1. First Do No Harm: Management of Atrial Septal Defect in Adult Patients Jimmy Klemis, MD Morbidity & Mortality Conference April 4, 2002

2. Case Presentation 68 Female presents with 3 rd admission in past 2yrs for “CHF” exacerbation. Notes progressive DOE, PND, Orthopnea, edema since prev admission 3 mos ago. Onset of sxs ~ 5-6 yrs ago. Denies any pleuritic CP, cough, F/C and compliant with medications/diet. PMHx: 1) HTN 2) CHF Meds: Lasix 40 Lisinopril 20 Dig.125

3. Case Presentation PE: HR 80 BP 140/80  HNT: jvp 8cm  CV: fixed split S2, RV heave  Resp: basilar rales  Ext: 2+ edema CXR pulm edema, CMG ECHO – biatrial enlargement, RV enlargement, PA 40’s, no shunt on color flow

4. Case Presentation Cardiology consult for hx of prev ECHO showing “intra-atrial shunt” – given exam and progressive sxs, R/L heart cath done R heart cath demonstrated O2 step up in high RA with demonstration of sinus venosus ASD and mod pulm HTN, PA systolic ~ 40 Medical mgmt chosen by pt

5. Historical Perspectives - ASD 1513 – Leonardo da Vinci describes “perforating channel” in atrial septum 1875 – Rokitansky first describes ASD 1941 – Bedford et al describe clinical features 1950’s – first successful open surgical repair 1980’s- present - transcatheter approaches to repair

6. ASD - Epidemiology 1/3 of all Adult congenital heart disease 2-3:1 female to male

7. Embryologic Development Braunwauld 6th ed

8. ASD - Anatomy Ostium Secundum -75% Ostium Primum - 15% Sinus Venosus - 10% Braunwauld 6 th ed

9. Associated conditions/ECG abnormalities Ostium Secundum  MVP (10-20%)  IRBBB, RAD Ostium Primum  MR/ cleft AMVL  LAD, 1 st degree AVB 75% Sinus Venosus  anomalous pulm venous drainage into RA or vena cavae  junctional/low atrial rhythm

10. Physiologic Consequences Shunt Flow  Size of defect  Relative compliance of ventricles  Relative resistance of pulmonary/systemic circulation L  R shunting results in diastolic overload of RV and increased pulmonary blood flow RV dilatation/failure and rarely severe pulm HTN (Eisenmenger’s) may ensue over time ~5% With age, deterioration chiefly due to 1  decrease LV compliance, increased L  R shunt  increase in atrial arrhythmias  pulm HTN develops, RV volume + pressure OL 1 Perloff, NEJM 1995

11. Clinical Symptoms Often asymptomatic until 3-4 th decade for moderate-large ASD, may present later in life for initially smaller ASD Fatigue DOE Atrial arrhythmias Paradoxical Embolus Recurrent Pulmonary infections

12. Physical Signs S2 – wide/fixed splitting RV/PA palpable impulse (if lg defect) systolic ejection murmur 2 nd L ICS mid-diastolic TV rumble

13. ECG

14. ECHO Subcostal view of Intraatrial Septum Color Flow/ Contrast Good for secundum, primum

15. Catheterization Oximetry Shunt Ratio (Qp/Qs) Grossman, Cardiac Cath. 6 th ed Ch 9

16. Catheterization/Oximetry Grossman; Keane JF et al, Grossman Cardiac Cath.6 th ed Chs 9,34

17. Treatment Medical : diuretics, ACEI, Aldactone Repair  Consider when sxs, Qp:Qs>1.5  Surgical Mortality 1-3% in most series PVR > 6-8 Woods Units - Contraindication  Interventional Only for secundum defects 94-96% success (Amplatzer)

18. Percutaneous Devices used for Closure of ASD Amplatzer FDA approved, over 9,000 used with excellent results

19. Early Studies of Prognosis/Natural History 1941 Bedford describes clinical features 1 1957, 1970 Campbell 2,3  untreated mortality 25% Age 30, 75% age 50, 90% age 60  noted that pattern of progressive disability began around 3 rd decade and included dyspnea, cardiac failure, atrial fibrillation and pulmonary HTN 1965 Markman 4  67 pt 1943-1963, all survived to age 40  40% died/disabled by 5 th decade  90% older than 60 were severely disabled 1 Bedford, et al. Br Heart J 1941; 2,3 Campbell M, et al. Br Heart J 1957,1970 4 Markman P, et al. Q J Med 1965

20. Early Studies of Prognosis/Natural History 1968 Craig and Selzer 1  128 pt age 18-56, hemodynamic + clinical data  Generally agreed with earlier studies 1 Craig RJ, Selzer A. Circulation 1968

21. Purpose of study was to analyze long term survival among pt who underwent ASD repair - up to then data had been poorly documented

22. Murphy JG, et al. 123 pt Mayo Clinic 1956-1960 ASD repair  62% female, mean age 26 (2-62) 27-32 year followup divided into groups according to age ( 41)and presence of mod- sev pulm HTN (PA s>40) at time of cath excluded primum ASD 75% symptomatic, older pt more likely to be on med Rx (Dig, diuretic, Quinidine)

23. Mortality followup at 27 years Age<2525-40>41 Repair93%84%40% Age/Sex Matched Control 97%91%59%

24. Survival Curves

25. Murphy JG, et al - Summary 28 deaths  13 (48%) Cardiac death  5 (19%) CVA (all in afib)  6 (21%) Noncardiac (cancer, sepsis, resp fail) Data on PVR available on only 42% of pt and was not included in statistical analysis A stated purpose of study was to determine employability and insurability of these pt and was not meant to be a “guideline” Led to consensus that repair age 41 had substantial increase in mortality Pts advised to have ASD repair because untreated prognosis thought to be poor

26. 82 pt (34 med 48 surgical)  70% asymptomatic, Mean PAP sys 34/30 25 year followup Outcome measures  Survival, symptoms, and complications

27. Outcomes/Follow-up at 25 years Medical (34)Surgical (48) PresentationFollow upPresentationFollow up CV Death1 (3%)2 (4%) NYHA I NYHA II NYHA III 25 (74%) 9 (26%) 0 (0%) 19 (56%) 15 (44%) 0 (0%) 34 (71%) 14 (29%) 0 (0%) 26 (54%) 22 (46%) 0 (0%) Atrial Fibrillation7 (20%)19 (56%)12 (25%)28 (53%)

28. Shah, et al. Conclusions Earlier data showing high morbidity and reduced survival was based on a group of highly selected pt b/c florid clinical signs of ASD were needed before catheterization considered (pre ECHO) In asymptomatic patients, ASD repair offered no benefit with regard to mortality, morbidity or progression to atrial arrhythmia Limitations: uncontrolled study, advanced pulm HTN excluded (these pt do better with surgery), 22% of original pt lost to followup

29. Children with sxs  ASD repair Asymptomatic  close followup and repair when sxs/hemodynamic deterioration Older pt >25, surgery may not benefit in terms of sxs/pulm HTN/mortality Questioned benefit of routine surgical repair of older pt with ASD

30. Sought to address issue of benefit/lack of benefit to ASD repair in middle aged-elderly pt Retrospective, 179 pt with ASD dx > age 40 between 1966-1991 47% surgery 53 % medical Mean followup of 8.9+-5.2 years Women 70%

31. Clinical / Baseline characteristics PVR, Qp/Qs Med Rx included Dig, diuretics or nitrates 94% of pt symptomatic

32. Results MedicalSurgery 10yr Surv.84%95% p=.02 NYHA worse34%11% NYHA better 1 3%32% Afib/flutter17%15% 1 69% improvement in NYHA III/IV

33. Konstantinides, et al - Summary 31% reduction in mortality among symptomatic pt, age > 40 with surgical repair Symptomatic improvement in NYHA functional class and less deterioration among surgically treated pt No effect on atrial arrhythmias First study to show benefit of surgery in older pt with ASD/ sxs Limitations – retrospective, nonrandomized; excluded pt with CAD or severe MR (prev study by same author showed no benefit in unselected pt 1 ) 1 Konstantinides, et al. Circulation 1994

34. Conclusions Age < 25, sxs, significant ASD – Repair Older age not contraindication and evidence supports mortality, symptomatic benefit for ASD repair in symptomatic pt with significant ASD