Ectopia Cordis

Table of Contents:

  1. What Is Ectopia Cordis?
  2. Causes
  3. Symptoms
  4. Diagnosis
  5. Treatment
  6. Comfort Care

 

What Is Ectopia Cordis?

 

Ectopia cordis is a very uncommon disease in which babies’ hearts are partly or completely outside their bodies when they are born. It usually occurs in conjunction with other heart or belly birth defects. 

 

Ectopia cordis affects just eight out of a million babies. Although the majority of them are stillborn or perish within three days of birth, there is room for optimism. Doctors, clinicians, occupational and physical therapists, and other professionals have helped certain infants with ectopia cordis survive for weeks or even years with the assistance of health care teams.

Causes

 

Ectopia cordis is a condition where no one knows what triggers. They do, though, have a few ideas. One is that physical adjustments occur in the baby when it grows through birth, limiting its normal development. 

 

Fibrous bands, for example, may form on the amniotic sac, which wraps the baby in the womb and restrain it. Alternatively, the baby’s chest can grow in an abnormal way, preventing the heart from moving down into its regular position, resulting in ectopia cordis and other cardiac issues. 

 

Ectopia cordis is often related to sternum (breastbone), pericardium (heart membrane), and abdominal wall abnormalities, as well as chromosomal anomalies like trisomy 18 and Turner syndrome. According to one hypothesis, certain embryos lack a gene named BMP2, which allows the formation of the heart and the front of the baby’s chest more difficult.

Symptoms

 

The major sign of ectopia cordis is the presence of the heart outside the bone. Other “midline defects” (problems in the line running up and down in the middle of the body, from the head to the groyne) are common in babies with this disorder, including:

 

  • Cranial cleft (a break in the shape of the baby’s face)
  •  Cleft lip/palate (a gap in the top of the baby’s mouth)
  • Lungs that haven’t completely matured
  •  Scoliosis (curved spine)
  • Abnormal hole in the diaphragm (the muscle between the chest and abdomen)

 

Diagnosis

 

You can find out whether your baby has ectopia cordis during a normal ultrasound as early as the first trimester or the beginning of the second trimester. It’s possible that you’ll require another round of ultrasound to confirm the diagnosis. These findings are crucial in determining how to continue. 

 

If you plan to keep the pregnancy going, the doctor can use a mixture of magnetic resonance imaging (MRI) and echocardiography, a procedure that uses sound waves to produce images of the heart, to monitor your baby’s growth. 

 

Your doctor may use the following terms to describe the location of the heart:

  • Thoracic: near the ribs and breastbone behind the abdomen (64 percent of ectopia cordis cases)
  • Thoracoabdominal: lower your chest into your belly (18 percent of ectopia cordis cases).
  • Abdominal: in the body’s lower tier (15 percent of ectopia cases).
  • Cervical:in the direction of the spine (3 percent of ectopia cordis cases).

 

Ectopia cordis may be a very stressful diagnosis for families. Link with the Feingold Center for Children and the nonprofit Birth Defect Research for Children for assistance and funding.

 

Treatment

 

You’ll need to do a C-section to better protect the baby. Your baby may need additional assistance to breathe. Doctors can place a flexible plastic tube in their windpipe to keep air flowing, as well as administer a special liquid through the tube that covers the lungs and ensures that they can take in enough oxygen. 

 

The primary aim in operation to cure ectopia cordis is to close the exposed chest wall. The surgeon and medical staff will also correct all other cardiac abnormalities as well as insert the heart inside the thorax. The type of ectopia cordis determines whether or not these operations are possible. The medical team will also implant some intestines or abdominal organs that are outside of the baby’s body into the belly. 

 

Surgeries are mostly performed in stages. The first obstacle is fitting the heart into a chest cavity that is much narrower than average. The sternum can be repaired until your baby’s doctor has completed this procedure. Transplanting a heart is normally not an alternative. 

The extent to which the heart is exposed, as well as whether it is protected by a serous membrane (tissue that lines body cavities) or ordinary skin, affects care and results.

Comfort Care

Your doctor can even provide you with “comfort care." This method of treatment is aimed at preventing and alleviating pain. It allows the kid to be soothed and at ease before the end of his or her life. The March of Dimes’ Share Your Story platform will help the family locate community groups and people in similar circumstances.

Sources

Referenced on  10.4.2021

  1. https://www.webmd.com/baby/ectopia-cordis-overview#1 
  2. Children’s Hospital Colorado: “Ectopia Cordis.”
  3. Texas Children’s Hospital: “Baby Born With Heart Outside Of Body (Ectopia Cordis) Makes History.”
  4. Pediatric Cardiology: The Fetus With Ectopia Cordis: Experience and Expectations From Two Centers.
  5. Clinical Anatomy: Ectopia Cordis: A Rare Congenital Anomaly.
  6. National Institutes of Health: “Pentalogy of Cantrell.”
  7. National Institute on Aging: “Providing Comfort at the End of Life.”

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