Volume 6: Issue 4

The case presentations and discussions in this issue of Advances illustrate both common and uncommon challenges in the diagnosis and management of patients with pulmonary hypertension. Although significant guidelines and evidence-based recommendations exist for patient evaluation and care, practical application of these guidelines and managing uncertainty in areas where evidence may be sparse are key elements in the daily activities of specialized pulmonary hypertension clinics.

The contributors present case-based analyses exploring important concepts and controversies in pulmonary venous hypertension, transplantation, vasoreactivity, chronic thromboembolic disease, and catastrophic antiphospholipid syndrome. Two of the cases not only illustrate important concepts but are examples

The scientific program of the Pulmonary Hypertension Association is guided by the association’s Scientific Leadership Council. The Council includes the following health care professionals:

David B. Badesch, MD, SLC Chair University of Colorado Health Sciences Center, Denver, Colorado
Robyn J. Barst, MD, SLC Immediate Past Chair, Columbia Presbyterian Medical Center Babies Hospital, New York, New York
Vallerie V. McLaughlin, MD, SLC Vice-Chair, University of Michigan Health System, Ann Arbor, Michigan
Raymond L. Benza, MD, University of Alabama, Birmingham, Alabama
Todd Bull, MD, University of Colorado Health Sciences Center, Denver, Colorado
Richard N. Channick, MD,

Chronic thromboembolic disease (CTED) remains an impor- tant exclusion in patients presenting with pulmonary hyper- tension. As illustrated by this case, however, exclusion must be rigorous with or without a suggestive history. Although it is a surgically treated disease, not all patients may be can- didates for or elect surgery, leaving medical management as the only remaining option.

Over the past decade effective medical treatments for idio- pathic pulmonary arterial hypertension (IPAH) have de- creased but not eliminated the need for lung transplantation for this disease. Timing of referral for transplant and listing remain critical judgments in the management of many PAH patients but are still subjects of significant debate. National initiatives that have increased donor organs overall and changed their distribution have changed previous decision- making for many centers. A case is presented that illustrates issues in managing the patient in whom medical therapy has failed.

Clinical dilemmas occur in practice. The experience and perspective on such cases may or may not be reflected in the published literature. Second opinions may be sought in an attempt to help determine a clinical course based on the best available information. The Pulmonary Hypertension Association as part of its mission of patient advocacy and education has created and electronic resource network as part of the Pulmonary Hypertension Clinicians and Researchers (PHCR) Program. Extraordinary access to vast clinical knowledge and experience now exists at the speed of e-mail, providing real-time second opinions as well as multifaceted review of clinically challenging cases. The following excerpted discussion exemplifies the possible results, combining the “old fashioned” experience and dedication of PHCR members with modern technology. The discussion has been edited with sequential replies and comments separated by division lines. A commentary from Keith McCrae, MD, follows. —R. Schilz, DO, Associate Editor

Marking a Milestone for the Journal and Previewing PHA's Upcoming Conference and Scientific Sessions

Pulmonary Hypertension: Challenges and Controversies in Diagnosis and Management

The Scientific Leadership Council of the Pulmonary Hypertension Association

Sir Magdi Yacoub: Surgeon, Knight, and “King of Hearts”

Pulmonary Venous Hypertension: A Diagnostic and Therapeutic Dilemma

Introduction

Commentary

History

The Vasoreactive Patient: Diagnosis, Treatment, and Follow-up

Introduction

Chronic Thromboembolic Disease: Underdiagnosis and Nonsurgical Options

Lung Transplantation and Idiopathic Pulmonary Arterial Hypertension: A Case Study

A Case of Catastrophic Antiphospholipid Syndrome

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