First Do No Harm:
Management of Atrial Septal
Defect in Adult Patients
Jimmy Klemis, MD
Morbidity & Mortality Conference
April 4, 2002
Case Presentation
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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
Case Presentation
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PE: HR 80 BP 140/80
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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
Case Presentation
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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
Historical Perspectives - ASD
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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
ASD - Epidemiology
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1/3 of all Adult congenital heart disease
2-3:1 female to male
Embryologic Development
Braunwauld 6th ed
ASD - Anatomy
Ostium Secundum -75%
Ostium Primum - 15%
Sinus Venosus
- 10%
Braunwauld 6th ed
Associated conditions/ECG abnormalities
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Ostium Secundum
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Ostium Primum
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MVP (10-20%)
IRBBB, RAD
MR/ cleft AMVL
LAD, 1st degree AVB 75%
Sinus Venosus
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anomalous pulm venous drainage into RA or
vena cavae
junctional/low atrial rhythm
Physiologic Consequences
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Shunt Flow
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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
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1Perloff,
decrease LV compliance, increased LR shunt
increase in atrial arrhythmias
pulm HTN develops, RV volume + pressure OL
NEJM 1995
Clinical Symptoms
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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
Physical Signs
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S2 – wide/fixed splitting
RV/PA palpable impulse (if lg defect)
systolic ejection murmur 2nd L ICS
mid-diastolic TV rumble
ECG
ECHO
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Subcostal view of
Intraatrial Septum
Color Flow/ Contrast
Good for secundum,
primum
Catheterization
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Oximetry
Shunt Ratio (Qp/Qs)
Grossman, Cardiac Cath. 6th ed Ch 9
Catheterization/Oximetry
Grossman; Keane JF et al, Grossman Cardiac Cath.6th ed Chs 9,34
Treatment
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Medical : diuretics, ACEI, Aldactone
Repair
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Consider when sxs, Qp:Qs>1.5
Surgical
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Mortality 1-3% in most series
PVR > 6-8 Woods Units - Contraindication
Interventional
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Only for secundum defects
94-96% success (Amplatzer)
Percutaneous
Devices used for
Closure of ASD
Amplatzer FDA approved,
over 9,000 used with
excellent results
Early Studies of Prognosis/Natural
History
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1941 Bedford describes clinical features 1
1957, 1970 Campbell 2,3
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untreated mortality
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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
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1Bedford,
67 pt 1943-1963, all survived to age 40
40% died/disabled by 5th decade
90% older than 60 were severely disabled
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
Early Studies of Prognosis/Natural
History
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1968 Craig and Selzer 1
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1Craig
128 pt age 18-56, hemodynamic + clinical data
Generally agreed with earlier studies
RJ, Selzer A. Circulation 1968
Purpose of study was to analyze long term survival among pt who underwent ASD
repair - up to then data had been poorly documented
Murphy JG, et al.
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123 pt Mayo Clinic 1956-1960 ASD repair
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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 modsev 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)
Mortality followup at 27 years
Age
<25
25-40
>41
Repair
93%
84%
40%
Age/Sex
Matched
Control
97%
91%
59%
Survival Curves
Murphy JG, et al - Summary
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28 deaths
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13 (48%) Cardiac death
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5 (19%) CVA (all in afib)
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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
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82 pt (34 med 48 surgical)
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70% asymptomatic, Mean PAP sys 34/30
25 year followup
Outcome measures
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Survival , symptoms, and complications
Outcomes/Follow-up at 25 years
Medical (34)
Surgical (48)
Presentation
Presentation
CV Death
Follow up
1 (3%)
Follow up
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%)
Shah, et al. Conclusions
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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
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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
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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%
Clinical / Baseline characteristics
•PVR, Qp/Qs
• Med Rx included Dig, diuretics
or nitrates
• 94% of pt symptomatic
Results
Medical
Surgery
10yr Surv.
84%
95% p=.02
NYHA worse
34%
11%
NYHA better1 3%
32%
Afib/flutter
15%
169%
17%
improvement in NYHA III/IV
Konstantinides, et al - Summary
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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
Conclusions
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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