Potts Shunt in a Child With End-Stage Pulmonary Hypertension After Late Repair of Ventricular Septal Defect

Abstract

We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology with right-to-left shunting. The shunt could be created either as an intracardiac or as an extracardiac shunt. We decided to create a Potts shunt, a direct anastomosis between the left pulmonary artery and the descending aorta. The Potts shunt functioned as a right-to-left shunt, thus reducing the afterload on the right ventricle. The boy’s clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart–lung transplantation, but because of the short life span after LTX, time was bought by postponing the time of transplantation.

Keywords

congenital heart surgery heart failure operations palliation pediatric pulmonary vascular resistance/hypertension shunts (Potts shunt)transplantation heart–lung lung

Get full access to this article

View all access options for this article.

References

Haworth

Hislop

. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart. 2009;95(4):312–317.

Benden

Aurora

Edwards

The registry of the International Society for Heart and Lung Transplantation: fourteenth pediatric lung and heart-lung transplantation report—2011. J Heart Lung Transplant. 2011;30(10):1123–1132.

Hopkins

Waggoner

. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am J Cardiol. 2002;89(1):34–38.

Potts

Smith

Gibson

. Anastomosis of the aorta to a pulmonary artery. J Am Med Assoc. 1946;132(11):627–631.

Blanc

Vouhe

Bonnet

. Potts shunt in patients with pulmonary hypertension. New Eng J Med. 2004;350(6):623.

Labombarda

Maragnes

Dupont-Chauvet

Serraf

. Potts anastomosis for children with idiopathic pulmonary hypertension. Ped Cardiol. 2009;30(8):1143–1145.

Baruteau

Serraf

Levy

Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg. 2012;94(3):817–824.

Hopkins

Ochoa

Richardson

Trulock

. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15(1 pt 1):100–105.