The hearts that beat...outside the chest? by Jodine Robin

Virsaviya Buron
Source: Bathsheba's Heart

I recently saw a video on Facebook of a six year old, named Virsaviya Borun with her heart outside of the rib cage, a condition called Pentalogy of Cantrell: Ectopia Cordis. I was rather quite intrigued and fascinated by this phenomenon! How is it possible for this little girl to live with heart just under her skin?? What could causes this? Can it be treated and how? I had a plethora of questions, but before I was able to find the answers I first had to understand what the condition was.
Pentalogy of Cantrell is a rare disorder characterized by birth defects of the sternum, diaphragm, abdominal wall and the heart. In Virsaviya’s case, her birth defect is of the heart which is Ectopia Cordis. This is a congenital heart defect where the heart is located partially or totally outside of the thoracic cavity. The four main ectopic positions are adjacent to the thorax (heart is completely outside rib-cage without soft tissue), abdominal, thoraco-abdominal and cervical.

The average heart is located within the thoracic cavity in the mediastinum which is the area between the lungs and the apex of the heart tilts to the left with a blunt point. The heart usually has multiple forms of protection from pericardium and thorax. The pericardium is a double-walled sac anchored to the diaphragm and connective tissue of major vessels of the heart. The pericardial sac includes the pericardial fluid (lubricant) between the visceral (smooth serous layer) and parietal pericardium (a tough fibrous layer of dense connective tissue) . The thoracic cavity consists of the rib cage, sternum and vertebrae that all surround the heart providing a hard shield like enclosure. As a result of ectopia cordis the heart is outside the thoracic cavity thus causing it to be much small than the cavity was intended to be. Also, Virsaviya's heart is prone to injury and numerous circulatory complications therefore it may become incapacitated and sensitive due to excess excess exposure .
  

Christopher Wall after multiple surgeries (left) and at his high school graduation (right)
Source: ABC News

The main causes of this malformation is a ventral wall defect that affects the abdomen, thorax or both. Another cause is the failure of the lateral mesoderm (middle layer) to migrate into the mid-line. The condition can be treated however, treatment is most effective when started at a young age. While Virsaviya was rejected for survey because she was weak, Christopher Wall was not. Wall was born in 1975 and was one of the first live 100 cases of this disorder. By age 3 he had received over 22 surgeries. The first stage of treatment occurs during the first hours of life, where the heart must be covered with skin grafts, synthetic or biological mesh and no attempt to move the heart into the thoracic cavity should be made. At several months- two years old, chest wall reconstruction and re-positioning surgery can occur.

Prenatal (32 weeks) diagnosis of ectopia cordis and extra-thoracic heart (arrow)
Source: Plastic Surgery Key

Both Virsaviya and Christopher Wall are miracle cases because most cases of ectopia cordis result in stillbirths or death within hours/days of birth. For every one million births only five to eight of them have ectopia cordis, hence it is even more amazing that they even have it. Christopher Wall had multiple surgeries that caused major side effects such as learning disabilities, however he would be 42 is 2017 which is remarkable (since 2009 it is unknown if Wall is alive). Virsaviya having received 0 surgeries lives a good but very careful life to keep her heart safe so that one day she can have surgery.