In this article, we discuss the topic of chronic thromboembolic pulmonary disease (CTEPD) and the growing role of balloon pulmonary angioplasty (BPA) in its treatment. We present the pathophysiology of CTEPD which arises from an incomplete resolution of thrombi in the pulmonary arteries and leads to stenosis and occlusion of the vessels. The article focuses mainly on the chronic thromboembolic pulmonary hypertension (CTEPH) subpopulation for which prognosis is very poor when left untreated. We describe a multimodal approach to treating CTEPH, including pulmonary endarterectomy (PEA), BPA, and pharmacological therapies. Additionally, the benefits of pharmacological pre-treatment before BPA and the technical aspects of the procedure itself are outlined. It is emphasized that BPA does not replace PEA but serves as a complementary treatment option for eligible patients. We summarized efficacy and treatment goals including an improvement in functional and biochemical parameters before and after BPA. Patients who received pre-treatment with riociguat prior to BPA exhibited a notable reduction in the occurrence of less severe complications. However, elderly patients are still perceived as an especially vulnerable group. It is shown that the prognosis of patients undergoing BPA is similar to PEA in the first years after the procedure but the long-term prognosis of BPA still remains unclear. The 2022 ESC/ERS guidelines highlight the significant role of BPA in the multimodal treatment of CTEPH, emphasizing its effectiveness and recommending its consideration as a therapeutic option for patients with CTEPD, both with and without pulmonary hypertension. This review summarizes the available evidence for BPA, patient selection, procedural details, and prognosis and discusses the potential future role of BPA in the management of CTEPH.

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.

Pulmonary embolism (PE) is a life-threatening condition and a leading cause of morbidity and mortality. There have been many advances in the field of PE in the last few years, requiring a careful assessment of their impact on patient care. However, variations in recommendations by different clinical guidelines, as well as lack of robust clinical trials, make clinical decisions challenging. The Pulmonary Embolism Response Team Consortium is an international association created to advance the diagnosis, treatment, and outcomes of patients with PE. In this consensus practice document, we provide a comprehensive review of the diagnosis, treatment, and follow-up of acute PE, including both clinical data and consensus opinion to provide guidance for clinicians caring for these patients.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a subgroup of pulmonary hypertension that differs from all other forms of PH in terms of its pathophysiology, patient characteristics and treatment. For implementation of the European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension in Germany, the Cologne Consensus Conference 2016 was held and last updated in spring of 2018. One of the working groups was dedicated to CTEPH, practical and controversial issues were commented and updated. In every patient with suspected PH, CTEPH or chronic thromboembolic disease (CTED, i.e. symptomatic residual vasculopathy without pulmonary hypertension) should be excluded. Primary treatment is surgical pulmonary endarterectomy (PEA) in a multidisciplinary CTEPH centre. Inoperable patients or patients with persistent or recurrent CTEPH after PEA are candidates for targeted drug therapy. There is increasing experience with balloon pulmonary angioplasty (BPA) for inoperable patients; this option, like PEA, is reserved for specialised centres with expertise in this treatment method.

Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery. These guidelines describe the current recommendations for the diagnosis and treatment of the different pulmonary hypertension groups.

Acute pulmonary embolism (PE) still represents a challenge regarding a rapid diagnosis and a risk-adapted therapy. In the 2014 guidelines of the European Society of Cardiology (ESC) on the diagnosis and management of acute PE, several new recommendations have been issued based on new study data. Some established scores for risk stratification were developed further and there is now good evidence for the use of age-adjusted D-dimer cut-off levels. For the risk stratification in patients without clinical features of shock, the utilization of the pulmonary embolism severity index (PESI) and simplified PESI (sPESI) scores is recommended. In patients with intermediate risk, right ventricular morphology and function can be evaluated by computer tomography or echocardiography and biomarkers facilitate further risk stratification. For the treatment of patients with venous thromboembolism with or without PE, the non-vitamin K-dependent oral anticoagulants (NOACs) are a safe alternative to the standard anticoagulation regimen with heparin and vitamin K antagonists. Systemic thrombolytic therapy should be restricted to patients with high risk or intermediate high risk with hemodynamic instability. Finally, new recommendations for the diagnosis and therapy of patients with chronic thromboembolic pulmonary hypertension (CTEPH), with cancer or during pregnancy are given.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as group IV in the WHO classification for pulmonary hypertension. The disease requires a very low index of suspicion for identification and needs a special diagnostic approach utilizing clinical, radiological, and hemodynamic tools. As CTEPH is potentially curable, all efforts should be consumed to reach the accurate diagnosis and subsequently evaluated for operability. Although pulmonary endarterectomy (PEA) is the only curative tool so far, recent updates concerning medical and interventional therapy have made significant advances in inoperable patients. In this review, we provide a detailed discussion on diagnostic algorithm, surgical operability criteria, PEA, and the medical therapy.