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Primary
Pulmonary Hypertension |
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Overview |
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Definition |
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Pulmonary hypertension can appear as a single disease entity (primary) or in
conjunction with another cardiopulmonary disease (secondary). It is
characterized by increased pulmonary artery pressure and pulmonary vascular
resistance. Three subtypes include thrombotic, plexogenic, and
veno-occlusive. |
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Etiology |
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Many cases of pulmonary hypertension are idiopathic or primary, especially in
young women. However, many diseases of the heart or respiratory system can also
cause pulmonary hypertension. All cause narrowing of the pulmonary blood
vessels, increasing resistance to blood flow through the lungs. To maintain
pulmonary blood flow, pulmonary arterial pressure increases. Possible causes
include the following.
- Congenital heart disease (e.g., atrial and ventricular septal
defects; patent ductus arteriosus)
- Mitral stenosis or regurgitation
- Chronic obstructive pulmonary disease (e.g., emphysema)
- Interstitial lung disease
- Obesity and the hypoventilation syndrome
- Pulmonary thromboembolism
- Dexfenfluramine and other diet
drugs
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Risk Factors |
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- Autoimmune disorders (e.g., systemic lupus erythematosus, rheumatoid
arthritis)
- Morbid obesity, especially accompanied by sleep apnea, or
hypoventilation syndrome
- Interstitial lung disease
- Family history of pulmonary hypertension (7% of cases)
- Human immunodeficiency virus
- Collagen vascular diseases
- Cocaine use
- Male gender
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Signs and Symptoms |
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All of the signs and symptoms are nonspecific, suggesting other more common
respiratory diseases. Pulmonary hypertension presents initially as dyspnea on
exertion; over time, however, the dyspnea occurs even at rest.
- Exertional dyspnea, often the only presenting symptom
- Excessive fatigue
- Exertional syncope or near syncope
- Cough
- Pleuritic chest pain
- Peripheral edema
- Hemoptysis
- Palpitations
Closer examination reveals the following signs.
- Loud P2
- Right ventricular lift
- Murmur of tricuspid or pulmonic insufficiency
- Right ventricular
S4
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Differential
Diagnosis |
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- Chronic obstructive pulmonary disease (COPD)
- Thromboembolic disease
- Pulmonary veno-occlusive disease
- Coronary artery disease
- Psychogenic dyspnea
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Diagnosis |
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Physical Examination |
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Classic findings include a reduced carotid pulse, increased jugular venous
pressure, an accentuated second heart sound (S2), right-sided third and fourth
heart sounds, tricuspid regurgitation, and right ventricular lift. There may be
peripheral cyanosis and edema. The right ventricle and central pulmonary
arteries are enlarged on chest X ray. The ECG and the echocardiogram reveal
right ventricular hypertrophy. Hypoxia and hypercapnia are almost always
present. |
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Pathology/Pathophysiology |
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Under normal circumstances the pulmonary vascular system is a high-flow,
low-pressure, and low-resistance system, even during exercise. In pulmonary
hypertension there is a decrease in the cross-sectional area of pulmonary blood
vessels because of vasoconstriction, obstruction, or obliteration. Pulmonary
arterial pressure and vascular resistance increase with increased flow and
eventually even with rest, leading to right ventricular hypertrophy and
deficient cardiac output. Other findings: remodeling of pulmonary vasculature
from chronic high flow (pressure), obliteration of the pulmonary vasculature,
vasoconstriction from interstitial lung disease, microthrombotic lesions in
small pulmonary arteries. |
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Imaging |
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- High-resolution computed tomography (CT)
- Echo-Doppler (shows right ventricular enlargement)
- Chest X ray (shows enlarged central pulmonary arteries)
- Pulmonary angiography
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Other Diagnostic
Procedures |
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- Open lung biopsy
- Balloon septostomy
- Ventilation and perfusion lung scanning
- ECG to detect right ventricular hypertrophy
These studies are used to exclude secondary causes of pulmonary
hypertension.
- Pulmonary function tests (e.g., spirometry, gas exchange
analysis)
- Chest X ray
- Two-dimensional M-mode, Doppler, and transesophageal
echocardiography
- Arterial blood gases
- Doppler or transesophageal echocardiography
- Pulmonary artery catheterization
- Sleep studies
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Treatment Options |
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Treatment Strategy |
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Treatment is entirely dependent on the cause. With secondary pulmonary
hypertension, the underlying disease must be treated. Hypoxia and hypercapnia
can be treated palliatively with supplemental oxygen, and vasodilators and
anticoagulants as needed. Patients must be counseled to avoid unnecessary
physical stress because pulmonary vascular resistance increases dramatically
with exercise. Withdrawal of appetite suppressant drugs sometimes, but not
always, may lead to stabilization or resolution of pulmonary
hypertension. |
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Drug Therapies |
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- Supplemental oxygen for hypoxemic patients
- Vasodilator therapy: adenosine, 50 mcg/kg/min, in increasing doses
until symptoms of pulmonary reactivity occur; prostacyclin, 2 ng/kg/min, every
30 minutes until side effects occur; nitric oxide may be effective in a subset
of patients with primary pulmonary hypertension, via inhalation of 5 to 10
parts/million, until no longer effective
- Calcium-channel blockers: nifedipine, 120 to 240 mg/day or diltiazem,
540 to 900 mg/day, to improve survival. Treatment should not be given, however,
without direct measurements of pulmonary artery pressures. As yet there is no
clear role for such therapies in secondary pulmonary hypertension.
- Anticoagulants (e.g., warfarin)—if the
primary disease is thromboembolic pulmonary disease
- Diuretics—for right ventricular
failure
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Complementary and Alternative
Therapies |
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Use to enhance respiratory function and strengthen cardiovascular and
pulmonary circulation. |
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Nutrition |
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- Coenzyme Q10 (100 mg bid) supports cardiac function, is an
antioxidant, and oxygenates tissues.
- L-carnitine (500 mg tid) improves endurance and is needed for
efficient cardiac function.
- Magnesium aspartate (200 mg bid to tid) increases efficiency of
cardiac muscle and decreases vascular resistance.
- Potassium aspartate (20 mg/day) improves heart contractility.
- Vitamin E (400 IU/day) is an antioxidant and is
cardioprotective.
- Vitamin C (1,000 to 1,500 mg tid) is an antioxidant, improves
vascular integrity, and stimulates immune function.
- Taurine (500 mg bid) enhances the efficiency of cardiac
function.
- Selenium (200 mcg/day) is a cardioprotective antioxidant.
- Choline (250 to 500 mg/day) and inositol (150 to 200 mg/day) are part
of the phospholipid membrane and positively affect parasympathetic activity and
vasodilation.
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Herbs |
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Herbs are generally a safe way to strengthen and tone the body's systems.
Ascertain a diagnosis first. Herbs may be used as dried extracts (capsules,
powders, teas), glycerites (glycerine extracts), or tinctures (alcohol
extracts). Unless otherwise indicated, teas should be made with 1 tsp. herb per
cup of hot water. Steep covered 5 to 10 minutes for leaf or flowers, and 10 to
20 minutes for roots. Drink 2 to 4 cups/day. Tinctures may be used singly or in
combination as noted.
The following herbs tonify the respiratory system, improve vascular tone,
lower blood pressure, and increase the efficiency of cardiac function. Combine
them in equal parts in tincture form and take 30 drops tid to qid.
- Hawthorn (Crataegus monogyna) increases cardiac output without
increasing cardiac load. Strengthens the integrity of vasculature and has mild
vasodilation activity.
- Garlic (Allium sativum) enhances expectoration, is
hypotensive, immune-stimulating, and anti-atherosclerotic.
- Rosemary (Rosmarinus officinalis) strengthens cardiac
function, is anti-atherosclerotic, antispasmodic, and improves circulation to
the lungs.
- Linden flowers (Tilia cordata) is an antispasmodic,
hypotensive, anti-atherosclerotic, respiratory relaxant, and expectorant. Also
stimulates immune function.
- Ginkgo (Ginkgo biloba) improves peripheral blood flow and
decreases platelet aggregation.
- Indian tobacco (Lobelia inflata) stimulates respiratory
function, is antispasmodic, and hypotensive. Used in high doses this herb can
have toxic side effects. Using small amounts in a formula (one-fourth or less)
will minimize the risk of toxicity.
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Homeopathy |
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An experienced homeopath should assess individual constitutional types and
severity of disease to select the correct remedy and potency.
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Physical Medicine |
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Castor oil pack. Apply oil directly to chest, cover with a clean soft cloth
(e.g., flannel) and plastic wrap. Place a heat source (hot water bottle or
heating pad) over the pack and let sit for 30 to 60 minutes. Use for three
consecutive days.
Contrast hydrotherapy. Alternating hot and cold applications to the chest
brings nutrients to the lungs and diffuses metabolic waste from inflammation.
The overall effect is decreased inflammation, pain relief, and enhanced healing.
Alternate three minutes hot with one minute cold. Repeat three times to complete
one set. Do two to three sets/day. For debilitated patients use cool and warm
applications.
Steams. Using three to six drops of essential oils in a humidifier,
vaporizer, atomizer, or warm bath will stimulate respiration and circulation.
Consider eucalyptus, rosemary, thyme, and/or lavender. This treatment will also
minimize risk of secondary infection due to COPD or interstitial lung disease.
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Acupuncture |
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May support treatment of symptoms through an increase in
circulation. |
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Patient Monitoring |
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Primary pulmonary hypertension is a relatively rare disease that most often
affects women in their 30s and 40s; however, it is seen at all ages from infancy
to over 60 years of age. |
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Other
Considerations |
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Prevention |
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Pulmonary hypertension has been associated with exogenous agents, collagen
vascular diseases, autoimmune disorders, and HIV. Weight control, avoiding diet
drugs, and smoking cessation all play a part in prevention. |
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Complications/Sequelae |
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Cor pulmonale, or hypertrophy of the right ventricle, is inevitable. Other
complications include thromboembolism, heart failure, and sudden
death. |
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Prognosis |
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Patients with primary pulmonary hypertension generally live only three to
five years from the time of the diagnosis. Patients usually die a sudden death
from heart failure. While the prognosis is generally poor, survival depends on
the severity of the pulmonary hypertension and the degree of right ventricular
hypertrophy. |
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Pregnancy |
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Pregnancy is contraindicated with pulmonary hypertension because it is
extremely dangerous for both the mother and the child.
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References |
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Bartram T. Encyclopedia of Herbal Medicine. Dorset, England: Grace
Publishers; 1995:195, 270, 276, 376.
Bordow RA, Moser KM. Manual of Clinical Problems in Pulmonary Medicine.
4th ed. Boston, Mass: Little, Brown; 1996:304-311, 353, 424, 431-434.
Fauci AS, Braunwald E, Isselbacher KJ, et al., eds. Harrison's Principles
of Internal Medicine. 14th ed. New York, NY: McGraw-Hill;
1998:1466-1468.
Fishman AP, Elias JA, Fishman JA, et al. Fishman's Pulmonary Diseases and
Disorders. 3rd ed. New York, NY: McGraw-Hill; l998:1261-1296.
Hinshaw HC, Murray JF. Disease of the Chest. 4th ed. Philadelphia, Pa:
WB Saunders Co; 1980:684-697.
Woodley M, Whelan A. Washington Manual of Therapeutics. 27th ed.
Boston, Mass: Little, Brown;
1992:211-212. |
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Copyright © 2000 Integrative Medicine
Communications This publication contains
information relating to general principles
of medical care that should not in any event be construed as specific
instructions for individual patients. The publisher does not accept any
responsibility for the accuracy of the information or the consequences arising
from the application, use, or misuse of any of the information contained herein,
including any injury and/or damage to any person or property as a matter of
product liability, negligence, or otherwise. No warranty, expressed or implied,
is made in regard to the contents of this material. No claims or endorsements
are made for any drugs or compounds currently marketed or in investigative use.
The reader is advised to check product information (including package inserts)
for changes and new information regarding dosage, precautions, warnings,
interactions, and contraindications before administering any drug, herb, or
supplement discussed herein. | |