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Posted on: April 14, 2019 at 9:21 am
Last updated: April 16, 2019 at 9:56 am

Nowadays, it’s totally cliché to describe a lady as ‘one in a million’. A little creativity would be appreciated. However, no one was trying to be cute or sweet when they described the late Rose Marie Bentley as ‘one in fifty million’. Doctors estimated that probably one out of 50 million people in the world may be as uniquely different as Bentley was.

The discovery was made at the Anatomy Dissection Lab at Oregon Health and Science University in Portland. A group of five medical students, led by Warren Nielsen, were excited about opening up the cadaver of a woman who died at the age of 99 to begin a heart examination.

Upon a closer look at her heart, they discovered that a person had lived for 99 years and died of totally natural causes, oblivious of the fact that most of her vital organs were laterally inverted.

Situs Inversus With Levocardia

Speaking to CNN, Cameron Walker, an assistant professor of Clinical Anatomy at the university describes the condition as extremely rare [1].

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“I think the odds of finding another person like her may be as remote as one in 50 million,” he said. “I don’t think any of us will ever forget it, honestly.”

The inferior vena cava (IVC) is a large vein that runs through the right side of the vertebral column, transporting blood from the lower half of the body into the right atrium of the heart [2]. Not only was Bentley’s IVC abnormally positioned in the left side of her vertebral column, according to Walker, “it continued through her diaphragm, along the thoracic vertebrae, up and around and over the aortic arch and then emptied into the right side of her heart,” Normally, it should have run under her liver and terminated directly into her heart.

The professors were initially appalled by the students’ seemingly poor observation skills.

“Her heart was missing a large vein that’s normally on the right side,” Nielsen said. “We asked the Professors: ‘Where’s the inferior vena cava? Are we missing it? Are we crazy?’ And they kind of rolled their eyes, like, ‘How can these students miss this big vessel?’ And they come over and that’s when the hubbub starts. They’re like ‘Oh, my God, this is totally backward!'”

Rosa Bentley had a condition known as Situs Invertus with levocardia, characterized by a left-sided cardiac apex (levocardia) and a crisscross of abdominal organs [3]. It usually comes with severe congenital heart defects, something Bentley never experienced.

She didn’t have only her IVC on the wrong side. Several other vital organs were inverted as well.

“And instead of having a stomach on the left, which is normal, her stomach was on the right,” Asst.  Professor Walker said. “Her liver, which normally occurs predominantly on the right, was predominantly on the left. Her spleen was on the right side instead of its normal occurrence on the left. And then the rest of her digestive tract, the ascending colon, was inverted as well.”

Her hepatic veins (liver drainage veins) took roots from the wrong positions, and she had two lobes on her right lung instead of three. The right atrium of her heart was also twice the normal size. Despite all these abnormalities, Bentley never suffered any heart defects. Aside from the persistent heartburn she suffered, she didn’t have any major cardiovascular or respiratory problem that could have been attributed to this condition. Bentley was a full-blown medical novelty all her life, and she never knew it.

Bentley’s Children Speak About Their Mom

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Bentley was a strikingly beautiful woman, even in her nineties. According to her eldest child, the mother of five had been a happy-go-lucky hairdresser who had been in love with the medical sciences, especially nursing. She was the youngest child of four kids, and as she used to say, she had been seriously spoiled as a kid.

“She would admit she was spoiled. She volunteered during World War II for one of the nurse’s aide corps,” said 78-year-old Patti Helmig. “And she was thrilled when someone reached out to her about doing a study on smallpox survivors, which she had as a child.”

“We had no reason to believe there was anything like that wrong,” 76-year-old Ginger Robbins, Rose’s third child said. “She was always very healthy. She was always doing something, taking us to Campfire Girls, fishing, and swimming. She was an excellent swimmer.”

According to her fourth child, Louise Allee, the surgeon had apparently discovered her appendix was in the wrong spot when she had it taken out, something he didn’t share with them. She had a cholecystectomy (gall bladder removal) and a hysterectomy, and none of the surgeons gave them any information.

Rose and her husband, Jim Bentley, who died 12 years before her, were passionate about donating their bodies for medical research. They took inspiration from the Robert Test poem, To Remember Me, which starts off with “Give my sight to the man who has never seen a sunrise, a baby’s face, or love in the eyes of a woman[4].

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More about Situs Inversus with Levocardia

The condition is extremely rare, also called isolated levocardia. One out of every 22,000 babies born in the world is affected by it [3]. It also occurs invariably with severe congenital heart defects, which is why Bentley is a truly special case.

The cause of the condition is not known. Many patients may suffer from dextrocardia rather than levocardia, which occurs when the heart is positioned on the right side of the chest cavity [5]. Bentley only had her vena cava wrongly positioned, along with a host of other inversions and abnormalities.

Only 5 – 13% of kids born with situs inversus with levocardia live past the age of five. The heart defects are always very severe and unmanageable, especially in infants. The inversions, according to Professor Walker are believed to occur within 30 – 45 days of pregnancy, before the organs begin to solidify. The doctors believe that Bentley managed to live so long due to the fact that she didn’t suffer any severe heart defects. She was also a very physically active person.

When asked about how her mother would have felt if she had found out about her condition, Allee said: “She would have had a big smile on her face.”

 

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