Down Syndrome Medical Interest Group Friday, 12 June Cardiac Surgery in patients with Down Syndrome

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1 Down Syndrome Medical Interest Group Friday, 12 June 2015 Cardiac Surgery in patients with Down Syndrome Mr. Attilio Lotto, FRCS CTh Congenital Cardiac Surgeon

2 Cardiac surgery in patients with Down syndrome Which are the issues? Is DS a risk for modern cardiac surgery? Results from 2 large population base studies EMCHC 5 years experience cavsd repair: Down vs non Down

3 Which are the issues? DS 1 2 per 1000 births 45 50% of DS have CHD: Septal defects, ToF, PDA Life expectancy: 60 years BUT Pulmonary hypertension Airway problems, sleep apnea Hypoplastic lungs, ToeF, laryngo tracheomalacia Pediatrics International (2014) 56,

4 Pulmonary Hypertension in Down Syndrome Abnormalities in vasoactive substances Endogenous N.O. lower Poor response to inhaled N.O. Small calibre blood vessels (lung and body) Poor thickening of media intimal thickening cavsd: DS vs non DS: Controversy same haemodynamics (PVR response to O 2 )?PAH develop easier in DS Pediatrics International (2014) 56,

5 Postoperative PAH Inconclusive evidence DS pts: Higher risk of PAH Longer intubation time Higher infection rate

6 Is Down Syndrome a risk for modern cardiac surgery? Congenital Heart Surgery Outcomes in down Syndrome: Analysis of a National Clinical Database Pediatrics Volume 126, Number 2, August 2010 Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease Circulation Cardiovascular Quality Outcome 2014;7

7 Congenital Heart Surgery Outcomes in down Syndrome: Analysis of a National Clinical Database Pediatrics, Volume 126, Number 2, August 2010 Review of STS Congenital database pts operated for CHD between Down Syndrome pts (cavsd 33%, VSD 19%, MV 7%, pavsd 6%, PDA 4%, ToF 4%, ASD 4%)

8 Congenital Heart Surgery Outcomes in down Syndrome: Analysis of a National Clinical Database Pediatrics, Volume 126, Number 2, August 2010 Mortality rates did not differ DS vs. non DS DS pts operated for ASD, VSD, TOF had prolonged LoS and higher complication rate (infections, respiratory, PAH, PPM) DS cavsd pt had similar LoS to non DS Univentricular palliation (unbal AVSD) in DS pts had significant higher mortality for the 3 stages: Stage % vs 19.4% Stage % vs 1.8% Stage % vs 1.6%

9 Congenital Heart Surgery Outcomes in down Syndrome: Analysis of a National Clinical Database Pediatrics, Volume 126, Number 2, August 2010 Conclusions DS does not confer additional risk in dealing with the most common CHD lesions Univentricular heart with DS have significant increased mortality rates

10 Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease Circulation Cardiovascular Quality Outcome 2014;7 Review of Kids database for year 2000, 2003, 2006, pts operated in those 4 years 4231 pts had Down Syndrome Risk Adjustment for Congenital Heart Surgery (RACHS 1) increasing risk categories 1 to 6 1: ASD, Coarctation 4: ASO+VSD 2: VSD, pavsd, ToF, Glenn shunt 5: TA+IAA 3: cavsd, ASO 6: HLHS

11 Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease Circulation Cardiovascular Quality Outcome 2014;7 MORTALITY RACHS 1 category DS Non DS 1 1.7% 1.9% 2* 1.0% 1.8% 3* 2.3% 5.1% 4 5.9% 8.1% % 22.0% 1 6* 1.9% 4.3% *p <0.05

12 Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease Circulation Cardiovascular Quality Outcome 2014;7 RACHS 1 category 2 VSD closure in DS pts had lower mortality (0.3% vs 1%*) Glenn shunts in DS had higher mortality (15.6% vs 2.1%*) *p<0.05

13 Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease Circulation Cardiovascular Quality Outcome 2014;7 RACHS 1 category 3 Systemic to PA shunt in DS had higher mortality (16.8% vs 11%*) cavsd repair in DS had lower mortality (1.4% vs 4.6%*) MV repairs in DS had lower mortality (0.8% vs 5.2%*) *p<0.05

14 Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease Circulation Cardiovascular Quality Outcome 2014;7 After multivariate logistic regression analysis controlling for RACHS 1, preterm, age and other non cardiac anomalies a diagnosis of DS is associated with lower odds of in hospital death CONCLUSIONS Children with DS and corrected CHD are more likely to survive than children with out DS

15 Complete AVSD Incomplete development of: Septum primum Inflow Outflow of IVS Atrio ventricular valves

16 Types: Partial: Ostium primum Separated TV and MV AVSD Classification Complete: Ostium primum Common A V valve (5 Leaflets) VSD Intermediate: Ostium primum Common AV valve VSD obliterated by fibrous tissue

17 cavsd Treatment Palliation PAB Limited to: Weight < 4 Kgs Failure < 3 months Complex extracardiac diseases Single ventricle strategy

18 cavsd Treatment Correction Complete repair Aims: Close the vsd Close the asd Competent L AV valve Competent R AV valve Technique: Median Sternotomy Normothermic CPB Repair through the RA Bovine pericardium patch for VSD Autologous pericardium patch for the ASD Cleft repair with interrupted stitches

19 cavsd Treatment Two patch technique Bovine pericardium patch for VSD Autologous pericardium patch for the ASD Cleft repair with interrupted stitches

20 AVSD Results Influenced by: Closure of the cleft Preop AV V regurgitation Technique employed Down vs non Down Degree of postop L AV valve regurg Degree of AV valve dysplasia

21 AVSD Late problems LAVV regurgitation LVOT obstruction Residual VSD

22 EMCHC Experience: Jan 2010 May 2015 Among 1521 patients operated, 76 had a diagnosis of CHD + Down Syndrome CHD DIAGNOSIS AVSD: VSD: cavsd ToF: 4 Tetralogy: 3 ASD: 2 31 complete 4 intermediate 2 partial 1 unbalance (univ.) 16 (1 + arch) LVOTO: 4 (2 in previous AVSD) PDA: 3 MV regurg: 3 (post cavsd) CHB: 1 PV regurg 2 (post ToF) OVERAL MORTALITY: 2/76 (2.6%) 1 child with unbalance cavsd on the univentricular route died after TCPC 1 neonate with Ebstein anomaly had PDA ligation died 1 months for cardiac failure

23 EMCHC Experience: Jan 2010 May Patients with CHD + Down Syndrome Age distribution at time of operation Neonates (0 1m) 2 Infants (1 12 m) 47 Children (1 16 yrs) 23 Adults (>16 yrs) 5

24 EMCHC Experience: Jan 2010 May cavsd cases: 25 first time complete repair 4 complete repair after PAB (2 first time PAB) cavsd repair Previous PAB Patients 25 4 Age at repair (months) 7.5 ± ±5.2 Weight at repair (Kg) 5.7 ± ±1.7 Gender M/F 12/13 2/2 Antenatal diagnosis 12/25 1/4 Length of Stay (days) 9.5 ± ±6 Mortality 0% 0%

25 Outcomes and reoperations after total correction of complete atrio ventricular septal defect Ali Dodge Khatamia,*Stefan Hergera, Valentin Roussonb, Maurice Combera, Walter Knirschc, Urs Bauersfeldc, René Prêtre Division of Congenital Cardiovascular Surgery, University Children s Hospital, Zürich, Switzerland Eur J Cardiothorac Surg 2008;34:

26 Freedom from reoperation between Down and non-down patients. Dodge-Khatami A et al. Eur J Cardiothorac Surg 2008;34:

27 Freedom from reoperation with regards to age at initial correction. Dodge-Khatami A et al. Eur J Cardiothorac Surg 2008;34:

28 Freedom from reoperation with regards to weight at initial correction. Dodge-Khatami A et al. Eur J Cardiothorac Surg 2008;34:

29 The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair Ruediger Lange, MD, PhD, Thomas Guenther, MD, Raymonde Busch, MS, John Hess, MD, PhD, Christian Schreiber, MD The Journal of Thoracic and Cardiovascular Surgery Volume 134, Issue 2, Pages (August 2007) DOI: /j.jtcvs Copyright 2007 The American Association for Thoracic Surgery Terms and Conditions

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33 Thank you Questions?