«Increased number of uni-ventricular hearts in adulthood. Are we ready?»

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1 «Increased number of uni-ventricular hearts in adulthood. Are we ready?» Augmentation du nombre de coeurs univentriculaires à l'âge adulte. Sommes nous prêts? P. Guérin L institut du thorax Nantes

2 Increased number of uni ventricular hearts in adulthood? Are we ready? Who? For what?

3 Paediatric survival? Moons et al. Circulation. 2010;

4 Paediatric survival? Moons et al. Circulation. 2010;

5 Increased number? Marelli et al. Circulation. 2007;115:

6 Increased number? Marelli et al. Circulation. 2014;130:

7 Lost of follow up in adults? Mackie et al. Circulation. 2009;120: )

8 Lost of follow up in adults? Mackie et al. Circulation. 2009;120: )

9 Lost of follow up in adults? Mackie et al. Circulation. 2009;120: )

10 What about termination of pregnancy in fetus with functional UVH? 13% 87% L. Idorn et al. Inter J Cardiol 167 (2013)

11 What about termination of pregnancy in fetus with functional UVH? Impact of antenatal screening L. Idorn et al. Inter J Cardiol 167 (2013)

12 What about termination of pregnancy in fetus with functional UVH? Impact of antenatal screening 20 year old in 2023 L. Idorn et al. Inter J Cardiol 167 (2013)

13 What about termination of pregnancy in fetus with functional UVH? Impact of antenatal screening Effect in GUCH population in 2040 L. Idorn et al. Inter J Cardiol 167 (2013)

14 Increased number of uni-ventricular Hearts in adulthood (at least until 2040

15 Ready for what? Ready for the follow-up? Ready to face long-term Complications in the Adult With Fontan Physiology?

16 Long-Term Complications in the Adult With Fontan Physiology Functional Status and Exercise: Maximal exercise capacity at baseline is lower than expected compared with healthy individuals and progressively declines (Paridon et al; JACC 2008; 52: Anderson et al. JACC 2008; 52: 85 98) Pulmonary vascular resistance: PVR and over time will lead to PA hypertension (Fredenburg et al. Radiographics 2011; 31: ) Arrhythmias: Atrial arrhythmias affecting more than 50% of patients. (Collins et al. Congenit Heart Dis 2009; 4: ) Fontan Failure Hypoxemia/Shunts Plastic Bronchitis

17 Long-Term Complications in the Adult With Fontan Physiology Thromboembolism: Commonly encountered complication of the Fontan circulation, occurring in up to 20% of patients both early and late in the surgery. (Monagle et al. Sem Th Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 36 47) Liver Disease: Ranges from abnormal liver function tests and coagulation factors to fibrosis and even liver failure. (Kiesewetter et al. Heart 2007; 93: ) Protein-Losing Enteropathy: Occurs in 3.7% of patients with Fontan. (Mertens et al. J Thorac Cardiovasc Surg 1998; 115: ) Systemic Ventricular Dysfunction: especially in case of RV univentricular heart (McGuirk et al. Eur J Cardiothorac Surg 2003; 24: 37 46)

18 Long-Term Complications in the Adult With Fontan Physiology Interventional cardiology Rythmology Cardiac failure

19 Are we ready? Who? Congenitalist pediatrics? Adult congenitalists? Adult cardiologists? Ready? Ready today? Ready for the future?

20 Congenitalist pediatricians?

21 Congenitalist pediatricians?

22 Adult congenitalists? Dr. Superman. MD Specialist in : CHD Rythmology Interventional cardiology Cardiac failure Thromboembolism Transplantation

23 Adult cardiologists? Dr. Superman. MD Specialist in : CHD Rythmology Interventional cardiology Cardiac failure Thromboembolism Transplantation How many of these «hyper specialists» do we need?

24 Adult cardiologists?

25 Adult cardiologists?

26 We need : Adult congenitalists cardiologists and we need Rythmologists, interventional cardiologists, specialists in heart failure, radiologists. with congenital education

27 We need it but will we get it? From Burdillat Martine, «Les nouvelles questions posées à la démographie médicale.», Regards croisés sur l'économie 1/2009 (n 5), p

28 We need it but will we get it? Quid of the effects of the french «hyper specialization» in CHD? Probably a positive effect for paediatric cardiology Probably a negative effect for GUCH From Burdillat Martine, «Les nouvelles questions posées à la démographie médicale.», Regards croisés sur l'économie 1/2009 (n 5), p

29 in adulthood : are we ready? Clearly NO! Thank you for your attention

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