Annie’s Story: Pulmonary Vein Stenosis Program Provides a ‘Hope that is Everything’

January 31, 2023

Annie was born prematurely at 26 weeks in February 2018, weighing in at only 1 pound and 6 ounces. After three months in the Neonatal Intensive Care Unit (NICU) at Prentice Women’s Hospital, she was transferred to Lurie Children’s NICU, where a team of specialists was ready to diagnose and treat the critical conditions affecting her heart and lungs.

To help Annie breathe, her parents, Dan and Ashley, and doctors agreed to put her on a ventilator. “It was a very hard decision for us because it was a life-changing thing,” Dan said. “But then again, we were already in a life-changing situation. We looked for second opinions, and everyone we spoke to kept agreeing: Lurie Children’s is the best place to be.”

After a battery of tests, doctors concluded Annie had pulmonary hypertension. In Annie’s case, pulmonary hypertension was caused by a rare and serious condition known as pulmonary vein stenosis. This condition causes the veins that connect the lungs to the heart to become narrowed, limiting the flow of oxygenated blood out of the lungs and to the heart.

Though pulmonary vein stenosis is historically an under-researched medical condition, Lurie Children’s team is committed to advancing understanding and improving treatment for the youngest patients with the condition.

To provide careful monitoring and specialized treatment for critically ill patients like Annie, Lurie Children’s offers a multidisciplinary Pulmonary Vein Stenosis Program. The team includes cardiologists with expertise in pulmonary hypertension, cardiac imaging, and interventional cardiology, neonatologists with expertise in lung disease, and cardiac surgeons.

Through the team’s dedication, led by Dr. Amanda Hauck and Dr. Paul Tannous, patients like Annie now have a much higher rate of survival.

To expand her narrowed veins and improve blood flow through her lungs, Annie underwent the first of multiple cardiac catheterization – or ‘cath’ – procedures with Dr. Tannous and his team to dilate and stent her pulmonary veins. She also began treatment with medications to manage the pulmonary hypertension and clear fluid from her lungs. Dr. Hauck closely monitored Annie’s pulmonary vein stenosis with echocardiograms and other tests to know when she might need more treatment or another catheterization to keep her pulmonary veins open and her heart and lungs healthy.

Ultimately, Annie spent 11 months in the NICU before joining her parents and big brother, Elliott, at home. To keep her healthy and strong, her parents learned to operate her respiratory therapy technology and administer her medications on their own at home. Annie also began a course of intensive therapies to help her build strength and hit key developmental milestones.

At three years old, Annie’s lungs were healthy enough and surgeons removed Annie’s tracheostomy – a major operation involving reconstruction of her airway. Post-surgery, she was put into a medical coma for two weeks so that she could fully heal.

Today, as she nears her fifth birthday, Annie enjoys the hallmarks of a healthy childhood: She loves attending school, singing, dancing and running. She continues to be followed by the Pulmonary Vein Stenosis Program team at Lurie Children’s.

“These days when I go to lay next to her and she’s breathing calmly, deeply and quietly, it’s so different than it used to be,” Dan said. “To have this hope that we’re really over the hill is everything.”

“The staff at Lurie Children’s is how we are able to have our family. We are very grateful.”