Guest Editors’ Memo

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is unique among the many etiologies of pulmonary hypertension (PH), in that it can be treated very effectively, even cured, with the surgical extraction of occlusive thrombo-fibrotic lesions via pulmonary thromboendarterectomy (PTE) surgery. More recently, balloon pulmonary angioplasty (BPA) has emerged as a viable option for patients who cannot or should not be operated upon, and for those with residual disease after PTE. Arguably, BPA is one of the major advancements in pulmonary vascular disease over the past 2 decades, although surgical therapy with PTE remains the treatment of choice whenever feasible. Effective PH-targeted medical therapy is also available, and increasingly multimodality approaches including a combination of these treatment options are being utilized in expert centers to achieve the best outcomes. And yet, CTEPH remains a vastly under-recognized disease, with considerable delays and confusion in its diagnosis. As a result, many patients are still not being effectively treated. This issue of Advances in Pulmonary Hypertension represents an effort to update our readers on the key current diagnostic and therapeutic approaches for CTEPH.In “Post-Pulmonary Embolism Follow-Up and Epidemiology of Chronic Thromboembolic Pulmonary Hypertension”, Jasuja and colleagues provide a comprehensive framework of the many issues that need to be addressed in follow-up after an acute pulmonary embolism (PE). In addition, the authors review the available evidence and challenges on the epidemiology and risk factors for CTEPH, as well as the evolving understanding of the post-PE syndrome.In the article “Diagnostic Evaluation of CTEPH”, Vaidya and Forfia describe the multiple steps and testing needed to arrive at the correct diagnosis of CTEPH, including several chest imaging studies that can accurately identify and quantify chronic thrombo-fibrotic occlusive disease of pulmonary arteries, as well as detailed imaging and hemodynamic studies that measure the impact of occlusive disease on right ventricular afterload.The article “Pulmonary Thromboendarterectomy: Patient Selection, Techniques, Outcomes, and Recent Advances” by Madani and Higgins provides a discussion on the current role of surgical therapy for CTEPH, which remains the treatment of choice for a majority of patients.In “Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension” Serfas and Krasuski share an expert insight into patient selection, procedural technique and complications of this relatively novel interventional treatment modality for CTEPH, which is gaining an increasingly important role in management of CTEPH in expert centers.In “Medical Management of Chronic Thromboembolic Pulmonary Hypertension” Goyanes and Heresi review the medical management of CTEPH, including lifelong anticoagulation to prevent recurrent PE and PH-targeted therapy (pulmonary vasodilators) directed at the concomitant microscopic vasculopathy. The authors detail the available literature and current knowledge gaps regarding choice of anticoagulant, patient selection for pulmonary vasodilator therapy, and the place of PH therapy in the context of PTE and BPA.Finally, in the round table discussion, Co-editors Drs. Heresi and Krasuski gather Drs. Auger, Tapson and Lang for a lively conversation on the practical aspects and current challenges in the diagnosis of CTEPH, including recognition of CTEPH after acute PE, and the use of detailed and precise imaging and hemodynamic techniques to phenotype CTEPH.We are confident our readers will find this issue informative and we sincerely thank all the contributors for their efforts and time devoted to this issue.