Cor pulmonale

Right-sided heart failure Last reviewed: May 1, 2011. Cor pulmonale is failure of the right side of the heart brought on by long-term high blood pressure in the pulmonary arteries and right ventricle of the heart.

Causes, incidence, and risk factors

Normally, the left side of the heart produces a higher blood pressure in order to pump blood to the body. The right side of the heart pumps blood through the lungs under much lower pressure. High blood pressure in the arteries of the lungs is called pulmonary hypertension. The right side of the heart has a harder time pumping blood against these higher pressures. If this high pressure is present for a longer period of time, it puts a strain on the right side of the heart, leading to cor pulmonale. Almost any chronic lung disease or condition causing prolonged low blood oxygen levels can lead to cor pulmonale. A few of these causes include:
y y y y y y

Chronic obstructive pulmonary disease (COPD) Chronic blood clots in the lungs Cystic fibrosis Scarring of the lung tissue (interstitial lung disease) Severe curving of the upper part of the spine (kyphoscoliosis) Obstructive sleep apnea, in which pauses occur during breathing because of airway inflammation

Symptoms
Shortness of breath or light-headedness during activity is often the first symptom. You may have a fast heart beat and feel like your heart is pounding. Over time, symptoms occur with lighter activity or even while at rest. They may include:
y y y y y

Fainting spells with activity Chest discomfort, usually in the front of the chest Chest pain Swelling of the feet or ankles Symptoms of underlying disorders (wheezing, coughing)

Signs and tests

The health care provider will perform a physical exam. This may reveal:
y y y y y y

Fluid build up in the belly area (abdomen) Abnormal heart sounds Bluish skin (cyanosis) Liver swelling Swelling (distension) of the neck veins, which is a sign of high right-heart pressures Ankle swelling

The following tests may help diagnose cor pulmonale:

y y y y y y y y y y

Blood antibody tests Blood test for brain natriuretic peptide (BNP) Chest x-ray CT scan of the chest Echocardiogram Lung biopsy (rarely performed) Measurement of blood oxygen by arterial blood gas (ABG) Pulmonary function tests Right heart catheterization Ventilation and perfusion scan of the lungs (V/Q scan)

Treatment
The goal of treatment is to control symptoms. It is important to treat medical disorders that cause pulmonary hypertension, which can lead to cor pulmonale. Many new treatment options are becoming available. Medicines that may be prescribed include:
y y y y y y

Ambrisentan (Letairis) Bosentan (Tracleer) Calcium channel blockers Diuretics Prostacyclin or similar medications Sildenafil

Your doctor will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well the medicine works for you. Never stop taking your medicines without talking to your doctor. Other treatments may include:
y y y

Blood thinners to reduce the risk of blood clots Oxygen, in some cases A lung or heart-lung transplant, if medication does not work

As the illness gets worse, you will need to make changes in the home environment and get more help around the home. Other important tips to follow:
y y y y y

Avoid strenuous activities and heavy lifting Avoid traveling to high altitudes Get yearly flu and pneumonia vaccines Stop smoking Talk to your doctor before becoming pregnant

Expectations (prognosis)
How well you do depends on what caused the condition.

Complications
Cor pulmonale may lead to:
y y y y

Life-threatening shortness of breath Severe fluid retention Shock Death

Calling your health care provider

Call your health care provider if you experience shortness of breath or chest pain.

Prevention
Avoiding behaviors that lead to chronic lung disease (especially cigarette smoking) may prevent the eventual development of cor pulmonale. Careful evaluation of childhood heart murmurs may prevent cor pulmonale caused by certain heart defects.

References
1. McGlothlin D, De Marco T. Cor pulmonale. In: Mason RJ, Broaddus VC, Martin TR, et al. Murray & Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 56. Review Date: 5/1/2011. Reviewed by: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; Denis

Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001153/figure/A000091.B17055/?report=objectonly

Emphysema

Emphysema is a lung disease involving damage to the air sacs (alveoli).There is progressive destruction of alveoli and the surrounding tissue that supports the alveoli. With more advanced disease, large air cysts develop where normal lung tissue used to be. Air is trapped in the lungs due to lack of supportive tissue which decreases oxygenation.

COPD (chronic obstructive pulmonary disorder)

Chronic obstructive pulmonary disease (COPD) refers to chronic lung disorders that result in blocked air flow in the lungs. The two main COPD disorders are emphysema and chronic bronchitis, the most common causes of respiratory failure. Emphysema occurs when the walls between the lung's air sacs become weakened and collapse. Damage from COPD is usually permanent and irreversible.

Respiratory system

Clubbing

Clubbing may result from chronic low blood-oxygen levels. This can be seen with cystic fibrosis, congenital cyanotic heart disease, and several other diseases. The tips of the fingers enlarge and the nails become extremely curved from front to back.

Clubbed fingers

Clubbed fingers is a symptom of disease, often of the heart or lungs which cause chronically low blood levels of oxygen. Diseases which cause malabsorption, such as cystic fibrosis or celiac disease can also cause clubbing.

Obstructive sleep apnea

Massively enlarged tonsils can cause episodes of cessation of breathing known as obstructive sleep apnea. Cessation of breathing can last 10 seconds or longer, causing extremely low levels of oxygen in the blood

Cardiac catheterization

Cardiac catheterization is used to study the various functions of the heart. Using different techniques, the coronary arteries can be viewed by injecting dye or opened using balloon angioplasty. The oxygen concentration can be measured across the valves and walls (septa) of the heart and pressures within each chamber of the heart and across the valves can be measured. The technique can even be performed in small, newborn infants

Swan Ganz catheterization

Right heart catheterization involves the passage of a catheter (a thin flexible tube) into the right side of the heart to obtain diagnostic information about the heart and for continuous monitoring of heart function in critically ill patients. Home > Tests and Treatments > Swan-Ganz - right heart catheterization Print

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Swan-Ganz - right heart catheterization

Right heart catheterization; Catheterization - right heart Last reviewed: July 10, 2010. Swan-Ganz catheterization is the passing of a thin tube (catheter) into the right side of the heart and the arteries leading to the lungs to monitor the heart's function and blood flow, usually in persons who are very ill.

How the test is performed

The test can be done while you are in bed in an intensive care unit (ICU) of a hospital or in special procedure areas such as a cardiac catheterization laboratory.

Before the test starts, you will be given a mild sedative to help you relax. An area of your body, usually the neck or groin, is cleaned and numbed with a local anesthetic. The health care provider will make a small cut in a vein in your neck or groin. Sometimes, the cut is made in another area. A thin hollow tube called a catheter is inserted through the cut and up into a vein. It is carefully moved up into the right atrium (upper chamber) of the heart. X-ray images help the doctor see where the catheter should be placed. The catheter is threaded through two heart valves (the tricuspid and pulmonary valve) and placed into the pulmonary (lung) artery. Once it is in place, the blood pressure in the pulmonary artery is measured. Blood may be removed from the catheter to measure the amount of oxygen in the blood. During the procedure, your heart's rhythm will be constantly watched using an electrocardiogram (ECG).

How to prepare for the test

You should not eat or drink anything for 8 hours before the test starts. You may need to stay in the hospital the night before the test. Otherwise, you will check in to the hospital the morning of the test. In critically ill patients, the test may be done in the intensive care unit. You will wear a hospital gown. You must sign a consent form before the test. Your health care provider will explain the procedure and its risks.

How the test will feel

You may be given sedation to help you relax before the procedure, but you will be awake and able to follow instructions during the test. You may feel some discomfort when the IV is placed into your arm and some pressure at the site when the catheter is inserted. In critically ill patients, the catheter may stay in place for several days.

Why the test is performed

The procedure is done to evaluate how the blood moves (circulates) in people who have:
y y y y

Abnormal pressures in the heart arteries Burns Congenital heart disease Heart failure

y y y

Kidney disease Leaky heart valves (valvular regurgitation) Shock

It may also be done to monitor for complications of heart attack and to see how well certain heart medications are working. Swan-Ganz catheterization can also be used to detect abnormal blood flow between two usually unconnected areas. Conditions that can also be diagnosed or evaluated with Swan-Ganz catheterization include:
y y y

Cardiac tamponade Pulmonary hypertension Restrictive cardiomyopathy

Normal Values
y y y y y y

Cardiac index is 2.8 to 4.2 liters per minute per square meter (of body surface area) Pulmonary artery systolic pressure is 17 to 32 millimeters of mercury (mmHg) Pulmonary artery mean pressure is 9 to 19 mmHg Pulmonary diastolic pressure is 4 to 13 mmHg Pulmonary capillary wedge pressure is 4 to 12 mmHg Right atrial pressure is 0 to 7 mmHg

What abnormal results mean

Abnormal results may indicate:
y y y

Circulatory flow problems such as heart failure or shock Heart valve disease Lung disease

What the risks are

Risks of the procedure include:
y y y

Bruising around the area where the catheter was inserted Injury to the vein Puncture to the lung if the neck or chest veins are used, causing lung collapse (pneumothorax)

Very rare complications include:

y

Cardiac arrhythmias

y y y y

Cardiac tamponade Embolism caused by blood clots at the tip of the catheter Infection Low blood pressure

References
1. Faxon DP. Catheterization and angiography. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 56. Review Date: 7/10/2010. Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Michael A. chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Albumin injection

Radioactive albumin injection is part of a nuclear scan test that is performed to measure the supply of blood through the lungs. After the injection, the lungs are scanned to detect the location of the radioactive particles as blood flows through the lungs.

Pulmonary ventilation/perfusion scan

V/Q scan; Ventilation/perfusion scan; Lung ventilation/perfusion scan Last reviewed: September 15, 2010. A pulmonary ventilation/perfusion scan involves two nuclear scan tests. These tests use inhaled and injected radioactive material (radioisotopes) to measure breathing (ventilation) and circulation (perfusion) in all areas of the lungs.

How the test is performed

A pulmonary ventilation/perfusion scan is actually two tests. These tests may be performed separately or together. During the perfusion scan, a health care provider injects radioactive albumin into your vein. You are placed on a movable table that is under the arm of a scanner. The machine scans your lungs as blood flows through them to find the location of the radioactive particles. During the ventilation scan, you breathe in radioactive gas through a mask while you are sitting or lying on a table under the scanner arm.

How to prepare for the test

You do not need to stop eating (fast), eat a special diet, or take any medications before the test. A chest x-ray is usually done before or after a ventilation and perfusion scan. You will sign a consent form and wear a hospital gown or comfortable clothing that does not have metal fasteners.

How the test will feel

The table may feel hard or cold. You may feel a sharp prick while the material is injected into the vein for the perfusion part of the scan. The mask used during the ventilation scan may make you feel nervous about being in a small space (claustrophobia). You must lie still during the scan. The radioisotope injection usually does not cause discomfort.

Why the test is performed

The ventilation scan is used to see how well air and blood flow moves through the lungs. The perfusion scan measures the blood supply through the lungs.

A ventilation and perfusion scan is most often done to detect a pulmonary embolus (blood clot in the lungs). It is also used to:
y y

Detect abnormal circulation (shunts) in the blood vessels of the lungs (pulmonary vessels) Test lung function in people with advanced pulmonary disease, such as COPD

Normal Values
The health care provider should take a ventilation and perfusion scan and then evaluate it with a chest x-ray. All parts of both lungs should take up the radioisotope evenly.

What abnormal results mean

If the lungs take up lower than normal amounts of radioisotope during a ventilation or perfusion scan, it may be due to:
y y y y y y y

Airway obstruction Chronic obstructive pulmonary disease (COPD) Pneumonia Narrowing of the pulmonary artery Pneumonitis Pulmonary embolus Reduced breathing and ventilation ability

What the risks are

Risks are about the same as for x-rays (radiation) and needle pricks. No radiation is released from the scanner. Instead, it detects radiation and converts it into an image. There is a small exposure to radiation from the radioisotope. The radioisotopes used during scans are short-lived. All of the radiation leaves the body in a few days. However, as with any radiation exposure, caution is advised for pregnant or breast-feeding women. There is a slight risk for infection or bleeding at the site where the needle is inserted. The risk with perfusion scan is the same as with inserting an intravenous needle for any other purpose. In rare cases, a person may develop an allergy to the radioisotope. This may include a serious anaphylactic reaction.

Special considerations

A pulmonary ventilation and perfusion scan may be a lower-risk alternative to pulmonary angiography for evaluating disorders of the lung blood supply. This test may not provide an definite diagnosis, especially in people with lung disease. Other tests may be needed to confirm or rule out the findings of a pulmonary ventilation and perfusion scan.

References
1. Tapson VF. Pulmonary embolism. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 99. Review Date: 9/15/2010. Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Pulmonary function tests

PFTs; Spirometry; Spirogram; Lung function tests Last reviewed: October 14, 2009. Pulmonary function tests are a group of tests that measure how well the lungs take in and release air and how well they move gases such as oxygen from the atmosphere into the body's circulation.

How the test is performed

In a spirometry test, you breathe into a mouthpiece that is connected to an instrument called a spirometer. The spirometer records the amount and the rate of air that you breathe in and out over a period of time. For some of the test measurements, you can breathe normally and quietly. Other tests require forced inhalation or exhalation after a deep breath. Lung volume measurement can be done in two ways:
y

The most accurate way is to sit in a sealed, clear box that looks like a telephone booth (body plethysmograph) while breathing in and out into a mouthpiece. Changes in pressure inside the box help determine the lung volume.

Lung volume can also be measured when you breathe nitrogen or helium gas through a tube for a certain period of time. The concentration of the gas in a chamber attached to the tube is measured to estimate the lung volume.

To measure diffusion capacity, you breathe a harmless gas for a very short time, often one breath. The concentration of the gas in the air you breathe out is measured. The difference in the amount of gas inhaled and exhaled measures how effectively gas travels from the lungs into the blood.

How to prepare for the test

Do not eat a heavy meal before the test. Do not smoke for 4 - 6 hours before the test. You'll get specific instructions if you need to stop using bronchodilators or inhaler medications. You may have to breathe in medication before the test.

How the test will feel

Since the test involves some forced breathing and rapid breathing, you may have some temporary shortness of breath or lightheadedness. You breathe through a tight-fitting mouthpiece, and you'll have nose clips.

Why the test is performed

Pulmonary function tests are done to:
y y y

Diagnose certain types of lung disease (especially asthma, bronchitis, and emphysema) Find the cause of shortness of breath Measure whether exposure to contaminants at work affects lung function

It also can be done to:

y y

Assess the effect of medication Measure progress in disease treatment

Spirometry measures airflow. By measuring how much air you exhale, and how quickly, spirometry can evaluate a broad range of lung diseases. Lung volume measures the amount of air in the lungs without forcibly blowing out. Some lung diseases (such as emphysema and chronic bronchitis) can make the lungs contain too much air. Other lung diseases (such as fibrosis of the lungs and asbestosis) make the lungs scarred and smaller so that they contain too little air. Testing the diffusion capacity (also called the DLCO) allows the doctor to estimate how well the lungs move oxygen from the air into the bloodstream.

Normal Values
Normal values are based upon your age, height, ethnicity, and sex. Normal results are expressed as a percentage. A value is usually considered abnormal if it is less than 80% of your predicted value. Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.

What abnormal results mean

Abnormal results usually mean that you may have some chest or lung disease.

What the risks are

The risk is minimal for most people. There is a small risk of collapsed lung in people with a certain type of lung disease. The test should not be given to a person who has experienced a recent heart attack, or who has certain other types of heart disease.

Special considerations
Your cooperation while performing the test is crucial in order to get accurate results. A poor seal around the mouthpiece of the spirometer can give poor results that can't be interpreted. Do not smoke before the test.

References
1. Mason RJ, Broaddus VC, Murray JF, Nadel JA. Murray and Nadel's Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: Saunders; 2005. Review Date: 10/14/2009. Reviewed by: Andrew Schriber, MD, FCCP, Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, New Jersey. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Disclaimer Copyright 2011, A.D.A.M., Inc.

Circulatory system

Blood used by the body is brought back to the heart and lungs by the veins of the body. Once the blood has gathered more oxygen from the lungs, it is pumped back out to the body through the arteries.

Echocardiogram
Transthoracic echocardiogram (TTE); Echocardiogram - transthoracic; Doppler ultrasound of the heart; Surface echo Last reviewed: May 23, 2011. An echocardiogram is a test that uses sound waves to create a moving picture of the heart. The picture is much more detailed than a plain x-ray image and involves no radiation exposure.

How the test is performed

TRANSTHORACIC ECHOCARDIOGRAM (TTE) TTE is the type of echocardiogram that most people will have.
y y

A trained sonographer performs the test, then a heart doctor interprets the results. An instrument called a transducer that releases high-frequency sound waves is placed on your ribs near the breast bone and directed toward the heart. Other images will be taken underneath and slightly to the left of your nipple and in the upper abdomen.

y y

The transducer picks up the echoes of sound waves and transmits them as electrical impulses. The echocardiography machine converts these impulses into moving pictures of the heart. Pictures can be two-dimensional or three-dimensional, depending on the part of the heart being evaluated and the type of machine. A Doppler echocardiogram uses a probe to record the motion of blood through the heart.

An echocardiogram allows doctors to see the heart beating, and to see the heart valves and other structures of the heart. Occasionally, your lungs, ribs, or body tissue may prevent the sound waves and echoes from providing a clear picture of heart function. If so, the sonographer may inject a small amount of liquid (contrast) through an IV to better see the inside of the heart. Very rarely, more invasive testing using special echocardiography probes may be needed. TRANSESOPHAGEAL ECHOCARDIOGRAM (TEE) The back of your throat is numbed and a scope is inserted down your throat. On the end of the scope is a device that sends out sound waves. An experienced technician will guide the scope down to the lower part of the esophagus. It is used to get a clearer echocardiogram of your heart.

How to prepare for the test

There is no special preparation for the test. For a TEE, you will be asked not to eat or drink for several hours before the test.

How the test will feel

You will be asked to remove your clothes from the waist up and lie on an examination table on your back. Electrodes will be placed on your chest to allow for an ECG to be done. A gel will be spread on your chest and then the transducer will be applied. You will feel a slight pressure on your chest from the transducer. You may be asked to breathe in a certain way or to roll over onto your left side.

Why the test is performed

This test is done to evaluate the valves and chambers of the heart in a noninvasive way. The echocardiogram allows doctors to diagnose, evaluate, and monitor:
y y y

Abnormal heart valves Atrial fibrillation Congenital heart disease

y y y y y y y

Damage to the heart muscle in patients who have had heart attacks Heart murmurs Infection in the sac around the heart (pericarditis) Infection on or around the heart valves (infectious endocarditis) Pulmonary hypertension The pumping function of the heart for people with heart failure The source of a blood clot after a stroke or TIA

Your health care provider may recommend a transesophageal echocardiogram (TEE) if:
y y

The regular or transthoracic echocardiogram is unclear because you have a barrel chest, lung disease, or obesity A much clearer picture of a certain area of the heart is needed

Normal Values
A normal echocardiogram reveals normal heart valves and chambers and normal heart wall movement.

What abnormal results mean

An abnormal echocardiogram can mean many things. Some abnormalities are very minor and do not pose significant risks. Other abnormalities are signs of very serious heart disease that will require further evaluation by a specialist. Therefore, it is very important to discuss the results of your echocardiogram in depth with your health care provider.

What the risks are

There are no known risks associated with this test.

Special considerations
Abnormal results may indicate heart valve disease, cardiomyopathy, pericardial effusion, or other heart abnormalities. This test may also be performed for the following conditions:
y y y y y y y y y

Alcoholic cardiomyopathy Aortic dissection Aortic insufficiency Aortic stenosis Arrhythmias Arterial embolism Atrial fibrillation/flutter Atrial myxoma Atrial septal defect

y y y y y y y y y y y y y y y y y y y y y y y y y y y y y

Cardiac tamponade Cardiomyopathy Coarctation of the aorta Heart attack Heart failure Hypertensive heart disease Mitral regurgitation; acute Mitral regurgitation; chronic Mitral stenosis Mitral valve prolapse Patent ductus arteriosus Patent foramen ovale Pericarditis; bacterial Pericarditis; constrictive Pericarditis; post-MI Peripartum cardiomyopathy Primary amyloidosis Pulmonary arterial hypertension Pulmonary valve stenosis Restrictive cardiomyopathy Right-sided heart failure Secondary systemic amyloidosis Senile cardiac amyloidosis Stroke Tetralogy of Fallot Transient ischemic attack (TIA) Transposition of the great vessels Tricuspid regurgitation Ventricular septal defect

References
1. Connolly HM, Oh JK. Echocardiography. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier;2011: chap 15. Review Date: 5/23/2011. Reviewed by: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; and Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Chronically low blood levels of oxygen may lead to pulmonary hypertension (high blood pressure in the lungs), and possibly to cor pulmonale. Cor pulmonale is also called right-sided heart failure, and is characterized by enlargement of the right ventricle. Treatment targets the underlying illness and may include supplemental oxygen, a low-salt diet or calcium channel blockers. Read more: http://www.umm.edu/imagepages/18131.htm#ixzz1k81uoz4v

http://www.e-radiography.net/radpath/c/cor_pulmonale.htm Definition Cor pulmona is characterized by hypertrophy of the right ventricle resulting from disease of the lungs, except for lung changes in diseases that primarily affect the left side of the heart and excluding

congenital heart disease; acute cor pulmonale is characterized by dilation and failure of the right side of the heart due to pulmonary embolism. In both types, characteristic electrocardiogram changes occur, and in later stages there is usually right-sided cardiac failure. Radiographic Appearance The chest x-ray may reveal right ventricular hypertrophy, manifest as filling of the retrocardiac space on the lateral film, and increased PA size. Image 2 Pathology Enlargement of the right ventricle occurs from pulmonary hypertension, elevated blood pressure in the lungs. Pulmonary hypertension is usually caused by lung disease (most commonly chronic bronchitis or emphysema). Any disease that causes chronically low blood levels of oxygen may lead to pulmonary hypertension and cor pulmonale. This condition does not include right ventricle enlargement that results from congestive heart failure or heart valve disease. Cor pulmonale is usually chronic (long-term), but it may be acute and reversible. Treatment: Diuretics to prevent fluid accumulation Digitalis to strengthen the force of heart-muscle contractions Antibiotics for recurrent infections Vasodilators to reduce the resistance of the blood vessels to promote improved blood flow Image 1

- hipocratism digital (deformarea extremitatilor degetelor, bombare si aplatizare)

ordul pulmonar cronic este urmarea repercusiunilor pe care le au asupra ventriculului drept unele boli pulmonare. In mod normal ventriculul drept trimite continutul sau de sange in artera pulmonara de unde ajunge in alveolele pulmonare, unde este eliminat bioxidul de carbon si are loc oxigenarea. Cordul pulmonar cronic (CPC) este definit ca hipertrofia ventriculului drept (HVD) consecutiva unei boli care afecteaza functia si/ sau structura plamanilor, exceptand situatiile cand acestea sunt urmarea bolilor cordului stang si cardiopatiilor congenitale. Ca frecventa, cordul pulmonar cronic se intalneste din ce in ce mai des, ajungand la 10-15% din bolile inimii, dupa bolile valvulare, cardiopatiile ischemice, hipertensiunea arteriala. Este mai des intalnit la barbati si apare de obicei intre 50 si 70 de ani.

Care sunt cauzele cordului pulmonar cronic?

Cauza cea mai frecventa a cordului pulmonar cronic sunt boli ale parenchimului pulmonar si ale cailor respiratorii intratoracice. Dintre acestea cele mai importante sunt boala obstructiva pulmonara cronica (BPOC), care impreuna cu astmul infectios cronic si bronsiectaziile difuze insumeaza aproximativ 70% din cauzele de CPC. CPC poate apare si in numeroase alte afectiuni precum: - boli ale parenchimului pulmonar si ale cailor aeriene intratoracice - afectari ale cutiei toracice - boli neuromusculare - tulburari ale centrului de control respirator - boala pulmonara vasculara ocluziva Toate acestea pot conduce la CPC daca evolueaza cronic si interfera cu functia sau/si structura plamanului - elemente care conditioneaza o alterare semnificativa a patului vascular pulmonar, cu hipertensiune pulmonara (HTP) persistenta si, cel mai adesea, progresiva.

Care sunt simptomele cordului pulmonar cronic?

Simptomele cordului pulmonar cronic sunt cel mai adesea dominate de boala pulmonara sau extrapulmonara cauzatoare. In aproape 70% din cazuri, tabloul clinic este de BPOC tip B cu predominanta bronsitei cronice in variante severe, de tip recurent purulent sau obstructiv (aspect "blue bloater"- "albastru buhait").

Bolnavii - de regula fumatori - au un istoric de tusitori cronici, cu sputa mucoasa sau mucopurulenta in cantitate variabila, survenind mai ales in anotimurile umede sau reci sau dupa infectii bacteriene. Frecvent, in perioadele de acutizare, apar accese de bronhospasm, cu dispnee expiratorie (dificultate in respiratie) si wheezing (respiratie zgomotoasa, suieratoare). Alte simptome clinice necaracteristice ale cordului pulmonar cronic apar ca urmare a hipertensiunii pulmonare la valori presionale foarte mari: - dispnee de efort (dificultate in respiratie) - sincopa de efort (pierderea cunostintei) - dureri precordiale

Care este diagnosticul cordului pulmonar cronic?

Investigatiile paraclinice necesare stabilirii diagnosticului pozitiv de cord pulmonar cronic includ: - explorari conventionale (radiografia toracica standard, electrocardiograma, probe ventilatorii) - explorari ale structurii si functiei VD (ecografie, scintigrafie) - explorari ale structurii si functiei circulatiei pulmonare (scintigrafie pulmonara, biopsie pulmonara) Diagnosticul de CPC este relativ usor de formulat in prezenta istoricului de boala pulmonara si a existentei simptomelor si semnelor pulmonare si cardiace relativ caracteristice, in special daca electrocardiograma sau/si examenul radiologic toracic arata semne de hipertrofie-dilatatie de cord drept si eventual artere pulmonare largi in hil.

Care este tratamentul cordului pulmonar cronic?

Tratamentul cordului pulmonar cronic urmareste combaterea bronsiolitei obstructive si a infectiilor bronsice prin utilizarea de bronhodilatatoare (bronhodilatin, eufilin), tratarea si prevenirea infectiilor bronsice dupa cercetarea antibiogramei, repaus intermitent, evitarea frigului sau a aerului rece. Ameliorarile obtinute cu diverse medicatii trebuie ajutate si de bolnav, care va cauta sa evite eforturi inutile, sa evite pranzurile abundente si va incerca sa faca zilnic, sistematic, gimnastica respiratorie. Respectand aceste norme va evita aparitia insuficientei ventriculare drepte, care este destul de rebela la preparatele digitalice. Profilaxia cordului pulmonar cronic consta in evitarea bronsitelor repetate la un bolnav emfizematos, evitarea prafului, a fumatului si a inhalarii aerului rece. In cazul unei persoane obeze se va urmari scaderea in greutate. Cordul pulmonar cronic (CPC) este definit clasic, ca hipertrofia si dilatatia ventriculului drept (HVD - hipertrofie de ventricul drept), consecutiva unor boli care afecteaza functia si/sau structura plamanilor, exceptand situatiile cand acestea sunt urmarea bolilor cordului stang sau cardiopatiilor congenitale.

HVD reprezinta elementul central de definire si diagnostic al CPC. CPC eventual se poate insoti de insuficienta cardiaca dreapta.

Cauze, clasificare

CPC apare in numeroase afectiuni ale parenchimului pulmonar si ale cailor aeriene intratoracice, in afectari ale cutiei toracice, in boli neuromusculare, in tulburari ale centrului de control respirator si in boala pulmonara vasculara ocluziva; toate acestea pot conduce la CPC daca evolueaza cronic si interfera cu functia sau/si structura plamanului - elemente care conditioneaza o alterare semnificativa a patului vascular pulmonar, cu hipertensiune pulmonara (HTP) persistenta sicel mai adesea progresiva.

1. Etiologia cea mai frecventa a CPC este reprezentata de boli ale parenchimului pulmonar si ale cailor respiratorii intratoracice. Dintre acestea se detaseaza boala obstructiva pulmonara cronica (BPOC), care impreuna cu astmul infectios cronic si bronsiectaziile difuze insumeaza aprox. 70% din cauzele de CPC.

Tendinta BPOC de a se transforma in CPC este cu atat mai mare cu cat componenta de bronsita cronica (tipul B de boala) este mai importanta. Pe de alta parte numai aprox. 20% din bolnavii cu BPOC dezvolta CPC, restul ramanand fara tulburari hemodinamice semnificative.

2. Bolile intersittiale fibrozante si bolile granulomatoase, mai frecvente si mai bine diagnosticate in prezent , insumeaza aprox. 15% din cauzele de CPC.

3. Un grup heterogen este reprezentat de afectarile cutiei toracice, boli neuromusculare si tulburarile centrului de control al respiratiei, care toate au in comun hipoventilatia alveolara cu plaman normal. Instalarea CPC se face progresiv, fara posibilitatea reversibilitatii sale. Din aceasta grupa, trebuie remarcate CPC la persoanele cu obezitate imoprtanta, la cei cu obezitate si tulburari de sensibilitate a centrului respirator (sindrom Pickwick), precum si in sindromul de apnee in somn. La aceste persoane reducerea ponderala poate constitui un mijloc de reducere sau stabilizare a tulburarilor hemodinamice.

4. Grupul de boli care produc ocluzia difuza a patului vascular pulmonar, cu HTP consecutiva, reprezinta aprox. 2% din cazurile de CPC; ele realizeaza o HTP severa, cu evolutie invariabil accelerata catre insuficienta cardiaca dreapta.

Obstructia patului vascular pulmonar seinttlneste in HTP primitiva, tromboembolismul pulmonar cronic sau invasculite pulmonare.

Sub aspect hemodinamic, CPC poate fi clasificat in CPC compensat si decompensat. In stadiul de CPC compensat exista HTP variabila de repaus si care se amplifica la efort, HVD, dar presiunea telediastolica in ventriculul drept, presiunea in atriul drept si fractia de ejectie sunt normale.

In CPC decompensat sunt prezente semnele de insuficienta cardiaca dreapta, elementele definitorii privesc HTP de repaus, de diverse grade, presiunea telediasolica in ventriculul drept si atriul drept crescute, fractia de ejectie scazuta.

Un bolnav poate prezenta in cursul evolutiei trecerea dintr-o categorie hemodinamica in altele, dupa cum evolueaza boala de fond, cu agravari si ameliorari temporare.

Diagnostic

Diagnosticul pozitv al CPC este relativ usor de formulat in prezenta istoricului de boala pulmonara si a existentei simptomelor si semnelor pulmonare si cardiace relativ caracteristice, daca electrocardiograma sau/si examenul radiologic toracic arata semne de hipertrofie-dilatatie de cord drept si eventual artere pulmonare largi in hil.

xplorarile paraclinice
Investigatiile paraclinice sunt necesare pentru stabilirea diagnosticului pozitiv, etiologic si functional al CPC. Ele pot fi grupate in: I. Explorari conventionale si II. Explorari speciale ale structurii si functiei circulatiei pulmonare.

I. Explorari conventionale reprezinta metode comune, usor accesibile, aproape toate obligatoriu de efectuat, atunci cand exista suspiciunea prezentei CPC.

1. Radiografia toracica standard poate arata modificari ale cordului, vaselor pulmonare si parenchimului pulmonar.

2. Electrocardiograma reprezinta metoda de explorare cea mai importanta prin specificitatea ei privind semnele de hipertrofie ventriculara dreapta si hipertrofie atriala dreapta. Atunci cand criteriile electrocardiografice sunt prezente, diagnosticul de CPC poate fi considerat precizat (specificitatea este de aproape 100%)

3. Ecocardiografia a devenit, odata cu cresterea accesibilitatii, metoda de explorare care furnizeaza neinvaziv numerose date morfologice si functionale in CPC.

4. Explorarea ventilatorie si dozarea gazelor sanguine furnizeaza date directe asupra tipului de disfunctie ventilatorie si de insuficienta respiratorie si date aproximative asupra prezentei HTP si a consecintelor ei. Aceste tipuri de explorari sunt in special utile in BPOC, si partial in bolile pulmonare restrictive.

II. Alte explorari.

Limitele metodelor de explorare conventionala a HVD si a CPC in etapele lor incipiente au condus la introducerea unor metode de explorare mai sensibile dar mai complexe, care sa deceleze modificari de functie si de structura a ventriculului drept si circulatiei pulmonare.

5. Scintigrafia miocradica cu Ta 201 este folosita pentru studiul HVD deoarece s-a demonstrat ca ventriculul drpet nu se vizualizeaza scintigrafic decat in conditii de hipertrofie.

6. Ventriculografia izotopica cmputerizata aduce informatii precise asupra functiei ventriculului drept.

7. Cateterismul arterei pulmonare si angiografia pulmonara de contrast raman indicatii de exceptie, atunci cand se suspecteaza prezenta unei TEP complicat cu HTP si CPC. Cateterismul arterei pulmonare furnizeaza datele cele mai precise hemodinamice.

8. Biopsia pulmonara prin toracotomie minima reprezinta o indicatie de exceptie, numai pentru elucidarea unoretiologii rare ale CPC (HTP primitva, boli interstitiale rare, ca de ex. Histiocitoza X sau hemangiomatoza). Metoda comporta riscuri serioase la bolnavii cu HTP severa si CPC. Ea poate fi utila inainte de transplantul pulmonar.

ratament
Tratamentul CPC trebuie diferentiat in functie de tipul etiopatogenic si de principalul mecanism generator al HTP.

Obiectivele tratamentului sunt: 1) reducerea sarcinii ventriculului drept prin scaderea valorilor HTP; 2) tratamentul insuficientei cardiace drepte acolo unde ea s-a instalat.

Mijloacele terapeutice prin care se pot realiza aceste obiective includ: 1. tratarea bolii de baza pulmonare sau extrapulmonare, generatoare a HTP; 2. oxigenoterapia; 3. utilizarea medicatiei vasodilatatoare pulmonare pentru reducerea HTP; 4. diuretice, in caz de retentie hisdrosalina; 5. digitala in unele forme de CPC decompensat; 6. sangerari repetate, la bolnavii cu policitemie; 7. evaluarea oportunitatii unei interventii chirurgicale corective a bolii de fond.

Tratarea bolii de baza are in vedere mai ales acele entitati etiologice care au un coeficient de reversibilitate. Deoarece 70% dintre bolnavii cu CPC recunosc drept etiologie BPOC, tratarea corecta a acesteia reprezinta un mod de a realiza profilaxia CPC, iar la cei cu CPC deja instalat de a impiedica agravarea sa. In tratamentul BPOC se urmareste: a. combaterea episoadelor de acutizare virobacteriana, realizata cu antibiotice; b. combaterea disfunctiei ventilatorii obstructive prin bronhodilatatoare betamimetice, anticolinergice si teofilina; c. combaterea sindromului obstructiv bronsic de natura inflamatorie prin corticoterapie; d. fluidificarea secretiilor.

Antibioticele de electie sunt tetraciclina, ampicilina sau amoxicilina si cotrimoxazolul. Durata curelor este de 7-10 zile; nu sunt justificate de obicei cure de mai lunga durata. Celelalte etiologii ale CPC parenchimatos prezinta coeficienti de reversibilitate net mai mici sub orice terapie (fibroze difuze, colagenoze, pneumoconioze).

Oxigenoterapia poate fi indicata fie in administrare de scurta durata, in timpul agaravarilor CPC, fie sub forma de terapie cronica la domiciliu. Oxigenoterapia cronica la domiciliu se poate efectua fie prin concentroare, fie prin mici rezervoare portabile de oxigen lichid. Este dovedit ca numai utilizarea continua (14-16 ore pe zi) are efecte semnificative favorabile, in timp ce administrarea intermitenta nu confera avantaje demonstrabile.

Vasodilatatoarele Tratamentul cronic cu PG12 este indicat bolnavilor c HTP primitiva sau alte etiologii severe de CPC, aflati pe lista de asteptare in vederea transplantului pulmonar.

Diureticele Sunt recomandate in tratarea retentei hidrosaline din cadrul CPC deompensat. Ele reduc expansiunea volemica si edemul periferic. Se utilizeaza diuretice de ansa (furosemid sau acid etacrinic) si antialdosteronice (spironolactona) sau alte diuretice care economisesc potasiul (de tipul amiloridului).

Digitala reprezinta a medicatie controversata in tratamentul CPC. Cu toate controversele, digitalizarea este recomandabila in: CPC cu tahiaritmii supraventriculare; CPC asociat cu disfunctie sistolica a ventriclului staang (ischemica sa hipertensiva); CPC cu insuficienta cardiaca dreapta la care se presupune un debit cardiac acazut (semne importante de HVD, hipotensiune).

Sangerarea este indicata la bolnavii cu poliglobulie secundara marcata, consecinta a hipoxemiei cronice. Sangerarile se pot repeta in ambulator, la un interval de 4+6 saptamani.

Interventiile chirurgicale pot fi corective a bolii de fond si in cazul bolilor severe, ireversibile, se poate tenta transplantul pulmonar. Interventiile corective se executa in cazul malformatiilor toracale (mai ales de coloana vertebrala), sau in sindromul de apnee in somn de cauza periferica, in care corectarea structurii osoase a fetei poate duce la o ameliorare marcata a bolii cauzatoare de HTP si CPC. Transplantul pulmonar este rezervat numai bolilor grave, avansate si ireversibile.

Tablou clinic

In tabloul clinic al CPC se regasesc simptome si semne intricate ale: a. Bolii pulmonare (mai rar extrapulmonare) generatoare de HTP; b. Sindromul de HTP; c. HVD, cu sau fara semne de insuficienta cardiaca dreapta.

A. Boala pulmonara sau extrapulmonara care produce CPC se afla, de obicei, pe primul plan al tabloului clinic, aproape in toate etapele de evolutie a CPC.

In aproape 70% din cazuri, tabloul clinic este de BPOC tip B. Bolnavii - de regula fumatori - au un istoric de tusitori cronic, cu sputa mucoasa sau mucopurulenta in cantitate variabila, survenind mai ales in anotimurile umede sau reci sau dupa infectii virobacteriene.

Frecvent, in perioadele de acutizare, apar accese de bronhospasm, cu dispnee expiratorie si wheezing. Dispneea, initial intermitenta, in perioadele de acutizari sau bronhospasm, tinde sa apara la eforturi mici sau chiar in repaus. Dupa ani de evolutie, cu agravari repetate ale simptomatologiei, se instaleaza insuficienta respiratorie, cu cianoza severa, extermitati calde si eventual encefalopatie hipercapnica.

B. Sindromul de HTP are simptome clinice necaracteristice sau aparind numai la valori presionale foarte mari. Dintre acestea asociatia: dispneea de efort, sincopa de efort si uneori durerile precordiale, poate sugerea prezensa de HTP; dispneea insa este o manifestare comuna a bolilor pulmonare si extrapulmonare aflate pe primul plan clinic.

C. HVD, urmare a HTP, si insuficienta cardiaca dreapta reprezinta elementele cele mai caracteristice din punct de vedere clinic, al instalarii CPC. Insuficienta cardiaca dreapta apare in fazelerelativ tardive ale evolutiei HTP si CPC si are de obicei drept factori precipitanti infectiile respiratorii, tromboembolismul pulmonar sau aritmiile cardiace.

Semnele de insuficienta respiratorie coexista - cel mai adesea - cu cele de decompensare cardiaca si foarte frecvent, evolueaza paralel.

Evolutie, complicatii, prognostic

Evolutia CPC este direct dependenta de boala de baza care a detrminat HTP, de gradul HTP, de elementele de revrsibilitate ale afectiunilor pulmonare si ale HTP, de insuficienta respiratorie coexistenta.

In cazul BPOC , evolutia depinde de tipul de BPOC (A sau B), de sevritatea obstructiei respiratorii, de ritmul si gravitatea acutizarilor virobacteriene si, in fianl, de gradul hipoxiei alveolare pe care o realizeaza.

In alte afectiuni pulmonare, cu ritm de progresie a bolii mai lent saau cu factori de reversibilitate limitati, ca de exemplu emfizemul pulmonar difuz sever, fibroze pulmonare primitive sau in pneumoconioze, evolutia spre CPC se face mai lent; in schimb insuficienta cardiaca dreapta odata instalata este greu tratabila, intrucat nu mai sunt prezenti factori de reversibilitate a HTP.

Dupa prima decompensare a cordului drept, mortalitatea in CPC secundar BPOC este de aprox. 40% in urmatorii 5 ani, dar oxigenoterapia cronica are efect pozitiv asupra HTP si reduce mortalitatea in mod semnificativ. Perioadele de decompensare reprezinta perioade de complicatii severe: aritmii, tromboembolism pulmonar, hemoragii digestive superioare, insuficienta renala functionala.