSIONS: Our study identified a new inflammatory pathway in IPAH, where T and NK cells drove heightened inflammation predominantly via the upregulation of CCL5, providing groundwork for the development of targeted therapeutics.


Assuntos
Quimiocina CCL5 , Hipertensão Pulmonar Primária Familiar , Células Matadoras Naturais , RNA-Seq , Análise de Célula Única , Linfócitos T , Animais , Humanos , Quimiocina CCL5/metabolismo , Quimiocina CCL5/genética , Células Matadoras Naturais/metabolismo , Células Matadoras Naturais/imunologia , Hipertensão Pulmonar Primária Familiar/genética , Hipertensão Pulmonar Primária Familiar/patologia , Hipertensão Pulmonar Primária Familiar/metabolismo , Linfócitos T/metabolismo , Linfócitos T/imunologia , Masculino , Comunicação Celular/genética , Ratos Sprague-Dawley , Pulmão/patologia , Ratos , Redes Reguladoras de Genes , Monocrotalina , Mapas de Interação de Proteínas/genética , Biologia Computacional
2.
BMC Cardiovasc Disord ; 24(1): 384, 2024 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-39054410

RESUMO

BACKGROUND: The risk stratification of pulmonary arterial hypertension proposed by the European Society of Cardiology /European Respiratory Society guidelines in 2015 and 2022 included two to three echocardiographic indicators. However, the specific value of echocardiography in risk stratification of pre-capillary pulmonary hypertension (pcPH) has not been efficiently demonstrated. Given the complex geometry of the right ventricular (RV) and influencing factors of echocardiographic parameter, there is no single echocardiographic parameter that reliably informs about PH status. We hypothesize that a multi-parameter comprehensive index can more accurately evaluate the severity of the pcPH. The purpose of this study was to develop and validate an echocardiographic risk score model to better assist clinical identifying high risk of pcPH during initial diagnosis and follow-up. METHODS: We studied 197 consecutive patients with pcPH. A multivariable echocardiographic model was constructed to predict the high risk of pcPH in the training set. Points were assigned to significant risk factors in the final model based on ß-coefficients. We validated the model internally and externally. RESULTS: The echocardiographic score was constructed by multivariable logistic regression, which showed that pericardial effusion, right atrial (RA) area, RV outflow tract proximal diameter (RVOT-Prox), the velocity time integral of the right ventricular outflow tract (TVIRVOT) and S' were predictors of high risk of pcPH. The area under curve (AUC) of the training set of the scoring model was 0.882 (95%CI: 0.809-0.956, p < 0.0001). External validation was tested in a test dataset of 77 patients. The AUC of the external validation set was 0.852. A 10-point score risk score was generated, with scores ranging from 0 to 10 in the training cohort. The estimate risk of high risk of pcPH ranged from 25.1 to 94.6%. CONCLUSIONS: The echocardiographic risk score using five echocardiographic parameters could be comprehensive and useful to predict the high risk of pcPH for initial assessment and follow-up.


Assuntos
Valor Preditivo dos Testes , Função Ventricular Direita , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Medição de Risco , Fatores de Risco , Reprodutibilidade dos Testes , Idoso , Estudos Retrospectivos , Prognóstico , Pressão Arterial , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/diagnóstico , Índice de Gravidade de Doença , Adulto , Técnicas de Apoio para a Decisão , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Ecocardiografia Doppler , Hipertensão Arterial Pulmonar/fisiopatologia , Hipertensão Arterial Pulmonar/diagnóstico por imagem , Hipertensão Arterial Pulmonar/diagnóstico
3.
Respiration ; 2024 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-39137746

RESUMO

INTRODUCTION: Balloon pulmonary angioplasty (BPA) is an effective intervention for patients with chronic thromboembolic pulmonary disease (CTEPD). We aim to identify the patient group with a low success rate or high complication rate of BPA, which is still unclear. METHODS: Both CTEPD patients with or without pulmonary hypertension (CTEPH and NoPH-CTEPD) were included. CTEPH patients were divided into groups with or without pulmonary endarterectomy (PEA-CTEPH and NoPEA-CTEPH). The efficacy and safety of BPA were compared among the groups. RESULTS: There were 450, 66, and 41 sessions in the NoPEA-CTEPH, PEA-CTEPH, and NoPH-CTEPD groups, respectively. The success rate (≥ 1 degree improvement in flow grade) in the PEA-CTEPH group was 94.5%, significantly lower than that in the NoPEA-CTEPH (97.1%) and NoPH-CTEPD (98.4%) groups (P=0.014). The percentage of complete flow recovery in treated vessels was also lower in PEA-CTEPH group. BPA-related complication rate in NoPEA-CTEPH, PEA-CTEPH, and NoPH-CTEPD patients was 6.1%, 6.0%, and 0.0%, respectively (P=0.309). One BPA-related death occurred (solely in NoPEA-CTEPH). Mean pulmonary artery pressure ≥ 41.5 mmHg was a predictor of BPA-related complications. NoPEA-CTEPH patients had more improvement in 6-minute-walk-distance (6MWD, 87±93 m -NoPEA-CTEPH vs 40±43 m -PEA-CTEPH vs 18±20 m -NoPH-CTEPD, P=0.012). CONCLUSIONS: BPA was safe and effective for all CTEPD groups with less improvement for the PEA-CTEPH and NoPH-CTEPD groups. The success rate of BPA was lower in the PEA-CTEPH group and the complication rate was lower in the NoPH-CTEPD group. Pre-BPA treatment to lower pulmonary artery pressure should not be overlooked in CTEPD patients.

4.
BMC Pulm Med ; 24(1): 194, 2024 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-38649898

RESUMO

BACKGROUND: Patients with congenital myopathies may experience respiratory involvement, resulting in restrictive ventilatory dysfunction and respiratory failure. Pulmonary hypertension (PH) associated with this condition has never been reported in congenital ryanodine receptor type 1(RYR1)-related myopathy. CASE PRESENTATION: A 47-year-old woman was admitted with progressively exacerbated chest tightness and difficulty in neck flexion. She was born prematurely at week 28. Her bilateral lower extremities were edematous and muscle strength was grade IV-. Arterial blood gas analysis revealed hypoventilation syndrome and type II respiratory failure, while lung function test showed restrictive ventilation dysfunction, which were both worse in the supine position. PH was confirmed by right heart catheterization (RHC), without evidence of left heart disease, congenital heart disease, or pulmonary artery obstruction. Polysomnography indicated nocturnal hypoventilation. The ultrasound revealed reduced mobility of bilateral diaphragm. The level of creatine kinase was mildly elevated. Magnetic resonance imaging showed myositis of bilateral thigh muscle. Muscle biopsy of the left biceps brachii suggested muscle malnutrition and congenital muscle disease. Gene testing revealed a missense mutation in the RYR1 gene (exon33 c.C4816T). Finally, she was diagnosed with RYR1-related myopathy and received long-term non-invasive ventilation (NIV) treatment. Her symptoms and cardiopulmonary function have been greatly improved after 10 months. CONCLUSIONS: We report a case of RYR1-related myopathy exhibiting hypoventilation syndrome, type II respiratory failure and PH associated with restrictive ventilator dysfunction. Pulmonologists should keep congenital myopathies in mind in the differential diagnosis of type II respiratory failure, especially in patients with short stature and muscle weakness.


Assuntos
Hipertensão Pulmonar , Debilidade Muscular , Insuficiência Respiratória , Canal de Liberação de Cálcio do Receptor de Rianodina , Humanos , Feminino , Canal de Liberação de Cálcio do Receptor de Rianodina/genética , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/genética , Insuficiência Respiratória/etiologia , Mutação de Sentido Incorreto , Imageamento por Ressonância Magnética , Doenças Musculares/genética , Doenças Musculares/diagnóstico , Doenças Musculares/complicações
5.
BMC Pulm Med ; 24(1): 26, 2024 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-38200493

RESUMO

BACKGROUND: Clinical characteristics of patients with pulmonary thromboembolism have been described in previous studies. Although very old patients with pulmonary thromboembolism are a special group based on comorbidities and age, they do not receive special attention. OBJECTIVE: This study aims to explore the clinical characteristics and mortality predictors among very old patients with pulmonary thromboembolism in a relatively large population. DESIGN AND PARTICIPANTS: The study included a total of 7438 patients from a national, multicenter, registry study, the China pUlmonary thromboembolism REgistry Study (CURES). Consecutive patients with acute pulmonary thromboembolism were enrolled and were divided into three groups. Comparisons were performed between these three groups in terms of clinical characteristics, comorbidities and in-hospital prognosis. Mortality predictors were analyzed in very old patients with pulmonary embolism. KEY RESULTS: In 7,438 patients with acute pulmonary thromboembolism, 609 patients aged equal to or greater than 80 years (male 354 (58.1%)). There were 2743 patients aged between 65 and 79 years (male 1313 (48%)) and 4095 patients aged younger than 65 years (male 2272 (55.5%)). Patients with advanced age had significantly more comorbidities and worse condition, however, some predisposing factors were more obvious in younger patients with pulmonary thromboembolism. PaO2 < 60 mmHg, eGFR < 60 mL/min/1.73m2, malignancy, anticoagulation as first therapy were mortality predictors for all-cause death in very old patients with pulmonary thromboembolism. The analysis found that younger patients were more likely to have chest pain, hemoptysis (the difference was statistically significant) and dyspnea triad. CONCLUSION: In very old population diagnosed with pulmonary thromboembolism, worse laboratory results, atypical symptoms and physical signs were common. Mortality was very high and comorbid conditions were their features compared to younger patients. PaO2 < 60 mmHg, eGFR < 60 mL/min/1.73m2 and malignancy were positive mortality predictors for all-cause death in very old patients with pulmonary thromboembolism while anticoagulation as first therapy was negative mortality predictors.


Assuntos
Neoplasias , Embolia Pulmonar , Idoso , Humanos , Masculino , Anticoagulantes/uso terapêutico , Gasometria , Oxigênio , Embolia Pulmonar/epidemiologia , Feminino
6.
BMC Pulm Med ; 24(1): 265, 2024 Jun 03.
Artigo em Inglês | MEDLINE | ID: mdl-38825688

RESUMO

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disorder with substantial morbidity and mortality, also a disease underdiagnosed and undertreated. It is potentially curable by pulmonary endarterectomy (PEA) in patients with surgically accessible thrombi. Balloon pulmonary angioplasty (BPA) and targeted medical therapy are options for patients with distal lesions or persistent/recurrent pulmonary hypertension after PEA. There is an urgent need to increase the awareness of CTEPH. Qualified CTEPH centers are still quite limited. Baseline characteristics, management pattern and clinical outcome of CTEPH in China needs to be reported. METHODS AND DESIGN: The CHinese reAl-world study to iNvestigate the manaGEment pattern and outcomes of chronic thromboembolic pulmonary hypertension (CHANGE) study is designed to provide the multimodality treatment pattern and clinical outcomes of CTEPH in China. Consecutive patients who are ≥ 14 year-old and diagnosed with CTEPH are enrolled. The diagnosis of CTEPH is confirmed in right heart catheterization and imaging examinations. The multimodality therapeutic strategy, which consists of PEA, BPA and targeted medical therapy, is made by a multidisciplinary team. The blood sample and tissue from PEA are stored in the central biobank for further research. The patients receive regular follow-up every 3 or 6 months for at least 3 years. The primary outcomes include all-cause mortality and changes in functional and hemodynamic parameters from baseline. The secondary outcomes include the proportion of patients experiencing lung transplantation, the proportion of patients experiencing heart and lung transplantation, and changes in health-related quality of life. Up to 31 December 2023, the study has enrolled 1500 eligible patients from 18 expert centers. CONCLUSIONS: As a real-world study, the CHANGE study is expected to increase our understanding of CTEPH, and to fill the gap between guidelines and the clinical practice in the diagnosis, assessment and treatment of patients with CTEPH. REGISTRATION NUMBER IN CLINICALTRIALS.GOV: NCT05311072.


Assuntos
Angioplastia com Balão , Endarterectomia , Hipertensão Pulmonar , Embolia Pulmonar , Humanos , Hipertensão Pulmonar/terapia , China , Embolia Pulmonar/complicações , Embolia Pulmonar/terapia , Doença Crônica , Qualidade de Vida , Resultado do Tratamento , Feminino , Terapia Combinada , Masculino , População do Leste Asiático
7.
Quant Imaging Med Surg ; 14(2): 1686-1698, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38415125

RESUMO

Background: Pulmonary artery sarcoma (PAS) is a very rare malignancy with a poor prognosis; however, its clinical manifestations and imaging findings are often indistinguishable from pulmonary thromboembolism (PTE). We thus aimed to accurately diagnose PAS by comparing the clinical and computed tomography pulmonary angiography (CTPA) and magnetic resonance imaging (MRI) imaging characteristics of PAS and PTE. Methods: This case-control study retrospectively enrolled 20 patients with PAS (from March 2017 to September 2022), 40 patients with central acute PTE, and 40 patients with central chronic PTE (from January 2021 to December 2022) in the China-Japan Friendship Hospital. The following clinical and imaging findings were compared between the three groups: initial symptoms; D-dimer, C-reactive protein, and N-terminal pro B-type natriuretic peptide levels; wall-eclipsing sign (WES); scope of lesion involvement; and morphological characteristics. Signal intensity was also observed on different MRI sequences. Results: The D-dimer level in PAS was significantly lower than that in central acute PTE (P<0.001). The WES was present in 17 cases of PAS (85.0%), which was a greater proportion than that of the central acute PTE and chronic PTE groups (all P values <0.001). The involvement of the pulmonary valve or right ventricular outflow tract was observed in five PAS cases but none of the central acute PTE or chronic PTE cases (all P values =0.001). In 19 PAS cases (95.0%), the lesions grew expansively in the central pulmonary artery. The proximal margin of 18 patients with PAS (90.0%) was bulging or lobulated. Nine cases of PAS (45.0%) showed aneurysm-like dilatation (grape-like sign) of the distal pulmonary artery, representing significantly greater proportion than that of the central acute PTE and chronic PTE groups (all P values <0.001). In 37 patients with central acute PTE (92.5%), the clots were observed to be floating in the pulmonary artery lumen with saddle, tubular or polypoid shape. Eccentric filling defects attached to the pulmonary artery wall were observed in 32 cases of central chronic PTE (80.0%). On MRI, PAS lesions were hyperintense on fat-suppressed T2-weighted imaging and diffusion-weighted imaging, demonstrating heterogeneous enhancement. Conclusions: Comprehensive analysis of the clinical data and imaging features on CTPA and MRI can aid in the accurate differential diagnosis of PAS and PTE.

8.
Quant Imaging Med Surg ; 14(1): 86-97, 2024 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-38223063

RESUMO

Background: Risk stratification for patients with acute pulmonary embolism (APE) is significantly important for treatment and prognosis evaluation. We aimed to develop a novel clot burden score on computed tomography pulmonary angiography (CTPA) based on deep learning (DL) algorithm for risk stratification of APE. Methods: The study retrospectively enrolled patients newly diagnosed with APE in China-Japan Friendship Hospital consecutively. We collected baseline data and CTPA parameters, and calculated four different clot burden scores, including Qanadli score, Mastora score, clot volume and clot ratio. The former two were calculated by two radiologists separately, while clot volume and clot ratio were based on the DL algorithm. The area under the curve (AUC) of four clot burden scores were analyzed. Results: Seventy patients were enrolled, including 17 in high-/intermediate-high risk and 53 in low-/intermediate-low risk. Clot burden was related to the risk stratification of APE. Among four clot burden scores, clot ratio had the highest AUC (0.719, 95% CI: 0.569-0.868) to predict patients with higher risk. In the patients with hemodynamically stable APE, only clot ratio presented statistical difference (P=0.046). Conclusions: Clot ratio is a new imaging marker of clot burden which correlates with the risk stratification of patients with APE. Higher clot ratio may indicate higher risk and acute right ventricular dysfunction in patients with hemodynamically stable status.

9.
Quant Imaging Med Surg ; 14(3): 2590-2602, 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38545067

RESUMO

Background: Single-photon emission computed tomography (SPECT) ventilation perfusion imaging is the main imaging method for the diagnosis of pulmonary embolism, and its application in the diagnosis and efficacy evaluation of chronic thromboembolic pulmonary hypertension (CTEPH) has been paid more and more attention. In recent years, with the development of computer software technology, ventilation/perfusion (V/Q) imaging quantitative analysis technology has become more and more mature. The objective of this study was to investigate the utility of quantitative analysis of pulmonary V/Q scintigraphy in evaluating the efficacy of balloon pulmonary angioplasty (BPA) in patients with CTEPH. Methods: In this retrospective analysis, we collected data of patients diagnosed with CTEPH who underwent BPA at the China-Japan Friendship Hospital from April 2018 to September 2020. The sample consisted of 23 males and 28 females, with an average age of 55.1±12.7 years. All patients underwent V/Q scintigraphy within one week before surgery, and we reviewed the pulmonary angiography within 1-3 months following the last BPA procedure. We repeated V/Q scintigraphy within 1 week before or after the pulmonary angiography, at the time of collecting clinical and hemodynamic parameters of these patients. We divided the patients into two groups based on the presence of residual pulmonary hypertension post-surgery and compared the pre- and post-operative quantitative pulmonary perfusion defect percentage scores (PPDs%) using the t-test. Results: In all, 102 V/Q scintigraphy scans were performed in 51 patients. The quantitative PPDs% were positively correlated with the hemodynamic indexes mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), and mean right ventricular pressure (RVP) (r=0.605, 0.391, and 0.464, respectively, all P<0.001) and negatively correlated with the 6-minute walking distance (6MWD) (r=-0.254, P=0.010). The average preoperative quantitative PPDs% were (49.0±15.6)% which significantly decreased to (33.5±13.9)% after surgery (t=11.249, P<0.001). The preoperative quantitative PPDs% were (54.7±15.7)% and (44.0±13.8)% in the residual pulmonary hypertension group and the non-residual pulmonary hypertension group, respectively (t=2.599, P=0.012). The postoperative quantitative PPDs% were (41.5±12.5)% and (26.3±11.0)%, in the residual pulmonary hypertension group and the non-residual pulmonary hypertension group, respectively (t=4.647, P<0.001). Conclusions: In this study, we found that quantitative analysis of SPECT pulmonary V/Q scintigraphy adequately reflected the pulmonary artery pressure and clinical status in patients with CTEPH. Our results demonstrate its definite utility in predicting residual pulmonary hypertension and in evaluating the postoperative efficacy of BPA in patients with CTEPH.

10.
Eur J Intern Med ; 124: 46-53, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38350784

RESUMO

BACKGROUND: The performance of existing prognostic scores including the simplified Pulmonary Embolism Severity Index (sPESI) for short-term mortality of non-high-risk PE in Chinese population has not been widely validated. METHODS: Non-high-risk patients were included from the prospective cohort of the China pUlmonary Thromboembolism REgistry Study (CURES). The sPESI, RIETE, Geneva, modified FAST, and Bova score were validated. The discriminatory performance was measured by the area under the curve (AUC). We also compared the sensitivity, odds ratio, specificity, positive predictive value and negative predictive value of these scores. RESULTS: A total of 6,873 non-high-risk patients with acute PE were included and 241 (3.5 %) patients died within 30 days. Compared to the Geneva, modified FAST, and Bova score, the AUCs for predicting 30-day death of sPESI and RIETE score were higher at 0.712 (95 % CI, 0.680, 0.743) and 0.723 (95 % CI, 0.691, 0.755) respectively. The sPESI demonstrated the highest sensitivity at 0.809, while the RIETE score, Geneva, Modified FAST and BOVA score showed sensitivities of 0.622, 0.568, 0.477 and 0.502 respectively. A sPESI ⩾1 point was associated with a 4.7-fold increased risk of 30-day all-cause mortality (95 % CI, 3.427, 6.563, p < 0.001), while a RIETE score of ⩾1 point was associated with a 4.5-fold increased risk (95 % CI, 3.127, 6.341, p < 0.001). The Geneva score, modified FAST and Bova score showed inferior performance. CONCLUSIONS: The implementation of the fewer-parameter, easier-to-calculate sPESI in Chinese patients with PE can help to discriminate patients with extremely low risk of short-term mortality for home treatment or early discharge.


Assuntos
Embolia Pulmonar , Sistema de Registros , Índice de Gravidade de Doença , Humanos , Embolia Pulmonar/mortalidade , Embolia Pulmonar/diagnóstico , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Estudos Prospectivos , China/epidemiologia , Prognóstico , Área Sob a Curva , Medição de Risco/métodos , Valor Preditivo dos Testes , Curva ROC , Doença Aguda
11.
iScience ; 27(2): 108930, 2024 Feb 16.
Artigo em Inglês | MEDLINE | ID: mdl-38333700

RESUMO

Determining novel biomarkers for early identification of chronic thromboembolic pulmonary hypertension (CTEPH) could improve patient outcomes. We used the isobaric tag for relative and absolute quantitation approach to compare the serum protein profiles between CTEPH patients and the controls. Bioinformatics analyses and ELISA were also performed. We identified three proteins including heparanase (HPSE), gelsolin (GSN), and secreted protein acidic and rich in cysteine (SPARC) had significant changes in CTEPH. The receiver operating characteristic curve analysis showed that the areas under the curve of HPSE in CTEPH diagnosis were 0.988. Furthermore, HPSE was correlated with multiple parameters of right ventricular function. HPSE concentrations were significantly higher in patients with a low TAPSE/sPAP ratio (≤0.31 mm/mmHg) (65.4 [60.5,68.0] vs. 59.9 [35.9,63.2] ng/mL, p < 0.05). The CTEPH patients treated by balloon pulmonary angioplasty had significantly lower HPSE levels. The study demonstrates that HPSE may be a promising biomarker for noninvasive detection of CTEPH.

12.
Pulm Circ ; 14(1): e12327, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38162296

RESUMO

Balloon pulmonary angioplasty (BPA) has been proven effective for addressing technically inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the effectiveness of BPA in technically operable CTEPH patients who, for various reasons, did not undergo the procedure remains an area requiring exploration. This study sought to assess the safety and efficacy of BPA in such cases. We collected and reviewed data from CTEPH patients who underwent BPA in a consecutive manner. Following multidisciplinary team (MDT) decisions, patients were classified into two groups: technically inoperable (group A) and operable (group B). Group B comprised patients deemed technically suitable for pulmonary endarterectomy (PEA) but who did not undergo the procedure for various reasons. All patients underwent a comprehensive diagnostic work-up, including right heart categorization at baseline and the last intervention. This study compared changes in hemodynamic parameters, functional capacity, and quality of life between the two groups. In total, 161 patients underwent 414 procedures at our center, with Group A comprising 112 patients who underwent 282 BPA sessions and group B comprising 49 patients who underwent 132 BPA sessions. Significantly, both groups exhibited improvements in hemodynamics, functional capacity, and quality of life. The occurrence rate of complications, including hemoptysis and lung injury, was similar [12 (63.2%) vs. 7 (36.8%), p = 0.68]. BPA demonstrated favorable outcomes in patients with proximal CTEPH who did not undergo pulmonary endarterectomy. However, the clinical impact of BPA in technically operable CTEPH was found to be less significant than in inoperable cases.

13.
J Interv Med ; 6(4): 199-202, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38312129

RESUMO

Takayasu arteritis (TA) is a rare systemic vasculitis of the aorta and its primary branches, which usually occurs in young women. Due to its insidious onset and lack of specific symptoms, this disease can be easily misdiagnosed or missed. Approximately 50 â€‹% of the patients having TA with pulmonary artery involvement develop pulmonary hypertension (PH). The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone. Early balloon pulmonary angioplasty (BPA) can improve the clinical symptoms and survival of patients with stable TA. To the best of our knowledge, this is the first case reported in the English literature in which a "Guidezilla" catheter was used during BPA to treat stenosis and occlusion of the pulmonary artery caused by Takayasu arteritis (TA).

14.
Quant Imaging Med Surg ; 13(12): 7910-7923, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-38106317

RESUMO

Background: In the 2022 European Society of Cardiology (ESC) and the European Respiratory Society (ERS) guidelines, the diagnostic criteria for pulmonary hypertension (PH) included a reduced mean pulmonary artery pressure (mPAP) of 20 mmHg (mPAP >20 mmHg). This study aimed to reassess cardiovascular metrics on computed tomography pulmonary angiography (CTPA) for chronic thromboembolic pulmonary hypertension (CTEPH) to optimize the timely diagnosis of patients with suspected PH. Methods: Patients with suspected CTEPH who underwent CTPA and right heart catheterization (RHC) between January 2019 and December 2022 in China-Japan Friendship Hospital were retrospectively included. They were grouped into CTEPH and non-PH groups according to the new and old criteria (2022 and 2015 ESC/ERS guidelines) for the diagnosis of PH. Cardiovascular metrics including the main pulmonary artery diameter (MPAd), Cobb angle, and right ventricular free wall thickness (RVWT), among others, were measured. The correlation of these metrics with hemodynamic data was analyzed with Spearman rank correlation analysis, while the differences in cardiovascular metrics between the updated (mPAP >20 mmHg) and old PH criteria (mPAP ≥25 mmHg) were compared with independent samples t-test or the Mann-Whitney test. Receiver operator characteristic (ROC) curve analysis was performed for the prediction model. Results: The study enrolled 180 patients (males n=86; age 55.5±12.0 years old). According to the old guidelines, 119 patients were placed into the PH group (mPAP ≥25 mmHg) , while according to the new guidelines, 130 patients were placed into the PH group (mPAP >20 mmHg). Cardiovascular metrics on CTPA between the updated and old guidelines were comparable (P>0.05). Compared to other metrics, an MPAd of 30.4 mm exhibited the highest area under the curve (AUC: 0.934±0.021), with a sensitivity of 0.88 and specificity of 0.90. MPAd [odds ratio (OR) =1.271], transverse diameter of the right ventricle (RVtd; OR =1.176), Cobb angle (OR =1.108), and RVWT (OR =3.655) were independent factors for diagnosing CTEPH (P<0.05). Cobb angle, right and left ventricular transverse diameter ratio, and right and left ventricular area ratio moderately correlated with mPAP (r=0.586, r=0.583, r=0.629) and pulmonary vascular resistance (PVR) (r=0.613, r=0.593, r=0.642). Conclusions: Cardiovascular metrics on CTPA were comparable between the new and old guidelines for CTEPH diagnosis. Cardiovascular metrics on CTPA can noninvasively assess the hemodynamics of patients with CTEPH.

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