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Pulmonary arterial hypertension (PAH) is an often misdiagnosed lung disorder occurring as a primary idiopathic disease or as a complication of a large number of respiratory and cardiac diseases. PAH can occur with or without an identifiable cause.
When a person has PAH, the blood pressure in the pulmonary artery rises far above normal levels. The pulmonary artery is the blood vessel carrying oxygen-poor blood from the right ventricle, one of the pumping chambers of the heart, to the lungs. In the lungs, the blood picks up oxygen and then flows to the left side of the heart, where it is pumped by the left ventricle to the rest of the body through the aorta.
Hypertension is the medical term for an abnormally high blood pressure. Normal mean pulmonary-artery pressure is approximately 14 mmHg at rest. In the PH patient, the mean blood pressure in the pulmonary artery is greater than 25 mmHg at rest and 30 mmHg during exercise. This abnormally high pressure (pulmonary arterial hypertension) is associated with changes in the small blood vessels in the lungs, resulting in an increased resistance to blood flowing through the vessels.
This increased resistance, in turn, places a strain on the right ventricle, which now has to work harder than usual against the resistance to move adequate amounts of blood through the lungs.
Over time, the lungs become so damaged and the heart so weakened, the heart will fail. That is why. PAH specialists are either pulmonologist or cardiologists. PAH is a lung disease that ultimately affects the heart.
People with PAH often go to their doctors complaining of shortness-of-breath and unusual fatigue. If they are overweight or getting a bit older, their doctors may tell them to lose some weight and get more exercise. For many, PAH may be present for years before it is diagnosed.
The preceding paragraphs were adapted from the following sources:
-National Heart, Lung, and Blood Institute (NHLBI)
-Guam Memorial Hospital Mortality and Morbidity Conference, June 24, 1998 Pulmonary Hypertension, by Rosie Villagomez, M.D.
NOTE: There has been a great deal of discussion among medical professionals and organizations related to Pulmonary Arterial Hypertension about nomenclature and classification of Pulmonary Hypertension. In the past, the term "Primary Pulmonary Hypertension (PPH)" was used to indicate pulmonary arterial hypertension without an identifiable cause. The term "Secondary Pulmonary Hypertension (SPH)" or occasionally just "Pulmonary Hypertension (PH)" was used to indicate pulmonary hypertension with an identifiable underlying cause. You will find a variety of these terms in use in the various sites listed below. No matter what it is called, the disease process is the same. For the most recent discussion of terminology, see the World Health Organization report, as listed below.
Lynn Z. Schaeffer, PHCentral Staff
Links to other descriptions of Pulmonary Hypertension:
World Health Organization (WHO)
EXECUTIVE SUMMARY from the
World Symposium on Primary Pulmonary Hypertension 1998 Evian, France, September 6-10, 1998
co-sponsored by The World Health Organization
edited by Stuart Rich, MD
TABLE OF CONTENTS
Introduction
The Pathology of Pulmonary Hypertension
Pathobiology of Pulmonary Hypertension
Risk Factors and Associated Conditions for Pulmonary Hypertension
Genetics of Pulmonary Hypertension
Diagnosis and Assessment of Pulmonary Hypertension
Medical Therapy of Pulmonary Hypertension
Atrial Septostomy for Pulmonary Hypertension
Transplantation for Pulmonary Hypertension
Nomenclature and Classification of Pulmonary Hypertension
American Lung Association
American Lung Association® Fact Sheet
Primary Pulmonary Hypertension (PPH)
August 1998
Includes definitions of PPH, SPH, Incidence of PPH, Symptoms, Detection, some treatment.
National Heart, Lung, and Blood Institute (NHLBI)
Can be downloaded as ASCII text, PDF format, or printed copies can be ordered.
Primary Pulmonary Hypertension Factsheet,
National Heart, Lung and Blood Institute
Twenty-three page definitive document includes:
Introduction
Incidence
Course Of The Disease
Symptoms
Diagnostic tools
Functional Classification
Treatment
The Primary Pulmonary Hypertension Patient
Registry, 1981-1988
Living With Primary Pulmonary Hypertension
Glossary
Pulmonary Hypertension Association
What is Pulmonary Hypertension?
Includes definitions of PPH and PH, briefly describes other medical conditions that may cause PH, survival rates.
The Pulmonary Hypertension Society of Ontario
What Is Pulmonary Hypertension?
Includes definitions of PPH and PH, briefly describes other medical conditions that may cause PH.
Guam Memorial Hospital
Guam Memorial Hospital Mortality and Morbidity Conference, June 24, 1998
Pulmonary Hypertension, by Rosie Villagomez, M.D.
What is pulmonary hypertension (PH)?
Includes definitions of PPH and PH, describes other medical conditions that may cause PH, Common symptoms of PH, diagnosis, treatments, prognosis, and a list of references.
PPH Cure Foundation
Learning About PPH
Includes brief description of the structure of lungs, and how PH affects cells and capillaries of the lungs.
Mayo Clinic, Rochester, Minnesota
An Introduction to Pulmonary Hypertension
What Is Pulmonary Hypertension?
Includes definitions of PPH and secondary PH, causes of secondary PH, incidence of PH, brief discussion of diagnostic methods, symptoms, prognosis, treatment options.
Published in the July 2004 issue of CHEST, the peer-reviewed journal of The American College of Chest Physicians: Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines
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