30 Days of PH: A Potts Shunt Is Buying My Daughter Precious Time
Day 27 of 30
This is Pam Minkey’s story:
My daughter Mara was diagnosed with pulmonary arterial hypertension (PAH) in February 2017 at age 9. She had been misdiagnosed for several years until finally having an echocardiogram. Her echo suggested PAH, so she underwent right heart catheterization (RHC).
Her PAH was considered severe, and she was put on triple therapy.
Mara’s initial response to the meds was good and she improved, which gave us hope. We settled into this “new normal” for several years, but it didn’t last. Her RHC in 2020 showed increased pressures and worsening heart function.
Her doctor mentioned “lung transplant” and we were again in shock, but she also mentioned a Potts shunt.
The Potts shunt procedure creates a connection from the left pulmonary artery to the descending aorta, diverting some of the high-pressure blood from the lungs. It relieves some of the strain on the right heart, and preserves right heart function.
Traditionally, most pediatric PAH centers didn’t do this procedure, regarding transplants as the standard of care when medicines fail. But by 2020, things had started to change, with several PH centers now offering it. In four years, there was a true paradigm shift in pediatric PAH care.
We started both transplant and Potts shunt evaluations in 2021. The process was emotional and traumatic. Telling your child her medicines are no longer working and that we must consider transplant is a hard conversation. We proceeded with a three-day lung transplant evaluation, but we always kept the Potts shunt in the back of our minds.
We ultimately decided on it, and in July 2021, Mara had the procedure. She had complications and was re-hospitalized for almost three weeks, receiving treatment for right heart failure, among other problems. This was by far the scariest part of the PAH journey. She did recover and is now feeling better than she did before surgery.
The Potts shunt has bought us precious time, and the opportunity to see what new medical advancements are on the horizon.
For now, our daughter is stable and enjoying life and we are adapting again to a new normal.
Pulmonary Hypertension News’ 30 Days of PH campaign is publishing one story per day from someone who has been affected by the disease for PH Awareness Month in November. Read the full series for more stories like this, and follow us on Facebook and Instagram, using the hashtag #30DaysofPH.