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Q21.80: Pentalogy of Fallot

You have a combined heart defect. These heart defects are congenital, which means you were born with them.

The heart pumps blood through the body. Inside the heart are 4 chambers. There are 2 heart atria and 2 ventricles. Both heart atria and both ventricles are separated from each other by a partition. Major, important blood vessels branch out of the heart. The main artery (aorta), originating from the left lower chamber (ventricle) of the heart, supplies the whole body with oxygen-rich blood. The right ventricle pumps oxygen-poor blood through the pulmonary artery to the lungs, where the blood is then enriched with oxygen.

You have several heart defects at the same time. You have a large hole in the wall separating the left side of your heart from the right (the cardiac septum). The hole is located between the two upper chambers (atria) and between the two lower chambers (ventricles) of your heart. This causes blood from the two ventricles to flow directly into the main artery (aorta). In your case, the pulmonary artery is also too narrow or blocked. As a result, the right heart has to pump the blood against a great deal of resistance. As a result, the right side of the heart becomes enlarged. A combined heart defect occurs while the heart is developing in the womb before birth. The exact reason is unknown.

The heart defect has changed normal blood flow in your heart. Normally, blood flows from the right ventricle into the pulmonary artery. However, your blood flows from the right heart directly into the left heart through the holes in the cardiac septum. In addition, your lungs are only supplied with a small amount of blood because of the narrow pulmonary artery, causing the blood to also be less enriched with oxygen. As a result, the blood in your main artery (aorta) has a low level of oxygen. The aorta then pumps oxygen-poor blood through the body. The body’s organs are supplied with less oxygen as a result.

The heart defects can lead to various symptoms. For example, the skin appears bluish in color. The fingers and nails may also look different. You often have severe shortness of breath and feel exhausted. In many cases, normal growth is affected. You can also lose consciousness or have seizures. The body produces more red blood cells to compensate for the lack of oxygen. Having lots of red blood cells makes it more likely that blood clots could develop.

Most symptoms often start shortly after birth.

Additional indicator

On medical documents, the ICD code is often appended by letters that indicate the diagnostic certainty or the affected side of the body.

  • G: Confirmed diagnosis
  • V: Tentative diagnosis
  • Z: Condition after
  • A: Excluded diagnosis
  • L: Left
  • R: Right
  • B: Both sides

Further information

Note

This information is not intended for self-diagnosis and does not replace professional medical advice from a doctor. If you find an ICD code on a personal medical document, please also note the additional indicator used for diagnostic confidence.
Your doctor will assist you with any health-related questions and explain the ICD diagnosis code to you in a direct consultation if necessary.

Source

Provided by the non-profit organization “Was hab’ ich?” gemeinnützige GmbH on behalf of the Federal Ministry of Health (BMG).