When Jessica Johnson, 36, began to experience labored breathing and heart palpitations, she quickly made an appointment to see a cardiologist. A CT scan showed she had two aortic aneurysms that could potentially require surgery.
As a child, Jessica was diagnosed with Marfan syndrome – a rare genetic disorder that affects the connective tissue in the body and can impact various systems, including the skeletal, cardiovascular, and ocular systems. But until then, she never had issues with her aorta, the main artery that carries blood from the heart to the rest of the body. In people with Marfan's disease, an aneurysm, or ballooning, can occur in the wall of the aorta and, if left untreated, can lead to a potentially deadly dissection or rupture.
Just days after her diagnosis, she received the most surprising news of all – she was expecting her first child.
"To get the news that I was needing aortic surgery and now I was pregnant, it was all so intense," she said.
That was the beginning of Jessica's journey to UTHealth Houston.
She reached out to physicians in the Canyon Lake area near San Antonio where she lived for prenatal care, but no one would establish her as a patient because she was considered too high risk due to her genetic condition.
"I went to four or five physicians who all confirmed that I was pregnant, but they told me they didn't think I would survive the pregnancy because my heart wasn't in good shape. It was the most helpless feeling," Jessica said.
In the middle of it all she lost her brother, who also suffered from Marfan syndrome, to an aortic dissection.
"After losing my brother, I really felt like I needed to fight for this pregnancy. I felt like it was worth the risk," she said.
She finally met with a physician who put her in touch with Rana Afifi, MD, a vascular surgeon with UTHealth Houston Heart & Vascular, who is considered an expert in the management of young women with aortic diseases during pregnancy.
"I remember getting a call from Dr. Afifi, and she told me I needed to make my way to Houston right away. I looked at my husband and we knew we found our team," she said.
"Jessica was referred to me by her physician in Austin. I wanted her to be evaluated by our team who specializes in complex aortic diseases and specifically in pregnant women with aortic disease," said Afifi, who is an associate professor of vascular surgery in the Department of Cardiothoracic and Vascular Surgery with McGovern Medical School at UTHealth Houston. "I was worried that she could be developing complications that may be life threatening, and I knew we would not have been able to manage her without seeing and evaluating her. I wanted to examine her, go over her imaging, and then decide together with our team of experts and Jessica on our plan for her care."
"When we arrived in Houston, she had an entire team waiting for us. She was a beacon of light in such a complicated and difficult time," Jessica said.
Jessica was introduced to Baha Sibai, MD, professor in the Department of Obstetrics, Gynecology, and Reproductive Sciences with McGovern Medical School.
When I first met Jessica, she was 16 weeks pregnant. I explained to her and her family that she had a life-threatening condition and would potentially face many challenges throughout her pregnancy given the physiological changes her body would go through, but I assured her that she was in the best medical center that cares for high-risk cardiac and obstetric patients."
Baha Sibai, MD, attending maternal-fetal medicine physician at Memorial Hermann-Texas Medical Center
Both Afifi and Sibai spent the next few hours explaining the potential risks Jessica and her husband might face for both Jessica and their unborn son. For the first time since finding out they were growing their family, both Jessica and her husband felt supported.
"Losing this pregnancy was never an option for us, and for the first time, we received nothing but support for that decision. The entire team took us in and told us they were going to do everything they could to not only keep me safe, but my son too," Jessica said.
Jessica spent the next few months in Houston in and out of the hospital for monitoring.
On October 21, 2023, two months before her original due date, hand-in-hand with Afifi, and surrounded by a team led by Sibai, Jessica delivered her son Damian via cesarean section.
A week after delivery, tests showed the size of her aortic aneurysms had remained the same.
According to Afifi, the aortic size may change during pregnancy due to increased blood flow. During pregnancy, it is more common and risky if there is a growth in the aortic diameter because it increases the risk of tearing in the inner layer of the aorta, causing dissection or rupture, which can be life threatening.
After delivery, the size of the aorta may remain the same, decrease in size, or enlarge, which is why continued follow-up is important.
"This story highlights the critical importance of having a specialized team to manage young women with aortic disease, particularly those who are pregnant or planning to become pregnant," said Afifi, who is an attending heart and vascular surgeon at Memorial Hermann-TMC. "Each case presents unique challenges, requiring an individualized care plan. Some patients may need aortic repair before or during pregnancy, while others may face risks that necessitate alternative counseling and decision-making. Because these cases are rare, available data is limited - making careful documentation and expert management even more essential. That's why referral to a specialized center, such as the one we have here at UTHealth Houston, is so important."
The family is still awaiting his test results to determine if Damian has Marfan syndrome.
"Damian is truly a miracle baby," Jessica said. "He is hitting all his milestones; he has no physical or mental delays. He is a happy-go-lucky baby, and I couldn't ask for a better situation. As far as I go, I'm doing well. I'm in very good spirits. I have a lot of support, not just through the doctors that took care of me, but my family and friends have been incredible."
Jessica and her family have since moved to Utah. She is currently in a wheelchair due to bone deterioration and hip pain. She will travel to Houston and continue her care with Afifi where she will be closely monitored. If Afifi notes an increase in the size of the aneurysm, she will undergo aortic repair surgery.
"I will always travel back to Houston for my care, because I know I am in good hands. It's challenging raising a child being wheelchair-bound, but mentally I am in a really great place, and I am taking everything one day at a time," Jessica said.
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