심장이 몸밖에 있는 소녀 VIDEO:Girl's Heart Beats Outside Her Chest: Rare Disorder Explained

Girl's Heart Beats Outside Her Chest: Rare Disorder Explained

By Rachael Rettner, Senior Writer | November 21, 2017

베르사비아가 소리를 내며 웃자, 갈비뼈 아래에 위치해있는 심장이 몸 밖으로 튀어나온다.

via youtube

설명하기 어려운 상황.

이 소녀의 심장은 몸 안에 있는 것이 아니라 밖으로 노출된 채 가슴에 달려 있다.

생명중추기관(vital organ)을 보호하는 얇은 피부층때문에 생기는 ‘칼트렐 증후군(Pentalogy of Cantrell)’을 앓고 있다. 

숨을 크게 쉬거나, 소리를 내며 웃으면 갈비뼈 밑에 얇은 막으로 싸인 심장이 몸 바깥쪽으로 튀어나온다.

러시아에서 태어난 베르사비아 바런(Virsaviya Borun)은 ‘치료 방법’을 찾기 위해 엄마의 손에 이끌려 2015년 미국 플로리다주로 이주했다. 

올해 8살이 됐지만 해맑은 모습으로 자라고 있다.

황기철 콘페이퍼 에디터 큐레이터

Ki Chul Hwang, conpaper editor, curator

A girl's heart appears to beat literally outside of her chest in a recent video, but what causes this to happen?

The video, which was uploaded to YouTube in September, shows a young girl laughing as her heart beats outside of her chest, with just a thin layer of skin protecting the vital organ. The girl appears to be Virsaviya Borun, an 8-year-old who was born with a rare condition called pentalogy of Cantrell. (Borun's story first went viral in 2015.)

Pentalogy of Cantrell is an often-fatal disorder involving several birth defects in the chest and abdominal area, which can result in the heart being located just beneath a person's skin, as opposed to behind layers of bone, tissue and muscle. The disorder can include defects of the diaphragm (the muscle that separates the chest cavity from the abdomen and helps with breathing); the breastbone (sternum); the pericardium (the membrane that lines the heart); the abdominal wall and the heart itself, according to the National Organization for Rare Disorders (NORD).

(The term "penta" in the condition's name comes from the Greek word for "five," because the disorder can include up to five defects. However, most infants with the disorder do not have all five defects, NORD said.)

In the most severe form of pentalogy of Cantrell, people are born with their heart completely or partially displaced outside of the thoracic cavity. This cavity is the chamber that typically holds the heart, and it is protected by the rib cage. The intestines and abdominal organs of such people can also partially protrude outside their body through the belly button, NORD said. [Top 10 Amazing Facts About Your Heart]

Pentalogy of Cantrell is very rare, occurring in an estimated 5 people per 1 million live births, according to NORD.

Infants born with the condition often do not survive very long — a 2008 review study found that, out of 58 infants with pentalogy of Cantrell, 37 (64 percent) died within days of birth. In most cases, the condition is fatal without surgery to repair the defects, NORD said.

Borun is originally from Russia but now lives in Florida, according to local news station NBC 6 in South Florida. Borun and her mother came to the United States because a doctor at Boston Children's Hospital had agreed to treat her, but later made the decision not to perform surgery because Borun had high blood pressure in her pulmonary artery, which carries blood from the heart to the lungs. In 2015, Borun's mother started a fundraising campaign to raise money for her daughter's medical expenses, and raised more than $71,000, although the page is no longer accepting donations.

https://www.livescience.com/60999-heart-beats-outside-chest-pentalogy-of-cantrell.html?utm_source=notification

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