First Do No Harm: Management of Atrial Septal Defect in Adult Patients Jimmy Klemis, MD

Morbidity & Mortality ConferenceApril 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 40’s, 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 1950’s – first successful open surgical repair 1980’s- 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 - AnatomyOstium Secundum -75%Ostium Primum - 15%Sinus Venosus - 10%

Braunwauld 6th ed

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

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

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 forClosure 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 1957,19704 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 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)

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 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) 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%)

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

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

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

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