Unusual cause of muscle weakness, type II respiratory failure and pulmonary hypertension: a case report of ryanodine receptor type 1(RYR1)-related myopathy.

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.

Evaluating the efficacy of balloon pulmonary angioplasty using quantitative analysis of SPECT pulmonary ventilation/perfusion scintigraphy in patients with chronic thromboembolic pulmonary hypertension.

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.

Performance of the Simplified Pulmonary Embolism Severity Index in predicting 30-day mortality after acute pulmonary embolism: Validation from a large-scale cohort.

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.

Serum proteome profiling reveals heparanase as a candidate biomarker for chronic thromboembolic pulmonary hypertension.

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.

Comparison of pulmonary artery sarcoma and pulmonary thromboembolism according to clinical and computed tomography pulmonary angiography and magnetic resonance imaging characteristics: a single-center retrospective study.

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.

Clot ratio, new clot burden score with deep learning, correlates with the risk stratification of patients with acute pulmonary embolism.

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.

Safety and efficacy of balloon pulmonary angioplasty for technically operable chronic thromboembolic pulmonary hypertension.

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.

Clinical characteristics and mortality predictors among very old patients with pulmonary thromboembolism: a multicenter study report.

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.

A machine learning model for diagnosing acute pulmonary embolism and comparison with Wells score, revised Geneva score, and Years algorithm.

BACKGROUND: Acute pulmonary embolism (APE) is a fatal cardiovascular disease, yet missed diagnosis and misdiagnosis often occur due to non-specific symptoms and signs. A simple, objective technique will help clinicians make a quick and precise diagnosis. In population studies, machine learning (ML) plays a critical role in characterizing cardiovascular risks, predicting outcomes, and identifying biomarkers. This work sought to develop an ML model for helping APE diagnosis and compare it against current clinical probability assessment models. METHODS: This is a single-center retrospective study. Patients with suspected APE were continuously enrolled and randomly divided into two groups including training and testing sets. A total of 8 ML models, including random forest (RF), Naïve Bayes, decision tree, K-nearest neighbors, logistic regression, multi-layer perceptron, support vector machine, and gradient boosting decision tree were developed based on the training set to diagnose APE. Thereafter, the model with the best diagnostic performance was selected and evaluated against the current clinical assessment strategies, including the Wells score, revised Geneva score, and Years algorithm. Eventually, the ML model was internally validated to assess the diagnostic performance using receiver operating characteristic (ROC) analysis. RESULTS: The ML models were constructed using eight clinical features, including D-dimer, cardiac troponin T (cTNT), arterial oxygen saturation, heart rate, chest pain, lower limb pain, hemoptysis, and chronic heart failure. Among eight ML models, the RF model achieved the best performance with the highest area under the curve (AUC) (AUC = 0.774). Compared to the current clinical assessment strategies, the RF model outperformed the Wells score ( P = 0.030) and was not inferior to any other clinical probability assessment strategy. The AUC of the RF model for diagnosing APE onset in internal validation set was 0.726. CONCLUSIONS: Based on RF algorithm, a novel prediction model was finally constructed for APE diagnosis. When compared to the current clinical assessment strategies, the RF model achieved better diagnostic efficacy and accuracy. Therefore, the ML algorithm can be a useful tool in assisting with the diagnosis of APE.

Comparison of cardiovascular metrics on computed tomography pulmonary angiography of the updated and old diagnostic criteria for pulmonary hypertension in patients with chronic thromboembolic pulmonary hypertension.

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.

Development and validation of a lung graph-based machine learning model to predict acute pulmonary thromboembolism on chest noncontrast computed tomography.

Background: Computed tomography pulmonary angiography (CTPA) is a first-line noninvasive method to diagnose acute pulmonary thromboembolism (APE); however, whether chest noncontrast CT (NC-CT) could aid in the diagnosis of APE remains unknown. The aim of this study was to build and evaluate a holistic lung graph-based machine learning (HLG-ML) using NC-CT for the diagnosis of APE and to compare its performance with that of radiologists and the YEARS algorithm. Methods: This study enrolled 178 cases (77 males; age 63.9±16.7 years) who underwent NC-CT and CTPA in the same day from January 2019 to December 2020. Of these patients, 133 (75% of cases; 58 males; age 65.4±15.6 years) were placed into a training group and 45 (25% of cases; 19 males; age 59.6±19.2 years) into a testing group. The other 43 cases (18 males; age 62.8±20.0 years) were used to externally validate the model between January 2021 and March 2022. A HLG was developed with a pulmonary radiomics descriptor derived from NC-CT images. The approach extracted local radiomics features and encoded these local features into a radiomics descriptor as a characterization of global radiomics feature distribution. Subsequently, 8 ML models were trained and compared based on the radiomics descriptor. In the validation group, area under the curves (AUCs) of the HLG-ML model in the diagnosis of APE were compared with those of the 3 radiologists and the YEARS algorithm. Results: Among the 8 ML models, gradient boosting decision tree demonstrated the best classification performance (AUC =0.772) on the training set. In the testing set, the AUC of gradient boosting decision trees was 0.857 [95% confidence intervals (CIs): 0.699-0.951]. In the validation set, the performance of gradient boosting decision tree (AUC =0.810; 95% CI: 0.669-0.952; Youden index =0.621) outperformed 3 radiologists (AUC =0.508, 95% CI: 0.335-0.681, Youden index =0.016; AUC =0.504, 95% CI: 0.354-0.654, Youden index =0.008; AUC =0.527, 95% CI: 0.363-0.691, Youden index =0.050) and the YEARS algorithm (AUC =0.618; 95% CI: 0.469-0.767; Youden index =0.237). Conclusions: Compared to all 3 radiologists and the YEARS algorithm, the proposed HLG-based gradient boosting decision tree model achieved a superior performance in the diagnosis of APE on the NC-CT and may thus serve as a valuable tool for physicians in the diagnosis of APE.

Cardiovascular metrics on CT pulmonary angiography in patients with pulmonary hypertension - re-evaluation under the updated guidelines of pulmonary hypertension.

PURPOSE: To re-assess cardiovascular metrics on computed tomography pulmonary angiography (CTPA) in predicting pulmonary hypertension (PH) under the 2022 ESC/ERS guidelines. MATERIALS AND METHODS: This observational study retrospectively included 272 patients (female 143, mean age = 54.9 ± 12.5 years old) with suspected PH. 218 patients were grouped to evaluate cardiovascular metrics on CTPA and develop a binary logistic regression model. The other 54 patients were grouped into the validation group to assess the performance of the prediction model under the updated criteria. Based on mean pulmonary artery pressure (mPAP), patients were divided into three groups: group A consisted of patients with mPAP ≤ 20 mmHg, group B included patients with 20 mmHg < mPAP < 25 mmHg, and group C comprised patients with mPAP ≥ 25 mmHg. Cardiovascular metrics among the three groups were compared, and receiver operating characteristic curves (ROCs) were used to evaluate the performance of cardiovascular metrics in predicting mPAP > 20 mmHg. RESULTS: The main pulmonary arterial diameter (MPAd), MPAd/ascending aorta diameter ratio (MPAd/AAd ratio), and right ventricular free wall thickness (RVFWT) showed significant differences among the three groups (p < 0.05). The area under curve (AUC) of MPAd was larger than MPAd/AAd ratio and RVFWT. A MPAd cutoff value of 30.0 mm has a sensitivity of 83.1% and a specificity of 90.4%. The AUC of the binary logistic regression model (Z = - 12.98187 + 0.31053 MPAd + 1.04863 RVFWT) was 0.938 ± 0.018. In the validation group, the AUC, sensitivity, specificity, and accuracy of the prediction model were 0.878, 92.7%, 76.9%, and 88.9%, respectively. CONCLUSION: Under the updated criteria, MPAd with a threshold value of 30.0 mm has better sensitivity and specificity in predicting PH. The binary logistic regression model may improve the diagnostic accuracy. CRITICAL RELEVANCE STATEMENT: Under the updated criteria, the main pulmonary arterial diameter with a threshold value of 30.0 mm has better sensitivity and specificity in predicting pulmonary hypertension. The binary logistic regression model may improve diagnostic accuracy. KEY POINTS: • According to 2022 ESC/ERS guidelines, a MPAd cutoff value of 30.0 mm has better sensitivity and specificity in predicting mPAP > 20 mmHg • A binary logistic regression model (Z = - 12.98187 + 0.31053 MPAd + 1.04863 RVFWT) was developed and had a sensitivity, specificity, and accuracy of 92.7%, 76.9%, and 88.9% in predicting mPAP > 20 mmHg. • A binary logistic regression prediction model outperforms MPAd in predicting mPAP > 20 mmHg.

Severe adverse reaction induced by albendazole and praziquantel for cystic echinococcosis.

Albendazole and praziquantel have been used to treat various parasitic infections for many years. Studies have confirmed the efficacy in the treatment of cystic echinococcosis (CE). We reported the case of a 45-year-old Chinese patient with pulmonary CE. He experienced diarrhea, stomachache, increase bilirubin, hair loss and acute fatal pancytopenia 10 days after albendazole and praziquantel treatment. We performed a literature review of severe adverse reaction caused by albendazole and praziquantel. It showed that severe adverse reactions such as bone marrow suppression caused by albendazole or praziquantel are rare, but patients with a course of treatment exceeding 10 days or with liver diseases are more likely to experience. Clinicians should pay attention to monitoring the patient's gastrointestinal tract reaction and peripheral blood cells (PBCs). If the patient showed a progressive disease, the medication should be immediately stopped. Supportive treatments should be considered, such as the administration of granulocyte colony-stimulating factor (G-CSF) against neutropenia or antibiotics to prevent infection.

Qualification of Ventricular Flow in Patients With Precapillary Pulmonary Hypertension With 4-dimensional Flow Magnetic Resonance Imaging.

PURPOSE: Our goal was to study both right and left ventricular blood flow in patients with precapillary pulmonary hypertension (pre-PH) with 4-dimensional (4D) flow magnetic resonance imaging (MRI) and to analyze their correlation with cardiac functional metrics on cardiovascular magnetic resonance (CMR) and hemodynamics from right heart catheterization (RHC). MATERIALS AND METHODS: 129 patients (64 females, mean age 47 ± 13 y) including 105 patients with pre-PH (54 females, mean age 49 ± 13 y) and 24 patients without PH (10 females, mean age 40 ± 12 y) were retrospectively included. All patients underwent CMR and RHC within 48 hours. 4D flow MRI was acquired using a 3-dimensional retrospectively electrocardiograph-triggered, navigator-gated phase contrast sequence. Right and left ventricular flow components including the percentages of direct flow (PDF), retained inflow (PRI), delayed ejection flow (PDE), and residual volume (PRVo) were respectively quantified. The ventricular flow components between patients with pre-PH and non-PH were compared and correlations of flow components with CMR functional metrics and hemodynamics measured with RHC were analyzed. Biventricular flow components were compared between survivors and deceased patients during the perioperative period. RESULTS: Right ventricular (RV) PDF and PDE significantly correlated with RVEDV and RV ejection fraction. RV PDF negatively correlated with pulmonary arterial pressure (PAP) and pulmonary vascular resistance. When the RV PDF was <11%, the sensitivity and specificity of RV PDF for predicting mean PAP ≥25 mm Hg were 88.6% and 98.7%, respectively, with an area under the curve value of 0.95 ± 0.02. When RV PRVo was more than 42%, the sensitivity and specificity of RV PRVo for predicting mean PAP ≥25 mm Hg were 85.7% and 98.5%, respectively, with an area under the curve value of 0.95 ± 0.01. Nine patients died during the perioperative period. Biventricular PDF, RV PDE, and PRI of survivors were higher than nonsurvivors whereas RV PRVo increased in deceased patients. CONCLUSIONS: Biventricular flow analysis with 4D flow MRI provides comprehensive information about the severity and cardiac remodeling of PH and may be a predictor of perioperative death of patients with pre-PH.

Current status of diagnosis and treatment of pulmonary hypertension in Chinese tertiary hospitals: A nationwide survey.

We intended to evaluate the diagnosis and treatment status of pulmonary hypertension (PH) in China and provide the basis for the design of the Chinese PH centers system. A questionnaire survey was conducted by sampling from Chinese Class A tertiary hospitals that have carried out the clinical work of PH, including the composition of PH clinical team, the current application of examinations related to PH diagnosis, the availability of PAH-specific medicine and the implementation of PH-related intervention and surgery. A total of 44 valid questionnaires from 20 provinces were collected in this survey. In the vast majority of centers (83.33%, n = 35), pulmonary artery catheterization was routinely performed under X-ray guidance. In 19.05% (n = 8) of centers, pressure measurements were determined at the right time (the end of normal expiration). Only 73.81% (n = 31) centers have carried out acute vasoreactivity testing. Prostacyclin analogues and prostaglandin receptor agonists were just prescribed in 45.45% (n = 20) of the centers. 19 centers (43.18%) were capable of performing balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA), while 25% (n = 11) were able to perform BPA, PEA, and lung transplantation. There was no significant difference in the diagnosis and treatment of PH between economic regions. The majority of Chinese tertiary hospitals were well equipped with the corresponding personnel, examinations and medicines related to PH, but the standardization and specialization of the management of PH need to be strengthened.

Genome-wide association analyses identified novel susceptibility loci for pulmonary embolism among Han Chinese population.

BACKGROUND: A large proportion of pulmonary embolism (PE) heritability remains unexplained, particularly among the East Asian (EAS) population. Our study aims to expand the genetic architecture of PE and reveal more genetic determinants in Han Chinese. METHODS: We conducted the first genome-wide association study (GWAS) of PE in Han Chinese, then performed the GWAS meta-analysis based on the discovery and replication stages. To validate the effect of the risk allele, qPCR and Western blotting experiments were used to investigate possible changes in gene expression. Mendelian randomization (MR) analysis was employed to implicate pathogenic mechanisms, and a polygenic risk score (PRS) for PE risk prediction was generated. RESULTS: After meta-analysis of the discovery dataset (622 cases, 8853 controls) and replication dataset (646 cases, 8810 controls), GWAS identified 3 independent loci associated with PE, including the reported loci FGG rs2066865 (p-value = 3.81 × 10-14), ABO rs582094 (p-value = 1.16 × 10-10) and newly reported locus FABP2 rs1799883 (p-value = 7.59 × 10-17). Previously reported 10 variants were successfully replicated in our cohort. Functional experiments confirmed that FABP2-A163G(rs1799883) promoted the transcription and protein expression of FABP2. Meanwhile, MR analysis revealed that high LDL-C and TC levels were associated with an increased risk of PE. Individuals with the top 10% of PRS had over a fivefold increased risk for PE compared to the general population. CONCLUSIONS: We identified FABP2, related to the transport of long-chain fatty acids, contributing to the risk of PE and provided more evidence for the essential role of metabolic pathways in PE development.

Right and left ventricular blood pool T2 ratio on cardiac magnetic resonance imaging correlates with hemodynamics in patients with pulmonary hypertension.

OBJECTIVES: Our objective is to compare the right/left ventricular blood pool T1 ratio (RVT1/LVT1), and right/left ventricular blood pool T2 ratio (RVT2/LVT2) on Cardiac Magnetic Resonance Imaging (CMR) between patients with pulmonary hypertension (PH) and normal controls, to analyze the correlation of RVT1/LVT1, RVT2/LVT2 and hemodynamics measured with right heart catheterization (RHC) in patients with PH. METHODS: Forty two patients with PH and 40 gender-and age-matched healthy controls were prospectively included. All patients underwent RHC and CMR within 24 h. The right and left ventricular blood pool T1 and T2 values were respectively measured, and RVT1/LVT1 and RVT2/LVT2 between the PH group and the healthy control were compared. Meanwhile, the correlation between RVT1/LVT1, RV/LVT2 ratio and hemodynamic parameters in patients with PH respectively was analyzed. RESULTS: In the control group, RVT2 was significantly lower than LVT2 (t = 6.782, p < 0.001) while RVT1 also was lower than LVT1 (t = 8.961, p < 0.001). In patients with PH, RVT2 was significantly lower than LVT2 (t = 9.802, p < 0.001) while RVT1 was similar to LVT1 (t = - 1.378, p = 0.176). RVT2/LVT2 in the PH group was significantly lower than that in the control group (p < 0.001). RVT1/LVT1 in PH patients increased in comparison with the control group (p < 0.001). RVT2/LVT2 negatively correlated with pulmonary vascular resistance (r = - 0.506) and positively correlated with cardiac index (r = 0.521), blood oxygen saturation in Superior vena cava, right atrium, right ventricle and pulmonary artery (r = 0.564, 0.603, 0.648, 0.582). CONCLUSIONS: RVT2/LVT2 on T2 mapping could be an additional CMR imaging marker that may assist to evaluate the severity of PH.

Incidence and risk factors of venous thrombotic events in patients with interstitial lung disease during hospitalization.

BACKGROUND: Studies on the incidence of venous thromboembolism (VTE) events in patients with interstitial lung disease (ILD) are limited and the results are inconsistent. The aim of this research was to investigate the incidence and risk factors of VTE in ILD during hospitalization. MATERIALS AND METHODS: In this retrospective, cross-sectional, observational study, a total of 5009 patients diagnosed with ILD from January 2016 to March 2022 in our hospital were retrospectively included. In ILD patients, VTE including pulmonary thromboembolism (PTE) and deep vein thrombosis (DVT) were screened from the electronic medical record system. Diagnosis of PTE and DVT were performed by CT pulmonary angiography (CTPA), CTV or ultrasound. And then the incidence and risk factors of VTE in different types of ILD were assessed. RESULTS: Among 5009 patients with ILD, VTE was detected in 129 (2.6%) patients, including 15(0.3%) patients with both PTE and DVT, 34 (0.7%) patients with PTE and 80 (1.6%) patients with DVT. 85.1% of patients with APE were in the intermediate-low risk group. The incidence of VTE in Anti-Neutrophil Cytoplasmic Antibodies -associated vasculitis related ILD (ANCA-AV-ILD), hypersensitivity pneumonitis and idiopathic pulmonary fibrosis (IPF) respectively was 7.9% and 3.6% and 3.5%. In patients with connective tissue disease-associated ILD (CTD-ILD), the incidence of VTE, DVT, PTE, combined PTE and DVT respectively was 3.0%, 2.3%, 0.4% and 0.3%. Among the various risk factors, different ILD categories, age ≥ 80 years (OR 4.178, 95% CI 2.097-8.321, P < 0.001), respiratory failure (OR 2.382, 95% CI 1.533-3.702, P < 0.001) and varicose veins (OR 3.718, 95% CI 1.066-12.964, P = 0.039) were independent risk factors of VTE. The incidence of VTE in patients with ILD increased with the length of time in hospital from 2.2% (< 7 days) to 6.4% (> 21 days). CONCLUSION: The incidence of VTE during hospitalization in ILD patients was 2.6%, with a 1.6% incidence of DVT, higher than the 0.7% incidence of PTE. Advanced age, ILD categories, respiratory failure and varicose veins as independent risk factors for the development of VTE should be closely monitored.

"Guidezilla" extension catheter combined with balloon technique for treating pulmonary artery stenosis caused by Takayasu arteritis.

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).