Figura 1. AL10 segmental branch with critical annular obstruction.

Figura 2. Balloon angioplasty with a 5.0-×-20-mm balloon on AL10 segmental branch.

Figura 3. Post-BPA angiography of AL10 segmental branch.

Figura 4. Anatomical classification of the pulmonary vascular tree and segment names according to th...

Tabla 1. RHC measurements for the different sessions.

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious complication of pulmonary embolism and one of the main causes of chronic pulmonary hypertension (PH), which can progress to right-heart failure and, ultimately, death1. In the last decade, the treatment of CTEPH has seen significant advances, with a multimodal approach recommended in current guidelines. This approach includes pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), and targeted medical therapies to address different patient profiles2. Growing evidence increasingly supports the safety and efficacy of BPA in patients with distal pulmonary artery lesions, especially in older individuals or those with comorbidities such as respiratory diseases or exercise intolerance. These characteristics mean that they are inoperable or high-risk candidates for pulmonary endarterectomy, positioning BPA as a valuable therapeutic option in such cases3.

Clinical case

The patient was a 60-year-old man with a history of obesity (BMI: 34.3), arterial hypertension, colon cancer in remission (surgical treatment + chemotherapy in 2010), heterozygous Factor V Leiden, recurrent deep vein thrombosis, and CTEPH (central and peripheral PE in 2020 and 2021), whose usual medications are rivaroxaban 20 mg/day, losartan 50 mg/day, and sildenafil 125 mg/day. He was referred by a clinical cardiologist in September 2023 due to New York Heart Association (NYHA) class IV functional dyspnea. He was assessed at a referral center in Buenos Aires City for PEA and was considered a high surgical risk patient; thus, BPA was indicated. A ventilation/perfusion lung scintigraphy was performed; it showed multiple bilateral lobar and segmental defects with ventilation/perfusion mismatch. Right-heart catheterization (RHC) and pulmonary angiography were conducted, reporting a mean pulmonary artery pressure (mPAP) of 48 mmHg, wedge pressure of 12 mmHg, minute volume (MV) of 6.4 L/min, and pulmonary vascular resistance (PVR) of 5.6 Wood units, with obstructions in bilateral lobar and segmental pulmonary arteries. In a multidisciplinary session, a decision was made to proceed with BPA. The patient underwent 2 BPA sessions, both performed under local anesthesia via right transfemoral access with an 8-French introducer, selective catheterization with a JR guide catheter, a 0.014” angioplasty guidewire, and progressive balloon inflations using 2.0-×-15-, 3.0-×-15-, 4.5-×-15-, and 5.0-×-20-mm balloons over the segmental branch (AL10) of the left lung in the first session (Figures 1, 2, and 3), and over the segmental branch (RA10) of the right lung in the second session. Figure 4 shows the anatomical classification of the pulmonary vascular tree and the names of the segments used in the latest ESC (Spanish Society of Cardiology) expert consensus on pulmonary circulation and right ventricular function. There were no intraprocedural or postprocedural complications. The patient evolved favorably with marked clinical improvement. A new BPA session was scheduled 30 days later. During the initial RHC, the mPAP was 31 mmHg, the minute volume (MV) of 6.77 L/min, and the pulmonary vascular resistance (PVR) was 1.79 Wood units (see Table 1 for RHC measurements in different sessions). Pulmonary angiography showed no residual lesions in the treated branches and normal anterograde flow. Therefore, due to marked clinical improvement, the scheduled session was suspended. To date, six months after undergoing BPA, the patient remains on the same initial medical therapy, under multidisciplinary follow-up, and without resting or exertional dyspnea.

Discussion

Chronic thromboembolic pulmonary hypertension is a rare complication of pulmonary embolism. It is characterized by the formation of intraluminal thrombi that partially or completely obliterate the lumen of large pulmonary arteries, resulting in increased pulmonary vascular resistance (PVR) and progressive pulmonary hypertension. All these changes lead to a microvasculopathy that includes pulmonary microvasculature remodeling and obstruction in both perfused and non-perfused areas of the lung, which contributes to the progressive nature of the disease. These alterations lead to right heart failure and, if untreated, death4.

The European CTEPH registry, conducted between 2007 and 2012, highlighted the significant impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH5. Since then, two more treatments have been added for patients with inoperable CTEPH: balloon pulmonary angioplasty (BPA) and an approved oral pharmacological therapy with soluble guanylate cyclase stimulator riociguat.

Riociguat was approved in 2013 for the treatment of patients with inoperable CTEPH and with residual pulmonary hypertension after PEA. At the same time, BPA, pioneered at Japanese centers, was introduced worldwide. Subsequently, the European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension recommended both medical treatment with riociguat and BPA for patients with inoperable CTEPH and with persistent or recurrent pulmonary hypertension after PEA6. CTEPH centers worldwide have published their individual results with BPA; however, most reports were limited to smaller populations and, in isolation, did not provide robust guidance on global trends in procedural safety. To address this knowledge gap, in 2023, Nishant Jain et al. conducted a systematic review and meta-analysis of all published articles to that date, reporting BPA outcomes worldwide, comparing procedure-related complication rates during the first five years (2013–2017) and the last five years (2018–2022). The final analysis was based on 26 reports from 4 continents and 18 countries, including 1714 patients with CTEPH undergoing a total of 7561 BPA sessions. This analysis yielded a cumulative incidence of hemoptysis and/or vascular injury of 11.1%, a reperfusion edema rate of 6.0%, a mechanical ventilation rate of 0.4% per BPA session, and an overall mortality of 1.3%. The review showed that these complications decreased over time due to refinements in patient and lesion selection, as well as in procedural technical aspects7.

In 2024, the World CTEPH Registry was published; it reported long-term outcomes with PEA, BPA, and medical therapy. Participation in this global international registry included 1009 patients from 34 specialized pulmonary hypertension centers in 20 countries. Results showed 3-year survival rates at 94% for the PEA group, 92% for the BPA group, and 71% for patients without mechanical intervention. Three-year survival in the PEA group improved by 5% compared to data collected between 2007 and 20128. Recently, a post-hoc analysis of the RACE trial comparing the effects of riociguat and BPA on right ventricular afterload and function was published. It showed that both therapies are effective in reducing right ventricular afterload, but BPA has a greater impact on improving right ventricular function9.

The presented case aligns with current evidence on the management of inoperable CTEPH. The decision to opt for BPA was based on the patient’s high surgical morbidity and mortality risk, consistent with studies supporting this strategy’s safety and efficacy in high-risk cases. Various registries have documented significant improvements in hemodynamic parameters after BPA, reflected in reductions in mean pulmonary artery pressure and pulmonary vascular resistance, as observed in this case. The patient’s marked clinical improvement reinforces findings from recent studies, where BPA not only reduces right ventricular afterload but also improves functional capacity and quality of life. The cancellation of a scheduled third session due to resolution of treated lesions highlights the procedure’s effectiveness and its potential to optimize therapeutic response with fewer interventions. This case underscores the importance of appropriate patient selection and multidisciplinary follow-up to maximize the clinical benefits of BPA.

Conclusions

BPA is a safe and effective option for patients with inoperable CTEPH, improving right ventricular function and quality of life. Its impact on the reduction of pulmonary vascular resistance reinforces its role in multimodal management of the disease.

1. N. Galiè, M. Humbert, J.L. Vachiery, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J., (2015), 46 pp. 903-975 http://dx.doi.org/10.1183/13993003.01032-2015 | Medline

2. M. Humbert, G. Kovacs, M.M. Hoeper, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J, 43 (2022), pp. 3618-3731.

3. X. Jais, P. Brenot, H. Bouvaist, et al. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med, 10 (2022), pp. 961-971.

4. Marion Delcroix et al. Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy. Circulation. 2024; 150:00–00. DOI: 10.1161/CIRCULATIONAHA.124.068610.

5. Mayer E, Jenkins D, Lindner J, D’Armini A, Kloek J, Meyns B, Ilkjaer LB, Klepetko W, Delcroix M, Lang I, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011; 141:702–710. Doi: 10.1016/j.jtcvs.2010.11.024.

6. K. Fukuda, H. Date, S. Doi, et al. Guidelines for the treatment of pulmonary hypertension (JCS 2017/JPCPHS 2017). Circ J, 83 (2019), pp. 842-945.

7. Nishant Jain et al. Periprocedural Complications With Balloon Pulmonary Angioplasty: Analysis of Global Studies. JACC: Cardiovascular Interventions, Volume 16, Issue 8, 24 April 2023, Pages 984-985. https://doi.org/10.1016/j.jcin.2023.01.361.

8. Marion Delcroix et al. Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy. Circulation. 2024; 150:00–00. DOI: 10.1161/CIRCULATIONAHA.124.068610.

9. Gerges et al. Effect of Balloon Pulmonary Angioplasty and Riociguat on Right Ventricular Afterload and Function in CTEPH: Insights From the RACE Trial. Circulation Cardiovasc Intervention. 2025; 18: e014785. DOI: 10.1161/CIRCINTERVENTIONS.124.014785.

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Chronic thromboembolic pulmonary hypertension: a multimodal approach with balloon pulmonary angioplasty. Case report

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