3/11/2017 10th International Conference on Neonatal & Childhood Pulmonary Vascular Disease Potts’ shunt in pediatric pulmonary arterial hypertension Damien Bonnet M3C-Necker Enfants malades, Université Paris Descartes Centre de référence des Malformations Cardiaques Congénitales Complexes Centre de référence des Maladies Cardiaques Héréditaires ICarP, Institut Hospitalo-Universitaire IMAGINE, Paris, France JAMA 1946 Different types of anastomoses Therapeutic algorithm in pediatric PAH Blalock Modified Taussig Blalock Thomas Deleval Potts shunt Waterston Potts Ivy DD et al. JACC 2013 1
3/11/2017 First Potts case in pediatric PAH in 2004 Lung transplantation in children (Lung Transplantations: january 1990 – june 2012) p = 0.4400 Pediatric PAH Pediatric PAH Median survival 5.8 years Median survival 5.8 years Death on list 7/26: 27% Death on list 7/26: 27% Operative death/acute graft failure 22% Operative death/acute graft failure 22% Median survival (years): Adult = 5.6; Pediatric = 5.1 2014 JHLT. 2014 Oct; 33(10): 1025-1033 Goldstein BS et al. J Heart Lung Transplant 2011 Blanc J, Vouhé P, Bonnet D. NEJM 2004 Stenting of tiny arterial duct in PAH Parisian series Mortality 15 % • 36 children: 19 surgical- 7 stenting of tiny ducts- 10 percutaneous Before PDA stenting After PDA stenting • 6 deaths after procedure • 3 major postoperative complications – One transient paraplegia – One chylothorax – One tracheal stenosis requiring subsequent surgery • 31 survivors – One late death (stenting) : severe RSV infection – One transplantation for recurrent hemoptysis after percutaneous closure of the Potts’shunt Boudjemline Y et al. Circ Cardiovasc Interv 2013 Baruteau A et al. Ann Thorac Surg 2012 Baruteau A et al. Eur J Cardiovasc Surg 2015 Baruteau A et al. Eur J Cardiovasc Surg 2015 2
3/11/2017 Outcomes after Potts ‘shunt Pros and cons Pros Cons � Improvement of WHO-FC High mortality of the procedure • � Convert iPAH in Eisenmenger syndrome supposed to have a better outcome Prognosis of Eisenmenger • � Wean patient from IV/SC prostanoids syndrome might not be so good � Improvement of RV function � No death on the waiting list � 15 % mortality compared to 27 % on the waiting list + 22% in post-transplantation � Normal oxygenation of brain and coronary arteries (vs. Atrioseptotomy) Boudjemline Y et al. Circ Cardiovasc Interv 2013 Baruteau A et al. Ann Thorac Surg 2012 Baruteau A et al. Eur J Cardiovasc Surg 2015 Immortality bias in Eisenmenger syndrome Pros and cons Pros Cons � Improvement of WHO-FC High mortality of the procedure • � Convert iPAH in Eisenmenger syndrome supposed to have a better outcome Prognosis of Eisenmenger syndrome • might not be so good � Wean patient from IV/SC prostanoids � Improvement of RV function Simple palliation not a cure of PAH • � No death on the waiting list Risk of polycythemia • � 15 % mortality compared to 27 % on the waiting list + 22% in post-transplantation Might increase the risk of bleeding • � Normal oxygenation of brain and coronary during a subsequent transplantation arteries (vs. Atrioseptotomy) Boudjemline Y et al. Circ Cardiovasc Interv 2013 Baruteau A et al. Ann Thorac Surg 2012 3
3/11/2017 When to do a Potts’ shunt ? Potts’ shunt at time of clinical worsening in children receiving triple therapy ? Potts Potts shunt ? shunt Ziljstra W et al. JACC 2014 Animal model of valved Potts Place of Potts shunt in the treatment strategy • Earlier ? –When prostanoids are indicated ? –Before right heart failure –In all children with suprasystemic PAH on triple therapy ? • Enlarging indications –PAH associated with CHD – convert an Eisenmenger syndrome with intracardiac shunt in an Eisenmenger syndrome with PDA –Valved Potts’shunt in infrasystemic PAH with syncope • Potts as a bridge to lung transplantation • Improve technique and reduce immediate risk –Percutaneous procedure Bui MT et al. JTCS 2011 Bui MT et al. JTCS 2011 Bui MT et al. JTCVS 2011;142:1223-8 Paradela M et al. JTCS 2013 Paradela M et al. JTCS 2013 Latus H et al. J Heart Lung Transplant 2014 Latus H et al. J Heart Lung Transplant 2014 Bhamra-Ariza et al. JACC 2014 Bhamra-Ariza et al. JACC 2014 4
3/11/2017 Percutaneous Potts’shunt Percutaneous Potts’shunts Technical aspects Initial experience Esch JJ et al. J Heart Lung Transplant 2013 Schranz D et al EuroIntervention 2015 Schranz D et al. Eurointervention 2015 Boudjemline Y et al. submitted Percutaneous Potts’shunt Optimization of perforation site Bronchial arteries between PA and Aorta Morphometric measurements on CT angiograms Pre Cath Lab simulation Contact area between PA and Aorta Sizarov A et al. Heart 2016 5
3/11/2017 Percutaneous Potts’shunt Percutaneous Potts’shunt Percutaneous Potts’shunt Percutaneous Potts’shunt 6
3/11/2017 Towards dedicated devices Mathematic modelization of Potts’shunt Pressure Flow velocity Courtesy from Younes Boudjemline Sizarov A, Boudjemline Y, et al. In preparation Conclusion The Potts shunt international cohort survey Surgical, percutaneous, stenting of tiny PDA in pediatric PAH If you want to participate to this study, please contact Damien BONNET or Mark GRADY damien.bonnet@aphp.fr grady@kids.wustl.edu Berger RMF & Bonnet D, Eur Respir Rev 2010 7
3/11/2017 M3C-Pediatric PH group Younes Boudjemline Marilyne Lévy Pascal Vouhé Olivier Raisky Diala Khraiche Magalie Ladouceur Isabelle Szezepanski Aleksander Sizarov Damien Bonnet 8
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