Volume 21: Issue 4

This issue of Advances in Pulmonary Hypertension highlights several associated conditions causing pulmonary arterial hypertension (PAH) that are of particular relevance globally and deserve to be on our radar: schistosomiasis, Human immunodeficiency virus (HIV), and liver disease. These well-known conditions provide unique challenges to our understanding and treatment of PAH.

Schistosomiasis affects a shocking 200 million or more throughout the world, with 5–10% developing PAH. Yet it can be easy to forget that schistosomiasis is cause of Group I PAH. Brian Graham reviews the background, mechanisms, diagnosis, and treatment schistosomiasis-associated pulmonary arterial hypertension. Graham leaves us with several

Schistosomiasis is a major cause of group 1 pulmonary arterial hypertension (PAH) worldwide. Schistosomiasis results from a parasitic infection present in over 200 million individuals worldwide. Schistosomiasis-associated PAH was initially thought to be obstructive due to egg embolization but has a pulmonary vascular pathology like other forms of group 1 PAH and can be treated using conventional PAH therapies. Mechanisms that underlie the development of schistosomiasis-associated PAH include type 2 inflammation which triggers TGF-β signaling; importantly, TGF-β signaling is a pathway shared with other PAH etiologies. However, many things which are unknown about this disease remain, including if the lung vascular pathology results from egg embolization causing localized inflammation and vessel remodeling, or if this is a form of portopulmonary hypertension resulting from schistosomiasis liver disease.

Human immunodeficiency virus (HIV) attacks the immune system and can cause acquired immunodeficiency syndrome. Thankfully, antiretroviral therapy is highly effective, and people infected with HIV can live long lives with proper adherence to therapy. One of the important noninfectious complications of HIV is the development of pulmonary arterial hypertension (PAH). This review will cover the epidemiology, pathogenesis, and clinical characteristics of HIV-associated PAH. It will also cover the current knowledge on use of vasodilator therapy in this population.

Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.

Full Issue PDF

Guest Editors’ Memo

Schistosomiasis-Associated Pulmonary Arterial Hypertension

Human Immunodeficiency Virus–Associated Pulmonary Arterial Hypertension

Portopulmonary Hypertension: A Review

PH Professional Network: The Role of the Advanced Practice Nurse With Patients Undergoing Pulmonary Thromboendarterectomy and Balloon Pulmonary Angioplasty for CTEPH

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease