3/11/2017. Different types of anastomoses. Therapeutic algorithm in pediatric PAH. Potts shunt in pediatric pulmonary arterial hypertension

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1 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 Taussig Thomas Modified Blalock Deleval Potts shunt Waterston Potts Ivy DD et al. JACC

2 Lung transplantation in children (Lung Transplantations: january 1990 june 2012) First Potts case in pediatric PAH in 2004 p = Pediatric PAH Median survival 5.8 years Death on list 7/26: 27% Operative death/acute graft failure 22% Median survival (years): Adult = 5.6; Pediatric = JHLT Oct; 33(10): Goldstein BS et al. J Heart Lung Transplant 2011 Blanc J, Vouhé P, Bonnet D. NEJM 2004 Stenting of tiny arterial duct in PAH Before PDA stenting After PDA stenting Parisian series Mortality 15 % 36 children: 19 surgical- 7 stenting of tiny ducts- 10 percutaneous 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

3 Outcomes after Potts shunt Pros and cons Pros Improvement of WHO-FC Convert ipah in Eisenmenger syndrome supposed to have a better outcome Wean patient from IV/SC prostanoids Improvement of RV function Cons High mortality of the procedure Prognosis of Eisenmenger syndrome might not be so good 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) Baruteau A et al. Eur J Cardiovasc Surg 2015 Boudjemline Y et al. Circ Cardiovasc Interv 2013 Baruteau A et al. Ann Thorac Surg 2012 Immortality bias in Eisenmenger syndrome Pros and cons Pros Improvement of WHO-FC Convert ipah in Eisenmenger syndrome supposed to have a better outcome Wean patient from IV/SC prostanoids 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) Cons High mortality of the procedure Prognosis of Eisenmenger syndrome might not be so good Simple palliation not a cure of PAH Risk of polycythemia Might increase the risk of bleeding during a subsequent transplantation Boudjemline Y et al. Circ Cardiovasc Interv 2013 Baruteau A et al. Ann Thorac Surg

4 When to do a Potts shunt? Potts shunt at time of clinical worsening in children receiving triple therapy? Potts shunt? Potts shunt Ziljstra W et al. JACC 2014 Earlier? When prostanoids are indicated? Before right heart failure Place of Potts shunt in the treatment strategy 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 Animal model of valved Potts Bui MT et al. JTCS 2011 Paradela M et al. JTCS 2013 Latus H et al. J Heart Lung Transplant 2014 Bhamra-Ariza et al. JACC 2014 Bui MT et al. JTCVS 2011;142: Bui MT et al. JTCS 2011 Paradela M et al. JTCS 2013 Latus H et al. J Heart Lung Transplant 2014 Bhamra-Ariza et al. JACC

5 s Initial experience Technical aspects 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 Optimization of perforation site Morphometric measurements on CT angiograms Bronchial arteries between PA and Aorta Pre Contact area between PA and Aorta Cath Lab simulation Sizarov A et al. Heart

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7 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 Berger RMF & Bonnet D, Eur Respir Rev

8 M3C-Pediatric PH group Younes Boudjemline Marilyne Lévy Pascal Vouhé Olivier Raisky Diala Khraiche Magalie Ladouceur Isabelle Szezepanski Aleksander Sizarov Damien Bonnet 8