Personal Stories

Window of Opportunity: A remarkable story of miracle and medicine

Aortopulmonary (AP) window: blood flows from the aorta into the pulmonary artery.

The bad news for the lovely young patient was that she was born and raised high in the mountains of Mexico, where medical care was rudimentary at best. No one there detected the heart defect present since birth: a gaping hole between her aorta and pulmonary artery that allowed blood to flow from one great vessel to the other. Left untreated, this aortopulmonary window, or "AP window," causes pulmonary hypertension and vascular disease, as well as irreversible heart and lung damage and premature death.

When the 22-year-old became pregnant and sought medical treatment in the U.S. in the fall of 2004, her physicians were justifiably alarmed. "Pregnancy in patients with pulmonary hypertension and pulmonary vascular disease is a very dangerous situation because of the tremendous changes in blood flow experienced during pregnancy," says Jonathan M. Chen, MD. "Many such women do not survive. In this case, her AP window was very large and it was remarkable that she had survived as long as she had, and especially given her pregnancy."

The good news was that she found the right medical care before she experienced a major problem. Recognizing the gravity of the situation, her first physicians referred her to the Pulmonary Hypertension Center at New York- Presbyterian Hospital/Columbia. At the Center, Drs. Robyn J. Barst and Erika Berman Rosenzweig worked closely with the obstetricians, anesthesiologists and surgeons to plan when and how to deliver the baby and repair the AP window. Dr. Jane Cleary Goldman delivered her healthy baby boy by Caesarian section under highly controlled conditions, with Dr. Chen and other cardiac surgeons standing by in the event that emergency surgery should be required. After a successful delivery that did not precipitate immediate heart surgery, she recovered for several days. Then, a team led by Dr. Chen performed open-heart surgery to repair her AP window.

The patient's pulmonary pressure has since improved, and after a two-week recovery in the hospital, she has returned home. "It is incredibly rare to see this condition in an adult," explains Dr. Chen. "The fact that she did not have irreversible heart or lung damage was remarkable." She will be monitored by Drs. Barst and Berman Rosenzweig and will receive specific treatment for residual pulmonary hypertension if needed.