When to close an Atrial Septal Defect (ASD) in adulthood?

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1 When to close an Atrial Septal Defect (ASD) in adulthood? Philippe ALDEBERT Hôpital de la Timone, CHU Marseille Département de cardiologie pédiatrique et congénitale médico-chirurgical

2 Abbott

3 Incidence Third most common type of congenital heart disease with an Estimated incidence of 56 per live births With improved recognition of clinically silent defects by echocardiography, recent estimates are about 100 per live births. 1.Hoffman JI et al. J Am Coll Cardiol 2002; 39: Botto Ld et al. Pediatrics 2001; 107: E32

4 Causes Most atrial septal defects are sporadic Familial clusters of secundum defects : NKX2-5 1, GATA4, TBX5, MYH6. Increased risk of secundum ASD in families with history of congenital heart disease, especially when present in a sibling 2 Trisomy 21 3 : secundum ASD =42% primum ASD=39% 1.Bjørnstad PG et al. Cardiol Young 2009; 19: Caputo S, et al. Eur Heart J 2005; 26: Freeman SB et al. Genet Med 2008; 10:

5 Anatomy (1) Tal Geva Lancet 2014; Lancet.383:

6 Secundum ASD-Anatomy - Within the fossa ovalis - Defects within septum primum - Septum secundum is wellformed in most patients. - Not confluent with : the vena cava right pulmonary veins coronary sinus atrioventricular valves. - The most common cause of an atrial-level shunt. Tal Geva Lancet 2014; Lancet.383:

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9 Primum ASD - Anatomy Incomplete Endocardial Cushion Defects = Primum Atrial Septal Defect = Partial Atrioventricular Canal = Partial Atrioventricular Septal Defect

10 Embryology 5 ème semaine de vie J 42 de vie Fermeture ostium primum 7 ème semaine de vie L.Houyel EMC 2009

11 Primum ASD- Elementary Lesions Primum ASD A common atrioventricular orifice with two distinct atrioventricular valve annuli completed by valve tissue adhering to the crest of the ventricular septum Valvular /sub-valvular anomaly of left atrioventricular valve ( LAVV). Sub Aortic root anomaly

12 Ostium Primum ASD

13 Common atrioventricular orifice and LAVV PQ Penkoske, RH Anderson, et al. JTCS 1985;90: S. Chauvaud. EMC ; 7(2):1-17

14 LAVV Cleft of LAVV towards admission IVS

15 Cleft and sub-valvular LAVV Anomaly Mitral LAVV M. Kanani, M. Elliot, R.H. Anderson, et al. JTCS 2006;132:640-6

16 Sub-Aortic Root Anomaly Fibro-muscular: -Unwedging -Mitro-aortic discontinuity -Longer outlet -aberrant chordae T. Ebels, RH Anderson, et al,ann Thorac Surg 1986;41:483-88

17 Sinus Venosus ASD ( SV)-Anatomy - 4 to 11 % of ASD - SV Superior : Communication between Right pulmonary vein and superior vena cava - SV inferior - Communication between Right pulmonary vein and inferior vena cava

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20 Physiopathology Right to left shunt : - Size of defect - Relative compliance LV/RV Shunt : of LV compliance = - LV hypertrophy / HTA /AS - Ischemic myocardium - RV enlargement with adverse ventricle interactions Valvular disease Abnormal Pulmonary Venous Return

21 Why should we close these ASDs?

22 Natural History Childhood adulthood -Cardiac murmur -Abnormal finding in chest Rx / EKG -Recurrent respiratory infection Second decade of life: -Shortness of breath with exertion -Palpitations Tal Geva Lancet 2014; Lancet.383: Fatigue exercise intolerance Syncope Peripheral oedema Thrombo-embolism Cyanosis Arrhythmia

23 179 patients : CIA OS, OP, SV: -84 operated -95 medical -Retrospective study FU moyen : 8.9 ans

24 Mean Follow up = 7.3 ans Primary end point CI of MACE : - Cardiac related death - Heart failure - Pulmonary/systemic embolism - Recurrent pulmonary infection - Sustained ventricular arrhythmia - Progression of PHT

25 2277 Patients born before 1994 with diagnostic of ASD between

26 HR = 1.7 ( ) HR = 1.4 ( ) HR = 1.7 ( ) HR = 1.6 ( )

27 ASD closure vs ASD without closure : HR = 1.6 ( ) Conclusions: -Increasing Mortality regardless of closure in childhood or adulthood -Closure improve survival -Need long term follow-up

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29 Am Heart J 2006;151: Retrospective study : consecutive patients

30 Rate of total complications: -Group A = 44 % -Group B= 6,9 % P<0,0001 Hospital stay - Group A = 8 +2,8 days - Group B = 3,2 + 0,9 days P= 0,001 Multivariate analysis: - Total complication: - OR=8,13 P<0,001 - Major complication - 4,03 P<0,001 P=0,002

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36 419 patients percutaneous ASD closure

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43 Rhythm and conduction Retrospective study patients 84%= OS 11%=SV 4%= OP

44 PARTIAL AVSD Rhythmic and conductive complications Somerville et al. Br. Heart J. 1965

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46 CONCLUSION ASD should be close once the diagnosis is confirmed if: Complication / Haemodynamically significant/ paradoxal embolism Absence of PAH Need good evaluation for a good treatment Need of regular follow up all the life