Effects of right ventricular remodeling in chronic thromboembolic pulmonary hypertension on the outcomes of balloon pulmonary angioplasty: a 2D-speckle tracking echocardiography study.

BACKGROUND: Balloon pulmonary angioplasty (BPA) improves the prognosis of chronic thromboembolic pulmonary hypertension (CTEPH). Right ventricle (RV) is an important predictor of prognosis in CTEPH patients. 2D-speckle tracking echocardiography (2D-STE) can evaluate RV function. This study aimed to evaluate the effectiveness of BPA in CTEPH patients and to assess the value of 2D-STE in predicting outcomes of BPA. METHODS: A total of 76 patients with CTEPH underwent 354 BPA sessions from January 2017 to October 2022. Responders were defined as those with mean pulmonary artery pressure (mPAP) ≤ 30 mmHg or those showing ≥ 30% decrease in pulmonary vascular resistance (PVR) after the last BPA session, compared to baseline. Logistic regression analysis was performed to identify predictors of BPA efficacy. RESULTS: BPA resulted in a significant decrease in mPAP (from 50.8 ± 10.4 mmHg to 35.5 ± 11.9 mmHg, p < 0.001), PVR (from 888.7 ± 363.5 dyn·s·cm-5 to 545.5 ± 383.8 dyn·s·cm-5, p < 0.001), and eccentricity index (from 1.3 to 1.1, p < 0.001), and a significant increase in RV free wall longitudinal strain (RVFWLS: from 15.7% to 21.0%, p < 0.001). Significant improvement was also observed in the 6-min walking distance (from 385.5 m to 454.5 m, p < 0.001). After adjusting for confounders, multivariate analysis showed that RVFWLS was the only independent predictor of BPA efficacy. The optimal RVFWLS cutoff value for predicting BPA responders was 12%. CONCLUSIONS: BPA was found to reduce pulmonary artery pressure, reverse RV remodeling, and improve exercise capacity. RVFWLS obtained by 2D-STE was an independent predictor of BPA outcomes. Our study may provide a meaningful reference for interventional therapy of CTEPH.

Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Disease: Success Rate and Complications among Different Patient Populations.

INTRODUCTION: 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.

Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.

BACKGROUND: Sarcoidosis-associated pulmonary hypertension (SAPH) is listed in Group 5 of the clinical classification of pulmonary hypertension, due to its complex and multifactorial pathophysiology. The most common cause of SAPH development is advanced lung fibrosis with the associated destruction of the vascular bed, and/or alveolar hypoxia. However, a substantial proportion of SAPH patients (up to 30%) do not have significant fibrosis on chest imaging. In such cases, the development of pulmonary hypertension may be due to the lesions directly affecting the pulmonary vasculature, such as granulomatous angiitis, pulmonary veno-occlusive disease, chronic thromboembolism or external compression of vessels by enlarged lymph nodes. Based on the case of a 69-year-old female who developed SAPH due to pulmonary arteries stenosis, diagnostic difficulties and therapeutic management are discussed. CASE PRESENTATION: The patient, non-smoking female, diagnosed with stage II sarcoidosis twelve years earlier, presented with progressive dyspnoea on exertion, dry cough, minor haemoptysis and increasing oedema of the lower limbs. Computed tomography pulmonary angiography (CTPA) showed complete occlusion of the right upper lobe artery and narrowing of the left lower lobe artery, with post-stenotic dilatation of the arteries of the basal segments. The vascular pathology was caused by adjacent, enlarged lymph nodes with calcifications and fibrotic tissue surrounding the vessels. Pulmonary artery thrombi were not found. The patient was treated with systemic corticosteroid therapy and subsequently with balloon pulmonary angioplasty. Partial improvement in clinical status and hemodynamic parameters has been achieved. CONCLUSIONS: An appropriate screening strategy is required for early detection of pulmonary hypertension in sarcoidosis patients. Once SAPH diagnosis is confirmed, it is crucial to determine the appropriate phenotype of pulmonary hypertension and provide the most effective treatment plan. Although determining SAPH phenotype is challenging, one should remember about the possibility of pulmonary arteries occlusion.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function.

The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines.

The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia.

Background: Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent pulmonary hypertension after pulmonary endarterectomy (PEA) include targeted medical therapy and balloon pulmonary angioplasty (BPA). BPA is an emerging treatment modality that has been reported to improve functional capacity, pulmonary hemodynamics, and right ventricular function. Reports from expert centers are promising, but more data are needed to make the results more generalizable. Materials and Methods: We conducted a prospective analysis of nine consecutive CTEPH patients who underwent balloon pulmonary angioplasty (BPA) sessions at Pauls Stradins Clinical University Hospital in Riga, Latvia between 1 April 2022 and 1 July 2023. We assessed World Health Organization (WHO) functional class, 6 min walk distance (6MWD), blood oxygen saturation (SpO2), brain natriuretic peptide (BNP) level at baseline and 3 months after the first BPA session. For two patients on whom repeated BPA sessions were performed, we additionally assessed cardiac output (CO), pulmonary vascular resistance (PVR), and mean pulmonary artery pressure (mPAP). Results: A total of 12 BPA procedures for nine patients were performed; repeated BPA sessions were performed for two patients. Our results show a reduction in BNP levels and improvement in WHO functional class, 6MWD, and SpO2 after the first BPA session. Improvement in 6MWD was statistically significant. Additionally, an improvement in pulmonary hemodynamic parameters was observed. Conclusions: Our data show that BPA is an effective interventional treatment modality, improving both the pulmonary hemodynamics and functional status. Moreover, BPA is safe and excellently tolerated.

Balloon Pulmonary Angioplasty for Inoperable Chronic Thromboembolic Pulmonary Hypertension: Insights from a Pilot Low-Volume Centre Study and a Comparative Analysis with Other Centres.

Background and Objectives: The aim of this study was to evaluate the effectiveness and safety of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) in the Vilnius Pulmonary Hypertension (PH) Referral Centre and to provide a comparative analysis with other centres. Materials and Methods: This study included all BPA procedures performed between 2019 and 2024 in a single tertiary centre. Invasive haemodynamic parameters and clinical variables were assessed at baseline; at the end of invasive treatment; and at the conclusion of follow-up, an average of 8.6 months after the last BPA. A literature review was also performed. Results: Twenty-six patients with inoperable CTEPH were enrolled. The mean age of the patients was 61.6 (40-80) years. Each patient underwent a mean of 3.84 (1-9) procedures. Follow-up data were available for 12 patients with an average of 6.08 (3-9) procedures. Mean pulmonary arterial pressure decreased by 32% (p < 0.001) and pulmonary vascular resistance by 41% (p = 0.001) at follow-up compared with the baseline measurements. There was also a significant 80% (p < 0.001) reduction in brain natriuretic peptide levels and a 30% (p = 0.04) increase in 6-min walk distance. The BPA procedures were generally safe in this low-volume centre setting, with only 17% of procedures having non-severe and non-fatal procedure-related complications. The most common complications included vessel dissection (10%), pulmonary vascular injury with haemoptysis (3%), and hyperperfusion pulmonary oedema (1%), which was successfully treated in all patients. Conclusions: The results of the present study demonstrate that the BPA procedure is an effective and safe treatment for individuals with inoperable CTEPH, being associated with significant improvements in hemodynamic parameters and functional capacity and a low risk of major complications in the low-volume tertiary PH centre setting.

Development and Validation of a Nomogram for Balloon Pulmonary Angioplasty-Related Complications in Patients with Chronic Thromboembolic Pulmonary Hypertension.

Background: 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. Methods: 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. Results: 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). Conclusions: 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.

Impact of psychiatric disorders on the hemodynamic and quality of life outcome of balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension: a retrospective study.

BACKGROUND: Balloon pulmonary angioplasty (BPA) has beneficial effects on pulmonary hemodynamics, exercise capacity, and quality of life (QOL) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Recently, emerging evidence suggests a relationship between CTEPH and psychiatric disorders (PD). However, data on the clinical efficacy of BPA in CTEPH patients with PD are lacking. METHODS: We retrospectively analyzed 75 patients with inoperable/residual CTEPH who underwent BPA and right-sided heart catheterization before the initial BPA and within 1 year after the last procedure. QOL was evaluated using the European Quality of Life Five Dimension (EQ-5D) scale in 27 patients before and after BPA sessions. Baseline and post-procedural hemodynamic, functional, and QOL parameters were compared between the patients with and without PD. RESULTS: Among the 75 participants, 22 (29.3%) patients were categorized in the PD group. Although PD group had a similar mean pulmonary artery pressure level compared with non-PD group (40 ± 7 vs. 41 ± 9 mmHg, p = 0.477), they tended to have unfavorable QOL status (0.63 ± 0.22 vs. 0.77 ± 0.19, p = 0.102). BPA significantly improved pulmonary hemodynamics, laboratory parameters and exercise tolerance in both groups. BPA also significantly improved EQ-5D scores in the non-PD group (from 0.77 ± 0.19 to 0.88 ± 0.13, p < 0.001), but the scores remained unchanged in the PD group (from 0.63 ± 0.22 to 0.67 ± 0.22, p = 0.770). During the long-term period [1,848 (1,055-2,565) days], both groups experienced similar mortality rates (PD 4.6% vs. non-PD 5.7%, p = 1.000). CONCLUSIONS: BPA improved hemodynamic and functional parameters irrespective of PD, but its effect on QOL was limited in patients with PD.

Electrocardiographic changes after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: Data from the Hellenic Registry.

BACKGROUND: Balloon pulmonary angioplasty (BPA) has provided an effective invasive treatment for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). The hemodynamic improvement achieved by BPA has significantly increased the long-term prognosis of these patients, mostly by reversing the negative remodeling of the right ventricle (RV). MATERIALS AND METHODS: In a cohort of 17 patients with symptomatic CTEPH hemodynamic data were collected before and after the completion of BPA sessions. After the confirmation of statistically significant hemodynamic improvement, we examined the changes in certain prespecified electrocardiographic (ECG) parameters (PR interval duration, QRS duration, QTc interval duration, R wave and S wave amplitude in lead I, R wave and S wave amplitude in precordial leads V1, V5 and V6) before the initiation and one month after the completion of BPA sessions. In addition, ECGs were qualitatively assessed before and after treatment for the presence of ECG abnormalities related to PH, proposed by the guidelines of the European Society of Cardiology (ESC). The term ESC criteria 1-6 was used for their description. RESULTS: Statistically significant correlation (p < 0.05) was found between the reduction in mean pulmonary artery pressure (mPAP) and the decrease of the depth of the S wave in leads I (p 0.0069), V5 (p 0.0003), V6 (p 0.0011) and in the R wave amplitude in leads V5 (p 0.0122) and V6 (p 0.0185). From the ESC criteria, RV strain pattern was the commonest in the initial cohort, with significant improvement after therapies. CONCLUSION: Hemodynamic improvement after BPA therapies is correlated with improved ECG amplitude parameters in leads I, V5 and V6. RV strain pattern is common among untreated patients with significant improvement after therapies.

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death. CTEPH is a progressive pulmonary vascular disease with a poor prognosis if left untreated. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), which is usually performed only in specialized centers. In recent years, balloon pulmonary angioplasty (BPA) and drug therapy for CTEPH have also shown good results. This review discusses the complex pathogenesis of CTEPH and presents the standard of care, PEA, as well as a new device called BPA, which is showing remarkable progress in efficacy and safety. Additionally, several drugs are now demonstrating established evidence of efficacy in treating CTEPH.

Bilateral versus unilateral balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension.

BACKGROUND: To evaluate the safety and efficacy of bilateral balloon pulmonary angioplasty (BPA) as compared with unilateral BPA for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). METHOD: We reviewed 210 consecutive BPA sessions for 92 CTEPH patients, including 124 unilateral BPA sessions and 86 bilateral BPA sessions. Radiation exposure, operation details, lesions characteristics and the occurrence of complications were compared between unilateral BPA and bilateral BPA. 131 BPA sessions with a hemodynamics follow-up were included for efficacy analysis, in which hemodynamics changes were compared. Logistic regression analysis was used to identify factors associated with the occurrence of complications. RESULT: Bilateral BPA treated more lobes, arteries and lesions [3 (2, 4) vs. 2 (1, 3) lobes, p < 0.001; 8 (5.5, 10) vs. 6 (4, 8) vessels, p = 0.003; 9 (7, 12) vs. 8 (5, 10) lesions, p = 0.01] in one single session than unilateral BPA in a comparable operation duration and amount of contrast media given. Overall, the occurrence of complications was similar between bilateral BPA and unilateral BPA [9 (10.5%) vs. 12 (9.7%), p = 0.83]. Hemodynamics effects didn't differ significantly between bilateral BPA and unilateral BPA in a single session [mPAP, - 4.5 ± 8.6 vs. - 3.6 ± 7.3 mmHg, p = 0.52; PVR, - 1.1 (- 3.5, 0.8) vs. - 1.8 (- 5.2, 0.3) Wood units, p = 0.21]. For the initial BPA session, bilateral BPA also treated more lobes, arteries and lesions than unilateral BPA [3 (2, 4) vs. 2 (1, 2) lobes, p < 0.001; 8.0 (5.8, 9.3) vs. 6.0 (4.0, 8.0) vessels, p = 0.04; 9 (6, 12) vs. 7 (4, 10) lesions, p = 0.02]. The occurrence of complications was also similar [5 (13.2%) vs. 5 (9.3%), p = 0.80], even in patients with poor baseline hemodynamics. Univariate regression analysis reveals the number of lobes treated/session, but not bilateral BPA, as predictive factors of complications. CONCLUSION: Bilateral BPA may be safely and effectively performed in patients with CTEPH without increasing operation duration and radiation burden, even in patients with unfavorable baseline hemodynamics.

The original and two new derivative versions of the COMPERA 2.0 risk assessment model: useful tools for guiding balloon pulmonary angioplasty.

BACKGROUND: The COMPERA 2.0 4-stratum (4-S) risk score has been demonstrated superior over the 3-stratum (3-S) one in patients with pulmonary arterial hypertension and medically managed patients with chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to determine the prognostic value of the original 4-S and 3-S COMPERA 2.0 risk score and two new derivative versions in CTEPH patients who underwent balloon pulmonary angioplasty (BPA). METHODS: We retrospectively enrolled 175 BPA-treated patients with CTEPH. We assessed the risk stratification before and after each BPA session of CTEPH patients by the original 4-S and 3-S COMPERA 2.0 risk score (by rounding decimal to the nearest integer) and two new proposed derivative versions: the modified version (by rounding decimal to the next integer) and a hybrid version that fuses the original and modified versions. The primary endpoint was clinical worsening events. The secondary outcomes were achieving low-risk profile and mean pulmonary arterial pressure (mPAP) < 30 mmHg at follow-up. We used the Kaplan-Meier curve analysis to assess the survival differences between stratified patients. The comparative model's performance was evaluated in terms of discrimination by Harrell's C-index. RESULTS: All versions of COMPERA 2.0 4-S model outperformed the 3-S one in discriminating the differences in echocardiographic and hemodynamic parameters and clinical worsening-free survival rates. The original and hybrid 4-S model could independently predict the primary and secondary endpoints, and the hybrid version seemed to perform better. The first BPA session could significantly improve risk profiles, and these changes were associated with the likelihood of experiencing clinical worsening events, achieving a low-risk profile and mPAP < 30 mmHg at follow-up. The number of BPA sessions required to achieve low risk/mPAP < 30 mmHg increased as the baseline risk score escalated. CONCLUSIONS: The COMPERA 2.0 4-S model outperformed the 3-S one in BPA-treated patients with CTEPH. The 4-S model, especially its hybrid version, could be used to predict clinical outcome before the initiation of BPA and monitor treatment response.

Comparison of fibrosing mediastinitis patients with vs. without markedly increased systolic pulmonary arterial pressure: a single-center retrospective study.

INTRODUCTION: Fibrosing mediastinitis (FM) complicated with pulmonary hypertension (PH) has been considered as an important cause of morbidity and mortality. This study was designed to observe the possible effects of abnormal hemodynamics on patients by conducting a between-group comparison according to the presence of markedly increased systolic pulmonary arterial pressure (SPAP), so as to provide more information for clinical management. MATERIALS AND METHODS: Fifty-one patients with clinically diagnosed FM were divided in two groups (SPAP < 50 mmHg group; SPAP ≥ 50 mmHg group) and retrospectively included in the study. Data mainly including demographic factors, echocardiographic data, results of right heart catheter and computed tomography (CT) examination were retrieved from the medical database. Echocardiographic parameters pre- and post- balloon pulmonary angioplasty (BPA) treatment were also collected in 8 patients. RESULTS: Significant changes in cardiac structure, hemodynamics and cardiac function were detected in patients complicated with markedly increased SPAP. Patients in the SPAP ≥ 50 mmHg group had increased right heart diameter, right heart ratio and velocity of tricuspid regurgitation (VTR) (p < 0.05). Deteriorated right heart function was also observed. There was no significant difference in CT findings between the two groups, except that more patients in the SPAP ≥ 50 mmHg group had pleural effusion (p < 0.05). After primary BPA in 8 patients, improvement in the right atrium proportion was observed. CONCLUSIONS: Changes due to significantly increased SPAP in patients with FM include adverse structure and function of the right heart, but differences in CT findings were not significant. Echocardiography has advantages as a noninvasive tool for the evaluation of cardiac structure, function and hemodynamics in patients with FM.

The long-term survival in patients with chronic thromboembolic pulmonary hypertension: experience from a single center in China.

The long-term prognosis of patients with chronic thromboembolic pulmonary hypertension (CTEPH) receiving different treatments is deserved to be analyzed in modern era of CTEPH treatment. From 2013 to 2019, a total of 364 patients diagnosed with CTEPH were retrospectively included, 14 patients were lost during follow-up. Among 350 patients included in the final analysis: 123 underwent pulmonary endarterectomy (PEA), 121 received balloon pulmonary angioplasty (BPA), and 106 treated with targeted drug alone. The median period of follow-up was 51.2 months, the estimated survival at 1-, 3-, 5- and 7-year was 97.1%, 93.3%, 86.9%, and 82.0% for the whole cohort; 100%, 99.20%, 96.5% and 92.5% in PEA group; 98.4%, 97.4%, 95.3% and 89.3% in BPA group;92.5%, 81.9%, 70.1% and 66.8% in patients who received targeted drug alone. In comparing with targeted treatment along, results of multivariate Cox analysis after adjusting the confounders showed that receiving PEA decreased the risk of death by 83% (HR [hazard ratio] 0.17, 95% CI [Confidence interval] 0.07-0.44) and receiving BPA decreased the risk of death by 89% (HR 0.11, 95% CI 0.04-0.29). In conclusion, the estimated survival of CTEPH patients at 1-, 3-, 5- and 7-year was 97.1%, 93.3%, 86.9%, and 82.0% respectively. The intervention of revascularization, including PEA and BPA, were preferred than treating with targeted drug alone in the view of long-term prognosis of CTEPH.

Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.

Sickle cell disease (SCD), a haemoglobinopathy characterized by chronic haemolysis with acute exacerbations and vascular occlusion episodes, may be complicated by pulmonary hypertension. The latter may be caused by chronic thromboembolic disease of pulmonary artery branches and its management is not well-defined. Herein, we present a case of SCD complicated by chronic thromboembolic pulmonary hypertension of subsegmental pulmonary artery branches successfully treated with endothelin receptor antagonists, orally administered prostacyclin analogs and balloon pulmonary angioplasty. This challenging case highlights the need for clinical awareness of chronic thromboembolic pulmonary hypertension as a specific and potentially curable form of pulmonary hypertension complicating SCD course that may necessitate combined pharmacologic and interventional management.

Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH), defined as precapillary pulmonary hypertension (PH) by right heart catheterization and imaging consistent with chronic thromboembolism, is a long-term complication of pulmonary embolism (PE). Pathobiological mechanisms involve pulmonary artery occlusion from organized thromboembolic material despite at least three months of uninterrupted therapeutic anticoagulation following acute PE and secondary microvasculopathy. Delay in diagnosis and management of CTEPH is associated with poor outcomes. High clinical suspicion, comprehensive assessment of residual dyspnea or exercise intolerance in the aftermath of PE and accurate interpretation of computed tomography pulmonary angiography (CTPA) are pivotal steps in the diagnosis. Ventilation-perfusion (V/Q) scan is the preferred initial radiologic screening tool as normal V/Q essentially rules out CTEPH. Any mismatched perfusion defect on the V/Q scan in the setting of PH or any finding compatible with chronic thromboembolism on CTPA should prompt referral to an expert CTEPH center. Once the diagnosis is verified, all eligible patients should be offered pulmonary thromboendarterectomy (PTE). Pulmonary vasodilators or balloon pulmonary angioplasty are safe and effective in inoperable or post-PTE persistent/recurrent CTEPH. During the course of their disease, a patient may receive a combination of treatments, at times consisting of all three strategies. Lifelong therapeutic anticoagulation is recommended for CTEPH.

Balloon Pulmonary Angioplasty in Patients With Inoperable or Recurrent/Residual Chronic Thromboembolic Pulmonary Hypertension: A Single-Centre Initial Experience.

BACKGROUND: Patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) are often treated with pulmonary arterial hypertension-specific drugs. However, most of these patients remain symptomatic, despite medical treatment. Balloon pulmonary angioplasty (BPA) is an emerging therapeutic intervention for patients with inoperable CTEPH. This study aimed to report the initial experience of BPA in a tertiary referral centre for CTEPH. METHODS: A total of 26 consecutive patients, who underwent 91 BPA sessions, were included in the study. All patients underwent a detailed examination, including 6-minute walking distance (6MWD), and right heart catheterisation at baseline and 3 months after the last BPA session. RESULTS: The mean age of the patients was 51±17 years. Fifteen (15) patients had inoperable CTEPH and 11 patients had residual or recurrent CTEPH post pulmonary endarterectomy (PEA). Functional class improved in 17 of 26 (65%) patients. The 6MWD increased from a mean 315±129 to 411±140 m (p<0.001), and NT pro-BNP reduced from a median 456 to 189 pg/mL (p=0.001). The number of patients who required supplemental oxygen decreased from 11 (42.3%) to five (19%) (p=0.031) after BPA treatment. The mean pulmonary artery pressure decreased from a mean 47.5±13.4 to 38±10.9 mmHg (p<0.001), the pulmonary vascular resistance decreased from a mean 9.3±4.7 to 5.8±2.8 Wood units (p<0.001), and the cardiac index increased from a mean 2.4±0.7 to 2.9±0.6 L/min/m2 (p=0.008). CONCLUSIONS: Balloon pulmonary angioplasty improved haemodynamics, 6MWD, and functional class, and reduced the requirement for supplemental oxygen, with an acceptable risk-benefit ratio in patients with inoperable CTEPH and with residual/recurrent CTEPH.

Effective Balloon Pulmonary Angioplasty in a Patient with Chronic Thromboembolic Complications after Ventriculoatrial Shunt for Hydrocephalus in von Hippel-Lindau Disease.

Von Hippel-Lindau (VHL) disease along with chronic thromboembolic pulmonary hypertension (CTEPH) is a unique and unusual severe complication of ventriculoatrial (VA) shunt implantation in the treatment of hydrocephalus. To the best of our knowledge, this can be the first reported case of an effective treatment with balloon pulmonary angioplasty in a patient with VHL after VA shunt placement. The patient underwent six balloon pulmonary angioplasty procedures. All invasive procedures resulted in haemodynamic and functional improvement.

Balloon pulmonary angioplasty vs. pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis.

Although balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) are effective in chronic thromboembolic pulmonary hypertension (CTEPH), the comparison of their efficacy and safety is still unclear. We identified studies through a systematic review of PubMed, Cochrane Library, and Embase and used a random effects meta-analysis model to synthesize estimates of weighted mean differences or combined effect size. In total, 54 studies were included in this meta-analysis. The survival rates at perioperative/in-hospital period, 2 years, and 3 years were 100%, 99%, and 97%, respectively, in BPA group and 93%, 90%, and 88%, respectively, in PEA group. The variation of 6-min walk distance was 141.80 m in BPA and 100.73 m in PEA when the follow-up was 1-6 months. At < 1-month, 1-6-month, and > 12-month follow-up, the changed results of mean pulmonary arterial pressure were - 18.31, - 17.00, and - 12.97 mmHg in BPA group and - 18.93, - 21.21, and - 21.35 mmHg in PEA group. At < 1-month and 1-6-month follow-up, the changed values of pulmonary vascular resistance were - 542.24 and - 599.77 dyne•s•cm-5 in PEA group and - 443.49 and - 280.00 dyne•s•cm-5 in BPA group. In addition, there was more wide variety of complications in PEA group than in BPA group. BPA might have higher survival rate (perioperative/in-hospital period, 2-year and 3-year follow-up) and fewer types of complications compared with PEA. The improvement in exercise capacity (1-6-month follow-up) in the BPA group might be more pronounced than in PEA group. Moreover, PEA might be superior in improvement of hemodynamic parameters (< 1-month, 1-6-month, and > 12-month follow-up).

Gadolinium contrast balloon pulmonary angioplasty for a patient with chronic thromboembolic pulmonary hypertension and severe iodine allergy.

Chronic thromboembolic pulmonary hypertension (CTEPH) is group 4 pulmonary hypertension caused by organized thrombi in the pulmonary arteries. Balloon pulmonary angioplasty (BPA) is an emerging treatment option for patients with inoperable CTEPH but needs contrast media. Therefore, management can be very difficult in patients who have severe iodine allergies. We present a case of a 61-year-old female with CTEPH. Right heart catheterization showed that the mean pulmonary arterial pressure (mPAP) was 47 mmHg. Her organized thrombi were not surgically accessible, so we performed BPA to improve her hemodynamic status. One session of BPA was performed, but the second session was halted because of iodine-induced anaphylactic shock. Despite the administration of pulmonary arterial hypertension-specific drugs for 3 months, the patient's mPAP was still 33 mmHg. CTEPH patients with mPAP ≥30 mmHg have a poor prognosis, so we decided to perform BPA using gadolinium contrast media. A total of six sessions of gadolinium contrast BPA (Gd-BPA) improved the patient's mPAP to the normal range. Gadolinium contrast media could also be used for visualizing pulmonary arteries during BPA. Our report is the first successful case of Gd-BPA, which improved the patient's hemodynamic status to the almost normal range. Gd-BPA may be an attractive treatment option for patients with inoperable CTEPH and severe iodine allergy.