Volume 19: Issue 1

Pulmonary hypertension, despite specific therapies, remains an incurable disease with a dreadful prognosis. A systemic-to-pulmonary shunt, if left unrepaired, can cause pulmonary arterial hypertension. With time, pulmonary vascular disease develops, and closure of the shunt becomes contraindicated. Operability criteria are not well defined and rely mainly on hemodynamic values that fail to predict long-term survival. Shunts can also be created in selected cases of advanced pulmonary hyper-tension, in view of off-loading the right ventricle and improving cardiac output at the cost of cyanosis. Shunt creation is not without risks and remains indicated only in selected severe cases.

Patients with chronic thromboembolic pulmonary hypertension (CTEPH), characterized by pulmonary artery obstruction with unresolved embolism, have poor prognosis. Although pulmonary endarterectomy is the treatment of choice in the management of CTEPH, some patients are not operable. Balloon pulmonary angioplasty (BPA) is a catheter-based interventional treatment for patients with CTEPH. BPA has been considered a high-risk procedure, although the beneficial effects were promising. BPA has been modified and refined over the last 10 years. Recent evidence about modern BPA has consistently demonstrated the beneficial clinical effects with acceptable risks. BPA is now recommended in nonoperable CTEPH patients in addition to targeted medical therapy, although several questions such as long-term prognosis remain unanswered. BPA is still evolving for its application in the CTEPH treatment strategy. Further investigations are still necessary to define the role of BPA.

This spring, Guest Editor Richard A. Krasuski, MD, Professor of Medicine at Duke University School of Medicine in Durham, North Carolina, convened a group of experts to discuss the present and future of invasive technologies in the diagnosis and treatment of pulmonary hypertension. The guests included Jamil A. Aboulhosn, MD, Professor of Medicine at the University of California Los Angeles David Geffen School of Medicine in Los Angeles, California; Raymond Benza, MD, Director of the Division of Cardiovascular Medicine and Professor of Medicine at The Ohio State University College of Medicine in Columbus, Ohio; and J. Eduardo Rame, MD, FACC, Louis R. Dinon MD Professor of Cardiovascular Medicine and Physiology and Chief for Advanced Cardiac and Pulmonary Vascular Disease at Thomas Jefferson University Hospital in Philadelphia, Pennsylvania.

The development of right ventricular (RV) failure in patients with pulmonary arterial hypertension (PAH) is associated with a dismal prognosis. While phosphodiesterase-5 inhibitors, prostacyclin analogs, endothelin receptor antagonists, and other medications have transformed the prognosis of PAH, these therapies have limited effectiveness. A subset of patients develops right heart failure in the face of severely elevated pulmonary vascular resistance (PVR) despite optimal medical management. The appropriate role for mechanical support of the failing RV in the context of PAH remains undefined.

There is rapidly growing experience with left ventricular assist devices (LVADs) to provide mechanical circulatory support, both percutaneous and

PRESENTATION

A 63 year-old morbidly obese female with chronic hypercarbic and hypoxic respiratory failure was referred to our program for severe pulmonary hypertension (PH) identified on right heart catheterization (RHC).

On presentation, she complained of increasing shortness of breath on exertion, in setting of PH directed therapy with macitentan and sildenafil prescribed 3 years before. She had initially improved on this regimen and supplemental oxygen. The patient remained compliant with a continuous positive airway pressure device and supplemental oxygen for obstructive sleep apnea. In clinic, vital signs were unremarkable; she had normal O2 saturation on 3 L/min supplemental oxygen. Physical

Full Issue PDF

Memos

Editor's Memo

Modern Right Heart Catheterization: Beyond Simple Hemodynamics

INTRODUCTION

INDICATIONS FOR RHC

Shunts: When to Close Them and When to Create Them for Palliation

The Evolving Role of Balloon Pulmonary Angioplasty in the Management of Chronic Thromboembolic Pulmonary Hypertension

Pulmonary Hypertension Roundtable: New Invasive Technologies in Pulmonary Hypertension

Ask the Expert: Is There a Role for Percutaneous or Surgically Implanted Right Ventricular Assist Devices in Pulmonary Arterial Hypertension?

PH Grand Rounds: Giant Pulmonary Artery Aneurysm and Severe Multifactorial Pulmonary Hypertension: A Case Report

PRESENTATION

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