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Background and aim: Noninvasive and simple surrogates of right ventricular-arterial coupling (RVAC) have been explored, such as the ratio of tricuspid annular plane systolic excursion / systolic pulmonary artery pressure (TAPSE/sPAP) and systolic volume / end-systolic volume (SV/ESV) deduced from the simplified formula of conductance catheterization. Thus we aimed to investigate the value of three-dimensional echocardiography (3DE) derived SV/ESV in evaluating RVAC of pre-capillary pulmonary hypertension (PH) and compare it with TAPSE/sPAP. We also explored the relationship between SV/ESV with right heart function, hemodynamic index, clinical indicators and prognostic risk stratification. Methods: The study included 59 pre-capillary PH patients who completed right heart catheterization (RHC), cardiac magnetic resonance imaging (CMR) and echocardiography within 7 days. The "gold standard" of RVAC indexes were derived by RHC and CMR data, including Ees/Ea for right ventricular pulmonary artery coupling, Ees for right ventricular end-systolic maximum elasticity and Ea for pulmonary artery effective elasticity. The corresponding 3DE coupling parameters were SV/ESV, 3D Ees and 3D Ea, respectively. Spearman test and Bland-Altman test were used to analyze the correlation and consistency between 3DE coupling parameters with RHC-CMR coupling standard and TAPSE/sPAP; With right ventricular ejection fraction (RV EF)＜35% measured by CMR as the standard diagnostic criterion of uncoupling, the ROC curve was adopted to determine the diagnostic threshold of SV/ESV and TAPSE/sPAP uncoupling. According to SV/ESV diagnostic threshold, patients were divided into coupling and uncoupling group. Independent sample t test and Chi-square test were used to compare the two groups in terms of right heart function, hemodynamic index, clinical indicators and prognostic risk stratification. Results: 3DE coupling parameters (SV/ESV, 3D Ees, 3D Ea) were strongly correlated with RHC-CMR coupling standard (Ees/Ea, Ees and Ea) , respectively (r = 0.880, 0.755, 0.759, P < 0.05). SV/ESV and TAPSE/sPAP were moderately related (r = 0.611, P < 0.05). 3DE coupling parameters and RHC-CMR coupling standard were in good agreement with a low bias, respectively (SV/ESV vs Ees/Ea:-0.053, 3D Ees vs Ees: 0.176 mmHg/mL/m2, 3D Ea vs Ea: 0.393 mmHg/mL/m2) and a satisfactory limits of agreement. The consistency of SV/ESV with TAPSE/sPAP was acceptable (bias: 0.379, 95% CI: -0.006, 0.765). The ROC curve analysis showed the area under the curve (AUC) of TAPSE/sPAP was 0.714 (95% CI, 0.570–0.832）with a optimal cut-off value 0.199 (sensitivity: 70%, specificity: 61.29%). The AUC of SV/ESV was 0.872 (95% CI, 0.759–0.945）with diagnostic threshold 0.533 (sensitivity: 85%, specificity: 74.36%). Right heart echocardiographic parameters (TAPSE, GLS, S’, FAC, RA area, RV/LV, RVOT act), hemodynamic index (mPAP, PVR), clinical indexes (6-minute walking distance, NT-proBNP, WHO FC) and prognostic risk stratification were statistically significant between coupling and uncoupling group (P < 0.05). Conclusion: 3DE derived SV/ESV can reliably evaluate RVAC as a new noninvasive surrogate. SV/ESV less than 0.533 predicted decreased right cardiac function, worsening hemodynamic and clinical status, and higher prognostic risk stratification.

Rong-Zheng Ma,1 Ping-Ping Han,1 Xin-Cao Tao,2 Huan Li,1 Ling Wang,1 Zhen-Guo Zhai,2 Li-Ping Fu1 1Department of Nuclear Medicine, China-Japan Friendship Hospital, Beijing, 100029, People’s Republic of China; 2Department of Pulmonary and Critical Care Medicine, China-Japan Friendship Hospital, National Center for Respiratory Medicine, Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, National Clinical Research Center for Respiratory Diseases, Beijing, 100029, People’s Republic of ChinaCorrespondence: Li-Ping FuDepartment of Nuclear Medicine, China-Japan Friendship Hospital, No. 2 of Yinghuayuan Road, Chaoyang District, Beijing, 100029, People’s Republic of ChinaTel +86 10 84205389Fax +86 10-84205389Email fulipjh@126.comZhen-Guo ZhaiDepartment of Pulmonary and Critical Care Medicine, China-Japan Friendship Hospital, No. 2 of Yinghuayuan Road, Chaoyang District, Beijing, 100029, People’s Republic of ChinaTel +86 10 84206269Fax +86 10 84206269Email zhaizhguo235@outlook.comObjective: The study is designed to evaluate the diagnostic ability of single-photon emission computed tomography (SPECT) pulmonary ventilation/perfusion (V/Q) imaging in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and investigate its feasibility in assessing patient risk.Methods: A total of 83 patients suspected of having CTEPH who received V/Q tomography were retrospectively analyzed. The consistency between SPECT V/Q imaging and pulmonary angiography was compared to investigate the correlation between the percentage of pulmonary perfusion defect score (PPDs%) and the hemodynamic indices. Patients were grouped according to the pulmonary arterial hypertension risk stratification, and the V/Q imaging results were compared between different groups.Results: For the 1494 pulmonary segments of the 83 patients, the sensitivity, specificity, and accuracy of identifying pulmonary segments with defects using V/Q imaging was 87.05%, 82.78% (668/807), and 84.74% (1266/1494), respectively. The average PPDs% (58.8 ± 12.6%) was positively correlated with the mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), and right ventricular pressure (RVP; r =0.316, 0.318, and 0.432, respectively; P < 0.05) and negatively correlated with the six-minute walk distance (6MWD; r = − 0.309; P < 0.05). There were 37 patients in the low-risk group and 46 in the medium-high-risk group. The number of pulmonary segments with perfusion defects (NPSPDs) and PPDs% were higher in the medium-high risk than in the low-risk group (t = − 6.721, − 5.032; P < 0.05). In the low- and medium-high-risk groups, the cut-off values for the NPSPDs (7.2 ± 2.1 and 10.2 ± 2.0) and PPDs% (51.9 ± 11.1% and 64.3 ± 11.1%,) were 8.5 and 61.25%, respectively.Conclusion: SPECT V/Q imaging achieved an accurate diagnosis of CTEPH. The semi-quantitative analysis index (PPDs%) was correlated with the hemodynamic indices and 6MWD. SPECT V/Q could be used for the preoperative risk assessment of patients with CTEPH.Keywords: pulmonary artery, thrombosis, pulmonary hypertension, pulmonary perfusion/ventilation imaging, 99mTc-MAA, risk stratification

The most common cause of pulmonary artery filling defects on computed tomography pulmonary angiography or magnetic resonance imaging is pulmonary thromboembolism, but not infrequently, the presentation of this finding lacks specificity. Given that the morbidity and mortality associated with pulmonary thromboembolism is high, proper diagnosis of the condition is essential. Unusual or more rarely encountered etiologies must be considered when clinical manifestations and imaging findings are inconsistent. With this review, our purpose is to describe possible causes of pulmonary arterial filling defects. We aim to provide clinicians with a comprehensive list of differential diagnoses to facilitate a measured approach to the assessment of pulmonary arterial filling defects on computed tomography pulmonary angiography or magnetic resonance imaging.

Background: Several echocardiographic methods to estimate pulmonary vascular resistance (PVR) in patients with pulmonary hypertension (PH) have been proposed. So far, most studies have focused on relatively low PVR. We aimed to clarify the clinical usefulness of our new echocardiographic index of evaluating markedly elevated PVR in pre-capillary PH patients. Methods: We studied 129 consecutive patients with pre-capillary PH. We estimated the mean pulmonary artery pressure using echocardiography (mPAPEcho) and measured LV internal diameter at end diastole (LVIDd). The ratio of mPAPEcho / LVIDd was then correlated with invasive PVR. Using receiver operating characteristic curve analysis, a cutoff value for the index was generated to identify patients with PVR > 15 Wood units (WU). Results: mPAPEcho / LVIDd correlated well with PVR (r = 0.70, P < 0.0001). There was a better correlation between PVR and mPAPEcho / LVIDd in patients with PVR > 15 WU compared with TRV2 /TVIRVOT and sPAPEcho / LVIDd. A cut-off value of 1.14 had an 80.0% sensitivity and 74.7% specificity to determine PVR > 15 WU (AUC=0.840, pConclusions: The index of mPAPEcho / LVIDd could be a valuable noninvasive and simple method of estimating markedly elevated PVR in pre-capillary PH patients.

Pulmonary artery sarcoma is a rare malignancy with poor prognosis. Lack of specific clinical manifestations, some patients are even confirmed postoperatively or at autopsy, that leads to the delay in treatment. Early diagnosis and radical surgical resection provide the possibility of prolonged survival. We retrospectively enrolled 13 patients diagnosed with pulmonary artery sarcoma at our hospital between 2015 and 2019. Their clinical, laboratory, radiological, and histopathological data were collected and analyzed. Published case series were also reviewed. Results show that, the median age of the patients was 53 years, with 6 (46.2%) males. The most common symptom is exertional dyspnea. Erythrocyte sedimentation rate and C-reactive protein were increased in 76.9% and 69.2% of these patients, while D-Dimer remained normal or elevated slightly. Metastasis was present at diagnosis in eight (61.5%) patients. Ten patients were diagnosed histologically: three were diagnosed after pulmonary endarterectomy, four by endobronchial ultrasound-guided transbronchial needle aspiration, two by percutaneous lung biopsy, and one by endovascular aspiration biopsy. Four patients underwent surgery and one is waiting for surgery. Nine patients received chemotherapy; and three of them received targeted therapy with anlotinib after chemotherapy. Two patients received anti-PD-1 monoclonal antibody. One patient died during endobronchial ultrasound-guided transbronchial needle aspiration. Two patients died 9 and 13 months after diagnosis, respectively; one refused invasive diagnostic procedures and died three months after clinical diagnosis. In conclusion, the most appropriate approach to get tissue specimen needs to be tailored to every pulmonary artery sarcoma patient. Pulmonary endarterectomy combined with chemotherapy and targeted therapy has prolonged their survival time.

BACKGROUND: Cardiac flow closely interact with function, however, the correlation of right ventricular (RV) flow and function remains unknown, thus our objective is to observe right ventricular flow with four-dimensional phase-contrast cardiovascular magnetic resonance imaging (4D flow CMR) in patients with pulmonary arterial hypertension (PAH) and to analyze flow components with RV function and hemodynamics. METHODS: This study retrospectively enrolled 30 patients with PAH (mean age: 49±13 years, 16 females) and 14 age- and sex-matched healthy volunteers as controls (mean age: 44±12 years, 9 females). All patients who underwent CMR and right heart catheterization (RHC) within 1 week between January 2019 and July 2020 were included. Hemodynamics were measured with RHC. RV flow components, including the percentages of direct flow (RVPDF), retained inflow (RVPRI), delayed ejection flow (RVPDEF) and residual volume (RVPRVo) were quantified using 4D flow CMR. The associations between RV flow components and other CMR metrics, clinical data, and hemodynamics were analyzed by Spearman’s correlation analysis. RESULTS: In patients with PAH, RVPDF was decreased and RVPRVo was increased compared with the normal control group. The sum of RVPDF and RVPDEF RV was significantly correlated with RV ejection fraction (RVEF) (r=0.802, P