Volume 6: Issue 3

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,

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

Despite recent advances in cardiac surgery that have allowed repair of congenital heart defects at a very young age, pulmonary arterial hypertension (PAH) secondary to congenital heart disease (CHD) remains a major problem. In its most severe form, the Eisenmenger syndrome, PAH and its complications result in a significant increase in morbidity and mortality and also greatly affect the quality of life of patients.

Pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH), as discussed throughout this issue of Advances in Pulmonary Hypertension, is one of the most commonly occurring causes of significant or severe morbidity and untimely mortality in CHD patients.1 Without surgical therapy, it is suggested that some 30% of CHD patients will develop PAH.2 Early recognition and treatment of CHD is often discussed, therefore, as the most effective preventive treatment measure for patients at risk for pulmonary vascular disease. Epidemiologic estimates of occur-rence of CHD-PAH have targeted approximately 15% of all CHD survivors as having PAH.3

Another Milestone for PHA Mission to Promote Awareness

Focusing on Pulmonary Arterial Hypertension in Adults with Congenital Heart Disease

The Scientific Leadershp Council of the Pulmonary Hypertension Association

Joseph K. Perloff, MD: Father of Adult Congenital Heart Disease

Pulmonary Arterial Hypertension In Adults With Congenital Heart Disease: General Overview of Disease Mechanisms

Evaluating Operability in Adults with Congenital Heart Disease and the Role of Pretreatment with Targeted Pulmonary Arterial Hypertension Therapy

Emerging Therapy of Congenital Heart Disease Associated with Pulmonary Hypertension

Pulmonary Arterial Hypertension in Adults with Congenital Heart Disease

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