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First Do No Harm: Management of Atrial Septal Defect in Adult Patients Jimmy Klemis, MD
Morbidity & Mortality ConferenceApril 4, 2002
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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
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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
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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
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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
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ASD - Epidemiology
1/3 of all Adult congenital heart disease 2-3:1 female to male
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Embryologic Development
Braunwauld 6th ed
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ASD - AnatomyOstium Secundum -75%Ostium Primum - 15%Sinus Venosus - 10%
Braunwauld 6th ed
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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
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Physiologic Consequences Shunt Flow
Size of defect Relative compliance of ventricles Relative resistance of pulmonary/systemic circulation
LR 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 LR shunt increase in atrial arrhythmias pulm HTN develops, RV volume + pressure OL
1Perloff, NEJM 1995
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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
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Physical Signs
S2 – wide/fixed splitting RV/PA palpable impulse (if lg defect) systolic ejection murmur 2nd L ICS mid-diastolic TV rumble
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ECG
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ECHO
Subcostal view of Intraatrial Septum
Color Flow/ Contrast Good for secundum,
primum
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Catheterization
Oximetry Shunt Ratio (Qp/Qs)
Grossman, Cardiac Cath. 6th ed Ch 9
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Catheterization/Oximetry
Grossman; Keane JF et al, Grossman Cardiac Cath.6th ed Chs 9,34
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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)
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Percutaneous Devices used forClosure of ASD
Amplatzer FDA approved, over 9,000 used with excellent results
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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 1957,19704 Markman P, et al. Q J Med 1965
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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
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Purpose of study was to analyze long term survival among pt who underwent ASD repair - up to then data had been poorly documented
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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)
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Mortality followup at 27 years
Age <25 25-40 >41
Repair 93% 84% 40%
Age/Sex Matched Control
97% 91% 59%
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Survival Curves
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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
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82 pt (34 med 48 surgical) 70% asymptomatic, Mean PAP sys 34/30
25 year followup Outcome measures
Survival , symptoms, and complications
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Outcomes/Follow-up at 25 years
Medical (34) Surgical (48)Presentation Follow up Presentation Follow up
CV Death 1 (3%) 2 (4%)
NYHA INYHA IINYHA 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%)
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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
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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%
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Clinical / Baseline characteristics
•PVR, Qp/Qs• Med Rx included Dig, diuretics or nitrates• 94% of pt symptomatic
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ResultsMedical 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
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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
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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