pulmonary hypertension

(redirected from Vaso-occlusive pulmonary hypertension)

Pulmonary Hypertension



Pulmonary hypertension is a rare lung disorder characterized by increased pressure in the pulmonary artery. The pulmonary artery carries oxygen-poor blood from the lower chamber on the right side of the heart (right ventricle) to the lungs where it picks up oxygen.


Pulmonary hypertension is present when the blood pressure in the circulation of the lungs is measured at greater than 25 mm of mercury (Hg) at rest or 30 mm Hg during exercise. Pulmonary hypertension can be either primary or secondary:
  • Primary pulmonary hypertension. The cause of pulmonary hypertension is unknown. It is rare, affecting one people per-million. The illness most often occurs in young adults, especially women.
  • Secondary pulmonary hypertension. Secondary pulmonary hypertension is increased pressure of the blood vessels of the lungs as a result of other medical conditions.
Regardless of whether pulmonary hypertension is primary or secondary, the disorder results in thickening of the pulmonary arteries and narrowing of these blood vessels. In response, the right side of the heart works harder to move the blood through these arteries and it becomes enlarged. Eventually overworking the right side of the heart may lead to right-sided heart failure, resulting in death.

Causes and symptoms

While the cause of primary pulmonary hypertension is uncertain, researchers think that in most people who develop the disease, the blood vessels are sensitive to certain factors that cause them to narrow. Diet suppressants, cocaine, and pregnancy are some of the factors that are thought to trigger constriction or narrowing of the pulmonary artery. In about 6-10% of cases, primary pulmonary hypertension is inherited.
Secondary pulmonary hypertension can be associated with breathing disorders such as emphysema and bronchitis, or diseases such as scleroderma, systemic lupus erythematosus (SLE) or congenital heart disease involving heart valves, and pulmonary thromboembolism.
Symptoms of pulmonary hypertension include shortness of breath with minimal exertion, general fatigue, dizziness, and fainting. Swelling of the ankles, bluish lips and skin, and chest pain are among other symptoms of the disease.


Pulmonary hypertension is rarely detected during routine physical examinations and, therefore, often progresses to later stages before being diagnosed. In addition to listening to heart sounds with a stethoscope, physicians also use electrocardiogram, pulmonary function tests, perfusion lung scan, and/or right-heart cardiac catheterization to diagnose pulmonary hypertension.


The aim of treatment for pulmonary hypertension is to treat the underlying cause, if it is known. For example, thromboendarterectomy is a surgical procedure performed to remove a blood clot on the lung that is causing the pulmonary hypertension. Lung transplants are another surgical treatment.
Some patients are helped by taking medicines that make the work of the heart easier. Anticoagulants, drugs that thin the blood, decrease the tendency of the blood to clot and allow blood to flow more freely. Diuretics decrease the amount of fluid in the body and reduce the amount of work the heart has to do. Calcium channel blockers relax the smooth muscle in the walls of the heart and blood vessels and improve the ability of the heart to pump blood.
One effective medical treatment that dilates blood vessels and seems to help prevent blood clots from forming is epoprostenol (prostacyclin). Prostacyclin is given intravenously to improve survival, exercise duration, and well-being. It is sometimes used as a bridge to help people who are waiting for a lung transplant. In other cases it is used for long-term treatment.
Some people require supplemental oxygen through nasal prongs or a mask if breathing becomes difficult.


Pulmonary hypertension is chronic and incurable with an unpredictable survival rate. Length of survival has been improving, with some patients able to live 15-20 years or longer with the disorder.


Since the cause of primary pulmonary hypertension is still unknown, there is no way to prevent or cure this disease. A change in lifestyle may assist patients with daily activities. For example, relaxation exercises help to reduce stress. Good health habits such as a healthy diet, not smoking, and getting plenty of rest should be maintained.

Key terms

Hypertension — The medical term for abnormally high blood pressure.
Perfusion lung scan — A scan that shows the pattern of blood flow in the lungs.
Pulmonary — Having to do with the lungs.
Pulmonary function test — A test that measures how much air the lungs hold and the air flow in and out of the lungs.
Right-heart cardiac catherization — A medical procedure during which a physician threads a catheter into the right side of the heart to measure the blood pressure in the right side of the heart and the pulmonary artery. The right heart's pumping ability can also be evaluated.



American Association for Respiratory Care. 11030 Ables Lane, Dallas, TX 75229. (972) 243-2272. http://www.aarc.org.
Pulmonary Hypertension Association. P.O. Box 24733, Speedway, IN 46224-0733. (800) 748-7274. http://www.phassociation.org.


"Primary Pulmonary Hypertensiton." National Heart, Lung, and Blood Institute. 〈http://www.nhlbi.nih.gov/nhlbi/lung/other/gp/pph.txt〉.
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.

pul·mo·nar·y hy·per·ten·sion

hypertension in the pulmonary circuit; may be primary, or secondary to pulmonary or cardiac disease, for example, fibrosis of the lung or mitral stenosis.
Farlex Partner Medical Dictionary © Farlex 2012

pulmonary hypertension

An idiopathic condition more common in women age 20 to 40, which is characterised by increased pulmonary arterial blood pressure in absence of other heart or lung disease. The major effect of pulmonary hypertension is increased right ventricular load which, when prolonged, predisposes patients to right ventricular failure, syncope, precordial pain, sudden death.
Idiopathic, secondary to Eisenmenger’s complex, respiratory failure in cystic fibrosis and COPD, with inhibition of endothelium-dependent pulmonary arterial relaxation due to decreased synthesis of nitric oxide or endothelium-derived growth factor.
High-dose calcium channel blockers may induce decreased pulmonary artery pressure and pulmonary vascular resistance, which may be combined with warfarin.

Causes of death
Arrhythmias, right and left ventricular dysfunction, left main coronary artery compression, dissection of pulmonary artery.

The average lifespan post-diagnosis is 3 years without therapy. The prognosis is worse in those who have severe symptoms, are older than 45 when diagnosed, present with right-sided heart failure, and do not respond to treatment.

Pulmonary hypertension aetiology 
Decreased cross-sectional area of the pulmonary vascular bed:
•  Parenchymal lung diseases; 
•  Lung resection;
•  Congenital hypoplasia.

Increased flow through the pulmonary arteries:
•  Systemic to pulmonary shunts.

Increased resistance to flow through large pulmonary arteries: 
•  Chronic thromboembolic disease;
•  Takayasu’s arteritis;
•  Congenital pulmonary artery stenosis; 
•  Mediastinal processes (fibrosis, tumours); 
•  Pulmonary artery tumours.
Increased resistance to flow through small pulmonary arteries:
•  Primary pulmonary arterial hypertension;
•  Pulmonary vasculitides;
•  Autoimmune diseases;
•  Chemical/toxic damage; 
•  Increased resistance to pulmonary venous drainage.
Increased resistance to pulmonary venous drainage:
•  Elevated left ventricular diastolic pressure;
•  Elevated left atrial pressure; 
•  Pulmonary venous obstruction.
Chronic alveolar hypoxia
•  Obesity-hypoventilation syndrome;
•  Chest wall disorders;
•  Neuromuscular disorders;
•  Parenchymal lung disease.
Miscellaneous conditions:
•  High altitude; 
•  Portopulmonary hypertension;
•  HIV infection;
•  Sickle haemoglobinopathies;
•  Pulmonary capillary hemangiomatosis.

Pulmonary Hypertension types
Systemic congestion due to mitral stenosis, left ventricular failure, left atrial myxoma, anomalous drainage of the pulmonary circulation.
Due to increased blood flow through lungs secondary to congenital heart defects.
Due to recurring vessel obstruction, seen in IV drug abuse and patients associated with hypoxia, alveolar hypoventilation (mitral stenosis, coarctation of aorta, Eisenmenger’s complex, ventricular septal defect).
Comprises 10-20% of cases, treated by addressing the underlying disease—e.g., unilateral renal artery stenosis, coarctation of aorta, primary aldosteronism, pheochromocytoma.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

pulmonary hypertension

Idiopathic pulmonary hypertension, primary pulmonary hypertension Internal medicine An idiopathic condition more common in ♀ age 20 to 40 characterized by ↑ blood pressure in pulmonary arteries in absence of other heart or lung disease; the major effect of PH is ↑ right ventricular load which, when prolonged, predisposes Pts to right ventricular failure, syncope, precordial pain, sudden death Types Idiopathic, 2º to Eisenmenger's complex, respiratory failure in cystic fibrosis and COPD, with inhibition of endothelium-dependent pulmonary arterial relaxation due to ↓ synthesis of nitric oxide or endothelium-derived growth factor Prognosis No longer thought to be universally fatal Treatment High-dose CCBs may induce ↓ pulmonary artery pressure and pulmonary vascular resistance, which may be combined with warfarin
Pulmonary Hypertension
Passive pulmonary hypertension, characterized by systemic congestion due to mitral stenosis, left ventricular failure, left atrial myxoma, anomalous drainage of the pulmonary circulation
Hyperkinetic pulmonary hypertension, with ↑ blood flow through lungs due to congenital heart defects
Vaso-occlusive pulmonary hypertension, due to recurring vessel obstruction, seen in IV drug abuse and PTE, associated with hypoxia, alveolar hypoventilation (mitral stenosis, coarctation of aorta, Eisenmenger's complex, ventricular septal defect).
Secondary pulmonary hypertension–comprises 10-20% of cases, treated by addressing the underlying disease, eg unilateral renal artery stenosis, coarctation of aorta, primary aldosteronism, pheochromocytoma  
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.

pul·mo·nar·y hy·per·ten·sion

(pul'mŏ-nar-ē hī'pĕr-ten'shŭn)
Hypertension in the pulmonary circuit; may be primary or secondary to pulmonary or cardiac disease (e.g., fibrosis of the lung or mitral stenosis).
Medical Dictionary for the Health Professions and Nursing © Farlex 2012

pulmonary hypertension

Abnormally high blood pressure in the arteries supplying the lungs. This occurs if the resistance to blood flow though the lungs is increased, as by PULMONARY FIBROSIS, so that the heart has to pump more strongly. Pulmonary hypertension causes the right side of the heart to enlarge and perhaps, eventually to fail. Endothelin-1 is known to have an important role in the disease.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005

Pulmonary Hypertension

DRG Category:315
Mean LOS:4 days
Description:MEDICAL: Other Circulatory System Diagnoses With CC

Pulmonary hypertension is diagnosed when the systolic pressure in the pulmonary artery exceeds 30 mm Hg. It is most commonly seen in preexisting pulmonary or cardiac disease but may occur (although rarely) as a primary condition when it is produced by fibrosis and thickening of the vessel intima. An increase in resistance of the vessels in the pulmonary vasculature bed occurs secondary to hypoxemia (oxygen deficiency). Chronic hypoxemia produces hypertrophy of the medial muscle layer in the smaller branches of the pulmonary artery, which decreases the size of the vessel lumen. Vasoconstriction, the pulmonary system’s response to hypoxemia, results in a pressure buildup in the right side of the heart because flow through the pulmonary system is impaired. When hypertension in the pulmonary system (measured as pulmonary vascular resistance) is greater than the ability of the right side of the heart to pump, the cardiac output falls and may cause shock.


The cause of primary pulmonary hypertension is unknown, but the disease tends to occur in families. Secondary pulmonary hypertension is caused by conditions that produce hypoxemia, such as chronic obstructive pulmonary disease, obesity, alveolar hypoventilation, smoke inhalation, and high altitude. Associated diseases include connective-tissue diseases, thyroid disease, liver cirrhosis, stimulant abuse, and HIV infection.

Genetic considerations

Familial primary pulmonary hypertension typically shows an autosomal dominant mode of inheritance and reduced penetrance and is more common in women than in men. Autosomal recessive transmission has also been documented. When pulmonary hypertension is inherited, it demonstrates the genetic concept of anticipation, where subsequent generations often have severe cases of the disease. Pulmonary hypertension is also seen in conjunction with other genetic problems such as hereditary hemorrhagic telangiectasia type 2. Mutations in the BMPR2 gene are associated with both heritable and sporadic forms of the disease, but for many cases, a gene mutation has not yet been identified.

Gender, ethnic/racial, and life span considerations

Pulmonary hypertension is most commonly seen in the elderly person with cardiac or pulmonary disease. It may occur at any age, however. Idiopathic primary pulmonary hypertension tends to occur more often in women between ages 20 to 40. Congenital causes may lead to occurrence in the pediatric population. There are no known racial or ethnic considerations.

Global health considerations

The global incidence rate is approximately 2 to 6 cases per 1 million individuals per year. Pulmonary hypertension exists around the globe at approximately the same incidence as in the United States.



Patients are usually without symptoms until late in the disease. Up to 50% of the pulmonary circulation may be impaired before significant hypertension is produced. Determine the presence of risk factors. Ask if the patient has experienced chest pain, labored and painful breathing (dyspnea), or syncope. Occasionally, the enlarged pulmonary artery compresses the left recurrent laryngeal nerve, producing hoarseness. Some patients may describe periods of heart palpitations.

Physical examination

Signs of right ventricular failure are common, such as dyspnea, weakness, and recurrent syncope likely accompanied by jugular venous distention, increased central venous pressure, and peripheral edema. Low cardiac output may produce central cyanosis, syncope, or chest pain. Auscultation of the heart may therefore reveal atrial gallop at the lower left sternal border, narrow splitting of S2 or increased S2 intensity, or ejection click at the second intercostal space, left sternal border. When palpating the precordium, you may detect a heave over the right ventricle or an impulse from the pulmonary artery itself. Signs of left ventricular failure, such as systemic hypotension (low blood pressure) and low urinary output, may coexist. Presentation may include hyperventilation, coughing, and eventually rapid breathing (tachypnea) or dyspnea. Initially, breath sounds may be clear or decreased, but you may hear crackles or wheezing.


The patient is experiencing a potentially life-threatening condition that requires the use of complex medical technology. Assess the anxiety level of the patient and plan interventions to place a minimum demand on the patient’s energy. Support of the patient is essential throughout hospitalization, from routine care such as placement and maintenance of the pulmonary artery catheter to attempts at averting a cardiac arrest.

Diagnostic highlights

TestNormal ResultAbnormality With ConditionExplanation
EchocardiogramNormal lung structures and circulationRight-to-left shunting across a patent foramen ovale (occurs in approximately 33% of patients with pulmonary hypertension)Estimate ventricular functioning; pulmonary hypertension may occur because of right to left shunting
Pulmonary artery pressure and pulmonary vascular resistance (PVR) (measurements made with a pulmonary artery catheter)Systolic: 15–20 mm Hg; diastolic: 8–15 mm Hg; PVR: 180–285 dynes/sec per cm–5 per m2Pressures elevated, with systolic pressure < 25 mm Hg; pulmonary artery systolic pressure may approach systemic arterial pressureSustained elevation of pulmonary vascular pressures

Other Tests: Antinuclear antibody to exclude autoimmune disorders, thyrotropin, HIV testing, pulmonary function tests, exercise testing, electrocardiogram, ventilation perfusion scan, pulmonary angiogram, chest x-rays, echocardiogram, high-resolution computed tomography

Primary nursing diagnosis


Impaired gas exchange related to changes in the alveolar membrane structure and increased pulmonary vascular resistance


Respiratory status: Gas exchange; Respiratory status: Ventilation; Comfort level; Anxiety control


Airway insertion and stabilization; Airway management; Respiratory monitoring; Oxygen therapy; Mechanical ventilation; Anxiety reduction

Planning and implementation


Primary pulmonary hypertension has limited therapy, and patients tend to have hemodynamic deterioration in spite of therapy. The median survival rate after diagnosis is 2.5 years. Supportive measures include supplemental oxygen for people who are hypoxemic and the use of diuretics in people who are fluid-overloaded. Relief of hypoxemia helps reduce pulmonary vasoconstriction. If the origin of the problem is structural, surgery may be attempted. Heart-lung transplantation is a consideration for severe conditions.

Pharmacologic highlights

Medication or Drug ClassDosageDescriptionRationale
DiureticsVaries by drugLoop diuretics, thiazide diureticsReduce both right and left ventricular failure
Sodium warfarin (Coumadin)5 mg PO initially, guided by coagulation studiesAnticoagulantPrevents microvascular thrombosis, venous stasis, and limitation of physical activity
VasodilatorsVaries by drugNitrates; calcium channel blockers; prostacyclin analogs: epoprostenol (Flolan), treprostinil (Remodulin), iloprost (Ventavis); endothelin antagonists: bosentan (Tracleer), ambrisentan (Letairis)Improve muscle tone in pulmonary vascular bed and reduce right ventricular workload
Sildenafil (Revatio)20 mg PO tidPhosphodiesterase type 5 (PDE5) inhibitorPromotes selective smooth muscle relaxation

Other Drugs: Bronchodilators to improve hypoxemia and reduce pulmonary vascular resistance (note that use of vasodilators is limited because it may produce systemic hypotension); oral prostacyclin (PG12) treprostinil extended-release tablets to cause vasodilation.


If the patient is critically ill, to minimize the risk of infection, use the sterile technique during setup and maintenance of the pulmonary artery catheter. Dressings should be changed according to policy, usually every 72 hours. Ask the patient to evaluate chest pain using a scale from 1 to 10 and provide comfort measures in addition to any ordered medication. Reduce energy demands by assisting the patient to a position of comfort, such as the semi-Fowler’s or Fowler’s position. Document pulmonary artery catheter readings and report significant changes to the medical team. Monitor the patient for the development of cardiac dysrhythmias.

Allow the patient to verbalize fears and assist in the development of a realistic perception as the patient appears ready. Because this is a disease that may affect young women in their 20s and 30s, consider the effects on their lifetime goals. Family considerations, especially if the patient is the mother of young children, requires sensitivity and possible referral. Incorporate family members and other support system members as appropriate. Help the patient adjust to the limitations imposed by this disorder. Advise against overexertion and suggest frequent rest periods between activities. The patient may need diversionary activities during periods of restricted activity. Be sure the patient understands dietary limitations and medication regimens.

Evidence-Based Practice and Health Policy

Sztrymf, B., Souza, R., Bertoletti, L., Jaïs, X., Sitbon, O., Price, L.C., …Humbert, M. (2010). Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. The European Respiratory Journal, 35(6), 1286–1293.

  • Right ventricular failure is a major complication of pulmonary hypertension and is associated with increased mortality.
  • Investigators conducted a study among 46 adult patients who were admitted to an intensive care unit (ICU) with acute right ventricular failure secondary to pulmonary hypertension and found that mortality in the ICU was 41.3%. The average time from pulmonary hypertension diagnosis to ICU admission was 6.3 years (range, 2.3 to 16.5 years).
  • Nonsurvivors had higher serum brain natriuretic peptide (BNP) levels (median, 1,415 pg/mL versus 628 pg/mL; p = 0.0007), higher serum creatinine levels (112 mol/L versus 95 mol/L; p = 0.04), and higher C-reactive protein levels (40 mg/L versus 12 mg/L; p = 0.01) upon admission. Nonsurvivors were also more likely to develop an infection while hospitalized compared to survivors (73.7% versus 22.2%; p = 0.0005).

Documentation guidelines

  • Vital signs, including pulmonary artery catheter readings
  • Cardiovascular and pulmonary physical assessment data, including breath and heart sounds as noted in previous sections
  • Responses to therapies and disease status, including medication, diet, fluids, oxygen administration, and psychological/family coping

Discharge and home healthcare guidelines

Risk for recurrent pulmonary embolism can be reduced by teaching the patient to minimize hypercoagulability, to reduce venous stasis, and to control risk factors such as obesity. Teach the patient to drink 2,000 mL of fluid a day unless restricted, to rest between activities, and to avoid overexertion. Teach the patient about the prescribed dosage, route, action, and follow-up laboratory work needed for all medications. If the patient is discharged on potassium-wasting diuretics, encourage a diet that is rich in high-potassium foods, such as apricots, bananas, oranges, and raw vegetables. The patient may also need instruction on a low-sodium diet. If the patient needs home oxygen, instruct the patient and significant others in oxygen use and oxygen safety. Arrange with social services for the delivery of oxygen equipment. If the patient smokes, teach strategies for smoking cessation or provide a referral for smoking-cessation programs. Assess the family situation and the effects of a chronic and debilitating disease that will affect occupational goals, child care, role fulfillment, and long-term health.

Diseases and Disorders, © 2011 Farlex and Partners