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Table of Contents Cardiopulmonary Syndrome Overview
Dyspnea and Coughing During Advanced Cancer
Malignant Pleural Effusions
Malignant Pericardial Effusions
Superior Vena Cava Syndrome
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Changes to This Summary (02/17/2009)
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Cardiopulmonary Syndrome Overview
Cardiopulmonary syndromes are heart and lung symptoms, such as dyspnea (shortness of breath), cough, chest pain, irregular heartbeats, and excess fluid around the lungs (pleural effusion) and/or heart (pericardial effusion). These may be caused by cancer or by other conditions. Four cardiopulmonary syndromes commonly caused by cancer are covered in this summary:
Back to Top Dyspnea and Coughing During Advanced Cancer
General Information about Dyspnea and Coughing
Dyspnea is difficult, painful breathing or shortness of breath. Patients may use different words to describe the feeling of breathlessness; terms such as "tightness in the chest" and "suffocating" are sometimes used. The distress caused by dyspnea is different for each patient, from mild discomfort in one patient to severe discomfort in another. Dyspnea is common in patients with advanced cancer, lung cancer, and in the last 6 weeks of life.
Causes of Dyspnea and Coughing
Many conditions may cause dyspnea and coughing. In cancer patients, causes may include the following:
- Direct effects of the tumor:
- Indirect effects of the tumor:
- Treatment-related effects:
- Conditions the patient may have unrelated to the cancer:
- Conditions with no known physical cause, such as anxiety.
Diagnosis of Dyspnea and Coughing
A diagnosis of the cause of the patient's dyspnea and coughing is helpful in planning treatment. Diagnostic tests and procedures may include the following:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Functional assessment: An exam to check for how the dyspnea affects the patient's ability to perform activities of daily living.
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan: A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- Complete blood count: A procedure in which a sample of blood is drawn and checked for the following:
- Oxygen saturation test: A procedure to determine the amount of oxygen being carried by the red blood cells. A lower than normal amount of oxygen may be a sign of lung disease or other medical conditions. One method uses a device clipped to the finger. The device senses the amount of oxygen in the blood flowing through the finger. Another method uses a sample of blood drawn from an artery, usually in the wrist, and tested for the amount of oxygen.
- Maximum inspiratory pressure (MIP) test: The MIP is the highest pressure that can be generated while breathing in. The MIP test measures this pressure and the strength of the muscles used to breathe. The patient breathes through a device called a manometer, which measures the pressure and sends the information to a computer.
Managing Dyspnea and Coughing
Management of Causes of Dyspnea
It may be possible to identify and treat the causes of dyspnea. Treatment may include the following:
Management of Symptoms of Dyspnea
Management of the symptoms of dyspnea may include the following:
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Oxygen therapy
Patients who cannot breathe enough oxygen from the air may be given supplemental oxygen to inhale from tanks or cylinders. Devices that concentrate oxygen already in the air may also be prescribed.
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Medicines
Opioids may reduce physical and mental distress and exhaustion, and improve the patient's quality of life. Other drugs may be used to treat dyspnea that is related to panic disorder or severe anxiety.
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General support
Supportive measures may be effective for some patients. These measures include the following:
Management of Chronic Cough
In some patients, chronic (long-term) coughing causes pain, interferes with sleep, and worsens dyspnea and fatigue. Treatments include the following:
Back to Top Malignant Pleural Effusions
General Information about Malignant Pleural Effusions
The pleural cavity is the space surrounding each lung in the chest. The pleura is the thin layer of tissue that covers the outer surface of each lung and lines the interior wall of the chest cavity, creating a sac that encloses the pleural cavity.
Pleural tissue normally produces a small amount of fluid that helps the lungs move smoothly in the chest while a person is breathing. A pleural effusion is an increased amount of fluid in the pleural cavity, which then presses on the lungs and makes breathing difficult.
Causes of Malignant Pleural Effusions
Pleural effusions may be malignant (caused by cancer) or nonmalignant (caused by a condition that is not cancer). Malignant effusions are a common complication of cancer. Lung cancer, breast cancer, lymphoma, and leukemia cause most malignant effusions. Effusions caused by cancer treatment, such as radiation therapy or chemotherapy, are called paramalignant effusions.
Not all pleural effusions found in cancer patients are malignant. Cancer patients often develop conditions
such as congestive heart failure, pneumonia, pulmonary embolism, and malnutrition, and these conditions may cause pleural effusions to occur.
Diagnosis of Malignant Pleural Effusion
The following symptoms may be caused by malignant pleural effusion:
The management of a malignant pleural effusion is different from the management of a nonmalignant effusion, so an accurate diagnosis is important. Diagnostic tests may include the following:
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan: A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- Thoracentesis: The removal of fluid from the pleural cavity using a needle inserted between the ribs. This procedure may be used to reduce pressure on the lungs and/or to check the fluid under a microscope to see if cancer cells are present.
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. If thoracentesis is not possible, a biopsy may be done during a thoracoscopy, a surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope (a thin, lighted tube) is inserted into the chest. Samples are then taken for biopsy.
Managing Malignant Pleural Effusions
Malignant pleural effusions often occur in advanced or unresectable cancer or in the last few weeks of life. The goal of treatment is usually palliative, to relieve the symptoms and improve the quality of life. The goals of therapy will depend on a number of factors, including the following:
- The prognosis (chance of recovery).
- The patient's preferences in regard to the risks and benefits of treatment.
- The patient's ability to perform activities of daily living.
- The type of primary cancer.
- The number and type of previous treatments. For example, patients whose cancer has not responded to chemotherapy are unlikely to obtain symptom relief with additional chemotherapy.
Treatment of the symptoms of malignant pleural effusion may include the following:
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Thoracentesis
(See Diagnosis of Malignant Pleural Effusion.) Removal of fluid from the pleural cavity using a needle and/or a thin, hollow plastic tube may help to alleviate severe symptoms in the short-term. A few days after thoracentesis, the effusion will begin to reform. Repeated thoracentesis has risks, however, including bleeding, infection, collapsed lung, fluid in the lungs, and low blood pressure.
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Pleurodesis
This is a procedure to close the pleural sac so that fluid
cannot collect there. Fluid is first removed by thoracentesis, using a chest tube. A drug or chemical that causes the sac to close is then inserted into the space through a chest tube. Chemical agents such as bleomycin or talc may be used.
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Surgery
Surgery may be done to implant a shunt (tube) to transfer the fluid from the pleural cavity to the peritoneal (abdominal) cavity, where the
fluid can be more easily removed. Another option is pleurectomy, removal of the part of the pleura that lines the chest.
Back to Top Malignant Pericardial Effusions
General Information about Malignant Pericardial Effusions
Pericardial effusion is an increased amount of fluid inside the pericardium, the thin layer of tissue that forms a sac surrounding the heart. The excess fluid causes pressure on the heart, which prevents it from pumping blood normally. Lymph vessels may also be blocked, and bacterial or viral infections often develop.
If fluid builds up very quickly, a condition called cardiac tamponade may occur, in which the pressure on the heart becomes life-threatening and must be treated promptly.
Causes of Malignant Pericardial Effusions
Pericardial effusions may be malignant or nonmalignant. Malignant pericardial effusions are caused by cancer that begins in the pericardium or the heart muscle, or by cancer that has spread there from the lung, esophagus, thymus, or lymph system. Malignant pericardial effusions are commonly caused by lung cancer in males and breast cancer in females. Nonmalignant causes include infection of the pericardium, heart attack, underactive thyroid gland, lupus, injury, surgery, and AIDS. Infection of the pericardium is a possible side effect of radiation therapy or chemotherapy.
Diagnosis of Malignant Pericardial Effusion
The following symptoms may be caused by malignant pericardial effusions:
- Dyspnea.
- Cough.
- Chest pain.
- Difficulty breathing while lying flat.
- Swelling in the upper abdomen.
- Hiccups.
- Extreme tiredness and weakness.
Because pericardial effusions usually occur in advanced cancer or in the last few weeks of life, extensive diagnostic testing may be less important than relief of symptoms. The following tests and procedure may be used to diagnose pericardial effusion:
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- Echocardiography:
A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs of the chest. The echoes form a picture of the heart's position, motion of the walls, and internal parts such as the valves.
- Electrocardiogram (EKG or ECG): A recording of the heart's electrical activity to evaluate its rate and rhythm. A number of small pads (electrodes) are placed on the patient’s chest, arms, and legs, and are connected by wires to the electrocardiograph machine. Heart activity is then recorded as a line graph on paper. Electrical activity that is faster or slower than normal may be a sign of heart disease or damage.
- Pericardiocentesis: The removal of fluid from the pericardium using a needle inserted through the chest wall. The physician may use an echocardiogram to view the movement of the needle inside the chest. This procedure can be used to drain fluid from an effusion and reduce pressure on the heart. To diagnose malignant pericardial effusion, the fluid is examined under a microscope to check for cancer cells. The fluid may also be checked for signs of infection.
Managing Malignant Pericardial Effusions
Large malignant pericardial effusions are managed by draining the fluid, unless the goals of therapy are to use a less invasive approach that may improve quality of life but not help the patient live longer. The goals of therapy depend on a number of factors, including the following:
- The patient's prognosis.
- The cost, risks, and invasiveness of treatment.
- Whether treatment will relieve symptoms and improve the patient's quality of life.
- Whether treatment will shorten the patient's hospital stay.
Treatment options include the following:
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Pericardiocentesis
(See Diagnosis of Malignant Pericardial Effusion.) In some patients, fluid may again collect in the pericardium after pericardiocentesis. A catheter may be inserted and left in place to allow continued drainage. This procedure may be used for patients with advanced cancer instead of more invasive surgery.
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Pericardial sclerosis
A procedure to close the pericardium so fluid cannot collect in the cavity. Fluid is first removed by pericardiocentesis. A drug or chemical that causes the pericardium to close is then injected through a catheter into the pericardial space. Three or more treatments may be needed to completely close the pericardium.
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Pericardotomy
A surgical incision is made in the chest and then in the pericardium to insert a drainage tube. This increases the quantity of fluid that can be drained from the pericardium.
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Pericardiectomy
Surgery to remove part of the pericardium. This may be done when there are chronic infections of the pericardium or to drain fluid quickly when cardiac tamponade occurs. This surgery is also called pericardial window.
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Balloon pericardiostomy
A catheter with a balloon tip is inserted through the chest and into the pericardium. The balloon is then inflated to enlarge the pericardial opening and allow fluid to drain into the pleural cavity. This may be used when an effusion has recurred (come back) after pericardiocentesis or as an alternative to more invasive surgery.
Back to Top Superior Vena Cava Syndrome
General Information about Superior Vena Cava Syndrome
Superior vena cava syndrome (SVCS) is a group of symptoms that occur when the superior vena cava becomes partially blocked.
The right atrium (chamber) of the heart receives blood from two major veins: the superior (upper) vena cava and the inferior (lower) vena cava.
- The superior vena cava returns blood from the upper body to the heart.
- The inferior vena cava returns the blood from the lower body to the heart.
The superior vena cava is thin-walled, and the blood is under low pressure. If a tumor forms in the chest or nearby lymph nodes become swollen (as from lymphoma), the superior vena cava can be squeezed. Blood flow slows. Complete blockage of the vein can occur. Sometimes, the other veins can become larger and take over for the superior vena cava if it is blocked, but this takes time. Superior vena cava syndrome (SVCS) is the group of symptoms that occur when this vein is partially blocked.
The location of the blocked area and how fast the blockage occurs affect the symptoms.
The symptoms will be more severe if the vein becomes blocked quickly. This is because the other veins do not have time to widen and take over the increased blood flow from the superior vena cava.
The location of the blocked area also affects how severe the symptoms will be:
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If the blockage is above where the superior and inferior vena cava veins join, other veins can become larger over time and take over the increased blood flow. The symptoms may be milder.
- If the blockage occurs below where the superior vena cava and inferior vena cava meet, the blood must be returned to the heart by the veins in the upper abdomen and the inferior vena cava, which require higher pressure. Symptoms may be more severe.
Common symptoms of SVCS include breathing problems and coughing.
The most common symptoms are these:
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Problems breathing.
- Coughing.
- Swollen face, neck, upper body, and arms.
Less common symptoms include the following:
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Hoarse voice.
- Chest pain.
- Problems swallowing and/or talking.
- Coughing up blood.
- Swollen veins in the chest or neck.
- Bluish color to the skin.
- Drooping eyelid.
Causes of Superior Vena Cava Syndrome
Superior vena cava syndrome (SVCS) is usually caused by cancer. In adults, SVCS most commonly occurs with lung cancer or non-Hodgkin lymphoma. A tumor in the chest or swollen lymph nodes can press on the superior vena cava, blocking the blood flow. There are other less common causes for the superior vena cava to become blocked:
Diagnosis of Superior Vena Cava Syndrome
The following tests may be done to diagnose SVCS and find the location of the blockage:
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the chest will be done to diagnose SVCS.
- Venography: A procedure to x-ray veins. A contrast dye is injected into the veins to outline them on the x-rays.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Ultrasound: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
The type of cancer can affect the type of treatment needed; for this reason, a diagnosis of suspected cancer should be made before treatment of SVCS is begun. Unless the airway is blocked or the brain is swelling, waiting to start treatment while a diagnosis is made usually presents no problem in adults. If lung cancer is suspected, a sputum sample and a biopsy may be taken.
Managing Superior Vena Cava Syndrome
This summary is about treatment for superior vena cava syndrome (SVCS) caused by cancer. Treatment will depend on the following:
- The type of cancer.
- The cause of the blockage.
- How severe the symptoms are.
- The prognosis (chance of recovery).
- The patient's wishes.
Treatment of SVCS may include the following:
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless symptoms appear or change. A patient who has good blood flow through other veins and mild symptoms may not need treatment.
The following may be used to relieve symptoms and keep the patient comfortable:
- Keeping the upper body raised higher than the lower body.
- Corticosteroids (drugs that reduce swelling).
- Diuretics (drugs that make excess fluid pass from the body in urine). Patients taking diuretics are closely monitored because these drugs can cause dehydration (loss of too much fluid from the body).
Radiation therapy
If the blockage of the superior vena cava is caused by a tumor that is not sensitive to chemotherapy, radiation therapy may be given. Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.
Chemotherapy
Chemotherapy is the usual treatment for tumors that respond to anticancer drugs, including small cell lung cancer and lymphoma. This treatment would not be changed for patients who have SVCS. Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).
Thrombolysis
SVCS may occur when a thrombus (blood clot) forms in a partially blocked vein. Thrombolysis is a method used to break up and remove blood clots. This may done using a drug put directly into the clot, through a catheter, or by a thrombectomy (the use of a device inserted into the vein).
Stent placement
A stent may be used to open up the blocked vein. A stent is a tube-like device that is inserted into the blocked area of a vein to allow blood to pass through. This helps most patients.
Patients may also receive an anticoagulant to keep more blood clots from forming.
Surgery
Surgery to bypass (go around) the blocked part of the vein is sometimes used for cancer patients, but is used more often for patients without cancer.
Social Considerations of Superior Vena Cava Syndrome
Superior vena cava syndrome is serious and the symptoms can be upsetting to the patient and family.
It is important that patients and family members receive information about the causes of superior vena cava syndrome and how to treat it. This can help relieve anxiety over symptoms such as swelling, trouble swallowing, coughing, and hoarseness.
When a patient has chosen not to receive aggressive treatment because of terminal cancer, palliative treatment can help keep the patient comfortable by relieving symptoms. Patients and family members can be taught how to provide palliative care to relieve symptoms and improve quality of life.
Superior Vena Cava Syndrome in Children
Superior vena cava syndrome in a child is a serious medical emergency because the child's windpipe can become blocked.
Superior vena cava syndrome (SVCS) in children can be life threatening. This is because blockage of the child's trachea (windpipe) can quickly occur along with SVCS. In adults, the windpipe is fairly hard, but in children, it is softer and can more easily be squeezed shut. Also, the diameter of a child's windpipe is smaller, so any amount of swelling can cause breathing problems. Squeezing of the trachea is called superior mediastinal syndrome (SMS). Because SVCS and SMS often occur together in children, the two syndromes are considered to be the same.
The most common symptoms of SVCS in children are similar to those in adults.
Common symptoms include the following:
- Coughing.
- Hoarseness.
- Problems breathing.
- Chest pain.
There are other less common but more serious symptoms:
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Fainting.
- Anxiety.
- Confusion.
- Tiredness.
- Headache.
- Vision problems.
- A sense of fullness in the ears.
The causes, diagnosis, and treatment of SVCS in children are not the same as in adults.
The most common cause of SVCS in children is non-Hodgkin lymphoma.
SVCS in children is rare; the most common cause is non-Hodgkin lymphoma. As in adults, SVCS may also be caused by a blood clot that forms as a side effect of using an intravenous catheter.
SVCS in children may be diagnosed and treated before a definite diagnosis of cancer is made.
A physical exam, chest x-ray, and medical history are usually all that are needed to diagnose superior vena cava syndrome in children. If cancer is suspected, a biopsy is not done unless the lungs and heart of the child with SVCS are able to handle the anesthesia needed. Other imaging tests may be done to help determine if anesthesia can be safely used. In most cases, treatment will begin before a definite diagnosis of cancer is made.
It is important that treatment begins right away.
The following treatments may be used for SVCS in children:
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Radiation therapy
Radiation therapy is usually used to treat a tumor that is causing the blocked vein. After radiation therapy, breathing may become more difficult because swelling narrows the windpipe. A drug to reduce swelling may be given.
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Drugs
Anticancer drugs, steroids, and/or other drugs may be used. If the tumor does not respond, it may be benign (not cancer).
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Surgery
This may include surgery to bypass (go around) the blocked part of the vein or to place a stent to open the vein.
Back to Top Get More Information From NCI
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Back to Top Changes to This Summary (02/17/2009)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
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PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
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