What are the other Names for this Condition? (Also known as/Synonyms)

• Aortic Coarctation
• CoA (Coarctation of the Aorta)

What is Coarctation of the Aorta? (Definition/Background Information)

• Congenital heart defects are relatively common birth defects involving the heart, and these may be of several types. A common but critical heart-related birth defect that occurs in children is narrowing of the aorta, caused by genetic or other underlying factors. It is known as Coarctation of the Aorta (CoA). This condition hinders normal blood flow to the body
• The aorta is the largest and most important (oxygenated and nutrient-rich) blood-supplying artery, originating from the heart. Aortic Coarctation may also be infrequently acquired. This may be because of atherosclerosis, severe injury to the region, or due to a disorder called Takayasu arteritis (a condition that causes swollen aorta)
• Severe Aortic Coarctation is easily diagnosed through the early manifestation of signs and symptoms. However, moderate to mild cases may be detected only much later (sometimes, even when the individual is well into adulthood)
• The condition can be potentially fatal when accompanying medical issues, such as hypertension, congestive heart failure, aortic dissection, heart infection, or hemorrhaging, are observed. Hence, surgical procedures to correct the condition(s) are performed at a very young age (usually by infancy itself), and the outcomes are usually good

Who gets Coarctation of the Aorta? (Age and Sex Distribution)

• Aortic Coarctation affects the newborn child. It may be present at birth, but sometimes not recognized until teenage, or even till much later, such as into adulthood. Some are diagnosed only by the age of 40 years
• The incidence of the condition is estimated to be around 1 in 1,800-2,500 babies
• Even though both genders are affected, males are twice likely to be affected than females
• No racial or ethnic predilection is generally observed. However, Asian countries have a lower incidence of the condition than North American and European regions

What are the Risk Factors for Coarctation of the Aorta? (Predisposing Factors)

The potential risk factors for Coarctation of the Aorta are the following:

• Family medical history of genetic disorders, such as Turner syndrome (which occur in girls)
• Often, congenital heart defects, such as ventricular septal defect, bicuspid aortic valve, stenosis of aortic/mitral valves, or patent ductus arteriosus, are commonly associated with Aortic Coarctation
• The predisposing factors for a late development of the condition include:
  • Any traumatic injury to the chest
  • Takayasu arteritis
  • Atherosclerosis causing fat deposits on the aortic walls

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Coarctation of the Aorta? (Etiology)

The exact cause of development of Coarctation of the Aorta is not well-understood. It may develop from a combination of factors that may be genetic and environmental.

• Certain genetic factors that are proposed may be inherited
• The condition may develop later from certain physiological (anatomical) anomalies including:
  • Obstructive tumorous growths
  • Overgrowth of tissue on the aorta
  • Constriction of the aorta soon after birth
  • Intrauterine blood flow abnormalities
• It is also believed that certain environmental influences and what the expectant mother consumes, such as food, drink, or even some medications, may be contributive

A coarctation may occur anywhere on the aorta. It can be focal (such as at certain discrete spots) or segmental (involving a large portion along its length). However, generally, they seem to be mainly observed at three particular sites, among which, a region known as the ductus arteriosus, is the most common site. The severity of the condition is based on its location and magnitude of narrowing of the aorta.

What are the Signs and Symptoms of Coarctation of the Aorta?

Mild cases of Aortic Coarctation may present itself without any sign or symptom for a long period. There is a possibility that it might suddenly develop into a life-threatening situation, particularly when other cardiac problems coexist. If narrowing of the aorta is acute, then the signs and symptoms may be visible at a very early stage.

The signs and symptoms observed in newborns and infants may include:

• Poor feeding
• Irritability
• Respiratory issues
• Heavy sweating
• Pale skin tone (pink-blue color)
• Irregular blood pressure value and low pulse (if the condition is severe)

The signs and symptoms observed in children and adults may include:

• High blood pressure
• Nosebleed
• Breathing difficulty, such as shortness of breath after light exercising
• Muscle cramps (lower limbs)
• Weakness
• Cold feet and legs
• Frequent headaches
• If condition is severe, then acute chest pain, sudden breathing difficulties, very high blood pressure, nausea, and unconsciousness, may be experienced

Many a times, multiple heart ailments may be observed in the affected individual.

How is Coarctation of the Aorta Diagnosed?

Diagnostic tests vary, depending on early- or late-onset symptoms of Aortic Coarctation, and these include:

• Physical exam with evaluation of medical history. This includes listening to the heart using a stethoscope, to detect any unusual ‘whooshing’ sound
• Evaluating pulse and blood pressure rates; varying blood pressure values can be detected on the arms and legs
• Assessment of the presenting signs and symptoms, particularly pale skin of the child, breathing difficulties, excess sweating, and irritability
• Newborn pulse oximetry screening may help the healthcare suspect the condition prior to symptom presentation
• Electrocardiogram (EKG), to measure electrical activity of the heart
• Echocardiogram may be performed on the fetus to detect any coarctation; it is commonly used to confirm the diagnosis
• Chest X-ray
• CT and MRI scan studies of the chest and abdomen 
• Cardiac catheterization

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Coarctation of the Aorta?

Complications due to Coarctation of the Aorta are mostly due to a delay in detection and treatment of the condition and/or due to the presence of other accompanying heart disorders. These include:

• Heart-related complications may include: Hypertension, coronary artery disease (taking place at an early age), rupture of the aorta, aortic aneurysm, infection of the heart, congestive heart failure, and aortic/mitral valve stenosis
• Brain-related complications may include: Stroke, brain aneurysm, hemorrhage, and paralysis of the lower limbs (or from below the hip)
• If coarctation is severe, then the heart may not be able to pump enough blood to match up to the requirements of other vital organs (such as the liver or kidney) and these may fail
• Post-surgical complications, such as recurrence of the condition, aneurysm or rupture of the repaired aortic portion, risk of heart infection, and hypertension

How is Coarctation of the Aorta Treated?

The treatment of Coarctation of the Aorta is based on the presenting signs and symptoms, the extent of constriction, the overall health condition and age of the adult/child, and other associated medical disorders (if any). Management of the condition involves correcting the defect(s) and providing symptomatic treatment that may include:

• Open heart surgery to repair the aorta: Techniques include removal of the affected aortic section, the use of a bypass graft, or the use of a synthetic patch to widen the narrowed portion of the aorta
• Balloon angioplasty to expand the blood vessel that may also involving stent placement. This may be performed during cardiac catheterization (diagnostic procedure)
• Use of medication to control high blood pressure, prior to and after surgery. The individual is requested to avoid stressful physical exercises during this period
• Post-operative care with control of blood pressure is important. A minimum activity level is to be ensured until complete healing takes place
• Regular health check-ups are essential

Bringing about changes in lifestyle to control blood pressure and taking steps to maintain a healthy heart after the surgery is important. This may be accomplished by:  

• Avoidance of smoking
• Avoiding high-fat diets (to prevent obesity)
• Limiting alcohol consumption
• Exercising regularly (per advise of the physician)
• Having a healthy diet
• Managing stress

How can Coarctation of the Aorta be Prevented?

• Presently, Coarctation of the Aortic is not a preventable disorder
• Detecting early signs and symptoms combined with a timely diagnosis, holds the key to managing the disorder
• Identifying other accompanying heart ailments and preventing further complications can help minimize the risk for fatalities

Regular medical screening at periodic intervals with tests, scans, and physical examinations are mandatory for those who have already undergone surgical correction of the defect. This is essential due to the possibility of other underlying conditions, including recurrence of the coarctation. Often, several years of active vigilance may be necessary.

What is the Prognosis of Coarctation of the Aorta? (Outcomes/Resolutions)

• Early diagnosis with successful medical (invasive) intervention to correct the constricted aorta (before age 10), long-term follow-up of the treated condition, control of blood pressure, and regular checkups and care are all factors that help yield the best prognosis
• When other heart illnesses are present along with Coarctation of the Aortic, and detection/treatment is delayed, then the prognosis is uncertain and is dependent on many factors
• An advancing age, post-surgical narrowing of the repaired aorta, bacterial infections, and persistently high blood pressure values are factors that may adversely affect the course of an outcome

Additional and Relevant Useful Information for Coarctation of the Aorta:

A healthy lifestyle can help improve the quality of the arteries. The factors include:

• Light exercising (walking, swimming, as recommended by the physician)
• Periodic health check-ups
• Keeping blood pressure under control
• Avoiding smoking and alcohol
• Taking a healthy food, less in sugar, salt, and fats

Such practices are good and beneficial for the heart and blood circulatory system.