Volume 14: Issue 3

Charles Burger,

Professor of Medicine, Mayo Clinic College of Medicine, Medical Director, PH Clinic, Jacksonville, FL

Editor's Memo

One of the many challenges in managing pulmonary hypertension patients encompasses the broad array of associated conditions. While advancement has occurred with clinical description and course as well as pathobiological relationships, there is much to be learned. Often, the best source of information is a detailed review of the current state of knowledge. For that very reason, I am extraordinarily proud of the current issue prepared under the insightful direction of Drs. Kelly Chin and Sonja Bartolome, guest editors. They have

In this issue of Advances, section editors Jonathan Rich, MD, and Oskana Shlobin, MD, review findings from 2 recently published studies and discuss their implications on treatment of patients with pulmonary arterial hypertension.

Sarcoidosis-associated pulmonary hypertension (SAPH) has been reported in 10% of all sarcoidosis patients. In the United States, the prevalence is similar to that reported for scleroderma. There are several possible mechanisms for SAPH, including vascular compression, granulomatous angiitis, left ventricular (LV) dysfunction, and fibrosis of lung tissue. The prognosis of those with LV dysfunction is significantly better than those with precapillary pulmonary hypertension. Screening for SAPH includes evaluation of dyspnea by 6-minute walk test, echocardiography, and measurement of pulmonary artery diameter on computer tomographic scan. Right heart catheterization remains the definitive test for characterizing SAPH. Treatment for precapillary hypertension has been shown to improve pulmonary hemodynamics. However, it is still unclear whether such treatments will change the natural course of the disease, especially in those with severe fibrosis.

Pulmonary hypertension (PH) associated with hereditary hemorrhagic telangiectasia (HHT) occurs most commonly as a consequence of high-output heart failure related to excessive shunting through visceral vascular malformations. A smaller number of HHT patients develop a form of heritable pulmonary arterial hypertension, characterized by an elevated pulmonary vascular resistance with normal left heart filling pressures. In this review, we will discuss the clinical manifestations of HHT in general, the pathophysiology behind the different forms of PH associated with HHT, and the clinical care of the HHT patient with PH.

Memos

Editor's Memo

Research Reviews

Sarcoidosis-Associated Pulmonary Hypertension: Diagnosis and Treatment

Distinct Forms of Pulmonary Hypertension Complicate Hereditary Hemorrhagic Telangiectasia

Pulmonary Hypertension in Chronic Myeloproliferative Disorders

Pulmonary Veno-occlusive Disease

Roundtable: Other Rare Disorders Associated with Pulmonary Hypertension

PH Grand Rounds: Confronting the Challenge of Sarcoidosis-Associated Pulmonary Hypertension

PH Professional Network: Filtering the Facts: Management of Portopulmonary Hypertension

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