慢性血栓塞栓性肺高血圧症

Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension arising from persistent major pulmonary artery obstruction after pulmonary embolism. Pulmonary endarterectomy (PEA) remains the standard of care, but up to 40% of patients in European and US registries are inoperable; balloon pulmonary angioplasty (BPA) has emerged as a more accessible interventional modality for suitable patients. This retrospective single-center cohort study evaluated functional improvement, hemodynamic response, and mortality in patients with CTEPH ineligible for PEA who underwent BPA at a Colombian institution (May 2022-October 2025). Risk was stratified using COMPERA 2.0. The primary outcome was improvement of at least one New York Heart Association (NYHA) functional class; secondary outcomes were change in mean pulmonary arterial pressure (mPAP) and all-cause mortality during follow-up. Of 33 patients (median age 63 years; 70% female), 5 (15.1%) were high risk at baseline, 16 (48.4%) intermediate-high, 10 (30.3%) intermediate-low, and 2 (6%) low risk. After BPA, 19 patients (58%) improved NYHA class (95% CI, 39.2%-74.5%), and mPAP fell from 60 (IQR 50-68) to 40 mmHg (IQR 37-48) after a median of three sessions (IQR 2-4) (mean paired reduction 14.05 mmHg, 95% credible interval 9.81-18.07; Hedges' g = 1.22, 95% CI 0.77-1.66). Two deaths (6.0%) occurred, both in high-risk patients. BPA improved NYHA class and pulmonary hemodynamics in severe, inoperable CTEPH, with a low complication rate despite advanced baseline disease. COMPERA 2.0 stratified baseline severity but did not predict the magnitude of hemodynamic response to BPA, consistent with the mechanical nature of the disease.

Objective: To investigate the etiological and across clinical classification features in children with pulmonary hypertension (PH). Methods: Case series study. This case series included 269 children with PH who were admitted to Department No. 2 of Respiratory Medicine, Beijing Children's Hospital, Capital Medical University, from January 2017 to December 2024. Demographic characteristics, clinical manifestations, and etiological evaluation results were collected. According to the etiology, PH was classified into pulmonary arterial hypertension (PAH), PH associated with left heart disease, PH associated with lung diseases and (or) hypoxia, chronic thromboembolic pulmonary hypertension (CTEPH), and PH with unclear and (or) multifactorial mechanisms, and others. Results: Among 269 patients with PH, 153 were boys and 116 were girls. The age of onset was 8.0 (3.2, 28.0) months, and 151 patients (56.1%) developed PH before one year of age. The cohort included 157 patients (58.4%) with PAH, 5 patients (1.9%) with PH associated with left heart disease, 63 patients (23.4%) with PH associated with lung diseases and (or) hypoxia, and 21 patients (7.8%) with PH of unclear and (or) multifactorial mechanisms. No cases of CTEPH were identified. The remaining 23 patients (8.6%) required supplementary etiological annotation, including immune-inflammatory-related PH in 9 patients (3.3%) and PH associated with genetic syndromes or chromosomal abnormalities in 14 patients (5.2%). Among 43 patients with heritable or idiopathic PAH, pathogenic or likely pathogenic genetic variants were identified in 27 patients (62.8%), involving 12 PAH-associated genes. Variants in BMPR2 gene and TBX4 gene were identified in 6 and 5 patients, respectively. Twenty-one patients (7.8%) had overlapping etiologies involving 2 or more potential contributing mechanisms. Conclusions: In this pediatric PH cohort, PAH and PH associated with lung diseases and (or) hypoxia represented the major etiological categories. Some patients had underlying genetic factors, immune-inflammatory conditions, genetic syndromes, or chromosomal abnormalities, and overlapping etiologies were observed.

Background: Chronic thromboembolic disease is a recognised sequela of acute pulmonary embolism and may occur with or without pulmonary hypertension. The purpose of this study was to evaluate differences in chronic thromboembolic burden, lesion type, and distribution between chronic thromboembolic pulmonary disease without pulmonary hypertension (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods: This retrospective single-centre study included patients with CTEPD or CTEPH by right heart catheterisation using 2022 ESC/ERS criteria between 2021 and 2022. Two CTEPH patients were randomly selected per CTEPD patient. CTPA studies were reviewed by a blinded thoracic radiologist, assessing 32 pulmonary vessels from the main to segmental arteries for chronic thromboembolic lesions. Groups were compared by disease distribution, most proximal lesion level, Qanadli obstruction index, lesion type, and location. Exploratory receiver operating characteristic (ROC) analysis assessed discriminatory performance. Results: The 44 CTEPD and 88 CTEPH patients included had no differences in age, BMI, or sex. CTEPH patients had shorter six-minute walk distance (p = 0.001), greater right ventricular dilation/dysfunction (p < 0.001), and lower prevalence of deep vein thrombosis (p = 0.03). CTPA identified more lesions in CTEPH (21.2 vs. 10.0 lesions/case, p < 0.001) across more lobes (4.8 vs. 3.4, p < 0.001), with more proximal main/lobar disease (p < 0.001). CT obstruction index was 51% in CTEPH and 26% in CTEPD (p < 0.001). Total lesion number best discriminated CTEPH from CTEPD (AUC 0.91; optimal cutoff, 16 lesions). Conclusions: CTEPH demonstrates a greater, more diffuse, and more proximal chronic thromboembolic lesion burden on CTPA than CTEPD.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a late complication of acute pulmonary embolism (PE), characterized by persistent elevation of pulmonary artery pressure and pulmonary vascular resistance due to narrowing and/or obstruction of pulmonary arteries. In some cases, thromboembolic obstruction of pulmonary artery branches may occur without pulmonary hypertension after PE (chronic thromboembolic disease - CTED). Importantly, treatment strategies for CTED and CTEPH differ. Difficulties arise in determining the optimal management for patients with previous PE, CTEPHtypical lesions on CT pulmonary angiography and mobile right heart mass (tumor, thrombus, vegetation), particularly when right heart catheterization carries a high risk of complications. We present a 50-year-old female with previous massive PE. Followup examination revealed a mobile right atrial mass interpreted as "myxoma/thrombus" and signs of thromboembolic involvement of the right pulmonary artery branch. The patient underwent surgical removal of the right atrial mass and pulmonary thromboendarterectomy from the right pulmonary artery branch. Morphological examination confirmed organized thrombi in pulmonary artery branches and a large "old" thrombus in the right atrial cavity attached to the Eustachian valve by thin connective tissue strands. This case illustrates diagnostic challenges of CTED/CTEPH in the presence of intracardiac masses and underscores the role of surgical methods in diagnosis and treatment.

OBJECTIVE: To evaluate the early outcomes of original controllable pulmonary thromboendarterectomy (PEA) in the treatment of CTEPH. MATERIAL AND METHODS: A single-center cohort retrospective-prospective study enrolled 74 CTEPH patients who underwent PEA between 2018 and 2024. All procedures were performed under deep hypothermic circulatory arrest using a patented method for assessing the completeness of endarterectomy (RF patent RU 2794557 C1). Original technique compares preoperative anatomical "map" of lesion (template No. 1) based on CT angiography and selective pulmonary angiography with intraoperative thromboembolic material placed on a sterile template No. 2. The primary endpoint was >50% reduction in mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR). Invasive assessment was performed immediately after surgery and at discharge. RESULTS: Median age was 52.5 years [43; 65]; 59.2% were male. A total of 73 patients (98.6%) completed the study. Cardiopulmonary bypass time was 216 min [188; 236], circulatory arrest time - 50 min [40; 60]. mPAP decreased by 62% [53.7; 68.2] at discharge from ICU; PVR dropped from 11.45 Wood units [7.4; 14.2] to 1.95 Wood units [1.4; 2.4] (p<0.001). Residual pulmonary hypertension (mPAP ≥25 mmHg and PVR ≥2.5 Wood units) was observed in 9 (12.3%) patients. The six-minute walk distance increased from 347 to 420 m (p<0.001); 85.7% of patients achieved NYHA class I-II. Complications included reperfusion pulmonary edema (41%), reintubation (8.1%), stroke (4.1%), and pneumonia (22.2%). In-hospital mortality was 1.4% (n=1; cause: generalized infection with multiple organ failure). CONCLUSION: Controllable pulmonary thromboendarterectomy provides objective intraoperative assessment of completeness of PEA without prolonged circulatory arrest time. Hemodynamic, clinical and functional outcomes are consistent with the best international standards. The incidence of residual pulmonary hypertension and mortality do not exceed those of expert centers.