Pulmonary Circulation最新文献

One of the great diagnostic challenges for children with pulmonary arterial hypertension is the need for general anesthesia (GA) to enable successful right heart catheterization. Here, for the first time, we describe how echocardiographic estimates of right ventricular function and pulmonary pressures change in pediatric patients during GA.

Tricuspid annular plane systolic excursion (TAPSE) is usually measured with M-mode using sector line, however, this may not align with the anatomical shortening of the right ventricular (RV). In this study, we compared the different methods to measure TAPSE using three different reference lines (sector line, anatomical line, and apico-annular line). We included 148 patients diagnosed with pulmonary arterial hypertension (PAH) who underwent TTE and right heart catheterization within 2 weeks of each other. TAPSE was measured by M-mode (sector, anatomical), 2D (sector, anatomical), or as tricuspid apico-annular displacement (TAAD). Agreement between measures was assessed using coefficient of variation (COV), Spearman's correlation, and Bland-Altman analysis. Receiver-operating characteristics and Kaplan-Meier analysis were used to explore associations with the combined outcome of death or lung transplantation at 5 years. There was a good concordance between anatomical and sector M-mode with a COV of 15.5 ± 1.6% and a bias of -0.6 ± 3.2 mm. In contrast, anatomical M-mode TAPSE and TAAD differed significantly with the mean difference of 3.3 ± 3.8 mm (COV 30.5 ± 6.1%; p < 0.0001). Among the different 2D methods, anatomical 2D agreed well with anatomical M-mode TAPSE (COV of 11.8 ± 2.0%; r = 0.89; p < 0.0001). Among the five methods, TADD had the strongest association with the combined endpoint of death or transplantation at 5 years (C-statistic 0.64, 95% confidence interval [CI] 0.57-0.71). We concluded that different measures of TAPSE are not interchangeable.

Balloon pulmonary angioplasty (BPA) to treat chronic thromboembolic pulmonary hypertension (CTEPH) is generally reserved for distal obstruction precluding pulmonary endarterectomy (PEA) but can be used in patients with proximal disease who are at high surgical risk or refuse surgery. This single-center retrospective study compared BPA efficacy in patients with proximal versus distal CTEPH. Of the 478 patients, 36 had proximal disease, follow-up was 11.6 months and mean number of BPA 6. After BPA, PVR, and mean pulmonary artery pressure decreased significantly in the proximal and distal groups (from 6.5 to 4.0 WU and 39 to 31 mmHg and from 7.6 to 3.8 WU and 44 to 31 mmHg, respectively, p < 0.001 for all comparisons). NYHA class also improved significantly in both groups, from 3 to 2, whereas the 6-min walk distance, cardiac output, and serum NT pro-BNP showed significant improvements only in the distal group. Thus, when PEA for CTEPH is technically feasible but not performed due to severe comorbidities or patient refusal, BPA can produce significant hemodynamic improvements, albeit less marked than in patients with distal disease. Better patient selection to BPA might improve outcomes in patients with proximal disease who are ineligible for PEA.

Treatment modalities for pulmonary arterial hypertension (PAH) improve quality of life and walk distance. However, none of these therapies alter the structural/functional pulmonary vascular integrity that results in vascular remodeling. PAH smooth muscle cells share biological characteristics with cancer cells, which may be potential therapeutic targets for PAH. We present a case of a patient with connective tissue disease (CTD)-associated PAH treated on triple therapy who developed metastatic lung adenocarcinoma. While on PAH triple-therapy, she received a combination of carboplatin, pemetrexed, and pembrolizumab. She eventually had a complete pathologic response, no evidence of cancer recurrence, and significant improvement of PAH/overall clinical status. After discontinuation of neoplastic therapy, her clinical status worsened, she eventually passed away, and lung biopsy findings revealed evidence of severe pulmonary smooth muscle cell hypertrophy and pulmonary veno-occlusive disease. This report suggests that combined chemotherapy and immunotherapy may influence the efficacy of PAH therapies and improve clinical status.

COVID-19 associates with a hypercoagulant state and an increased risk for venous thromboembolic events (VTEs). Whether severe COVID-19 infection requiring extracorporeal membrane oxygenation (ECMO) support might lead to chronic pulmonary perfusion abnormalities and chronic thromboembolic pulmonary disease/hypertension remains unclear. The purpose of this study was to evaluate chronic pulmonary perfusion abnormalities in long-term survivors of COVID-19-related severe acute respiratory distress syndrome (ARDS) treated by ECMO at our institution. Pulmonary perfusion was examined by ventilation/perfusion (V/Q) single-photon emission computed tomography or V/Q planar scintigraphy at least 3 months after ECMO explantation, comorbidities and incidence of thromboembolic events were recorded as well. Of 172 COVID-19 patients treated by ECMO for severe COVID-19 pneumonia between March 2020 and November 2021, only 80 were successfully weaned from ECMO. Of those, 37 patients were enrolled into the present analysis (27% female, mean age 52 years). Median duration of ECMO support was 12 days. In 24 (65%) patients VTE was recorded in the acute phase (23 patients developed ECMO cannula-related deep vein thrombosis, 5 of them had also a pulmonary embolism, and one thrombus was associated with a central catheter). The median duration between ECMO explantation and assessment of pulmonary perfusion was 420 days. No segmental or larger mismatched perfusion defects were then detected in any patient. In conclusion, in long-term survivors of COVID-19-related ARDS treated by ECMO, no persistent pulmonary perfusion abnormalities were detected although VTE was common.

The present study aimed to evaluate the efficacy of long-term riociguat sequentially combined with balloon pulmonary angioplasty (BPA) for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Eight inoperable CTEPH patients were enrolled in this study, who have been administrated riociguat 2.5 mg three times daily for about 8 years, then underwent several sessions of BPA procedures. Data are prospectively collected to evaluate clinical outcomes, hemodynamics, exercise capacity, and right heart size and function by echocardiography at baseline, 8 years after riociguat, and 3 months after the final BPA. Eight patients (mean age 54.9 ± 11.4 years) were treated with riociguat 2.5 mg three times daily for 95.0 ± 10.7 months. Cardiac index (CI) (1.5 ± 0.5 L/min/m² to 2.4 ± 0.6 L/min/m², p = 0.005), 6 min walking distance (6MWD) (329.6 ± 87.5 m to 418.1 ± 75.8 m, p = 0.016), and pulmonary vascular resistance (PVR) (1336.9 ± 320.2 dyn·s·cm^-5 to 815.4 ± 195.6 dyn·s·cm^-5, p = 0.008) were significant improvement after riociguat treatment. Mean 4.1 ± 1.6 additional combinational BPA sessions and mean 18.8 ± 8.1 balloon dilations were performed. Mean pulmonary artery pressure (54.1 ± 11.1 mmHg to 33.6 ± 7.7 mmHg, p = 0.002) and PVR (815.4 ± 195.6 dyn·s·cm^-5 to 428.3 ± 151.2 dyn·s·cm^-5, p＜0.001) were further decreased. CI (2.4 ± 0.6 L/min/m² to 2.7 ± 0.7 L/min/m², p = 0.028) and 6MWD (418.1 ± 75.8 m to 455.7 ± 100.0 m, p = 0.038) were increased significantly. After long-term riociguat treatment, sequential combination with BPA delivered considerably incremental benefits on exercise capacity and pulmonary hemodynamics, as well as right heart size and function of technically inoperable CTEPH patients.

Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts.

In pulmonary hypertension (PH) associated with chronic lung disease (CLD), identifying patients who would benefit from pulmonary vasodilators is a significant clinical challenge because the presence of PH is associated with poorer survival. This study evaluated the severity of pulmonary circulation impairment in patients with CLD-PH using pulmonary perfusion single-photon emission computed tomography/computed tomography (SPECT/CT). This single-center, observational study enrolled patients with CLD-PH who had a mean pulmonary arterial pressure (PAP) ≥ 25 mmHg, as confirmed by right heart catheterization. The primary outcome was to measure the percentage of pulmonary perfusion defect (%PPD), calculated by dividing the perfusion defect volume from perfusion SPECT images by the lung volume from CT scan images. The secondary outcome was to assess the correlation between %PPD and baseline characteristics. The median %PPD was 52.4% (interquartile range, 42.5%-72.3%) in 22 patients. In multivariate linear regression analysis, both forced vital capacity (β = 0.58, p = 0.008) and mean PAP (β = 0.68, p = 0.001) were significantly correlated with %PPD. In conclusion, significant correlation between mean PAP and %PPD in patients with CLD-PH was observed. This noninvasive assessment of %PPD may be useful for evaluating the severity of pulmonary circulation impairment in CLD-PH.