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.

ANATOMY AND PHYSIOLOGY

Human Cardiovascular System
The cardiovascular system consists of the heart, which is an anatomical
pump, with its intricate conduits (arteries, veins, and capillaries) that traverse the
whole human body carrying blood. The blood contains oxygen, nutrients, wastes,
and immune and other functional cells that help provide for homeostasis and
basic functions of human cells and organs.[1, 2]
The pumping action of the heart usually maintains a balance between
cardiac output and venous return. Cardiac output (CO) is the amount of blood
pumped out by each ventricle in one minute. The normal adult blood volume is 5
liters (a little over 1 gallon) and it usually passes through the heart once a minute.
Note that cardiac output varies with the demands of the body.[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). A normal heart rate is approximately 72 beats/minute,
and the cardiac cycle spreads over 0.8 seconds. The heart sounds transmitted
are due to closing of heart valves, and abnormal heart sounds, called murmurs,
usually represent valve incompetency or abnormalities.[4]
Blood is transported through the whole body by a continuum of blood
vessels. Arteries are blood vessels that transport blood away from the heart, and
veins transport the blood back to the heart. Capillaries carry blood to tissue cells
and are the exchange sites of nutrients, gases, wastes, etc.[5]
Heart
The heart is a muscular organ weighing between 250-350 grams located
obliquely in the mediastinum. It functions as a pump supplying blood to the body
and accepting it in return for transmission to the pulmonary circuit for gas
exchange.
The heart contains 4 chambers that essentially make up 2 sides of 2
chamber (atrium and ventricle) circuits; the left side chambers supply the
systemic circulation, and the right side chambers supply the pulmonary
circulation. The chambers of each side are separated by an atrioventricular valve
(A-V valve). The left-sided chambers are separated by the mitral (bicuspid) valve,
and right-sided chambers are divided by the tricuspid valve. 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).

Unique properties of cardiac muscle

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

Cardiac muscle cells.

Myocardial gap junctions.

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. From the left ventricle,
oxygen rich blood is pumped to all organs of the human body through the aortic
semilunar valve (see the image below).

Systemic and pulmonary circulation.

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

Coronary circulation.

Vessel Anatomy
An artery is a blood vessel that carries blood away from the heart to
peripheral organs (see the image below). They are subdivided into larger
conducting arteries, smaller distributing arteries, and the smallest arteries, known
as arterioles, that supply the capillary bed (the site of active tissue cells gas
exchange).

Arterial cross-section.
Capillaries are vessels that are microscopic in size and provide a site of
gas, ion, nutrient, and cellular exchange between blood and interstitial fluid. They
have fenestrations that allow for and enhance permeability for exchange of gas,
ion, nutrient, and cellular elements (see the image below).

Capillary structure.

A vein is a blood vessel that has a larger lumen, and sometimes veins
serve as blood reservoirs or capacitance vessels, containing valves that prevent
backflow. This system of vessels in general returns blood to the heart from the
periphery (see the image below).

Veins: blood flow and valve structure.

PATHOPHYSIOLOGY
Cor pulmonale usually presents chronically, but 2 main conditions can
cause acute cor pulmonale: pulmonary embolism (more common) and acute
respiratory distress syndrome (ARDS). The underlying pathophysiology in
massive pulmonary embolism causing cor pulmonale is the sudden increase in
pulmonary resistance. In ARDS, 2 factors cause right ventricular (RV) overload:
the pathologic features of the syndrome itself and mechanical ventilation.
Mechanical ventilation, especially higher tidal volume, requires a higher
transpulmonary pressure.
In chronic cor pulmonale, RV hypertrophy (RVH) generally predominates.
In acute cor pulmonale, right ventricular dilatation mainly occurs. In the case of
ARDS, cor pulmonale is associated with increased possibility of right-to-left shunt
through the patent foramen ovale and carries a poorer prognosis.[2]

Several different pathophysiologic mechanisms can lead to pulmonary

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

polymorphism could explain the differing rates of hypertrophy of the right and left
ventricles.[3]
RV overload
Right ventricular overload is associated with septal displacement toward
the left ventricle. Septal displacement, which is seen on echocardiography, can
be another factor that decreases LV volume and output in the setting of cor
pulmonale and RV enlargement.
Causes
High blood pressure in the arteries of the lungs is called pulmonary
hypertension. It is the most common cause of cor pulmonale.
In people who have pulmonary hypertension, changes in the small blood
vessels inside the lungs can lead to increased blood pressure in the right side of
the heart. This makes it harder for the heart to pump blood to the lungs. If this
high pressure continues, it puts a strain on the right side of the heart. That strain
can cause cor pulmonale.
Chronic lung conditions that cause low blood oxygen levels in the blood
over a long time can also lead to cor pulmonale. 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, 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 also have a fast heartbeat and feel like your
heart is pounding.
Over time, symptoms occur with lighter activity or even while you are at
rest. Some symptoms you may have are:
Fainting spells during activity
Chest discomfort, usually in the front of the chest
Chest pain

Swelling of the feet or ankles

Symptoms of lung disorders, such as wheezing or coughing
Lips and fingers that turn blue (cyanosis)

Exams and Tests

Your health care provider will perform a physical exam. This may show:
Fluid buildup in your belly
Abnormal heart sounds
Bluish skin
Liver swelling
Swelling of the neck veins, 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. It is important to treat
medical problems that cause pulmonary hypertension, because they can lead to
cor pulmonale.
Many treatment options are available. Your doctor will decide which
medicine is best for you. In general, the cause of your cor pulmonale will
determine which treatment you receive.
If your doctor prescribes medicines, you may take them by mouth (oral),
receive them through a tube that goes into a vein (intravenous or IV), 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. Never stop taking
your medicines without first talking to your doctor.

Other treatments may include:

Blood thinners to reduce the risk of blood clots
Oxygen therapy at home
A lung or heart-lung transplant, if medicine does not work
Important tips to follow:
Avoid strenuous activities and heavy lifting.
Avoid traveling to high altitudes.
Get a yearly flu vaccine, as well as other vaccines, such as the
pneumonia vaccine.
If you smoke, stop.
Use oxygen if your doctor prescribes it.
Do not get pregnant (women).
Outlook (Prognosis)
How well you do depends on the cause of your cor pulmonale.
As your illness gets worse, you will need to make changes to your home
so that you can manage as well as possible. You will also need help around your
house.
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.
Prevention
Avoid cigarette smoking to help prevent lung disease, because lung
disease can lead to cor pulmonale.
Alternative Names
Right-sided heart failure