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.

OBJECTIVE: This research evaluates the effect of balloon pulmonary angioplasty (BPA) on cardiac electrophysiological changes in patients with chronic thromboembolic pulmonary hypertension (CTEPH).
METHODS: Involving a retrospective analysis of 39 CTEPH patients (average age 61 ± 11), who had at least two BPAs and paired ECGs pre- and post-surgery, we examined changes in ECG indicators of right ventricular hypertrophy and their correlation with hemodynamic results.
RESULTS: BPA yielded marked improvements in cardiac function and hemodynamics. ECG parameters, specifically the Lewis criteria and Butler-Leggett score, correlated strongly with hemodynamics and were predictive of a mean pulmonary arterial pressure (mPAP) ≥ 35mmHg. Notably, QRS complex axis normalization was observed in 25 patients, with 14 fully normalizing (range - 30° to + 90°). The qR pattern in V1 vanished in 9 cases, and 75% of the patients in qR pattern in V1 group had QRS complex electrical axis completely returned to normal range. The qR V1 group had higher mPAP and pulmonary vascular resistance (PVR), and lower cardiac output and index compared to the non-qR V1 group, alongside a higher Butler-Leggett score.
CONCLUSIONS: BPA enhances cardiac function and hemodynamics in CTEPH patients, with certain ECG measures such as Lewis criteria and Butler-Leggett score reflecting the severity of hemodynamic impairment. The reversal of QRS axis deviation and the disappearance of the qR pattern in lead V1 may serve as valuable indicators for assessing post-BPA satisfaction in CTEPH patients.

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UNASSIGNED: Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension (CTEPH) is limited by a lack of safe and effective tools for crossing these lesions. We aim to identify a safety window for an intraluminal crossing device in this vascular bed by studying the piercing properties of pulmonary arterial vessel walls and intraluminal CTEPH lesion specimens. As a secondary objective, we also describe the histopathologic features of CTEPH lesions.
UNASSIGNED: Specimens were procured from 9 patients undergoing pulmonary endarterectomy. The specimens were subsampled and identified grossly as arterial wall or intraluminal CTEPH lesions. The force needed for tissue penetration was measured using a 0.38-mm (0.015-in) diameter probe in an ex vivo experimental model developed in our lab. Concurrent histology was also performed.
UNASSIGNED: The mean force needed to penetrate the arterial wall and intraluminal CTEPH lesions was 1.75 ± 0.10 N (n = 121) and 0.30 ± 0.04 N (n = 56), respectively (P < .001). Histology confirmed the presence of intimal hyperplasia with calcium and hemosiderin deposition in the arterial wall as well as an old, organized thrombus in the lumen.
UNASSIGNED: The pulmonary arterial wall is friable and prone to perforation during instrumentation with workhorse coronary guide wires. However, the results of this study demonstrate that a much lower force is needed for the 0.38-mm (0.015-in) probe to penetrate an intraluminal CTEPH lesion compared to pulmonary arterial intima. This finding suggests the existence of a safety window for lesion-crossing devices, enabling effective balloon pulmonary angioplasty.

Outcomes of balloon pulmonary angioplasty (BPA) using intravascular ultrasound (IVUS) with a rounded tip to cross a total occlusion lesion in chronic thromboembolic pulmonary hypertension without passing a conventional guidewire remain unclear. Even in initially unsuccessful cases of total occlusion lesions, improved blood flow may be observed in the postprocedural phase as a result of breaking the thrombosis cap surface. The aim of the study was to verify the initial success of BPA with a new technique using IVUS for total occlusion lesions and to evaluate peripheral blood flow in the postprocedural phase in initially unsuccessful cases.
Initial success rate and associated factors were evaluated in 50 total occlusion lesions by attempted IVUS passage using a new technique without a preceding guidewire from August 2016 to February 2024. Peripheral blood flow improvement in the postprocedural phase among initially unsuccessful cases was investigated via subsequent angiographic examination or during follow-up.
The success rate was 54%, and the sole determinant of success was the angle of the lesion. Peripheral perfusion improved in 15 of 23 initially unsuccessful cases in the postprocedural phase. Even in the unsuccessful cases, significant improvement in peripheral perfusion occurred in the postprocedural phase in the patients undergoing dilatation with the balloon advanced partway into the total occlusion lesion to confirm intravascular location of the balloon (P = .0257).
BPA treatment of total occlusion lesions may improve perfusion in the postprocedural phase even following an initially unsuccessful treatment. Maximizing use of IVUS may provide an adjunctive role in BPA of total occlusion lesions.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is an effective intervention for patients with chronic thromboembolic pulmonary disease (CTEPD). We aimed 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 mm Hg was a predictor of BPA-related complications. NoPEA-CTEPH patients had more improvement in 6-min 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.

UNASSIGNED: Balloon pulmonary angioplasty (BPA) is currently performed at select centers worldwide, with the current standard of practice being postprocedural inpatient monitoring for 24 to 72 hours. We sought to evaluate the safety and efficacy of BPA in a cohort of patients with chronic thrombo-embolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH) and outline a protocol for implementation in the outpatient setting.
UNASSIGNED: All patients with distal, inoperable CTEPH, residual symptoms after pulmonary endarterectomy, or symptomatic CTEPD from July 1, 2020, to June 30, 2022, were evaluated by a multidisciplinary chronic thromboembolic pulmonary hypertension team for consideration of BPA. Patients undergoing each BPA session adhered to a regimented protocol developed and implemented at our institution. Safety and efficacy were retrospectively evaluated with a mean follow-up time of 8.5 months.
UNASSIGNED: Eighteen patients underwent a total of 78 BPA sessions. Overall, there was a significant improvement in World Health Organization functional class and mean improvement in 6-minute walking distance of +67 m. Hemodynamic parameters significantly improved with a mean decrease in mean pulmonary artery pressure and pulmonary vascular resistance of 7.3 ± 5.8 mm Hg and 1.7 ± 1.5 Wood units, respectively (P <.05). Complication rates were low with 3 (3.9%) of 78 patients developing scant hemoptysis and 1 (1.3%) of 78 experiencing vascular injury requiring inpatient hospitalization.
UNASSIGNED: BPA is both safe and effective when implemented in the outpatient setting using a regimented protocol provided there are necessary contingencies in place.

UNASSIGNED: Balloon pulmonary angioplasty (BPA)-related complications are not uncommon and could contribute to perioperative mortality. However, there is a lack of a prediction model for BPA-related complications.
UNASSIGNED: Data from consecutive patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) who underwent BPA were retrospectively analyzed. The primary outcome was BPA-related complications. The secondary outcomes were mortality and hemodynamics after BPA.
UNASSIGNED: A total of 207 patients with 614 BPA sessions were included. Complications occurred during 63 sessions (10.26%) in 49 patients. Hemoptysis or hemosputum (6.51%) was the most common complication, whereas pulmonary reperfusion edema was rare (0.49%). Multivariable logistic regression identified that disease duration, mean pulmonary arterial pressure (mPAP) and the proportion of occlusion lesions were correlated with BPA complications. A nomogram was constructed accordingly, which had the highest area under curve (0.703) and was superior to previously reported predictors [nomogram vs. mPAP, net reclassification index (95% confidence interval (CI)), 0.215 (0.002, 0.427), p = 0.047; integrated discrimination index (95% CI), 0.059 (0.010, 0.109), p = 0.018]. The nomogram was found to be accurate based on validation and calibration (slope 0.978, Bier score 0.163). After adjusting for the number of BPA sessions in multivariable linear regression, the occurrence of complications was not associated with hemodynamic improvement after BPA. The 3-year survival was also comparable between patients with and without complications (98.0% vs. 94.8%, log-rank p = 0.503).
UNASSIGNED: The nomogram, comprising mPAP, the proportion of occlusion lesions and disease duration, could better predict BPA-related complications than previously reported single parameters. Distinctively, the occurrence of complications did not impair the beneficial impact of BPA on hemodynamics and survival. The occurrence of complications should not discourage patients from continuing BPA sessions.

Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH, and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassaemia and 14.3% with sickle cell disease, with a diagnosis of PH confirmed by right heart catheterization were included in the study. The patients were divided into three groups according to the PH haemodynamic definition and overall diagnostic approach: 42.9% had precapillary PH (pulmonary arterial hypertension-PAH group), 25% had post-capillary PH, and 32.1% had chronic thromboembolic PH (CTEPH) (29% of b-thalassemia and 50% of SCD patients). The therapeutic approach in each group and its impact on the outcome and haemodynamics were recorded. PAH-specific drug therapy received 82.1% of patients, and balloon pulmonary angioplasty (BPA) was performed in six patients with CTEPH. There were statistically significant differences in baseline mPAP and PVR values between the CTEPH-haemolytic anaemia group and other groups. PAH-specific drug therapy resulted in haemodynamic improvement for the PAH group. Patients who underwent BPA had improved pulmonary haemodynamics. The median survival time was 162 months, and the survival rate was 1 year-100%; 2, 3, 4, 5, and 6 years-96%; 9 years-90%; and 13 years-78%. In patients with haemolytic anaemia, the wide spectrum of induced PH highlighted the importance of a correct predominant diagnosis. BPA in CTEPH patients and specific-PAH drug therapy for PAH patients represent potential therapeutic strategies; however, the management should be offered in expert PH centres under individualized approaches for patients.

UNASSIGNED: We aimed to investigate the response to balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH) using semi-quantitative analysis of lung perfusion SPECT/CT.
UNASSIGNED: This is a single-center retrospective study of patients with CTEPH who underwent BPA and pre- and post-BPA lung perfusion SPECT/CT between 2015 and 2022. Segmental defects on SPECT/CT were visually assessed and semi-quantitatively scored as 1 (large defect) or 0.5 (moderate defect) in accordance with modified PIOPED II criteria. The perfusion defect score was defined as (Σ segmental defect scores/18) × 100 (%). Associations between perfusion defect score and hemodynamic or functional parameters including WHO functional class, six-minute walking distance (6MWD), serum B-type natriuretic peptide (BNP), mean arterial pulmonary pressure (mPAP), pulmonary vascular resistance (PVR), and tricuspid regurgitation pressure gradient (TRPG) on echocardiography were statistically analyzed.
UNASSIGNED: A total of 24 consecutive patients were included. The perfusion defect score significantly improved after BPA (median 58.3% vs. 47.2%, P < 0.001), in conjunction with the WHO functional class, 6MWD, serum BNP, mPAP, and TRPG. Perfusion defect scores were significantly correlated with 6MWD (rho = - 0.583, P < 0.001), serum BNP (rho = 0.514, P < 0.001), mPAP (rho = 0.583, P < 0.001), and PVR (rho = 0.575, P < 0.001). The improvement in the perfusion defect score was significantly associated with improvement in mPAP (rho = 0.844, P < 0.001).
UNASSIGNED: Our results suggest that semi-quantitative analysis of lung perfusion SPECT/CT can provide a potential imaging biomarker for monitoring the efficacy of BPA.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s13139-024-00858-1.