Title: First Do No Harm: Management of Atrial Septal Defect in Adult Patients
1First Do No Harm Management of Atrial Septal
Defect in Adult Patients
- Jimmy Klemis, MD
- Morbidity Mortality Conference
- April 4, 2002
2Case 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
3Case 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
4Case 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
5Historical 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
6ASD - Epidemiology
- 1/3 of all Adult congenital heart disease
- 2-31 female to male
7Embryologic Development
Braunwauld 6th ed
8ASD - Anatomy
Ostium Secundum -75 Ostium Primum -
15 Sinus Venosus - 10
Braunwauld 6th ed
9Associated 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
10Physiologic 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
11Clinical 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
12Physical Signs
- S2 wide/fixed splitting
- RV/PA palpable impulse (if lg defect)
- systolic ejection murmur 2nd L ICS
- mid-diastolic TV rumble
13ECG
14ECHO
- Subcostal view of Intraatrial Septum
- Color Flow/ Contrast
- Good for secundum, primum
15Catheterization
- Oximetry
- Shunt Ratio (Qp/Qs)
Grossman, Cardiac Cath. 6th ed Ch 9
16Catheterization/Oximetry
Grossman Keane JF et al, Grossman Cardiac
Cath.6th ed Chs 9,34
17Treatment
- 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)
18Percutaneous Devices used for Closure of ASD
Amplatzer FDA approved, over 9,000 used with
excellent results
19Early 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
20Early 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
21Purpose of study was to analyze long term
survival among pt who underwent ASD repair - up
to then data had been poorly documented
22Murphy 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)
23Mortality followup at 27 years
Age lt25 25-40 gt41
Repair 93 84 40
Age/Sex Matched Control 97 91 59
24Survival Curves
25Murphy 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
27Outcomes/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)
28Shah, 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
31Clinical / Baseline characteristics
- PVR, Qp/Qs
- Med Rx included Dig, diuretics
- or nitrates
- 94 of pt symptomatic
32Results
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
33Konstantinides, 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
34Conclusions
- 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