Young Patient Reflects on ‘Whirlwind’ Diagnosis to the Novel Heart Surgery That Saved Her Life
After getting up for work one morning in October 2021, 27-year-old Allegra Campagna experienced an excruciating chest pain unlike anything she had ever felt before.
“I had this ripping, bursting feeling I described as like a flower blooming in my chest,” she recalled.
Then a clinical psychology graduate student living in Washington state, Campagna enjoyed an active lifestyle filled with hiking, ballet and other exercise. She never imagined she might have a serious health condition at such a young age.
When her symptoms did not resolve, she went to a local hospital, where doctors discovered a ruptured aneurysm in her aorta, the body’s largest artery. She was quickly transported by air ambulance to a hospital in Spokane for emergency surgery and was later diagnosed with Marfan syndrome.
That turbulent and frightening day changed the course of Campagna’s life. Now, nearly a year and a half later, she is grateful to the multidisciplinary team of experts at the Brigham who performed a lifesaving, complex and innovative cardiac procedure that has set her on the path to healing and getting back to what she loves most.
Marfan syndrome is a genetic disorder that occurs in about one in 5,000 people and affects the body’s connective tissue, which are the fibers that provide support for and anchor the organs, as well as other structures in the body. The condition can cause complications in the heart, blood vessels, skin, lungs and bones, among other organs. If the aorta is affected, as in Campagna’s case, Marfan syndrome can become life-threatening.
“That was a whirlwind,” she recalled. “I went from pretty normal and healthy to, ‘You have this genetic condition that affects multiple areas of your body.’”
Although Campagna’s emergency surgery in 2021 succeeded in saving her life, her diagnosis required a complete lifestyle change and monitoring to prevent another episode. According to the Marfan Foundation, patients with Marfan syndrome are strongly recommended to avoid strenuous physical activity in order to avoid injuring their heart.
Shortly after moving to Boston in June 2022 to begin her final year of training for her clinical psychology program, Campagna had another health scare when she experienced severe abdominal pain one day. She went to the Brigham’s Emergency Department (ED), and a CT scan showed that she had a second aneurysm in her aorta.
After being cared for by the ED’s multidisciplinary Code Aorta team, Campagna was referred to cardiologist Rajat M. Gupta, MD, of the Heart and Vascular Center, and Kim de la Cruz, MD, FACS, FACC, FCCP, section chief of Aortic Surgery in the Division of Thoracic Surgery, co-director of the Aortic Disease Center, to determine the most appropriate treatment.
“In someone with her condition, we recommend surgery to address an aortic aneurysm before there’s a rupture,” Gupta said.
De la Cruz evaluated Campagna for an open thoracoabdominal aortic aneurysm (TAAA) repair. The surgery involves replacing large portion of the aorta with a graft and reconnecting the smaller arterial vessels to the respective organs. In Campagna’s case, her aorta needed to be replaced almost completely from the chest to the abdomen, a section known as the thoracoabdominal aorta. Then, the blood vessels branching off it — which supply blood to organs like the liver, kidneys, stomach, intestines and lower extremities — needed to be reattached.
“The normal size of the aorta is about an inch wide. An aneurysm is any abnormal enlargement of the aorta. An aortic aneurysm needs surgery when it grows to over two inches,” de la Cruz explained. “In Allegra’s case, her thoracoabdominal aorta grew rapidly to about the size of a soda can, meeting criteria for repair. Otherwise, without repair, her aorta is in danger of bursting or leaking out blood, and that causes pain, organ damage and death.”
A Safer Approach
Because patients with Marfan syndrome have soft aortic walls, this extensive surgery is the best approach, de la Cruz explained. Patients without this complication are usually better candidates for endovascular repair, which is a minimally invasive procedure that involves inserting a graft through blood vessels in the groin.
Still, open TAAA repair is not without risks. Because of the complex nature of the surgery, the risk of paralysis, renal failure and death after surgery is high.
De la Cruz is an international expert in a version of the procedure known as TAAA repair with left heart bypass, which has significantly lower rates of complications. De la Cruz was recruited to the Brigham for his expertise in performing this procedure, which he learned in Houston from one of the world’s most experienced and leading experts in it, Joseph Coselli, MD, FACS.
What makes this form of TAAA repair safer for Marfan patients, yet also far more complex to perform, is that it uses a left bypass, rather than the standard full bypass, de la Cruz explained. This allows the operating team to transfer blood to other parts of the aorta not being worked on during the surgery. It results in far less bleeding, a reduced dose of blood thinners and a significantly lower rate of complications compared to the full bypass procedure.
The Brigham is the only hospital in New England to offer this approach.
“We’ve done four of these procedures in the hospital so far,” de la Cruz said, “and all of the patients have walked out of the hospital with good outcomes.”
Stronger Each Day
Although the prospect of heart surgery was daunting, Campagna said her team’s honest communication and patient-centered approach alleviated many of her worries. She underwent the procedure in November 2022.
“Dr. de la Cruz was really good at explaining what was going on,” Campagna said. “He had goals for every day — stabilize blood pressure, increase hemoglobin level — written on the whiteboard in my room, and it felt very collaborative. I really appreciated that.”
The surgery left Campagna with a scar across her stomach, left ribcage and back, and a painful healing process immediately after. Despite the initial challenges, Campagna said her strength continues to improve, and she remains grateful for the support of her loved ones.
“I know it sounds like cliche, but you definitely have your ups and downs,” Campagna said. “I have to remember that, on average, I’m doing a lot better than I was last week, and I’m doing better today than I was yesterday.”
Now four months out from surgery, Campagna must return for yearly follow-ups to ensure her heart and aorta remain in good condition. While she remains cautious about placing undue strain on her body as it continues to heal, she has been cleared to resume all the activities she enjoyed before, including hiking, walking and ballet.
Gupta expressed his admiration for Campagna’s optimism throughout her diagnosis and treatment.
“She’s just really a remarkable person who has been a pleasure to take care of,” Gupta said. “I think she’s been through a lot, yet she’s always had really good attitude and has been eager to get better and help others with Marfan Syndrome.”