Volume 12: Issue 4

Our understanding and management of pulmonary arterial hypertension (PAH) has advanced tremendously over the last 30 years. Numerous scientific discoveries have helped to elucidate underlying mechanisms. Registries dating from the National Institutes of Health (NIH)-sponsored Patient Registry for Primary Pulmonary Hypertension (PPH Registry) of the 1980s to the recent REVEAL and French National registries have provided valuable information on PAH's epidemiology, natural history, risk factors, and prognostic indicators. Clinical classifications and diagnostic algorithms have been developed and periodically updated through international collaboration. Most importantly, numerous pulmonary vasomodulating drugs have been developed, and their widespread use has been associated with longer

Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as precapillary pulmonary hypertension (PH) as assessed by right heart catheterization, and results from incomplete resolution of the vascular obstruction associated with acute pulmonary embolism (PE). Pulmonary thromboendarterectomy (PTE) is the therapy of choice for CTEPH patients with surgically accessible thrombi.

Although associated with potential risks, PTE has been found to improve, and in many cases normalize pulmonary hemodynamics, functional status, and long-term survival. It is critical to undergo careful diagnosis and preoperative selection of patients who will most likely benefit from surgery. We have used published literature along with our personal experiences to review diagnosis of CTEPH and evaluation in advance of the PTE procedure.

In patients with PH or suspected PH, a complete diagnostic workup should be performed to identify the underlying etiology of the disease. Pulmonary angiography and right heart catheterization are the preferred assessment tools to diagnose CTEPH. PTE remains the treatment of choice, and for further evaluation of operability and preoperative risk patients should be referred to a CTEPH expert center.

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

Chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary hypertension (PH) secondary to chronic emboli, obstructing the pulmonary arteries. This results in increased pulmonary vascular resistance and right ventricular failure. CTEPH is the only form of PH that is potentially curable, through a surgical procedure that removes the chronic emboli: pulmonary thromboendarterectomy (PTE). The first step in managing patients diagnosed with CTEPH is to determine if they are operable. The use of medical therapy should never delay referral for surgery, which should be done at a specialized center with expertise in CTEPH. Since a significant proportion of CTEPH patients are not surgical candidates, or are among the 10% to 15% of patients that have persistent or recurrent PH after surgery, there is a need for effective medical therapy.

The use of several pulmonary arterial hypertension (PAH)-targeted agents have been studied, mostly in small uncontrolled trials. A recent Phase 3 clinical trial found riociguat, a stimulator of soluble guanylate cyclase (sGC), to be effective for inoperable CTEPH.

What Should FDA Know about PAH?

Encourage your patients to join a rare conversation with FDA drug reviewers about living with PAH and the impact of current treatments on daily life.

Tuesday May 13, 2014
1:00–5:00 p.m. EST
10903 New Hampshire Avenue Silver Spring, MD 20993 (FDA Campus)

In-person attendance is encouraged, but webcast participation is available. PHA will provide buses from our New York and Maryland offices. All members of the PHA community are welcome to attend, but FDA will focus on speaking with persons currently living with PAH. Learn more and register at www.PHAssociation.org/FDARegistration

Memos

Editor's Memo

Pulmonary Hypertension Care Centers

Diagnosis and Preoperative Evaluation of Chronic Thromboembolic Pulmonary Hypertension

Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

Medical Therapy for Chronic Thromboembolic Pulmonary Hypertension

Pulmonary Hypertension Roundtable: CTEPH Experiences and Expertise

PHPN: Post-Pulmonary Endarterectomy: What to Expect During the First Year and How to Handle the Unexpected

Ask the Expert: Do Patients With Pulmonary Arterial Hypertension Benefit From Referral to a Specialized Center?

News To Use

What Should FDA Know about PAH?

Help Your

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