Sarah Pickard, MD, clinical fellow in cardiology at Boston Children’s Hospital, is taking a new look at the long-term risk of stroke and coronary artery disease in patients with coarctation of the aorta (CoA) under the mentorship of Ashwin Prakash, MD.

Pickard presented an oral abstract of her research at the American Heart Association (AHA) scientific sessions in November 2017: “Stroke and Coronary Artery Disease in Adults with Coarctation of the Aorta: A National Population-based Study.” For this research, Pickard was selected as a finalist for the AHA’s 2017 Cardiovascular Disease in the Young Early Career Investigator Award, and was awarded a Matthew’s Hearts of Hope grant as well as the Boston Children’s Hospital Alumni Association’s Trainee Travel Award.

Pickard, second from right, and Sarah de Ferranti, MD, MPH, right, at the AHA’s 2017 Scientific Sessions

“We knew that the long-term survival in patients with CoA is generally lower than that of the general population, and that two etiologies of reduced long-term survival are stroke and coronary artery disease,” says Pickard. “But there hadn’t been a contemporary-era evaluation of stroke or coronary artery disease (CAD) in patients with CoA in the U.S. Our primary question was: are patients with CoA still suffering stroke and CAD at a younger age than the general population?”

They discovered the answer is “yes.”

Utilizing the National Inpatient Sample database from 2005-2014, they found that among 4,894,582 stroke discharges, 207 had a diagnosis of CoA. “We found that patients with CoA suffered strokes at significantly younger age compared to patients without CoA,” says Pickard. Specifically, 18.7 years younger for all-cause stroke 15.7 years younger for ischemic stroke, and 28.2 years younger for hemorrhagic stroke.

“We were especially surprised by the age at which patients were affected by hemorrhagic stroke,” says Pickard. “We expected they would be younger because of the risk of intracranial aneurysms, but we had no idea it would be so much younger.”

The results were similar, though not as marked, for CAD. “In our preliminary data, we found that patients with CoA also suffered CAD earlier than the general population, but we need further analysis to better understand that.”

Looking ahead, Pickard hopes to use this research to better understand how to manage patients with CoA as they age, as well as how to best provide counseling about long-term prognosis for families.

“It remains a question how to best prevent premature stroke. Our guidelines tell us to screen these patients for intracranial aneurysms, but we don’t know how often to screen or when to screen,” she says. “Our next step is to try to determine optimal screening strategies for adults with CoA.”

Pickard believes this research has larger implications for all patients with congenital heart disease. “It highlights the fact that we need to be studying long-term issues that affect our patients with congenital heart disease, even those who seem to have been adequately treated in childhood and don’t seem to have residual lesions. We need to better understand how their heart disease will affect their long-term survival and morbidity.”

She says that the research also further highlights the need to do more as clinicians to keep these patients under the care of a cardiologist who understands the specific risks of CHD. “Many of our patients think of themselves as cured and there’s significant loss of follow-up as they transition into adulthood.”