Transitions in Adolescent MedicineCommentary by Megumi J. Okumura, MD, MAS, and Roberta G. Williams, MD
A 21-year-old woman with a history of Marfan syndrome complicated by aortic root dilation presented to the emergency department with abdominal pain and was found to be pregnant. This was her second pregnancy, as she had had a therapeutic abortion 4 years previously. At that time, the patient was being followed by a pediatrician and a pediatric cardiologist. They had advised her of the high-risk nature of the pregnancy, including a 10% chance that she could have an aortic rupture during pregnancy. She decided to terminate the pregnancy and was advised that she should undergo surgical repair of her aortic root in the near future. Shortly thereafter, she turned 18 years old and was referred to an adult primary care physician and cardiologist for continued care. The patient never saw her new physicians and was never scheduled for the recommended cardiac surgery procedure. In fact, over the next 3 years she did not receive regular follow-up care at all, although she remained relatively healthy. She also never received pre-conception counseling or contraception, despite the risk to her health should she become pregnant.
Upon learning of the current pregnancy, the patient decided against another abortion. She was evaluated by adult cardiology and cardiac surgery, and she was advised to undergo an aortic root repair during her second trimester in order to minimize potential harm to the fetus while preserving her own health. Understandably, this decision caused significant anxiety for the patient and her family. She did undergo the procedure without complications and proceeded to have a healthy child at full term.