Every year, 100,000 babies don't make it to their first birthday because of CHD.
Johanna Sarnstrom

Age: 27

Birthday: August 1st

Defect: Ebstein’s Anamoly

Surgeries: Too many to count (25+)

Her best attribute? Never giving up in her fight against CHD

Fun Fact: She loves LeAnn Rimes. She has been to 22 of her live shows and has met her 20 times.

Johanna was born cyanotic (blue fingers, toes, and lips). Unable to determine a diagnosis right away, Doctors told her parents she only had a few days to live, if they were lucky. A diagnosis came 16 hours later after being taken to Children’s Hospital by an ambulance – Ebstein’s Anomaly.

Her first open-heart surgery occurred when she was 5 years old in July of 1996; surgeons tried (without success) to repair her damaged valve, so they used a pig’s valve instead. 5 years later, her body started to reject this valve so a balloon angioplasty was performed to try to open up the valve to buy some more time. Unfortunately this did not work and she had a mechanical heart valve surgery in December of 2002. She went into complete heart block shortly after that operation and a pacemaker was implanted just a few weeks later. It was soon after this operation that she developed a deadly infection called pseudomonas due to the fact that the surgeon had forgotten to remove a pacemaker lead. This infection caused destruction all over her body; in a 5-year period she underwent over 22 surgeries. These surgeries included multiple hernia repairs, mesh implants, partial sterum and muscle removal, and many others. With the infection wreaking havoc on her body for so long, her options for survival began to dwindle by the time 2007 came around, doctors gave her only a few months to live. Luckily, another hospital was willing to take a look at her case - they performed a variety of tests and determined the safest plan was to go through her back to remove the lead.

Despite the infection, all the surgeries, and odds stacked against her, Johanna pulled through and continues her fight with CHD to this day. She takes blood thinners to accommodate her mechanical heart valve and beta blockers for her irregular heart rhythm; both medications she will need for life. She cannot play contact sports and lives a life of extreme caution due to risk of injury that could be fatal as a result of her heart condition and the fact that she is on blood thinners. Although she has no surgeries scheduled at this time, there is always a possibility she will need more. There is a chance she will need a heart transplant in the future as Ebstein's can lead to heart failure.

Johanna says she constantly worries, she says it is a struggle to have to think about how everything she does might affect her heart. She must always factor that into everything she does, but adds, “Each new day I am here is a blessing to me.

About Johanna’s Defect

ebstein's anomaly

Anatomy: With Ebstein’s Anomaly, the leaflets of the tricuspid valve are abnormally formed and sit lower in the right ventricle than normal.

Complications: Due to this malformation, blood may leak back through the valve, making the heart work less efficiently and leaving the body with low oxygen saturations. It may also lead to enlargement of the heart or heart failure.

Symptoms: Mild forms may cause no symptoms but patients with more severe cases may experience shortness of breath - especially with exertion, fatigue, heart palpitations or abnormal heart rhythms (arrhythmias), and a bluish discoloration of the lips and skin (cyanosis).

Treatment: Depending on the severity of the case, Ebstein’s Anomaly can be treated with medications, surgery, or heart transplant.

Prevalence: This is a very rare defect, occurring in about 1 out of every 20,000 live births, affecting boys and girls equally.

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