Volume 18: Issue 1

Atrial septal defects (ASDs) are common congenital heart defects in children and adults. Pulmonary arterial hypertension (PAH) is found in subsets of both pediatric and adult patients with atrial defects under varied clinical contexts. The pulmonary hypertension specialist is often faced with questions surrounding timing and method of defect closure, which may have significant impact on procedural and long-term morbidity and survival. This review highlights important differences in management between children and adults with ASDs associated with PAH, highlighting indications for closure, operability, types of closure, and long-term outcomes.

Despite significant improvements in the surgical and postoperative care for patients with single-ventricle physiology culminating in the Fontan circulation, significant late morbidity and mortality remains. In the setting of passive (ie, non-“pump” driven) pulmonary blood flow, pulmonary vascular resistance (PVR) plays a key role in determining cardiac output, and even slight elevations in PVR may result in significant morbidity. There is now great interest to treat Fontan patients with pulmonary vasodilators in an attempt to “optimize” PVR (and by extension, quality of life) and/or improve an elevated PVR. This review discusses the hemodynamic implications of the Fontan circulation, the evidence for use of pulmonary vasodilator therapy, and possible target physiologic mechanisms.

Guest editor Dunbar Ivy, MD, Chief of Pediatric Cardiology and Director of the Pediatric Pulmonary Hypertension Program at the University of Colorado School of Medicine and Children's Hospital of Colorado led a discussion among Editor-in-Chief Harrison (Hap) Farber, MD, then Professor of Medicine and Director of the Pulmonary Hypertension Center at Boston University/Boston Medical Center; Mary P. Mullen, MD, PhD, Assistant Professor of Pediatrics at Harvard Medical School, associate cardiologist at Boston Children's Hospital and Associate Director of the Pulmonary Hypertension Service as well as a member of the adult congenital heart program; Jeffrey R. Fineman, MD, Professor and Vice Chair of Pediatrics, Director of Pediatric Critical Care Medicine and Pulmonary Hypertension, University of California, San Francisco, Benioff Children's Hospital; and Gareth Morgan, MD, Associate Professor of Pediatrics-Cardiology at the University of Colorado School of Medicine and Director of the Cardiac Catheterization Lab at Children's Hospital of Colorado.

Pulmonary hypertension (PH) is a common complication of congenital heart disease (CHD), often developing as a result of vascular remodeling from long-standing left-to-right shunting and pulmonary overcirculation. The most severe form of CHD-associated PH is Eisenmenger's syndrome (ES), in which pulmonary vascular remodeling has led to irreversible changes, systemic pulmonary pressure, and shunt reversal, resulting in cyanosis. While the ever-increasing armamentarium of pulmonary vasodilators has given clinicians several effective treatment strategies, the disease can still progress to the point where advanced therapies such as lung transplantation, with or without heart transplantation, must be considered.

The optimal timing of transplantation in

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Memos

Editor's Memo

Atrial Septal Defect–Associated Pulmonary Hypertension: Outcomes of Closure With a Fenestrated Device

Challenges in the Patient With Pulmonary Hypertension and Atrial Septal Defect: Understanding When and How to Close the Defect

Pulmonary Vascular Disease in the Single-Ventricle Patient: Is it Really Pulmonary Hypertension and if So, How and When Should We Treat it?

Pressure vs Flow-Induced Pulmonary Hypertension

Pulmonary Hypertension Roundtable: The Crossover From Child to Adult With PH and Congenital Heart Disease

Ask the Expert: Transplantation in Eisenmenger Syndrome: Getting to the Heart of the Matter

PH Grand Rounds: Eisenmenger Syndrome: When Less Is More

PH Professional Network: Comprehensive Evaluation and Ongoing Approach to Children With Down Syndrome Who Have Pulmonary Hypertension or Are at Risk of Developing Pulmonary 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