Ruth, a 25-year-old, couldn't understand the level of fatigue she was experiencing. Her pregnancy and childbirth were uneventful,
and her past medical history was negative. The baby was a year old—she should start feeling herself by now. But she had to
stop and rest when doing something as simple as making the bed, and she didn't feel like eating much, napping when the baby
did. Even when extra help was available to let Ruth have time to herself, she inevitably did nothing, never feeling refreshed.
After a series of exasperating appointments—where she was instructed to get more rest, lose weight, exercise, and consider
anti-depressants—she was transferred to a major teaching hospital for more extensive testing. Eventually, Ruth was diagnosed
with pulmonary artery hypertension (PAH), a rare and complicated disorder most commonly affecting women between 20 and 40
years of age.
With an average delay of 3 years between symptoms and diagnosis of PAH, it is no wonder that such cases are easily misdiagnosed.
Add to this the fact that pulmonary artery hypertension only affects one to two individuals per million people and it is easy
to see the diagnostic challenge. Given that the use of diet pills increases incidence to 25 to 50 cases per million, it is
essential to question patients about weight management behaviors. Unfortunately, regardless of the potential causes of PAH—and
despite advances in drug therapy—the prognosis for this condition remains poor. Mortality may be as high as 50 percent within
just a few years from symptom development. New research, however, supports increasing survival rates, with some patients living
15 to 20 years, or more, after diagnosis.
If one of the patients in your critical care unit presented with these symptoms, would you know how to handle such a situation?
Intensive care units (ICUs) may be the first place you encounter individuals like Ruth, either with the initial dyspnea before
diagnosis or because of accidental disconnection from a drug called epoprostenol (Flolan), one lifeline for PAH. By becoming
familiar with the disorder, assessment techniques, and treatment modalities, you can actively assist in managing this incurable
condition.
PAH pathophysiology Classified as idiopathic or primary when the cause is un-known, PAH may also be categorized as familial or genetic. The latter two classifications comprise 6 to 12 percent of cases, often the result of an altered pulmonary arterial wall.
Teri Miller, MSN, RN, ANP
The disorder is termed secondary pulmonary hypertension if the cause is known. More common, this variation is often the result
of emphysema or bronchitis. Less frequent catalysts include collagen vascular disease, such as scleroderma or systemic lupus
erythematosus; human immunodeficiency virus; shunting, as in atrial septal defects; and portal hypertension. PAH is also linked
to use of drugs or toxins like weight reduction medications (fenfluramine and dexfenfluramine or Phen-Phen), appetite suppressants,
cocaine, or amphetamines. Other potential catalysts include hypoxemia associated with chronic obstructive pulmonary disease
(COPD), interstitial lung disease, sleep apnea, obstruction to pulmonary circulation by thromboemboli or tumor, and sickle-cell
disease.
Regardless of underlying conditions, endothelin-1, an amino acid peptide and powerful vasoconstrictor, may be the culprit
that leads to pulmonary artery hypertension. It mediates vasoconstriction, inflammation, hypertrophy, and fibrosis. Too much
endothelin leads to narrowing of the pulmonary arteries, causing high blood pressure within the pulmonary system.
The mechanisms in place include decreased prostacyclin, a vasodilator; decreased nitric oxide, a powerful vasodilator produced
by the lungs; and increased secretion of thromboxane, a vasoconstrictor. As the heart tries to overcome the abnormal pressure,
the right ventricle enlarges. Ultimately, the pulmonary artery becomes so constricted that right-sided heart failure develops.
If this condition occurs as a result of lung dysfunction, it is called cor pulmonale. When the right ventricle can't pump enough blood into the lungs, there isn't enough oxygen to meet the body's demands. As
the disease progresses, cardiac output (CO) drops and the patient dies of heart failure.
Pulmonary BP Primer
Assessment techniques To assist other members of the healthcare team in making an accurate diagnosis, you need to realize that signs and symptoms
of PAH will mimic heart failure. Exertional dyspnea is the most frequent presenting symptom. Even a simple task like taking
a shower can cause tremendous fatigue. The patient may experience dizziness or lightheadedness and have chest pain. Typically,
the reported fatigue is vastly out of proportion to what is usual for the activity.
Teri Miller, MSN, RN, ANP, the clinical editor for Healthcare Traveler, is a nurse practitioner for the Cleveland Cancer Institute. She received her masters degree from Case Western Reserve University, Cleveland, Ohio.
Articles by Teri Miller, MSN, RN, ANP
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