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Look Up > Conditions > Primary Pulmonary Hypertension
Primary Pulmonary Hypertension
Overview
Definition
Etiology
Risk Factors
Signs and Symptoms
Differential Diagnosis
Diagnosis
Physical Examination
Pathology/Pathophysiology
Imaging
Other Diagnostic Procedures
Treatment Options
Treatment Strategy
Drug Therapies
Complementary and Alternative Therapies
Patient Monitoring
Other Considerations
Prevention
Complications/Sequelae
Prognosis
Pregnancy
References

Overview
Definition

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.


Etiology

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

Risk Factors
  • 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

Signs and Symptoms

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

Differential Diagnosis
  • Chronic obstructive pulmonary disease (COPD)
  • Thromboembolic disease
  • Pulmonary veno-occlusive disease
  • Coronary artery disease
  • Psychogenic dyspnea

Diagnosis
Physical Examination

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.


Pathology/Pathophysiology

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.


Imaging
  • High-resolution computed tomography (CT)
  • Echo-Doppler (shows right ventricular enlargement)
  • Chest X ray (shows enlarged central pulmonary arteries)
  • Pulmonary angiography

Other Diagnostic Procedures
  • 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

Treatment Options
Treatment Strategy

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.


Drug Therapies
  • 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

Complementary and Alternative Therapies

Use to enhance respiratory function and strengthen cardiovascular and pulmonary circulation.


Nutrition
  • 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.

Herbs

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.

Homeopathy

An experienced homeopath should assess individual constitutional types and severity of disease to select the correct remedy and potency.


Physical Medicine

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.


Acupuncture

May support treatment of symptoms through an increase in circulation.


Patient Monitoring

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.


Other Considerations
Prevention

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.


Complications/Sequelae

Cor pulmonale, or hypertrophy of the right ventricle, is inevitable. Other complications include thromboembolism, heart failure, and sudden death.


Prognosis

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.


Pregnancy

Pregnancy is contraindicated with pulmonary hypertension because it is extremely dangerous for both the mother and the child.


References

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.


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.