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DEFINITION

is a condition that causes the right side of the heart to fail. Long-term high
blood pressure in the arteries of the lung and right ventricle of the heart can lead
to cor pulmonale.
Description
This term describes impairment in right ventricular function as a result of
respiratory disease leading to increased resistance to blood flow in the
pulmonary circulation.
The structure and function of the right ventricle is adversely affected by
pulmonary arterial hypertension, induced by a disease process affecting the
lungs, their ventilation or blood supply. For cor pulmonale to come about, mean
pulmonary arterial pressure is usually >20 mm Hg. Complete right ventricular
failure usually ensues if mean pulmonary arterial pressure is ≥40 mm Hg. It is
thought that chronic hypoxia leads to pulmonary arteriolar constriction through
excessive action of the physiological mechanism that acts to maintain the
balance of ventilation and perfusion in the lungs.
Other mechanisms that may raise mean pulmonary arterial pressure in
cases of cor pulmonale include:
 Chronic hypercapnoea and respiratory acidosis causing pulmonary
vasoconstriction.
 Anatomic disruption of the pulmonary vascular bed due to primary
lung
disease (for example,
in emphysema,
pulmonary
thromboembolic disease and pulmonary fibrosis).
 Increased blood viscosity due to lung disease and its effects (for
example, in secondary polycythaemia).
A wide range of pulmonary and cardiopulmonary disease processes may
cause the condition. It is usually a chronic and progressive process, but does
occur acutely due to sudden causes of pulmonary hypertension, usually following
pulmonary embolism.
If right-heart failure occurs due to primary disease of the left side of the
heart, or because of a congenital cardiac lesion then it is not normally considered
to be cor pulmonale.

and immune and other functional cells that help provide for homeostasis and basic functions of human cells and organs. The heart sounds transmitted are due to closing of heart valves. and veins transport the blood back to the heart. A normal heart rate is approximately 72 beats/minute. The blood contains oxygen. nutrients. and the cardiac cycle spreads over 0. and right-sided chambers are divided by the tricuspid valve. gases. Cardiac output (CO) is the amount of blood pumped out by each ventricle in one minute.[1. The normal adult blood volume is 5 liters (a little over 1 gallon) and it usually passes through the heart once a minute. called murmurs.8 seconds. The chambers of each side are separated by an atrioventricular valve (A-V valve). which is an anatomical pump. The left-sided chambers are separated by the mitral (bicuspid) valve. wastes. with its intricate conduits (arteries. and the right side chambers supply the pulmonary circulation. Blood flows through the heart in only one direction enforced by a valvular system that regulates opening and closure of valves based on pressure gradients (see image below). and abnormal heart sounds. The heart contains 4 chambers that essentially make up 2 sides of 2 chamber (atrium and ventricle) circuits. etc. wastes. the left side chambers supply the systemic circulation.ANATOMY AND PHYSIOLOGY Human Cardiovascular System The cardiovascular system consists of the heart. . usually represent valve incompetency or abnormalities.[4] Blood is transported through the whole body by a continuum of blood vessels.[3] The cardiac cycle refers to events that occur during one heart beat and is split into ventricular systole (contraction/ejection phase) and diastole (relaxation/filling phase). 2] The pumping action of the heart usually maintains a balance between cardiac output and venous return.[5] Heart The heart is a muscular organ weighing between 250-350 grams located obliquely in the mediastinum. Capillaries carry blood to tissue cells and are the exchange sites of nutrients. Arteries are blood vessels that transport blood away from the heart. Note that cardiac output varies with the demands of the body. veins. It functions as a pump supplying blood to the body and accepting it in return for transmission to the pulmonary circuit for gas exchange. and capillaries) that traverse the whole human body carrying blood.

The myocardium behaves as a functional syncytium because of electrical coupling action provided by gap junctions. . the molecule that provides energy for cellular function (see the images below). uninucleate (single nucleus) cells that contain myofibrils. Cardiac muscle cells. Adjacent cardiac cells are connected by intercalated discs containing desmosomes and gap junctions. Cardiac muscle has abundant mitochondria that depend on aerobic respiration primarily to generate adenosine tri-phosphate (ATP).Unique properties of cardiac muscle Cardiac muscle cells are branching striated.

Myocardial gap junctions. Systemic and pulmonary circulation. . oxygen rich blood is pumped to all organs of the human body through the aortic semilunar valve (see the image below). From the left ventricle. Systemic Circulation The systemic circuit originates in the left side of the heart and functions by receiving oxygen-laden blood into the left atrium from the lungs and flows one way down into the left ventricle via the mitral valve.

leading to the pulmonary trunk that takes the oxygen deprived blood to the lungs for gas exchange. Oxygen-poor systemic blood reaches the right atrium via 3 major venous structures: the superior vena cava. Venous blood collected by the cardiac veins (great. Once gas exchange occurs in the lung tissue. Delivery of oxygen-rich blood to the myocardial tissue occurs during the heart relaxation phase (see the image below). The right and left coronary arteries branch from the ascending aorta and. This blood is pumped down to the right ventricle via the tricuspid valve and eventually through the pulmonic valve. marginal and circumflex arteries). small.Pulmonary Circulation The pulmonary circuit is on the right side of the heart and serves the function of gas exchange. Coronary Circulation Coronary circulation is the circulation to the heart organ itself. Coronary circulation. middle. and coronary sinus. supply the heart muscle (myocardial) tissue. . inferior vena cava. through their branches (anterior and posterior interventricular. and anterior) flows into the coronary sinus. the oxygenladen blood is carried to the left atrium via the pulmonary veins. hence completing the pulmonary circuit (see the image above).

ion. known as arterioles. Capillary structure. Arterial cross-section. smaller distributing arteries. ion. They have fenestrations that allow for and enhance permeability for exchange of gas. They are subdivided into larger conducting arteries. that supply the capillary bed (the site of active tissue cells gas exchange). nutrient. Capillaries are vessels that are microscopic in size and provide a site of gas. . and cellular elements (see the image below). and cellular exchange between blood and interstitial fluid. nutrient. and the smallest arteries.Vessel Anatomy An artery is a blood vessel that carries blood away from the heart to peripheral organs (see the image below).

In ARDS. containing valves that prevent backflow. especially higher tidal volume. Mechanical ventilation.[2] .A vein is a blood vessel that has a larger lumen. Veins: blood flow and valve structure. 2 factors cause right ventricular (RV) overload: the pathologic features of the syndrome itself and mechanical ventilation. The underlying pathophysiology in massive pulmonary embolism causing cor pulmonale is the sudden increase in pulmonary resistance. right ventricular dilatation mainly occurs. PATHOPHYSIOLOGY Cor pulmonale usually presents chronically. requires a higher transpulmonary pressure. cor pulmonale is associated with increased possibility of right-to-left shunt through the patent foramen ovale and carries a poorer prognosis. In the case of ARDS. In acute cor pulmonale. This system of vessels in general returns blood to the heart from the periphery (see the image below). and sometimes veins serve as blood reservoirs or capacitance vessels. In chronic cor pulmonale. but 2 main conditions can cause acute cor pulmonale: pulmonary embolism (more common) and acute respiratory distress syndrome (ARDS). RV hypertrophy (RVH) generally predominates.

RV and LV output The RV is a thin-walled chamber that is more a volume pump than a pressure pump. adult respiratory distress syndrome. subsequently. to cor pulmonale. interstitial lung disease. This . emphysema. originates from the aorta. These pathogenetic mechanisms include the following:  Pulmonary vasoconstriction due to alveolar hypoxia or blood acidemia – This can result in pulmonary hypertension and if the hypertension is severe enough. pulmonary thromboembolism. which supplies the RV free wall. decreased LV output diminishes blood pressure in the aorta and decreases right coronary blood flow. and rheumatoid disorders) – These conditions can cause elevated pulmonary blood pressure. macroglobulinemia)  Increased blood flow in pulmonary vasculature  Idiopathic primary pulmonary hypertension The result of the above mechanisms is increased pulmonary arterial pressure. a further increase in the degree of pulmonary arterial pressure produces significant RV dilatation. It adapts better to changing preloads than afterloads. sickle cell disease. it causes cor pulmonale.  Anatomic compromise of the pulmonary vascular bed secondary to parenchymal or alveolar lung disorders (eg. With an increase in afterload.Several different pathophysiologic mechanisms can lead to pulmonary hypertension and. What ensues is a vicious cycle between decreases in LV and RV output. polycythemia vera. Because the right coronary artery. and RV circulatory collapse. At a point. A decrease in RV output with a decrease in diastolic left ventricle (LV) volume results in decreased LV output. RV and LV morphogenesis Genetic investigations have confirmed that morphogenesis of the right and left ventricle originated from different sets of progenitor cells and sites. Chronic obstructive pulmonary disorder is the most common cause of cor pulmonale. the RV increases systolic pressure to keep the gradient. an increase in RV end-diastolic pressure. and some connective tissue disorders with pulmonary involvement may result in pulmonary hypertension and cor pulmonale.  Increased blood viscosity secondary to blood disorders (eg.

it puts a strain on the right side of the heart. symptoms occur with lighter activity or even while you are at rest. changes in the small blood vessels inside the lungs can lead to increased blood pressure in the right side of the heart. can be another factor that decreases LV volume and output in the setting of cor pulmonale and RV enlargement. Septal displacement. usually in the front of the chest  Chest pain . Causes High blood pressure in the arteries of the lungs is called pulmonary hypertension. That strain can cause cor pulmonale. Some symptoms you may have are:  Fainting spells during activity  Chest discomfort. It is the most common cause of cor pulmonale. Chronic lung conditions that cause low blood oxygen levels in the blood over a long time can also lead to cor pulmonale. In people who have pulmonary hypertension. You may also have a fast heartbeat and feel like your heart is pounding.[3] RV overload Right ventricular overload is associated with septal displacement toward the left ventricle.polymorphism could explain the differing rates of hypertrophy of the right and left ventricles. A few of these are:  Chronic obstructive pulmonary disease (COPD)  Chronic blood clots in the lungs  Cystic fibrosis (CF)  Scarring of the lung tissue (interstitial lung disease)  Severe curving of the upper part of the spine (kyphoscoliosis)  Obstructive sleep apnea. This makes it harder for the heart to pump blood to the lungs. which is seen on echocardiography. If this high pressure continues. Over time. which causes stops in breathing because of airway inflammation Symptoms Shortness of breath or light-headedness during activity is often the first symptom of cor pulmonale.

you may take them by mouth (oral). In general.   Swelling of the feet or ankles Symptoms of lung disorders. . Many treatment options are available. such as wheezing or coughing Lips and fingers that turn blue (cyanosis) Exams and Tests Your health care provider will perform a physical exam. Your doctor will decide which medicine is best for you. It is important to treat medical problems that cause pulmonary hypertension. the cause of your cor pulmonale will determine which treatment you receive. receive them through a tube that goes into a vein (intravenous or IV). Never stop taking your medicines without first talking to your doctor. because they can lead to cor pulmonale. This may show:  Fluid buildup in your belly  Abnormal heart sounds  Bluish skin  Liver swelling  Swelling of the neck veins. If your doctor prescribes medicines. or breathe them in (inhaled). You will be closely monitored during treatment to watch for side effects and to see how well the medicine works for you. which is a sign of high pressure in the right side of the heart  Ankle swelling These tests may help diagnose cor pulmonale:  Blood antibody tests  Blood test to check for a substance called brain natriuretic peptide (BNP)  Chest x(rarely done)  Ventilation and perfusion scan of the lungs (V/Q scan) Treatment The goal of treatment is to control symptoms.

you will need to make changes to your home so that you can manage as well as possible. Prevention Avoid cigarette smoking to help prevent lung disease. Alternative Names Right-sided heart failure . You will also need help around your house. Outlook (Prognosis) How well you do depends on the cause of your cor pulmonale. Possible Complications Cor pulmonale may lead to:  Life-threatening shortness of breath  Severe fluid buildup in your body  When to Contact a Medical Professional Call your doctor or nurse if you have shortness of breath or chest pain.  If you smoke. if medicine does not work Important tips to follow:  Avoid strenuous activities and heavy lifting. such as the pneumonia vaccine.  Use oxygen if your doctor prescribes it. stop. because lung disease can lead to cor pulmonale. As your illness gets worse.  Get a yearly flu vaccine.  Avoid traveling to high altitudes.  Do not get pregnant (women).Other treatments may include:  Blood thinners to reduce the risk of blood clots  Oxygen therapy at home  A lung or heart-lung transplant. as well as other vaccines.