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If your doctor has given you a diagnosis of PH, tests have revealed vascular changes in the lungs that are causing your symptoms.
If you have received a diagnosis of PAH, you are in a subset of PH patients with the primary difference being that the pressure
in your pulmonary artery is also elevated above normal levels.
The pulmonary artery is the blood vessel that carries oxygen-poor blood from the right ventricle of the heart - one of the pumping
chambers - to the lungs. In the lungs, the blood picks up oxygen which is then carried to the left side of the heart, where it is pumped
by the left ventricle to the rest of the body through the aorta.
Normal mean pulmonary-artery pressure is approximately 14 mmHg at rest. In the PAH 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.
The elevation in pulmonary artery pressure and possible narrowing and thickening of this important vessel causes the right side of the
heart to work harder than normal. Over time, the lungs become so damaged and the heart so weakened, the heart will eventually fail. This
is why an early diagnosis is so important in the outcome of PAH.
People with PH 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, PH may be present for years before it is
properly diagnosed.
Once you have been diagnosed, we encourage patients to seek out the care of a PH specialist rather than simply a cardiologist or
pulmonologist who may not be up on the latest treatments and clinical trials. A PH specialist will also be either a cardiologist or
a pulmonologist, but will have more experience in treating this complicated disease.
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.
11/13/08 - Page Reviewed and Updated.
Links to other descriptions of Pulmonary Hypertension:
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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