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Pulmonary Arterial Hypertension (PAH)

I have just been diagnosed (after extensive tests including angiogram, and echo cardiogram which showed elevated arterial pressure) with PAH.

My symptoms include being out of breath at rest, my heart is having arrhythmia,  I can hardly talk because I run out of breath, and I am having difficulty swallowing.

Looking back on everything, I have been having some problems since 2007 and last August I was hospitalized for what they thought was an MI, however tests did not prove that theory.

I would like to know what the common prognosis is (life expectancy) with this class 3 to 4 PAH with chronic pulmonary heart failure diagnosis. Also if there are any new treatments coming around for this disease. Thank you.
1 Responses
242587 tn?1355427710
MEDICAL PROFESSIONAL
The description you have provided suggests that you may have rather severe, rapidly progressive pulmonary  hypertension, presumably Primary Pulmonary Hypertension (PPH) also referred to as Pulmonary Arterial Hypertension (PAH).  However, neither, 1) the severity of the hypertension (expressed as measured pressure) nor, 2) the rapidity of progression of hypertension necessarily add up to a dire prognosis.  First, because of the wide variation in the NYHA/WHO functional class assessments made by clinicians who care for and conduct clinical research (See report from the Mayo Clinic, that follows and the November, 2009 New England Journal of Medicine article, attached)  And, secondly because any determination of severity must include not only the absolute level of pressure in the untreated individual, but other factual information, including the degree of heart failure (clinically and per echocardiogram) (ECHO) and the response to pharmacotherapy.

The preceding description alone supports my strong recommendation that you arrange to be seen by an expert or a team of experts, skilled in the diagnosis and management of PPH.  There has been a great resurgence of interest in PPH during the past 5-15 years and a veritable explosion in research on the number and pharmacologic types of drugs for the treatment of this condition.  Given the rapid progression in our understanding of the pathophysiology of this disease and the number of drugs being investigated on the basis of this understanding (in humans and animals), you would be best served by those on the “cutting edge” of this field.  Examples of such clinics and physicians include those cited in the abstract below:  at the University of Pennsylvaina, the Mayo Clinic in Rochester MN, & Washington University School of Medicine in St. Louis.  To these I would add Northwestern University School of Medicine & the University of Chicago Department of Medicine, and the University of Colorado Health Sciences in Denver, Colorado (Dr. David Badisch).

Whatever you decide, do not delay.  The longer your PAH remains untreated or sub-optimally treated, the greater will be the risk of permanent/irreversible end-organ damage, most notably to the pulmonary arteries and their branches and to your heart.


A sampling of newly developed drugs mentioned in recent reports includes the following

1) Simvastatin and sildenafil combine to attenuate pulmonary hypertension.

2) Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension.

3) Riociguat, an oral soluble guanylate cyclase stimulator for the treatment of pulmonary hypertension. [Review] [36 refs] (animal experimentation)

The following article is illustrative of new approaches to therapy of PAH.  See the first paragraph below.  Unfortunately, I cannot attach the article but you and your doctors could easily find it at the nearest Medical Library.

Phosphodiesterase type 5 inhibitors for pulmonary arterial hypertension. [Review] [39 refs]

Archer SL. Michelakis ED.

New England Journal of Medicine. 361(19):1864-71, 2009 Nov 5.

[Journal Article. Research Support, N.I.H., Extramural. Research Support, Non-U.S. Gov't. Review]

UI: 19890129

Authors Full Name
Archer, Stephen L. Michelakis, Evangelos D.  

The Clinical Problem
Pulmonary arterial hypertension, a disease of the pulmonary vasculature, is diagnosed
when there is both an increased mean pulmonary-artery pressure (>25 mm Hg
at rest or 30 mm Hg with exercise) and a pulmonary-capillary wedge pressure of less
than 15 mm Hg. The diagnosis also requires that secondary pulmonary hypertension
due to lung disease, hypoxia, thromboembolism, and left ventricular muscle or
valve disease be ruled out.1 Pulmonary arterial hypertension occurs in a rare idiopathic
form (in which 10% of cases are familial) but is more commonly associated
with other conditions, including connective-tissue diseases, congenital heart disease,
portopulmonary disease, and human immunodeficiency virus (HIV) infection1
or the use of anorexigens (see the Table in the Supplementary Appendix,
available with the full text of this article at NEJM.org). The functional classification
system of the New York Heart Association has been adapted by the World Health
Organization (WHO) for use in classifying symptoms in patients with pulmonary
hypertension (Table 1).

Although idiopathic pulmonary arterial hypertension is rare, this syndrome in
association with other conditions is increasingly recognized, particularly with the
common use of echocardiography. National databases in France3 and Scotland4
report incidences of 2.4 cases and 7.1 to 7.6 cases per 1 million persons per year,
respectively, and prevalences of 15 cases and 26 to 52 cases per 1 million, respectively.
The prevalence of pulmonary arterial hypertension is expected to increase
Copyright © 2009 Massachusetts Medical Society. All rights reserved.

Mayo Clin Proc. 2009 July; 84(7): 586–592.
PMCID: PMC2704130
Copyright © 2009 Mayo Foundation for Medical Education and Research

Wide Variation in Clinicians' Assessment of New York Heart Association/World Health Organization Functional Class in Patients With Pulmonary Arterial Hypertension

Darren B. Taichman, MD, PhD, Michael D. McGoon, MD, Michael O. Harhay, MBe, Chris Archer-Chicko, MSN, Jeffrey S. Sager, MD, Meena Murugappan, MD, Murali M. Chakinali, MD, Harold I. Palevsky, MD, and Robert Gallop, PhD

From the Pulmonary, Allergy and Critical Care Division, University of Pennsylvania, Philadelphia (D.B.T., M.O.H., C.A.-C., H.I.P.); Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (M.D.M.); Santa Barbara Cottage Health System, Santa Barbara, CA (J.S.S.); Division of Pulmonary and Critical Care Medicine, Washington University School of Medicine, St. Louis, MO (M.M., M.M.C.); and Applied Statistics Program of the Department of Mathematics, West Chester University, West Chester, PA (R.G.)

Individual reprints of this article are not available. Address correspondence to Darren B. Taichman, MD, PhD, Pulmonary, Allergy and Critical Care Division, University of Pennsylvania, Ste 120 PHI, PPMC, 51 N 39th St, Philadelphia, PA 19104 (Email: darren.***@****).

Discussion:

We found a wide variation in the NYHA/WHO functional class assessments made by clinicians who care for and conduct clinical research in patients with PAH. The findings were consistent in 2 independent groups of clinicians assessed with paper and online presentations of patients with PAH. Agreement on a single functional class was not seen for any patient, and in most cases judgments spanned 2 or 3 classes. Consistent with the discrepant conclusions reached was the reported wide variation in factors considered when assessing functional class. Although some clinicians use only patient symptoms to judge functional class, others use varying combinations of symptoms, demographics, exercise, and hemodynamic variables. Indeed, many report using multiple factors not mentioned in the originally defined NYHA or WHO functional class definitions.1,6 Other potential reasons for the variation in approach might include differences in physician training; inappropriate assumptions regarding the relationships among hemodynamics, exercise capacity and functional class; and a perceived need to “adjust” assignments to meet restrictions in the availability of treatments.
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