Chest. 2004;126(1_suppl):2S-3S. doi:10.1378/chest.126.1_suppl.2S
Executive Summary*: Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines
Chest. 2004;126(1_suppl):4S-6S. doi:10.1378/chest.126.1_suppl.4S

An expert committee representing multiple disciplines has developed “Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines.” This committee was impaneled at the request of the American College of Chest Physicians (ACCP) Health and Science Policy Committee, with the endorsement of the ACCP Board of Regents, who had selected pulmonary hypertension as a priority for evidence-based guideline development in 2001. Ten years ago, the ACCP formed a consensus panel on primary pulmonary hypertension (PPH), which provided a well-received and concise general review of the pathobiology, diagnosis, and treatment of PPH. The objective of the current project was to create a guideline broader in scope and more evidence based than the earlier consensus statement. The Duke University Center for Clinical Health Policy Research provided the review and summaries of the current evidence on this subject. The resulting evidence-based recommendations are targeted toward an audience of cardiologists, pulmonologists, rheumatologists, and primary care physicians, as well as other health-care providers who treat pulmonary arterial hypertension (PAH).

Introduction*: Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines
Chest. 2004;126(1_suppl):7S-10S. doi:10.1378/chest.126.1_suppl.7S

Interposed between the two sides of the heart and embedded within the matrix of the lung, the pulmonary circulation plays a pivotal role in the cardiopulmonary functions of gas exchange and oxygen transport. Its central position, however, renders the pulmonary circulation vulnerable to injury as a result of developmental or acquired disorders affecting the heart or lungs, as well as conditions that may also affect the systemic vasculature. Accordingly, pulmonary vascular disease is not the exclusive domain of any medical discipline: specialists in respiratory medicine, cardiology, rheumatology, infectious diseases, hematology, and pediatrics, among others, must all provide care for patients with these conditions. The most serious and potentially devastating chronic disorder of the pulmonary circulation is pulmonary hypertension (PH), a hemodynamic abnormality of diverse etiology and pathogenesis that challenges physicians with both its diagnosis and treatment. This document, developed by a multidisciplinary panel of experts, is intended to provide physicians with comprehensive, evidence-based guidelines for the approach to patients with pulmonary arterial hypertension (PAH).

Chest. 2004;126(1_suppl):11S-13S. doi:10.1378/chest.126.1_suppl.11S

The American College of Chest Physicians assembled a multidisciplinary, geographically diverse panel of experts in the treatment of pulmonary hypertension to develop clinically relevant practice guidelines for the diagnosis and treatment of pulmonary hypertension in its many variations. That group of experts produced recommendations covering five topic areas, each related to a distinct set of patient-management decisions. This article describes the approach used to develop the guidelines, including identifying, evaluating, and synthesizing the evidence, assessing the strength of evidence pertinent to individual guidelines, and grading guideline recommendations.

Screening, Early Detection, and Diagnosis of Pulmonary Arterial Hypertension*: ACCP Evidence-Based Clinical Practice Guidelines
Chest. 2004;126(1_suppl):14S-34S. doi:10.1378/chest.126.1_suppl.14S

Pulmonary arterial hypertension (PAH) occurs as an idiopathic process or as a component of a variety of disease processes, including chronic thromboembolic disease, connective tissue diseases, congenital heart disease, and exposure to exogenous factors including appetite suppressants or infectious agents such as HIV. This article reviews evidence for screening in susceptible patient groups and the approach to diagnosing PAH when it is suspected, and provides specific recommendations for applying this evidence to clinical practice.

Medical Therapy For Pulmonary Arterial Hypertension*: ACCP Evidence-Based Clinical Practice Guidelines
Chest. 2004;126(1_suppl):35S-62S. doi:10.1378/chest.126.1_suppl.35S

Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. This chapter will provide evidence-based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.

Surgical Treatments/Interventions for Pulmonary Arterial Hypertension*: ACCP Evidence-Based Clinical Practice Guidelines
Chest. 2004;126(1_suppl):63S-71S. doi:10.1378/chest.126.1_suppl.63S

While considerable advances have been achieved in the medical treatment of pulmonary arterial hypertension (PAH) over the past decade, surgical and interventional approaches continue to have important roles in those patients for whom medical therapy is unavailable or has been unsuccessful. These techniques include pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension, thoracic transplantation, and atrial septostomy. This chapter will provide evidence-based recommendations for the selection and timing of surgical and interventional treatments of PAH for physicians involved in the care of these complex patients.

Pulmonary Artery Hypertension and Sleep-Disordered Breathing*: ACCP Evidence-Based Clinical Practice Guidelines
Chest. 2004;126(1_suppl):72S-77S. doi:10.1378/chest.126.1_suppl.72S

The objective of this article is to review the available data on the relationship between sleep-disordered breathing (SDB) and pulmonary arterial hypertension (PAH), with a focus on the prevalence of SDB in patients with idiopathic PAH (IPAH); the prevalence of PAH in patients with SDB; and the effects of SDB treatment on PAH. The evidence to date suggests that PAH may occur in the setting of SDB, although the prevalence is low. However, pulmonary hypertension (PH) in SDB is most strongly associated with other risk factors, such as left-sided heart disease, parenchymal lung disease, nocturnal desaturation, and obesity. The limited data available also suggest that SDB is uncommon in patients with IPAH. Treatment of SDB with continuous positive airway pressure may lower pulmonary artery pressures when the degree of PH is mild.

Chest. 2004;126(1_suppl):78S-92S. doi:10.1378/chest.126.1_suppl.78S

Although idiopathic pulmonary arterial hypertension is perceived as a progressive disease with a uniformly poor outcome, the natural history of disease is heterogeneous, with some patients dying within months of diagnosis and others living for decades. The course of the disease has also been altered by advances in medical therapies. The outcome of patients with other types of pulmonary arterial hypertension (PAH) has been less well characterized. Assessment of prognosis of such patients is important, as it influences both medical therapy and referral for transplantation. This chapter will provide evidence based recommendations to assess the prognosis of patients with PAH.

Sign In to Access Full Content


Want Access?


Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.


Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543