A Routine Scan Uncovers a Life-Saving Aortic Dissection Linked to Marfan Syndrome

In August 2023 the author postponed a scheduled echocardiogram that was set for August 6. A follow-up CT scan on August 17 revealed that her ascending aorta had enlarged to 5 cm, prompting an emergency call to the ER. She was diagnosed with an aortic dissection and underwent a Bentall procedure the next day, replacing the aortic valve with a mechanical prosthesis and repairing the ascending aorta with a graft. The author describes the postoperative period as “extremely difficult,” noting that she awoke intubated and was later extubated after intensive care.

Newsweek

The author’s father, Bert Harvey Veen, died on September 7, 2010, at age 55 from an aortic dissection that extended from his heart to his legs despite open-heart surgery. After her own pregnancy-related symptoms, the author was referred to a cardiologist and a geneticist. Genetic testing identified a mutation in the FBN1 gene, confirming Marfan syndrome—a connective-tissue disorder that explains her tall, slender build, long limbs, and vision issues, as well as her father’s condition.

Newsweek

According to the author, Marfan syndrome affects roughly one in every 5,000 people and carries a 50 percent chance of being passed to each child.

Newsweek

Following the surgery, the author has been monitored with bi-weekly echocardiograms during pregnancy, annual MRIs in January, and echocardiograms in July. She notes that routine imaging will be a lifelong requirement because her descending aorta remains within normal range but could change. The diagnosis prompted broader family testing: her aunt was found to have the condition, her daughter tested positive, and her son tested negative.

Newsweek

The author plans to maintain regular cardiac imaging and follow-up appointments to track aortic dimensions, emphasizing that early detection and monitoring are critical for managing Marfan-related vascular risks.

Newsweek