First Do No Harm: Management of Atrial Septal Defect in Adult Patients

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 3rd 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 6th ed

9. Associated conditions/ECG abnormalities • Ostium Secundum • MVP (10-20%) • IRBBB, RAD • Ostium Primum • MR/ cleft AMVL • LAD, 1st 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 1Perloff, NEJM 1995

11. Clinical Symptoms • Often asymptomatic until 3-4th 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 2nd 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. 6th ed Ch 9

16. Catheterization/Oximetry Grossman; Keane JF et al, Grossman Cardiac Cath.6th 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 3rd decade and included dyspnea, cardiac failure, atrial fibrillation and pulmonary HTN • 1965 Markman4 • 67 pt 1943-1963, all survived to age 40 • 40% died/disabled by 5th decade • 90% older than 60 were severely disabled 1Bedford, et al. Br Heart J 1941; 2,3Campbell 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 1Craig 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 (<11, 12-24, 25-40, and >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

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 24 had nl mortality, between age 25-41 good survival but less than expected, and > 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

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 169% 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 pt1) 1Konstantinides, 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