By Pirooz Eghtesady, MD, PhD; R. Mark Grady, MD
Introduction
Children with severe pulmonary hypertension (PH) who have failed medical therapy have limited treatment options and often die from right heart failure. A surgical procedure known as a Potts shunt is an old technique, originally developed for “blue babies,” that is showing promise as an alternative therapy for treating children with severe, drug-resistant PH, possibly delaying or even negating the need for lung transplantation. A Potts shunt is a side-to-side connection from the left pulmonary artery to the descending aorta, resulting ideally in a right-to-left shunt in a child with suprasystemic right heart pressures (Figure 1). The 1940s version was the reverse and was created for children who didn’t have enough pulmonary blood flow. This new twist on the procedure diverts de-oxygenated blood down the descending aorta, reducing pressures within the right ventricle, and hopefully preserving its function.
The first case series published using the Potts shunt in children with severe PH came from the French group in 2012 (Baruteau et al.) Their initial and subsequent experience demonstrated that children with severe disease treated with a Potts shunt showed significant and sustained clinical improvement often with a reduction in onerous medical therapy. They theorized that by reducing right ventricular pressures to systemic levels, they would recapitulate the same physiology seen in Eisenmenger’s Syndrome (ES) and hopefully confer the same superior survival rates seen with Eisenmenger patients, which has been reported as for long as a median of 30-40 years (Kempny et al. Eur Heart Journal, 2017). While such long-term survival remains to be seen, the French group has cohorts of children post-Potts shunt who have sustained their improvement beyond the median survival of similar children undergoing lung transplantation (Baruteau et al 2014; Goldstein et al 2011). In 2013, we began applying this technique at St. Louis Children’s Hospital. We have thus far, the largest experience in North America for this modality of therapy (15 children to date). We recently performed the procedure on a four-month-old male patient, to our knowledge, making him the world’s youngest recipient of a “reverse” Potts shunt.
To read the full article, please go to the March 2018 Issue of CCT, where it was originally published.
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