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

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

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Title: 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
    40s, 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
  • 1950s first successful open surgical repair
  • 1980s- present - transcatheter approaches to
    repair

6
ASD - Epidemiology
  • 1/3 of all Adult congenital heart disease
  • 2-31 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
    (Eisenmengers) 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, QpQsgt1.5
  • Surgical
  • Mortality 1-3 in most series
  • PVR gt 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 (lt11,
    12-24, 25-40, and gt41)and presence of mod-sev
    pulm HTN (PA sgt40) 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 lt25 25-40 gt41
Repair 93 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 ltage 24 had nl
    mortality, between age 25-41 good survival but
    less than expected, and gt 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) Medical (34) Surgical (48) Surgical (48)
Presentation Follow up Presentation Follow up
CV Death 1 (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 Fibrillation 7 (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 gt25, 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 gt 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
Medical Surgery
10yr Surv. 84 95 p.02
NYHA worse 34 11
NYHA better1 3 32
Afib/flutter 17 15
169 improvement in NYHA III/IV
33
Konstantinides, et al - Summary
  • 31 reduction in mortality among symptomatic pt ,
    age gt 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 lt 25, sxs, significant ASD Repair
  • Older age not contraindication and evidence
    supports mortality, symptomatic benefit for ASD
    repair in symptomatic pt with significant ASD
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