The Link Between Congenital Heart Block and Autoimmune Disease

Congenital Heart Block (CHB) is a rare but serious condition that occurs more frequently in newborns born to mothers with autoimmune disease. Image courtesy of Insider.com.

What is Congenital Heart Block?

According to the National Organization for Rare Disease, Congenital Heart Block, or CHB for short, is the interference of the transfer of electric nerve impulses that regulate the pumping of the heart muscle.

As long as electrical impulses are transmitted normally between the heart’s chambers – the atria and the ventricles – the heart contracts normally, allowing for blood to be pumped throughout the body. If the transmission of the signal is impeded, the blocked electrical transmission is known as heart block, or atrioventricular (AV) block.

Though heart block can happen to anyone of any age, it is called congenital heart block if it occurs in a fetus or newborn up to 28 days old.

Why Does CHB Occur in Children Born to Women with Autoimmune Disease?

Autoimmune-associated CHB has been found in a variety of maternal autoimmune disorders, including Sjogren’s syndrome, systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid syndrome (APS), mixed connective tissue disorders, and undifferentiated connective tissue disease.

It is believed that CHB may result when maternal antibodies cross the placenta, enter the fetus, and attack the fetal cardiac conduction system. The antibodies that were originally produced by the mother’s body to fight infections mistakenly recognize parts of the fetal heart’s conduction system as foreign; for this reason, the immune system attacks and damages the tissues, resulting in inflammation and scarring, which in turn leads to faulty conduction. 

What Is the Risk of Congenital Heart Block if I Have an Autoimmune Disease?

A 2017 study conducted by Chinese medical professionals Kai-Yu Zhou and Yi-Min Hua of the West China Second University Hospital, Department of Pediatric Cardiology, revealed that more than half of CHB cases (between 60 and 90%) are associated with maternal autoimmune disease.

Among the general population, CHB occurs in 1 out of every 20,000 live births – an incidence of only 0.00005%. The study found that autoimmune-associated CHB, however, occurs at much more frequent rates, affecting between 2–5% pregnancies with positive anti-Ro/SSA and La/SSB antibodies. The study also found that when a woman had a child with CHB, the recurrence rate of CHB was 12–25% for a subsequent pregnancy.

Mortality Rate & Treatment for Congenital Heart Block

The perinatal mortality rate of a newborn with CHB is up to 30%, and even higher in the presence of endocardial fibroelastosis (EFE) or dilated cardiomyopathy (DCM), which are other potential complications associated with CHB.

If CHB is detected in utero by a fetal electrocardiography (ECG) and echocardiography, your OB/GYN may prescribe an adrenocorticosteroid such as dexamethasone, which works to decrease inflammation and the number of circulating maternal antibodies in the fetus.

Once born, other studies have shown that between that 64 and 70% of CHB survivors require surgery to permanently implant a pacemaker, a medical device which stimulates the heart to contract so that it can pump blood.

How to Prevent Congenital Heart Block

A 2016 report by the American College of Rheumatology states that there are no official guidelines about the prevention, screening, and treatment of CHB due to maternal Ro antibodies.

However, in the same report, it was stated that in a survey of 330 women with autoimmune conditions, 67% were told by their rheumatologists to use hydroxychloroquine (also known as Plaquenil) to prevent CHB. In addition, 62% were told to start the drug prior to pregnancy, in order to prevent the condition from developing.

Another study published in The Journal of the American College of Cardiology stated that hydroxychloroquine reduces the recurrence of CHB below the historical rate by more than 50%, further demonstrating the promise of this drug in the prevention of CHB.

Have you or someone you love been affected by congenital heart block (CHB)? Let us know in the comments below!

Autoimmune Patients Urge Public to Take Coronavirus Seriously

Close-up image of the coronavirus (COVID-19) from the Centers for Disease Control & Prevention.
Image of the coronavirus (COVID-19) courtesy of the Centers for Disease Control and Prevention (CDC).

The coronavirus (COVID-19) pandemic has taken the world by storm, causing what many perceive to be a case of mass hysteria. But for those living with a compromised immune system, the ‘hysteria’ is not unwarranted.

Amber Beckley, a 33-year-old mother from Sandusky county, Ohio, suffers from a rare autoimmune condition called common variable immunodeficiency (CVID). The condition makes her bone marrow unable to produce the antibodies that fight against infections. As a result, she’s terrified of contracting the coronavirus, since she’s a high-risk patient.

“My immune system is at five percent,” said Beckley. “If I caught it, even with treatment from doctors and hospital and ICU, me fighting it off is just not going to happen.” 

Amber Beckley, a 33-year old mother, suffers from CVID, leaving her at-risk for death if she contracts the coronavirus.

Beckley also added that the only way to treat her condition is to get an infusion of antibodies from healthy patients, a treatment she’s been receiving for the last seven years. Unfortunately, her nurse has advised her against leaving the house, to reduce her risk of being exposed to COVID-19 – as a result, she can’t get the life-saving treatment she needs. She also thinks that healthy patients aren’t taking the disease seriously enough.

That’s a position with which Angela Michelle of San Antonio, Texas agrees. Michelle suffers from an autoimmune disease as well – antiphospholipid syndrome (APS), which causes clotting in her arteries and veins. The condition has caused her to have a stroke, and also affects her lung function by causing her to develop pulmonary hypertension. Having a lung disease puts her at an even greater risk if she were to catch the coronavirus, since the virus is respiratory in nature.

“I think it’s been really disheartening for us to see the general public not take it as seriously as we feel like it should be because they don’t think it’s going to affect them. And for us, it does affect us,” she said.

Angela Michelle of San Antonio, Texas, is an antiphospholipid syndrome patient who feels the public isn’t taking the coronavirus as seriously as they should.

Michelle had a medical procedure planned in San Diego, California, but since her flight was cancelled, she’s no longer able to have the procedure done. What really worries her is that medical facilities may become so overwhelmed dealing with the outbreak, that they won’t be able to effectively treat her should she get infected.

Heather Millen, a 42-year-old from Brooklyn, New York, has multiple sclerosis (MS), an autoimmune condition that damages the myelin sheath coating one’s nerves in the brain and spinal cord. She feels that she and others with compromised immune systems have been brushed off by media and politicians alike.

“I feel like people with MS and other people who are high risk are constantly being dismissed,” she lamented. “I feel like the coronavirus is being so underplayed.”

Heather Millen (R) pictured here with her sister (L), an autoimmune disease patient who passed away from H1N1 in 2009.

Millen’s own sister, Denise, also suffered from an autoimmune disease, but passed away when she contracted the H1N1 virus (also known as the ‘swine flu’) back in 2009. Seeing the global health crisis now brings back terrible memories of watching her sister’s body shut down.

“Any time people with MS get any kind of infection, it can be a trigger for their symptoms and make them worse,” said Amesh Amalja, MD and infectious disease expert at the John Hopkins Center for Health Security. “Many are on immune-suppressing medications…So if they do get infected, it could be severe.”

Dr. Murray Cohen, an epidemiologist, adds that it’s important to assess your personal risk for the disease, especially since even mild cases of the disease could lead to pneumonia.

“When we have pulmonary disease, coronary disease, autoimmune diseases — we’ve got no way to fight this virus since there is no treatment,” Dr. Cohen explained. “The only defense you have if you get infected is your immune system fighting that virus. One of you is going to win, and one of you is going to lose.”

That’s why it’s extremely important that even if you’re not high-risk for succumbing to the coronavirus, that you take precautions to help prevent the spread of the virus to those who are immunocompromised. Ultimately, ‘being seen’ is what autoimmune disease patients like Millen want.

“Those people that are being discounted by every news program and government official, they’re people. What about those people?” she countered.

Thank you for reading! If you’re an autoimmune disease patient, what precautions are you taking against the spread of COVID-19? Let us know in the comments below!