Aortic Coarctation

Why does coarctation of the aorta occur? Find all the information about the causes, symptoms, and treatment of this condition.

Symptoms and Causes

Aortic coarctation is a narrowing of a section of the aorta that hinders blood flow through it. This causes the heart to work harder, pumping with greater force to ensure adequate circulation to the lower half of the body. The coarctation is usually located in the descending thoracic aorta. Generally, it is a congenital malformation present from birth, often associated with other heart defects.

Based on the segment where the coarctation occurs, it is classified as:

  • Preductal coarctation: Located proximal to the ductus arteriosus, the vessel that connects the aorta to the pulmonary artery.
  • Ductal coarctation: Located at the insertion of the ductus arteriosus.
  • Postductal coarctation: The narrowing is distal to the ductus arteriosus.

Depending on the presence or absence of associated lesions, the types are:

  • Simple aortic coarctation: Occurs in isolation.
  • Complex aortic coarctation: Occurs along with associated conditions.

In some cases, symptoms appear from birth (neonatal aortic coarctation), while in others, they do not manifest until childhood (pediatric aortic coarctation) or even adulthood.

Symptoms

The symptoms of aortic coarctation depend on the degree of narrowing. When mild, it is often asymptomatic and may not be diagnosed until adolescence or adulthood. If symptoms appear, they include:

  • Chest pain
  • Headaches
  • High blood pressure and a difference in blood pressure between the arms and legs
  • Nosebleeds
  • Leg pain or cramps
  • Cold feet
  • A heart murmur may be present

Infants with moderate coarctation may present the following symptoms:

  • Rapid or difficult breathing
  • Accelerated heart rate
  • Pale skin
  • Difficulty feeding
  • Irritability or lethargy

Causes

The exact causes of aortic coarctation are not fully understood, and it is believed to result from various abnormalities during fetal development. In rare cases, it may not be congenital and instead be caused by other factors, such as trauma, inflammation of the arteries (Takayasu arteritis), or severe artery hardening (atherosclerosis).

Risk Factors

Aortic coarctation is often associated with the presence of other congenital heart conditions, such as:

  • Turner syndrome: A chromosomal disorder that causes heart malformations.
  • Bicuspid aortic valve: The aortic valve has two leaflets instead of three.
  • Subaortic stenosis: A narrowing in the left ventricular outflow tract below the aortic valve.
  • Patent ductus arteriosus: The ductus arteriosus remains open after birth.
  • Ventricular septal defect: An opening in the septum between the ventricles allows oxygen-rich blood from the left side to mix with oxygen-poor blood from the right side.
  • Mitral valve stenosis: A narrowing of the mitral valve that restricts blood flow between the left atrium and ventricle.

Complications

Untreated aortic coarctation can lead to:

  • Chronic high blood pressure: The most common complication, which can cause serious health problems in the medium and long term.
  • Left ventricular hypertrophy due to the increased effort required to maintain normal systolic function.
  • Weakening of the aorta or arteries in the arms or brain due to excessive pressure.
  • Aneurysms, ruptures, and spontaneous tears resulting from arterial weakening, potentially leading to hemorrhages, strokes, or other vascular events.
  • Organ damage due to reduced blood supply in severe cases of coarctation.
  • In newborns, it can lead to heart failure or death.

Prevention

Since aortic coarctation is a congenital malformation with no established cause, it cannot be prevented. However, early detection of associated heart diseases can facilitate a timely diagnosis.

Which Doctor Treats Aortic Coarctation?

Aortic coarctation is treated in cardiovascular medicine and surgery units.

Diagnosis

Aortic coarctation is often suspected during a routine medical examination if a heart murmur is detected or if there is a significant difference in blood pressure between the arms and legs. If suspected, further tests are conducted to confirm the diagnosis:

  • Doppler echocardiogram: Uses ultrasound waves to create moving images of the heart, allowing the detection of the location and severity of the coarctation, as well as other associated congenital defects.
  • Magnetic resonance angiography or computed tomography angiography: Imaging studies combined with a special dye injection to visualize blood flow through the veins and arteries, locate the coarctation, and determine if other vessels are affected. These tests are often used if echocardiography is inconclusive.
  • Electrocardiogram (ECG): Records the heart’s electrical activity and may show signs of left ventricular hypertrophy.

Treatment

Treatment depends on the patient’s age and the severity of the coarctation. Options include:

  • Emergency drug treatment for newborns with severe aortic coarctation (preparation for surgery):
    • Prostaglandins, to keep the narrowed ductus arteriosus open.
    • Inotropic agents and diuretics, to support ventricular function.
  • Surgical repair of the narrowing:
    • Resection with end-to-end anastomosis: The narrowed segment of the aorta is removed, and the healthy sections are reconnected. This is the preferred procedure for moderate and severe coarctations in infants and young children.
    • Patch aortoplasty: An incision is made at the coarctation site, and a synthetic patch is implanted to expand the artery.
    • Left subclavian flap aortoplasty: Similar to the previous procedure, but using a portion of the left subclavian artery as a patch to widen the narrowed area.
    • Balloon angioplasty: A catheter is used to place a balloon at the narrowed area of the aorta. The balloon is inflated to widen the aorta, and a mesh tube (stent) is usually placed to keep the artery open.
    • Graft interposition: The obstructed tissue is removed, and a graft is placed in its place.
  • Medications to control high blood pressure: Even after repair, hypertension may persist and require treatment with drugs that relax blood vessels, such as:
    • Angiotensin-converting enzyme (ACE) inhibitors
    • Angiotensin II receptor blockers (ARBs)
    • Calcium channel blockers
    • Diuretics
    • Beta-blockers
    • Vasodilators
  • Follow-up: After surgery, lifelong follow-up is necessary to monitor blood pressure and detect possible recoarctations.
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