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The aorta is the main artery that carries blood from the heart to the major arteries
that go to all parts of the body.

Coarctation of the aorta is a common congenital (present at birth) heart defect.

Coarctation comes from the Latin coartare, meaning "to press together." In
coarctation of the aorta, the aorta is pinched in or narrowed, either in a single
location or along a portion of its length. This narrowing restricts normal blood
flow through the aorta.

  




  




Coarctation of the aorta occurs in about 1 in 10,000 births and accounts for 5 to 10 percent of all
congenital heart defects.





 


  

  




Complications can develop from untreated coarctation of the aorta, the result of long-term high
blood pressure caused by the coarctation. Some of the most severe complications include stroke,
early-onset coronary artery disease, and brain aneurysm or aortic rupture. If the coarctation is
severe and remains untreated for a long period, kidney and liver failure can develop.

More than half of people with coarctation of that aorta also have a congenital heart valve defect
(bicuspid aortic valve). Over time, the valve condition usually worsens and may require surgical
valve repair or replacement. People who have valve disease are also at risk for an aortic
aneurysm (weakening in the walls of the aorta that causes it to bulge out and poses a risk of
rupture).





    

  




Symptoms depend on the severity of the narrowing. Severe cases often are diagnosed at birth or
within the first months of life. In mild cases, the individual may be symptom±free into
adulthood. Symptoms in an adult may include exercise intolerance, headache, shortness of
breath, chest pain, nose bleeds, cold feet or leg pain after exercise.
_  

  






›ortic coarctation usually occurs between the site where the blood vessels branch off
the aorta to the upper body and the site where the blood vessels branch to the lower
body. Because of this positioning, aortic coarctation causes high blood pressure in
the arms and upper body and low blood pressure in the lower body and legs. This
blood pressure difference is one of the most distinguishing diagnostic factors for
coarctation of the aorta. Other distinguishing clinical features include differences in
the pulse at the groin and the neck and a distinctive harsh heart murmur that can be
heard with a stethoscope placed over the patient¶s back.

To help define the extent of the problem, a cardiologist may order several different types of
imaging tests that may include:

u? Ñlectrocardiogram (ÑCG)
u? Chest X-ray
u? Ñchocardiography
u? Chest computed tomography (CT or C›T) scan
u? Magnetic resonance imaging (MRI) of the chest
u? Cardiac catheterization

_  

  








Twenty years ago, surgery was the only treatment available for aortic coarctation. Surgery is still
considered the gold standard, but today treatment options for adults with this condition also
include balloon angioplasty and stenting. The choice of treatment is based on the individual¶s
overall health, the size and severity of the coarctation and its precise location.

›n individual who is diagnosed with coarctation of the aorta should be under the care of an
experienced congenital heart specialist at a major medical center. › cardiac surgeon experienced
in the procedures used to treat this condition is best able to determine the optimal timing and
choice of treatment.

Ñnd-to-end anastamosis



When the coarctation is relatively small, the surgeon can remove the
narrowed section of aorta and re-join the two ends. This is called an end-to-end anastamosis and
is often the best surgical option to treat the condition.

Other surgical options include various types of bypass surgery in which a graft is stitched onto
the aorta to divert blood around the area of the defect. In patients with valve disease, a bypass
can be performed in combination with a valve repair or replacement procedure.
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If you have had heart surgery or angioplasty to repair a defect, your doctor will prescribe
preventive antibiotics for you to take before certain medical procedures for at least 6 months
after the repair procedure to reduce the risk of infective endocarditis. Your doctor can provide
specific guidelines about when to take antibiotics. ›ccording to the ›merican Heart ›ssociation,
there are insufficient data to make recommendations for continuing preventive antibiotic therapy
longer than 6 months.

For more information about endocarditis, visit:

www.clevelandclinic.org/heart

 
    



›ngioplasty is another option for treating coarctation of the aorta, and at some medical centers, it
is the preferred treatment. The procedure is similar to angioplasty for coronary artery disease: a
long, thin tube (a catheter) with a balloon on the end of it is passed into the aorta through the
blood vessels, entering at the groin. When the catheter reaches the coarctation, the congenital
interventionalist inflates the balloon to expand the aorta. When the narrowed area is fully
expanded, the balloon and the catheter are withdrawn.

› small metal mesh tube called a stent may be placed at the site of the coarctation to keep the
aorta open after the balloon is removed. The current trend is to recommend stenting in cases of
severe, long areas of coarctation and in cases when differences in blood pressure persist after
angioplasty alone. Stenting appears to lower the risk for aneurysm formation and rupture of the
aorta compared with balloon angioplasty alone.


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Following treatment for coarctation of the aorta, there is a small risk that the problem will
redevelop. ›fter surgical treatment, the risk of recurrence is between 5 and 10 percent. ›fter
angioplasty or stenting, the risk of recurrence is between 11 and 15 percent. However, long-term
data on outcomes, risks and complications associated with angioplasty and stenting are not yet
available.

In the event that coarctation does recur after any treatment, angioplasty with stenting is the
preferred treatment.

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People who have undergone treatment for coarctation of the aorta should be under the ongoing
care of a congenital heart disease specialist. Regular visits that include measurements of blood
pressure and a clinical exam will be required every year. In addition, Doppler ultrasound and
MRI tests should be performed at regular intervals, usually every two to five years.
People who have normal blood pressure after treatment likely will have no activity limitations.
Individuals who continue to have high blood pressure may be advised to avoid certain strenuous
activities or sports.


  
 


  

Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the
heart to the lungs become narrowed, making it difficult for blood to flow through the vessels. ›s
a result, the blood pressure in these arteries -- called pulmonary arteries -- rises far above normal
levels. This abnormally high pressure strains the right ventricle of the heart, causing it to expand
in size. Overworked and enlarged, the right ventricle gradually becomes weaker and loses its
ability to pump enough blood to the lungs. This could lead to the development of right heart
failure.

Pulmonary hypertension occurs in individuals of all ages, races, and ethnic

backgrounds although it is much more common in young adults and is
approximately twice as common in women as in men.

  
 




 

Scientists believe that the process starts with injury to the layer of cells that
line the small blood vessels of the lungs. This injury, which occurs for
unknown reasons, may cause changes in the way these cells interact with
the smooth muscle cells in the vessel wall. ›s a result, the smooth muscle
contracts more than normal and narrows the vessel.





     
 

  

Symptoms of pulmonary hypertension do not usually occur until the condition has progressed.
The first symptom of pulmonary hypertension is usually shortness of breath with everyday
activities, such as climbing stairs. Fatigue, dizziness, and fainting spells also can be symptoms.
Swelling in the ankles, abdomen or legs; bluish lips and skin, and chest pain may occur as strain
on the heart increases. Symptoms range in severity and a given patient may not have all of the
symptoms.

In more advanced stages of the disease, even minimal activity will produce some of the
symptoms. ›dditional symptoms include irregular heart beat (palpitations or strong, throbbing
sensation), racing pulse, passing out or dizziness, progressive shortness of breath during exercise
or activity, and difficulty breathing at rest. Ñventually, it may become difficult to carry out any
activities as the disease worsens.




 
 

  

The following are some known causes of pulmonary hypertension:

 u? Ô



 ›lthough the appetite suppressant "fen-phen"
(dexfenfluramine and phentermine) has been taken off the market, former fen-phen users
have a 23-fold increase risk of developing pulmonary hypertension, possibly years later.
u? !





 
     Pulmonary hypertension also can
occur as a result of other medical conditions, such as chronic liver disease and liver
cirrhosis; rheumatic disorders such as scleroderma or systemic lupus erythematosus
(lupus); and lung conditions including tumors, emphysema, chronic obstructive
pulmonary disease (COPD), and pulmonary fibrosis.
u? c






 Heart diseases including aortic valve disease, left heart failure,
mitral valve disease, and congenital heart disease can also cause pulmonary hypertension.
u? Ô  
 


 › blood clot in a large pulmonary artery can result in the
development of pulmonary hypertension.
u? !  !
    High altitude living, obesity, and sleep apnea can also lead to
the development of pulmonary hypertension.
u? è


    Pulmonary hypertension is inherited in a small number of cases.
Knowing that someone in the family had or has pulmonary hypertension should prompt
you to seek early evaluation should symptoms occur.

Pulmonary hypertension may also be caused by other conditions, and in some cases, the cause is
unknown.

_   
 


  



Because pulmonary hypertension may be caused by many medical conditions, a complete

medical history, physical exam, and description of your symptoms are necessary to rule out other
diseases and make the correct diagnosis. During the physical exam, your health care provider
will:

u? listen for abnormal heart sounds such as a loud pulmonic valve sound, a systolic murmur
of tricuspid regurgitation, or a gallop due to ventricular failure.
u? examine the jugular vein in the neck for engorgement.
u? examine the abdomen, legs, and ankles for fluid retention.
u? examine nail beds for bluish tint.
u? look for signs of other underlying diseases that might be causing pulmonary
hypertension.

Other tests that might be ordered include:

u? v 
 "
‘? Complete metabolic panel (CMP): Ñxamines liver and kidney function
‘? ›utoantibody blood tests, such as › ›, ÑSR, and others: Screens for collagen
vascular diseases
‘? Thyroid stimulating hormone (TSH): › screen for thyroid problems
‘? HIV: › screen for human immunodeficiency virus
‘? ›rterial blood gases (›BG): Determines the level of oxygen in arterial blood.
‘? Complete blood count (CBC): Tests for infection, elevated hemoglobin, and
anemia
 ‘? B-type natriuretic peptide (B P): › marker for heart failure
u?   

 
 
" Uses sound waves to show the function of the right ventricle,
to measure blood flow through the heart valves, and then calculate the systolic pulmonary
artery pressure.
u? c
#
" Shows an enlarged right ventricle and enlarged pulmonary arteries.
u? D

$
" Determines exercise tolerance level and blood oxygen saturation
level during exercise.
u? Ä 
 
 " Ñvaluates for other lung conditions such as chronic
obstructive pulmonary disease and idiopathic pulmonary fibrosis among others.
u? Ä   
  
  !
" Screens for sleep apnea (results in low oxygen
levels at night).
u?  




%
 " Measures various heart pressures (ie, inside the pulmonary
arteries, coming from the left side of the heart), the rate at which the heart is able to pump
blood, and finds any leaks between the right and left sides of the heart.
u? A

 
   
&A
 
'" Looks for evidence of blood clots along the
pathway to the lungs.
u? Ä 

 
" Looks for blood clot blockages in the pulmonary arteries.
u? c
cÔ 
" Looks for blood clots and other lung conditions that may be contributing
to or worsening pulmonary hypertension.

_   
 


  




›ppropriate diagnosis and analysis of the problem is necessary before starting any treatment.
Treatment varies per individual based on the different underlying causes but generally includes
taking medications; making lifestyle and dietary changes; having surgery, if necessary; and
seeing your doctor regularly. Listed below are medication and surgical treatment approaches.

(

  

Many different types of medications are available to treat pulmonary hypertension. Treatment
choices, such as those listed below, depend on the severity of pulmonary hypertension, the
likelihood of progression, and individual drug tolerance.

u? þ!
 ² replaces the low oxygen in your blood.
u? 

    
  such as warfarin sodium (Coumadin) ² decreases
blood clot formation so blood flows more freely through blood vessels. ) 
"when
taking anticoagulant medications, it is important for you to monitor bleeding
complications and have regular lab work to monitor the level of medication in your
bloodstream.
® ®
u?  
  

  [such as furosemide (Lasix ), spironalactone (›ldactone )]
² removes extra fluid from the tissues and bloodstream, which reduces swelling and
makes breathing easier.
®
u? Ä 
 (such as K-dur ) ² replaces potassium (an essential nutrient) that may be lost
with increased urination when taking diuretics.
u? ˜  

 (such as digoxin) ² improves the heart¶s pumping ability.
 u? Vasodilators [such as nifedipine (Procardia®) or diltiazem (Cardizem®)] ² lowers
pulmonary blood pressure and may improve the pumping ability of the right side of the
heart.
* *
u? v

&Ô


 ' 


&!

 ' ² helps block the action of endothelin,
a substance that causes narrowing of lung blood vessels. These medications require
monthly labwork to monitor liver function.
* * *
u?   
 & 
 ' 
  &
  Ô
'   
*
&A

 ' ² dilates pulmonary arteries and helps prevent blood clots from forming.
* *
u? 

&

 ' 


&
' ² relaxes pulmonary smooth muscle cells,
which leads to dilation of the pulmonary arteries.







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 "If present, a large clot in the pulmonary artery may be
surgically removed to improve blood flow and lung function.

!
 

 "Currently, this is the only cure for primary pulmonary hypertension.
Transplantation is reserved for advanced pulmonary hypertension that is not responsive to
medical therapy. The right side of the heart will generally return to normal after the lung/lungs
have been transplanted. ›bout 1,000 lung transplants are performed annually in the United
States. Many people are on the waiting list, yet a shortage of donors is the major limiting factor.
Your health care provider will discuss transplantation if it is an appropriate treatment option for
your condition.

_

 
 

 "This type of double organ transplant is very rare but is necessary
for all patients who have combined lung and left heart failure