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

Jimmy Klemis, MD Morbidity & Mortality Conference April 4, 2002

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

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

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

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

ASD - Epidemiology

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

Ostium Secundum

MVP (10-20%) IRBBB, RAD MR/ cleft AMVL LAD, 1st degree AVB 75% anomalous pulm venous drainage into RA or vena cavae junctional/low atrial rhythm

Ostium Primum

Sinus Venosus

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 (Eisenmengers) 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
NEJM 1995

1Perloff,

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

Physical Signs

S2 wide/fixed splitting RV/PA palpable impulse (if lg defect) systolic ejection murmur 2nd L ICS mid-diastolic TV rumble

ECG

ECHO

Subcostal view of Intraatrial Septum Color Flow/ Contrast Good for secundum, primum

Catheterization

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

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)

Percutaneous Devices used for Closure of ASD

Amplatzer FDA approved, over 9,000 used with excellent results

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

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

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.

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 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
Repair Age/Sex Matched Control

<25
93%

25-40
84%

>41
40%

97%

91%

59%

Survival Curves

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

82 pt (34 med 48 surgical)

70% asymptomatic, Mean PAP sys 34/30

25 year followup Outcome measures

Survival , symptoms, and complications

Outcomes/Follow-up at 25 years
Medical (34)
Presentation Follow up

Surgical (48)
Presentation Follow up

CV Death NYHA I NYHA II NYHA III Atrial Fibrillation 25 (74%) 9 (26%) 0 (0%) 7 (20%)

1 (3%) 19 (56%) 15 (44%) 0 (0%) 19 (56%) 34 (71%) 14 (29%) 0 (0%) 12 (25%)

2 (4%) 26 (54%) 22 (46%) 0 (0%) 28 (53%)

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

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

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
10yr Surv. 84%

Surgery
95% p=.02

NYHA worse

34%

11%
32% 15%

NYHA better1 3% Afib/flutter

169%

17%

improvement in NYHA III/IV

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)
et al. Circulation 1994

1Konstantinides,

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