Volume 11: Issue 4

Summaries and commentaries from the section editors and invited reviewers present a clinical context for practitioners' application of the latest published research relevant to the care of patients with pulmonary hypertension. In this issue, Kelly Chin discusses the role of computed tomography and 6-minute walk distance in the diagnosis of pulmonary hypertension patients.

Advances in disease awareness, earlier diagnosis, and additional therapeutic options for treatment of pediatric pulmonary hypertension (PH) in the last 2 decades have dramatically improved survival, leading to the first generation of pediatric patients surviving to adulthood.1–4 Pediatric care is generally a family-centered approach, whereas adult care is more patient-centered. When patients become young adults they must move from dependency on parental involvement and oversight to independence and individual accountability. It is difficult for young adult patients to navigate this transition successfully without coordinated support from their family, pediatric, and adult care providers.

Recent analyses of pediatric PH

Adult congenital heart disease represents a growing population of patients. Many patients survive to adulthood and lead functional, productive lives. In fact, there are more adults living with congenital heart disease than pediatric patients. Many adult patients will have had prior surgical repair as children. However, some patients present in adulthood with a new diagnosis of congenital heart disease. Furthermore, there are a variety of complications associated with each individual congenital lesion and specific surgical repair procedure.

Pulmonary arterial hypertension (PAH) is a hemodynamic and pathophysiologic condition defined as an increase in mean pulmonary artery pressure (MPAP) of ≥25 mm Hg at rest measured at right heart catheterization (RHC).1 2 Patients with PAH associated with congenital heart disease (PAH-CHD) are a growing population consisting of an anatomically and phenotypically heterogeneous group, where differences among specific cardiac defects, along with their varied clinical course and prognosis, influence treatment choices for the individual patient.

1.
Aggressive monitoring for the development of PAH should occur in Fontan patients because:
1. They often have a mean pulmonary pressure >25 mm Hg at right heart catherization
2. An Eisenmenger physiology is associated in most cases
3. A conduit obstruction may lead to cardiac decompensation
4. Even slight increase in PVR may have significant hemodynamic consequences
2.
Which of the following forms of congenital heart disease is most likely to lead to the development of PAH?
1. Partial anomalous pulmonary venous return without repair
2. Secundum atrial septal defect without repair
3. An unrestricted ventricular septal defect without prior repair
4. Patent foramen ovale without repair

Guest Editor's Memo

Pulmonary Hypertension Associated with Congenital Heart Disease: It's Not All the Same

Review of the Latest Published Research

Transitioning the Pediatric Pulmonary Hypertension Patient

Advances in Pulmonary Hypertension

Anatomy of Congenital Heart Disease Lesions Associated With Pulmonary Arterial Hypertension

The Essential Role of Imaging in the Evaluation of Patients With Pulmonary Arterial Hypertension in Association With Congenital Heart Disease

Targeted Pulmonary Arterial Hypertension Therapies and a Combined Medical-Surgical Approach for Congenital Heart Disease Patients

The Role of Catheter-Based and Surgical Treatments in Patients With Congenital Heart Disease and Pulmonary Hypertension

Self-Assessment Examination

Management of PAH in Adults with Congenital Heart Disease: Impact and Dilemmas

Congenital Heart Disease With Associated Pulmonary Arterial Hypertension. Who and When to Operate: A Therapeutic Dilemma

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