Volume 14: Issue 1

Presentation: A 40-year-old African American female with past history of congenital heart murmurs of unknown etiology, iron deficiency anemia, and intellectual disability secondary to newborn shaken baby syndrome presented to the emergency department with worsening dyspnea, cough, sore throat, and fatigue. Family history was significant for Wegener's granulomatosis and lung cancer. Social history was negative for tobacco, alcohol, or illicit drug use. She lives at home with her sister as her legal caregiver. Workup revealed tachycardia, III/VI holosystolic murmur, right axis deviation on electrocardiogram (EKG), and respiratory alkalosis on arterial blood gas (ABG). She was found to have bilateral

In this edition of Advances, new section editors Jonathan Rich, MD, and Oksana Shlobin, MD, review the findings from 3 recent investigations than lend support to the argument that vasoreactive PAH is a distinct phenotype and probably a distinct genotype of PAH. Watch for their reports on emerging research with implications for clinicians in future issues of Advances.

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that leads to exercise limitation, right heart failure, and death. There is a need for biomarkers that can aid in early detection, disease surveillance, and treatment monitoring in PAH. Several potential molecules have been investigated; however, only brain natriuretic peptide is currently recommended at diagnosis and for follow-up of PAH patients. This review will focus on potential biomarkers in PAH and will discuss their pathophysiology, prognostic significance, as well as their limitations.

Pulmonary arterial hypertension (PAH) is a progressive, often lethal condition originating in the pulmonary arteriolar tree. It is typically manifested in stereotypical changes in the right ventricle (RV). RV dysfunction is an important mediator of patient symptoms in PAH, and RV failure is the most common cause of mortality in PAH patients. Because of the physiologic importance of the RV, RV imaging is critical in the initial diagnostic evaluation and serial assessment of PAH patients, and can provide indirect insight into the status of the disease at the level of the pulmonary vasculature. This article will focus on whether technological advances in imaging have shifted the debate toward which modality is optimal both for routine clinical practice and for a possible surrogate endpoint in PAH clinical trials.

Pulmonary hypertension (PH) does not discriminate based on social support, health literacy, emotional bandwidth, or socioeconomic status. Evaluating these factors provides an elemental foundation to best foster the patient's adherence and success. The non-physician clinician plays a pivotal role in assessment of the new patient.

Evaluation of the patient begins with first contact, whether in person, by telephone or records review. This offers a glimpse of the patient's journey. Telephone screening provides insight into the patient's current knowledge base. Understanding when and how the patient learned of his or her diagnosis, the location and results of testing done thus far,

Memos

Editor's Memo

Editorial Prognostication

PH Grand Rounds: Rare Coexistence of Major Lung Pathologies

Research Reviews: Vasoreactivity in PAH

Circulating Biomarkers in Pulmonary Arterial Hypertension

Consensus or Controversy: Do Recent Advances Shift the Debate for the Use of Echocardiography Versus Cardiac Magnetic Resonance Imaging of the Right Ventricle in Pulmonary Arterial Hypertension?

Prognostication in Pulmonary Arterial Hypertension and Use of Current Risk Prediction Models

Ask the Expert: What Is the Utility of Evaluating Patients for Exercise-Induced Pulmonary Hypertension?

EXERCISE-INDUCED PULMONARY HYPERTENSION

Pulmonary Hypertension Roundtable: Prognostic Indicators for Pulmonary Hypertension

PH Professional Network: Holistic Assessment of the New Patient With Pulmonary Hypertension: The Role of the Non-Physician Clinician

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