Monographic Issue on Pulmonary Hypertension

Joan Albert Barberà, MD, PhD

Marc Humbert, MD, PhD

Pulmonary hypertension (PH) is a pathophysiological disorder characterized by an abnormally increased pulmonary arterial pressure that may result from or be associated with a variety of clinical conditions. PH is a major global health issue, with an estimated prevalence of 1% of the global population, mainly because it is a frequent complication of left heart disease and chronic lung disease. Nevertheless, the most severe forms of PH, requiring highly specialized management, are those that occur in rare diseases of the pulmonary vasculature, such as pulmonary arterial hypertension (PAH) or chronic thromboembolic PH (CTEPH).

In recent years, substantial progress has been made in the detection and management of PAH and CTEPH that have yielded to a significant increase in survival of patients. In some way, these diseases have converted to chronic diseases and patients may face episodes of decompensation or other comorbidities, deeming management not only by PH specialists but also by other specialists. Furthermore, optimal management of the patient with PH requires the collaboration and engagement of several health care professionals participating in multidisciplinary teams. We can certainly say that nowadays PH is no longer a disease managed by select super-specialists, but rather by a wide spectrum of professionals who interact with each other, even between different levels of care, for a patient-centered management.

The European Society of Cardiology (ESC) and the European Respiratory Society (ERS) have issued recently the fourth edition of the Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension that incorporate significant novelties with respect to previous editions, based on the most recent evidence.[1] [2]

In this monographic issue of Seminars in Respiratory and Critical Care Medicine, we aim to provide a state-of-the-art on the current management of patients with PH, covering a wide range of clinical conditions and trying to translate the ESC/ERS guidelines to the clinical arena. The monograph is addressed not only to the PH specialist but also to the nonspecialist physician who may be interested in specific aspects of disease management or the best way to face complex clinical situations. The 13 review articles of the monograph cover the general approach to the patient, management of the different types of PH, and that of specific situations patients with PH might face in their clinical course.

In the first article, Lechartier et al analyze the new hemodynamic definition of PH, proposed at the 6th World Symposium on Pulmonary Hypertension and now endorsed and refined in the ESC/ERS guidelines and review the evidence behind that change. The authors also review the current clinical classification providing examples for an appropriate diagnosis. Torbicki et al analyze thoroughly the diagnostic approach to PH providing strategies aimed at the early diagnosis and referral of PAH and CTEPH, which require specific and complex therapies.

The right ventricle plays a pivotal role in PH. In their review, Wessels et al describe the normal structure and function of right ventricle, changes that occur during adaptation to increased afterload, and consequences of its failure.

Weatherald et al review the current management of PAH, discussing the specific approach to those patients with associated cardiopulmonary comorbidities, a concept introduced in the recent guidelines. Baratto et al address PH associated with left heart disease, which is the most frequent form of PH, and provide a stepwise approach to its diagnosis paying special attention to the differential diagnosis between heart failure with preserved ejection fraction with significant pulmonary vascular disease and idiopathic PAH. In their review, Blanco et al discuss PH associated with chronic lung disease, another prevalent form of PH, addressing the different clinical phenotypes that may present and recent advances in its management.

Patients with PH may face complex clinical situations in their course. Savale et al discuss the complex management of the acutely decompensated patient, which may be a life-threatening situation. In his article, Hoeper provides an overview on the use of extracorporeal life support that might be a life-saving treatment in some patients, particularly when used as a bridge to transplantation. Seitler et al provide an updated review on the assessment and management of acute medical emergencies in patients with PH, and Condliffe et al review the challenging issue of surgery in patients with PH, analyzing the risks related to patient condition, type of surgery, and anesthetic procedure, proposing a perioperative pathway that involves a multidisciplinary team.

CTEPH is currently managed with different treatment modalities that are not mutually exclusive. Delcroix et al review the management of inoperable patients with PH drugs and balloon pulmonary angioplasty. Pepke-Zaba et al review pulmonary endarterectomy, its indications, specific surgical and perioperative strategies, and postoperative monitoring and management.

Lastly, Ali and Sahay address the challenging issue of end-of-life care for the patient with PH, analyzing the existing barriers and providing a guidance for an early intervention to palliate symptoms and reduce psychosocial disease burden.

Overall, this monography provides a comprehensive state-of-the-art of the management of the patient with PH. We hope it will be of interest to the readers, not only those devoted to PH in their usual practice but also those who face patients with PH occasionally.

Publication History

Article published online:
30 October 2023

• References

• 1 Humbert M, Kovacs G, Hoeper MM. et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43 (38) 3618-3731
• 2 Humbert M, Kovacs G, Hoeper MM. et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2023; 61 (01) 2200879