Catherine O’Hara, the actress and comedian who got her start on the Canadian sketch series “Second City Television” and then went on to play iconic moms in “Beetlejuice,” “Home Alone” and “Schitt’s Creek,” died last week at age 71.
The cause of her death has not yet been confirmed, although O’Hara previously shared she was born with a rare congenital condition called situs inversus that affects roughly 1 in 10,000 people. She reportedly discovered her diagnosis over 20 years ago when undergoing routine medical tests. “When the doctor told us that my heart was on the right side, and my organs were flipped, my husband immediately said, ‘No, her head’s on backwards,’” O’Hara said during a “Virtual Happy Hour” with Kathryn Hall in 2020.
There is no evidence that situs inversus — a condition in which the internal organs are essentially a mirror image of where they typically occur — contributed to O’Hara’s death. However, the condition occasionally co-occurs with a respiratory disorder that impairs lung function. We asked two cardiologists, neither of whom had treated O’Hara, about the causes, symptoms and complications of situs inversus.
What is situs inversus?
“In normal circumstances, the heart, spleen and stomach are on the left, and the liver and appendix are on the right. With situs inversus, all of those things are inverted,” said Adam Small, a cardiologist with NYU Langone Health. Various genetic mutations can cause situs inversus, which may be inherited from a parent or occur randomly.
There are a few ways the conditions presents. With situs inversus totalis, or dextrocardia with situs inversus — the type O’Hara had — all of the internal organs, including the heart, are flipped in a mirror-image inversion of a typical body. “In the olden days, people would often think the X-ray was backwards, so they’d take it off the light board and flip it upside-down to make it look normal,” Small said.
The other main subcategory is known as heterotaxy syndrome. In this situation, the organs are not inverted but in atypical positions. For example, the liver may be in the middle of the body, or there may be multiple spleens or severe heart issues. This could also look like most organs being flipped, except for the heart (known as levocardia). “The body gets very confused,” said Small, noting that this unusual arrangement of organs can lead to serious health problems.
What are possible complications of situs inversus?
The vast majority of people with situs inversus totalis will never develop symptoms or face any health consequences. “The heart pumps normally, and the liver works normally, they’re just in the wrong place,” Small said, adding that he has a number of patients with situs inversus totalis “who have no real problems at all.”
Though largely asymptomatic, the disorder does come with an increased risk of relatively minor congenital heart issues, such as holes between the heart chambers and subtle valve abnormalities that may be associated with atrial fibrillation, that can be picked up on an echocardiogram, said Martina Brueckner, a pediatric cardiologist with Yale Medicine. (Atrial fibrillation is one of the most common heart arrhythmias and is characterized by a fast, irregular heart rhythm.)
Roughly 20 to 30 percent of people with situs inversus also have primary ciliary dyskinesia (PCD), a disorder that can lead to chronic respiratory tract infections. With PCD, the cilia — microscopic hairlike structures that extend from cells — develop abnormally. Typically, cilia in the lungs help clear out mucus and particles; impaired cilia cause mucus-clearance problems, which can result in chronic lung infections, Small said. Over time, this can lead to bronchiectasis, a condition in which the airways of the lungs may become dilated or collapse due to scarring and tissue breakdown. “That can be a very challenging condition,” Small said. There are many treatments available, including inhalers, antibiotics, devices that help you cough and hypertonic saline.
How is situs inversus diagnosed?
O’Hara said she discovered her internal organs were reversed only when she underwent an electrocardiogram for an unrelated health exam. Small said that most people get diagnosed this way — essentially, by accident. “Someone comes into the emergency room for problem X, Y or Z, and they get a CT scan or X-ray, and the computer will show the heart is on the right side,” he said.
Individuals with PCD tend to seek care for respiratory symptoms, including chronic cough and wheezing, or recurrent sinus and ear infections. PCD is often identified in childhood or adolescence, Small said, but sometimes it can be missed — and misdiagnosed as severe asthma or ear infections — until later in life.
To make a PCD diagnosis, the cilia can be biopsied and evaluated under a microscope to ensure they move properly, Bruecker said. Genetic testing can also determine if you have abnormal genes linked to the disorder. Heterotaxy syndrome, on the other hand, is typically diagnosed shortly after birth if there are heart problems that cause symptoms, if not in utero during routine fetal ultrasounds.
What is the life expectancy of someone with situs inversus?
The prognosis for situs inversus varies from person to person, depending on the severity of their condition and the subtype they have. The life expectancy for people with situs inversus totalis is in line with that of the general population. “Because it’s a mirror image, everything ends up being hooked up properly,” Small said.
In the case of heterotaxy syndrome, you may have changes that affect the structure and function of the walls, valves and chambers of the heart, Small said. In this case, a person’s prognosis is dictated by the severity of their heart malformation.
Finally, PCD does not necessarily affect life expectancy, but complications are quite variable from person to person. For example, while some may experience mild asthma symptoms, others may have complex lung issues that require a transplant. As Brueckner explained, “it depends how severe your respiratory conditions are and how well those are managed.”
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