Atrial Septal Defects Ali Mahajerin Non-Invasive Cardiology Conference December 12, 2007

Atrial Septal Atrial Septal DefectsDefectsAli MahajerinAli Mahajerin

Non-Invasive Cardiology Non-Invasive Cardiology ConferenceConference

December 12, 2007December 12, 2007

IntroductionIntroduction

Atrial septal defect (ASD) is detected Atrial septal defect (ASD) is detected in 1 child per 1500 live births, and in 1 child per 1500 live births, and accounts for 5-10% of congenital accounts for 5-10% of congenital heart defects.heart defects.

ASDs make up 30-40% of all ASDs make up 30-40% of all congenital heart disease detected in congenital heart disease detected in adults (second only to bicuspid adults (second only to bicuspid aortic valve).aortic valve).

ASDs occur in women 2-3 times as ASDs occur in women 2-3 times as often as men.often as men.

IntroductionIntroduction

ASDs can occur in different anatomic ASDs can occur in different anatomic portions of the atrial septum.portions of the atrial septum.

ASDs can be isolated or occur with ASDs can be isolated or occur with other congenital cardiac anomalies.other congenital cardiac anomalies.

Functional consequences of ASDs are Functional consequences of ASDs are related to the anatomic location of the related to the anatomic location of the defect, its size, and the presence or defect, its size, and the presence or absence of other cardiac anomalies.absence of other cardiac anomalies.

EmbryologyEmbryology

ClassificationClassification Primum ASDPrimum ASD

Secundum ASDSecundum ASD

Sinus venosus Sinus venosus defectsdefects

Coronary sinus Coronary sinus defectsdefects

(Patent foramen (Patent foramen ovale)ovale)

Primum ASDPrimum ASD Make up ~15% of all ASDs.Make up ~15% of all ASDs. Occur if the septum primum does not fuse Occur if the septum primum does not fuse

with the endocardial cushions, leaving a defect with the endocardial cushions, leaving a defect at the base of the interatrial septum that is at the base of the interatrial septum that is usually large.usually large.

Usually not isolated – primum ASDs are Usually not isolated – primum ASDs are typically associated with anomalies of the AV typically associated with anomalies of the AV valves (such as cleft mitral valve) and defects valves (such as cleft mitral valve) and defects of the ventricular septum (VSDs) or a common of the ventricular septum (VSDs) or a common AV canal.AV canal.

Secundum ASDSecundum ASD Make up ~70% of all ASDs.Make up ~70% of all ASDs. Occur twice as often in females.Occur twice as often in females. Typically located within the area Typically located within the area

bordered by the limbus of the fossa bordered by the limbus of the fossa ovalis.ovalis.

Defects vary in size, from <3 mm to >20 Defects vary in size, from <3 mm to >20 mm.mm.

Secundum ASDSecundum ASD May be associated with other ASDs.May be associated with other ASDs. Multiple defects can be seen if the floor Multiple defects can be seen if the floor

of the fossa ovalis (AKA valve of the of the fossa ovalis (AKA valve of the foramen ovale) is fenestrated.foramen ovale) is fenestrated.

Ten to twenty percent have a functional Ten to twenty percent have a functional mitral valve prolapsemitral valve prolapse May be related to changing LV geometry May be related to changing LV geometry

associated with RV volume overloadassociated with RV volume overload

Sinus venosus ASDSinus venosus ASD Make up ~10% of ASDs.Make up ~10% of ASDs. Characterized by malposition of the insertion of Characterized by malposition of the insertion of

the SVC or IVC straddling the atrial septum.the SVC or IVC straddling the atrial septum. Often associated with anomalous pulmonary Often associated with anomalous pulmonary

venous return – the RUL/RML pulmonary veins venous return – the RUL/RML pulmonary veins may connect with the junction of the SVC and may connect with the junction of the SVC and RA in the setting of a superior sinus venosus RA in the setting of a superior sinus venosus ASD.ASD.

Coronary Sinus Septal Coronary Sinus Septal DefectsDefects

Less than 1% of ASDsLess than 1% of ASDs Defects in the inferior/anterior atrial Defects in the inferior/anterior atrial

septum region that includes the coronary septum region that includes the coronary sinus orifice.sinus orifice.

Defect of at least a portion of the common Defect of at least a portion of the common wall separating the coronary sinus and wall separating the coronary sinus and the left atrium – AKA “unroofed coronary the left atrium – AKA “unroofed coronary sinus”sinus”

Can be associated with a persistent left Can be associated with a persistent left SVC draining into the coronary sinus.SVC draining into the coronary sinus.

Patent Foramen OvalePatent Foramen Ovale

Not truly an “ASD” because Not truly an “ASD” because no septal tissue is missing.no septal tissue is missing.

Oxygenated blood from the Oxygenated blood from the IVC crosses the foramen ovale IVC crosses the foramen ovale in utero.in utero.

At birth, the flap normally At birth, the flap normally closes due tocloses due to Reduced right heart pressure Reduced right heart pressure

and PVRand PVR Elevated LA pressure.Elevated LA pressure.

Flap fusion is complete by age Flap fusion is complete by age two in 70-75% of children; the two in 70-75% of children; the remainder have a PFO.remainder have a PFO.

Pathophysiology of ASDsPathophysiology of ASDs Not an issue in utero – flow is occurring Not an issue in utero – flow is occurring

through the foramen ovale.through the foramen ovale. After birth, generally LA pressure > RA After birth, generally LA pressure > RA

pressure:pressure: PVR falls (lungs have expanded)PVR falls (lungs have expanded) SVR rises (placenta has been removed)SVR rises (placenta has been removed) Pulmonary venous blood flow is increased; all flows Pulmonary venous blood flow is increased; all flows

into LAinto LA Left-to-right shunting occurs across the ASD – Left-to-right shunting occurs across the ASD –

this depends on the size of the defect, the this depends on the size of the defect, the relationship of PVR and SVR, and the relationship of PVR and SVR, and the compliance of RV and LV.compliance of RV and LV.

Brief R-to-L shunting also occurs during Brief R-to-L shunting also occurs during cardiac cycle in children (during inspiration, cardiac cycle in children (during inspiration, LA pressure decreased and RA pressure LA pressure decreased and RA pressure increased) – causes mild neonatal cyanosis.increased) – causes mild neonatal cyanosis.

Pathophysiology of ASDsPathophysiology of ASDs

Generally L-to-R flow across ASDGenerally L-to-R flow across ASD Occurs mainly in late ventricular systole Occurs mainly in late ventricular systole

and early diastole; some augmentation and early diastole; some augmentation during atrial systole.during atrial systole.

The volume of pulmonary blood flow is The volume of pulmonary blood flow is greater than systemic blood flow because greater than systemic blood flow because of this circuit.of this circuit.

Qp/Qs can be as high as 8:1, though in Qp/Qs can be as high as 8:1, though in asymptomatic young adults is usually 2:1 asymptomatic young adults is usually 2:1 to 5:1.to 5:1.

Pathophysiology - Pathophysiology - consequencesconsequences

Right-sided volume overload leads to Right-sided volume overload leads to dilation of right-sided chambers.dilation of right-sided chambers.

Main pulmonary arteries dilate, and Main pulmonary arteries dilate, and pulmonary vascularity is increased.pulmonary vascularity is increased.

Eventual development of pulmonary Eventual development of pulmonary hypertension.hypertension.

RV function can become decreased.RV function can become decreased. Eisenmenger syndrome, with RV Eisenmenger syndrome, with RV

failure and right-to-left shunting of failure and right-to-left shunting of blood.blood.

Clinical ManifestationsClinical Manifestations Children may be asymptomatic; may have easy Children may be asymptomatic; may have easy

fatigability, exertional dyspnea. fatigability, exertional dyspnea. Underdeveloped, more prone to respiratory Underdeveloped, more prone to respiratory infections.infections.

Most patients with shunt flow ≥ 2:1 will be Most patients with shunt flow ≥ 2:1 will be symptomatic and require correction by age 40.symptomatic and require correction by age 40.

Exercise intolerance, fatigue, dyspnea, and Exercise intolerance, fatigue, dyspnea, and overt heart failure are the common overt heart failure are the common presentations in adulthood.presentations in adulthood.

Risk of atrial arrhythmias increases with age Risk of atrial arrhythmias increases with age and PA pressure.and PA pressure.

Pulmonary hypertension and Eisenmenger Pulmonary hypertension and Eisenmenger syndrome – 50% occurrence in unoperated syndrome – 50% occurrence in unoperated ASDs.ASDs.

Physical Exam FindingsPhysical Exam Findings

Wide, fixed splitting of S2 (delayed closure Wide, fixed splitting of S2 (delayed closure of pulmonic valve with reduced respiratory of pulmonic valve with reduced respiratory variation)variation)

Midsystolic pulmonary flow or ejection Midsystolic pulmonary flow or ejection murmur murmur Usually over 2Usually over 2ndnd intercostal space intercostal space Peaks in early-to-mid systole, ends before S2Peaks in early-to-mid systole, ends before S2

Palpable RV heavePalpable RV heave Usually no audible murmur across the ASDUsually no audible murmur across the ASD Eisenmenger’s sequellae: cyanosis, clubbingEisenmenger’s sequellae: cyanosis, clubbing Murmur of MR if cleft MV also present Murmur of MR if cleft MV also present

(primum ASD)(primum ASD)

EKG FindingsEKG Findings Right atrial enlargement d/t vol overload (tall P Right atrial enlargement d/t vol overload (tall P

wave)wave) RVH – RAD, RSR’ in V1, R>S in V1.RVH – RAD, RSR’ in V1, R>S in V1. Atrial tachyarrhythmias – a.fib, atrial flutterAtrial tachyarrhythmias – a.fib, atrial flutter AV delay – often with primum ASD in AV delay – often with primum ASD in

association with LAFB and RBBB (the rim of an association with LAFB and RBBB (the rim of an ostium primum defect is near the His bundle).ostium primum defect is near the His bundle).

Chest X-Ray FindingsChest X-Ray Findings

Dilation of RA and RVDilation of RA and RV Enlarged main Enlarged main

pulmonary arteries pulmonary arteries and pulmonary and pulmonary vessels, without vessels, without redistribution to redistribution to apical vessels.apical vessels.

Left atrial Left atrial enlargement if enlargement if associated mitral associated mitral regurgitation.regurgitation.

Echocardiography and Echocardiography and ASDsASDs

Some clues to the presence of ASD:Some clues to the presence of ASD: Abrupt discontinuity of the septum, and slight Abrupt discontinuity of the septum, and slight

thickening at its terminationthickening at its termination RA enlargement, RV enlargement/dilationRA enlargement, RV enlargement/dilation Dilated pulmonary arteriesDilated pulmonary arteries Increased flow velocity in the PA and across Increased flow velocity in the PA and across

TVTV Paradoxical motion and diastolic flattening of Paradoxical motion and diastolic flattening of

the ventricular septumthe ventricular septum TTE is usually definitive in secundum TTE is usually definitive in secundum

ASDs.ASDs. TEE will help with sizing defects, and TEE will help with sizing defects, and

identifying sinus venosus defects.identifying sinus venosus defects.

Two-Dimensional TTETwo-Dimensional TTEApical four-chamber Apical four-chamber

viewview Can often see ostium primum Can often see ostium primum

ASD in this view.ASD in this view. Shadowing and echo dropout Shadowing and echo dropout

(especially in the area of the (especially in the area of the fossa ovalis) may lead to fossa ovalis) may lead to false positives.false positives.

Subcostal viewSubcostal view Often more reliable - can Often more reliable - can

visualize entire atrial visualize entire atrial septum.septum.

Sensitivity for ASD detection:Sensitivity for ASD detection: Primum ASD: 100%Primum ASD: 100% Secundum ASD: 89%Secundum ASD: 89% Sinus venosus ASD: 44%Sinus venosus ASD: 44%

Color Doppler TTEColor Doppler TTE Can confirm the presence of Can confirm the presence of

the ASD, estimate the defect the ASD, estimate the defect size, and evaluate the size, and evaluate the efficacy of surgery.efficacy of surgery.

Flow extends from mid-Flow extends from mid-systole to mid-diastole; systole to mid-diastole; second phase of flow second phase of flow coincident with atrial systole.coincident with atrial systole.

May have brief R-L shunting.May have brief R-L shunting. Usually not a high velocity Usually not a high velocity

jet.jet. Must avoid confusing the Must avoid confusing the

low-velocity shunt flow with low-velocity shunt flow with normal venous and AV valve normal venous and AV valve flow.flow.

Contrast EchoContrast Echo Administer agitated saline contrast through IV.Administer agitated saline contrast through IV. Apical four-chamber view is usually optimal.Apical four-chamber view is usually optimal. Bubbles in the LA suggests right-to-left Bubbles in the LA suggests right-to-left

shunting shunting at the atrial level if 3 bubbles within 3 cardiac at the atrial level if 3 bubbles within 3 cardiac

cycles following complete opacification of the cycles following complete opacification of the RA. Delayed bubbles may be due to pulmonary RA. Delayed bubbles may be due to pulmonary AVMs – may be less phasic in appearance. AVMs – may be less phasic in appearance.

Large ASDs may have nearly continuous Large ASDs may have nearly continuous shunting, but smaller ASDs may be more shunting, but smaller ASDs may be more phasic with respiration.phasic with respiration.

May see “negative contrast effect” if mainly May see “negative contrast effect” if mainly left-to-right shunt.left-to-right shunt.

Contrast Echo - PFOsContrast Echo - PFOs

Often a small, hemodynamically Often a small, hemodynamically insignificant left-to-right shunt insignificant left-to-right shunt present in PFO based on the unsealed present in PFO based on the unsealed overlap of foraminal valve.overlap of foraminal valve.

The shunt is often phasic with The shunt is often phasic with respiration.respiration.

Maneuvers such as Valsalva or cough, Maneuvers such as Valsalva or cough, which transiently increase R heart which transiently increase R heart pressure, may allow the occult R-to-L pressure, may allow the occult R-to-L shunt component of a PFO to become shunt component of a PFO to become evident.evident.

Contrast EchoContrast Echo

Negative contrast effectNegative contrast effect

Transesophageal EchoTransesophageal Echo TEE is superior to TTE in visualizing the TEE is superior to TTE in visualizing the

interatrial septum and identifying all types of interatrial septum and identifying all types of ASDs.ASDs.

With contrast or Doppler, TEE can detect any With contrast or Doppler, TEE can detect any brief right-to-left shunting that may occur with brief right-to-left shunting that may occur with transient increases in right-sided pressure.transient increases in right-sided pressure.

TEE is much more sensitive than TTE for TEE is much more sensitive than TTE for detection of left-to-right shunt as negative right detection of left-to-right shunt as negative right atrial contrast (93% vs. 58% in one study).atrial contrast (93% vs. 58% in one study).

TEE can detect flow through multiple ASDs.TEE can detect flow through multiple ASDs.

Transesophageal EchoTransesophageal Echo Estimation of defect size using the diameter Estimation of defect size using the diameter

of the Doppler color flow jet correlates with of the Doppler color flow jet correlates with surgical findings.surgical findings.

Since ASDs are not necessarily round, TEE Since ASDs are not necessarily round, TEE helps with determining both their size and helps with determining both their size and shape. This is especially important when shape. This is especially important when percutaneous closure is being contemplated.percutaneous closure is being contemplated.

TEE is often used when contrast echo TEE is often used when contrast echo suggests shunting, but a defect can’t be suggests shunting, but a defect can’t be visualized on TTE. The TEE then helps to visualized on TTE. The TEE then helps to differentiate between a PFO and a true ASD.differentiate between a PFO and a true ASD.

TEE is particularly helpful for diagnosis of TEE is particularly helpful for diagnosis of sinus venosus ASDs.sinus venosus ASDs.

TEETEE

TEETEE

Cardiac MRCardiac MR

Can provide excellent Can provide excellent details regarding:details regarding:

Shunt flowShunt flow Defect sizeDefect size Pulmonary venous Pulmonary venous

returnreturn

Large sinus venosus ASDLarge sinus venosus ASD Qp/Qs 2.7Qp/Qs 2.7 Anomalous return of Anomalous return of

right upper pulmonary right upper pulmonary vein to RAvein to RA

Increased RV cavity size, Increased RV cavity size, normal RV functionnormal RV function


Qp/Qs = 2.0Qp/Qs = 2.0 Dilated RA, increased RV cavity Dilated RA, increased RV cavity

size, evidence of RV volume size, evidence of RV volume overloadoverload

Normal pulmonary veinsNormal pulmonary veins

Cardiac MRCardiac MR Severely increased RV Severely increased RV

size, mild RV free wall size, mild RV free wall hypokinesis, volume hypokinesis, volume overload, dilated RAoverload, dilated RA

No significant AR or MR, No significant AR or MR, normal LVnormal LV

Sinus venosus ASD with Sinus venosus ASD with significant L-to-R shuntsignificant L-to-R shunt

Qp/Qs 3.03Qp/Qs 3.03 Normal pulmonary venous Normal pulmonary venous

return – the right upper return – the right upper pulmonary vein enters the pulmonary vein enters the LA at its junction with the LA at its junction with the RA and empties in the RA and empties in the region of the ASD.region of the ASD.


Large primum ASD, Qp/Qs 2.3; Large primum ASD, Qp/Qs 2.3; possible associated membranous possible associated membranous VSD.VSD.

Normal LV cavity size; LVEF Normal LV cavity size; LVEF 66%, effective forward LVEF 66%, effective forward LVEF 43%. Increased RV size, RVEF 43%. Increased RV size, RVEF 51%.51%.

Main PA diameter 37Main PA diameter 37

Paradoxical interventricular Paradoxical interventricular septal motion c/w RV volume septal motion c/w RV volume overloadoverload

Mod-severe MR with likely Mod-severe MR with likely cleft anterior leaflet of MVcleft anterior leaflet of MV

Biatrial enlargementBiatrial enlargement

3D Echo3D Echo

Estimation of Shunt Flow Estimation of Shunt Flow RatioRatio

Operative closure of an ASD traditionally Operative closure of an ASD traditionally recommended when the ratio of pulmonary recommended when the ratio of pulmonary blood flow to systemic blood flow (Qp/Qs) is blood flow to systemic blood flow (Qp/Qs) is greater than 1.5:1 or 2:1.greater than 1.5:1 or 2:1.

Can estimate Qp/Qs from TTE Can estimate Qp/Qs from TTE measurements using Pulsed Doppler measurements using Pulsed Doppler echocardiography. Cardiac MR is also echocardiography. Cardiac MR is also useful for further assessment of Qp/Qs ratio.useful for further assessment of Qp/Qs ratio.

Correlation between Doppler imaging and Correlation between Doppler imaging and cardiac catheterization techniques for this cardiac catheterization techniques for this measurement is good.measurement is good.


First measure stroke volume through each First measure stroke volume through each valve:valve:

Stroke Volume (Q) = CSA x VTIStroke Volume (Q) = CSA x VTI Left-sided stroke volume is measured from LVOT Left-sided stroke volume is measured from LVOT

(diameter measured in parasternal long axis (diameter measured in parasternal long axis view).view).

Maximum Doppler flow velocity apical to aortic Maximum Doppler flow velocity apical to aortic valve (VTIvalve (VTILVOTLVOT) taken in apical four-chamber view.) taken in apical four-chamber view.

Right-sided velocity time integral (VTIRight-sided velocity time integral (VTIPAPA) ) measured in PA well before bifurcation. measured in PA well before bifurcation.

PA diameter measured at the same level as VTIPA diameter measured at the same level as VTIPAPA..


Substitution into stroke volume ratio gives:Substitution into stroke volume ratio gives:

Qp/Qs = (PAQp/Qs = (PAdiamdiam))22 x VTI x VTIPAPA

--------------------------------------------------------------------------

((LVOTLVOTdiamdiam))22 x VTI x VTILVOTLVOT

Diameters of LVOT and PA are squared – exact Diameters of LVOT and PA are squared – exact measurement of these values is especially measurement of these values is especially important.important.

PA diameter can be difficult to assess in some PA diameter can be difficult to assess in some patients; this is the term that is most often patients; this is the term that is most often responsible for inaccurate estimates of the shunt responsible for inaccurate estimates of the shunt ratio.ratio.

Natural History of ASDsNatural History of ASDs

Most ASDs <8mm close spontaneously Most ASDs <8mm close spontaneously in infants.in infants.

Spontaneous closure is unusual in Spontaneous closure is unusual in children and adults; defects often children and adults; defects often become progressively larger.become progressively larger.

Most patients with a significant shunt Most patients with a significant shunt flow ratio (Qp:Qs > 2:1) will be flow ratio (Qp:Qs > 2:1) will be symptomatic and require closure by age symptomatic and require closure by age 40.40.

Increasing size of the ASD may preclude Increasing size of the ASD may preclude percutaneous closure.percutaneous closure.

Natural History of ASDsNatural History of ASDs

Life expectancy is not normal, though Life expectancy is not normal, though many patients live to advanced age.many patients live to advanced age.

Natural survival beyond age 40-50 is Natural survival beyond age 40-50 is <50%.<50%.

The attrition rate after age 40 is ~6% per The attrition rate after age 40 is ~6% per year.year.

Advanced pulmonary hypertension seldom Advanced pulmonary hypertension seldom occurs before the third decade.occurs before the third decade.

Atrial fibrillation is a late complication; Atrial fibrillation is a late complication; stroke is a potential complication of ASD stroke is a potential complication of ASD (ongoing investigation into this issue).(ongoing investigation into this issue).

Indications for Defect Indications for Defect ClosureClosure

1.) Symptoms1.) Symptoms Exercise intolerance, fatigue, dyspnea, Exercise intolerance, fatigue, dyspnea,

heart failureheart failure Atrial tachyarrhythmias?Atrial tachyarrhythmias?

Occur in 20% and often the presenting Occur in 20% and often the presenting symptomsymptom

Not an indication by itself (incidence may Not an indication by itself (incidence may not be reduced after surgery).not be reduced after surgery).

Indications for Defect Indications for Defect ClosureClosure

2.) Defect Size and Qp/Qs2.) Defect Size and Qp/Qs Larger ASDs impose a greater hemodynamic Larger ASDs impose a greater hemodynamic

burden on the RV. burden on the RV. In the absence of pulmonary hypertension, In the absence of pulmonary hypertension,

Qp/Qs is closely correlated with the size of the Qp/Qs is closely correlated with the size of the ASD.ASD.

Qp/Qs > 2:1 is a well-established indication, Qp/Qs > 2:1 is a well-established indication, though many authors advocate 1.7:1 or even though many authors advocate 1.7:1 or even 1.5:1.1.5:1.

AHA recommends a threshold Qp/Qs ≥ 1.5:1, AHA recommends a threshold Qp/Qs ≥ 1.5:1, but these guidelines exclude patients > 21 but these guidelines exclude patients > 21 years of age.years of age.

Canadian Cardiac Society recommends Qp/Qs Canadian Cardiac Society recommends Qp/Qs >2:1, or >1.5:1 in the presence of reversible >2:1, or >1.5:1 in the presence of reversible pulmonary hypertension.pulmonary hypertension.

Surgical ClosureSurgical Closure Median sternotomy is the Median sternotomy is the traditional approach; minimally traditional approach; minimally invasive approaches are emerging.invasive approaches are emerging. Pericardial or Dacron patches Pericardial or Dacron patches are used. are used. Primary closure of the defect is not Primary closure of the defect is not

recommended.recommended. Can repair other defects at the same time Can repair other defects at the same time

(such as cleft mitral valve if primum ASD).(such as cleft mitral valve if primum ASD). Intraoperative TEE useful to assess adequacy Intraoperative TEE useful to assess adequacy

of repair; can also assess for any new TR or of repair; can also assess for any new TR or MR that is occurring from tension of the MR that is occurring from tension of the repair.repair.

Surgery: EfficacySurgery: Efficacy Does surgery still benefit patients who are Does surgery still benefit patients who are

older at the time of diagnosis?older at the time of diagnosis? Attie et al. prospectively evaluated 473 pts. Attie et al. prospectively evaluated 473 pts.

over age 40 diagnosed with secundum over age 40 diagnosed with secundum ASDs, randomized to surgery vs. medical RxASDs, randomized to surgery vs. medical Rx Mean age 51Mean age 51 Median follow-up of 7.3 years.Median follow-up of 7.3 years. Qp/Qs ratio 2.3 ± 0.7Qp/Qs ratio 2.3 ± 0.7 Primary endpoints = death, PE, major Primary endpoints = death, PE, major

arrhythmia, embolic CVA, recurrent pulmonary arrhythmia, embolic CVA, recurrent pulmonary infection, functional class deterioration, or heart infection, functional class deterioration, or heart failure.failure.

Attie F, et al. JACC 2001; 38(7): 2035-42.

Attie F, et al. JACC 2001; 38(7): 2035-42.

Composite primary endpoint occurred more frequently Composite primary endpoint occurred more frequently with medical than surgical therapy (21 vs 11 percent, with medical than surgical therapy (21 vs 11 percent, H.R. 2.0).H.R. 2.0).

Overall mortality not statistically different, but there Overall mortality not statistically different, but there was a nonsignificant trend toward higher sudden death was a nonsignificant trend toward higher sudden death rate with medical treatment (2.9 vs 0.9 percent).rate with medical treatment (2.9 vs 0.9 percent).

Multivariate analysis (adjusted for age at entry, mean Multivariate analysis (adjusted for age at entry, mean PASP > 35 mmHg, previous atrial tachyarrhythmia, and PASP > 35 mmHg, previous atrial tachyarrhythmia, and C.I. < 3.5 L/mC.I. < 3.5 L/m22) had a significantly higher mortality with ) had a significantly higher mortality with medical management (H.R. 4.1).medical management (H.R. 4.1).

Surgery: EfficacySurgery: Efficacy Konstantinides et al. retrospectively evaluated Konstantinides et al. retrospectively evaluated

179 patients with isolated ASDs diagnosed after 179 patients with isolated ASDs diagnosed after age 40 (91% secundum, 3% primum, 6% sinus age 40 (91% secundum, 3% primum, 6% sinus venosus)venosus) Compared 84 pts. (47%) who underwent surgery Compared 84 pts. (47%) who underwent surgery

vs. 95 pts. (53%) who were treated medically.vs. 95 pts. (53%) who were treated medically. Mean age 54±7 years for the surgery group, Mean age 54±7 years for the surgery group,

57±10 years for the medical therapy group.57±10 years for the medical therapy group. Mean follow-up period 8.9±5.2 yearsMean follow-up period 8.9±5.2 years Cardiac symptoms reported in 94% at Cardiac symptoms reported in 94% at

presentation.presentation. ““The decision not to operate was based on the The decision not to operate was based on the

judgment of the cardiologists and cardiac judgment of the cardiologists and cardiac surgeons involved in each case.”surgeons involved in each case.”

Konstantinides S, et al. NEJM 1995; 333(8): 469-73.


Surgery: EfficacySurgery: Efficacy

Multivariate analysis revealed a reduced Multivariate analysis revealed a reduced mortality from all cause in the surgical group mortality from all cause in the surgical group ((relative risk 0.31relative risk 0.31; 95% C.I. 0.11-0.85).; 95% C.I. 0.11-0.85).

Ten-year survival rate was Ten-year survival rate was 95%95% in surgical in surgical group, group, 84%84% in medically treated group. in medically treated group.

Surgical treatment prevented functional Surgical treatment prevented functional deterioration (deterioration (11% vs. 34%, relative risk 11% vs. 34%, relative risk 0.210.21, 95% C.I. 0.08-0.55) and improved , 95% C.I. 0.08-0.55) and improved functional status (functional status (32% vs. 3%,32% vs. 3%, p=0.002). p=0.002).

Incidence of new atrial arrhythmias or stroke Incidence of new atrial arrhythmias or stroke was not significantly different.was not significantly different.


Long-Term Surgical Long-Term Surgical OutcomesOutcomes

Surgery before the age of 25 yields in 30-year Surgery before the age of 25 yields in 30-year survival rates comparable to age- and sex-survival rates comparable to age- and sex-matched controls.matched controls.

At 25-40 years of age, surgical survival is At 25-40 years of age, surgical survival is reduced, though not significantly if PA reduced, though not significantly if PA pressures are normal.pressures are normal.

If PASP > 40 mmHg, late survival is 50% less If PASP > 40 mmHg, late survival is 50% less than control rates, though life expectancy in than control rates, though life expectancy in surgically treated older patients is better than surgically treated older patients is better than that of medically treated patients.that of medically treated patients.

No benefit of surgery in reducing the incidence No benefit of surgery in reducing the incidence of AF, though the patient’s age at the time of of AF, though the patient’s age at the time of closure is the most important predictor of the closure is the most important predictor of the development of atrial arrhythmias.development of atrial arrhythmias.

Percutaneous ClosurePercutaneous Closure An alternative to surgical An alternative to surgical

closure for secundum closure for secundum ASDs with appropriate ASDs with appropriate anatomic characteristics.anatomic characteristics. Defect < 30mm diameterDefect < 30mm diameter Prefer a rim of tissue at least Prefer a rim of tissue at least

5mm around the defect to 5mm around the defect to prevent obstruction of prevent obstruction of coronary sinus, R pulmonary coronary sinus, R pulmonary veins, vena cavae, or AV veins, vena cavae, or AV valves.valves.

Approximately half to two-Approximately half to two-thirds of secundum ASDs thirds of secundum ASDs in adults meet these in adults meet these criteria.criteria.

Amplatzer Occlusion Amplatzer Occlusion DeviceDevice

Introduced in 1996.Introduced in 1996. Approved for Approved for

percutaneous ASD percutaneous ASD closure in 2001 by F.D.A.closure in 2001 by F.D.A.

Over 90,000 have been Over 90,000 have been manufactured and manufactured and delivered to date.delivered to date.

Consists of two round Consists of two round disks made of Nitinol disks made of Nitinol (nickel + titanium) wire (nickel + titanium) wire mesh linked together by mesh linked together by a short connecting waist.a short connecting waist.

Amplatzer Occlusion Amplatzer Occlusion DeviceDevice

Advantages over other devices:Advantages over other devices: Can be delivered through Can be delivered through

smaller catheterssmaller catheters It is self-centering but can be It is self-centering but can be

repositioned easilyrepositioned easily Has round retention disks Has round retention disks

that extend radially beyond that extend radially beyond the defect, which results in a the defect, which results in a much smaller overall size much smaller overall size and firmer contact with the and firmer contact with the atrial septumatrial septum

Shape enhances Shape enhances endothelialization and endothelialization and reducing the risk of residual reducing the risk of residual shuntingshunting



Amplatzer - catheterAmplatzer - catheter

Amplatzer – crossing the Amplatzer – crossing the septumseptum

Amplatzer – deployment of Amplatzer – deployment of left atrial discleft atrial disc

Amplatzer – seating against Amplatzer – seating against interatrial septuminteratrial septum

Amplatzer – deployment of Amplatzer – deployment of right atrial discright atrial disc

Amplatzer – releasing the Amplatzer – releasing the devicedevice

Amplatzer – minimal Amplatzer – minimal residual shuntresidual shunt

Post-Procedure TTEPost-Procedure TTE




Percutaneous Closure - Percutaneous Closure - OutcomesOutcomes

Chan et al. showed successful implantation Chan et al. showed successful implantation of Amplatzer device in 93 of 100 patients of Amplatzer device in 93 of 100 patients (mean age 13.3 years).(mean age 13.3 years). Procedure time ranging 30 to 180 minutes.Procedure time ranging 30 to 180 minutes. Seven failuresSeven failures Total ASD occlusion rate at 3 months = 99% for Total ASD occlusion rate at 3 months = 99% for

the 93 successes.the 93 successes. Masura et al. showed no deaths or Masura et al. showed no deaths or

significant complications, along with all significant complications, along with all defects remaining completely closed, in 151 defects remaining completely closed, in 151 patients (mean age 11.9 years) at long-term patients (mean age 11.9 years) at long-term follow-up (median time 78 months). follow-up (median time 78 months).

Chan KC, et al. Heart 1999; 82(3): 300-6.Masura J, et al. JACC 2005; 45(4): 505-7.

Percutaneous Closure - Percutaneous Closure - ComplicationsComplications

Early complicationsEarly complications Device embolization or malposition requiring Device embolization or malposition requiring

surgery (2.4%)surgery (2.4%) Atrial fibrillation (2.4%)Atrial fibrillation (2.4%) Heart block, effusion, thrombus in LAA (2.2%)Heart block, effusion, thrombus in LAA (2.2%)

Thrombus formation (both in LA and RA)Thrombus formation (both in LA and RA) Need aspirin and plavix for at least 6 monthsNeed aspirin and plavix for at least 6 months

Rare complications: cardiac perforation, Rare complications: cardiac perforation, sudden deathsudden death

Long-term complication: device erosion (0.1% Long-term complication: device erosion (0.1% of cases) – risk factors include deficient aortic of cases) – risk factors include deficient aortic rim (25/28 cases), deficient superior rim, and rim (25/28 cases), deficient superior rim, and oversized device.oversized device.

Surgery vs. Percutaneous Surgery vs. Percutaneous Repair?Repair?

Du et al. evaluated 596 patients with secundum Du et al. evaluated 596 patients with secundum ASDs and Qp/Qs ≥ 1.5:1 – surgery vs. ASDs and Qp/Qs ≥ 1.5:1 – surgery vs. Amplatzer ASOAmplatzer ASO Nonrandomized; assigned according to patient’s Nonrandomized; assigned according to patient’s

option.option. Median age 9.8 years in Amplatzer arm (442 Median age 9.8 years in Amplatzer arm (442

patients), 4.1 years surgical arm (154 patients; patients), 4.1 years surgical arm (154 patients; p<0.001)p<0.001)

Twelve-month follow-upTwelve-month follow-up Procedural success significantly higher with Procedural success significantly higher with

surgery (100 vs. 96 percent, p=0.006).surgery (100 vs. 96 percent, p=0.006). Percutaneous closure had significant reduction Percutaneous closure had significant reduction

in complication rates (7 vs. 24 percent) and in complication rates (7 vs. 24 percent) and mean hospital stay (1.0 vs. 3.4 days).mean hospital stay (1.0 vs. 3.4 days).

Mortality 0% for both groups.Mortality 0% for both groups.Du ZD, et al. JACC 2002; 39(11): 1836-44.


Du ZD, et al. JACC 2002; 39(11): 1836-44.


Bialkowski et al. prospectively compared Bialkowski et al. prospectively compared closure and complication rates in 91 closure and complication rates in 91 children with secundum ASDs over mean children with secundum ASDs over mean follow-up 3.9 years.follow-up 3.9 years. Mean age 8.1±4.7 years for surgery (44 pts.), Mean age 8.1±4.7 years for surgery (44 pts.),

10.1±4.9 years for Amplatzer (47 pts.)10.1±4.9 years for Amplatzer (47 pts.) Surgery if ASDs unsuitable for percutaneous Surgery if ASDs unsuitable for percutaneous

closure; 3 patients’ parents also requested closure; 3 patients’ parents also requested surgery.surgery.

Closure rate similar in the two groups (95.5% Closure rate similar in the two groups (95.5% vs. 97.5%).vs. 97.5%).

Hospital stay 7.5 days vs. 2.2 days (p<0.001)Hospital stay 7.5 days vs. 2.2 days (p<0.001) No deaths reported.No deaths reported.

Bialkowski J, et al. Tex Heart Inst J 2004; 31: 220-3.


Mild complicationsMild complications: small pericardial effusions, : small pericardial effusions, headaches, AV delay, atrial rhythm disturbancesheadaches, AV delay, atrial rhythm disturbances

Moderate complicationsModerate complications: pneumonia, : pneumonia, paroxysmal SVT, AV junctional rhythmparoxysmal SVT, AV junctional rhythm

Severe complicationsSevere complications: bleeding requiring : bleeding requiring reoperation, transient neurologic events.reoperation, transient neurologic events.

Bialkowski J, et al. Tex Heart Inst J 2004; 31: 220-3.


Butera et al. retrospectively evaluated 1268 Butera et al. retrospectively evaluated 1268 patients with secundum ASDs.patients with secundum ASDs. Surgical group: mean age 22.4±18.9 years (range 1-81 Surgical group: mean age 22.4±18.9 years (range 1-81

years), female-male ratio 393:124.years), female-male ratio 393:124. Percutaneous group: mean age 29±19.8 years (range 9 Percutaneous group: mean age 29±19.8 years (range 9

mo-81 years), female-male ratio 415:336.mo-81 years), female-male ratio 415:336. No post-operative deaths.No post-operative deaths. Overall complications higher for surgery (44 vs. Overall complications higher for surgery (44 vs.

6.9 percent, p<0.0001).6.9 percent, p<0.0001). Surgery was independently strongly related to Surgery was independently strongly related to

the occurrence of total complication (OR 8.13, the occurrence of total complication (OR 8.13, 95% CI 5.75-12.20) and of major complications 95% CI 5.75-12.20) and of major complications (OR 4.03, 95% CI 2.38-7.35).(OR 4.03, 95% CI 2.38-7.35).

Hospital stay was shorter for percutaneous Hospital stay was shorter for percutaneous closure (3.2±0.9 vs. 8.0±2.8 days, p<0.0001).closure (3.2±0.9 vs. 8.0±2.8 days, p<0.0001).

Butera G, et al. AHJ 2006; 151: 228-34.


Butera G, et al. AHJ 2006; 151: 228-34.


Most reports show comparable Most reports show comparable procedural success.procedural success.

Rate of complications is consistently Rate of complications is consistently lower with percutaneous closure.lower with percutaneous closure.

Percutaneous closure associated with Percutaneous closure associated with shorter hospital stays.shorter hospital stays.

Many centers prefer implantation of Many centers prefer implantation of percutaneous device to surgical repair percutaneous device to surgical repair when percutaneous approach seems when percutaneous approach seems feasible. feasible.

Stroke Risk?Stroke Risk? Data are widely conflicting on the relationship Data are widely conflicting on the relationship

between PFO, atrial septal aneurysm, and/or ASD between PFO, atrial septal aneurysm, and/or ASD and recurrent cerebral emboli.and recurrent cerebral emboli. Increased prevalence of PFO and ASA in cryptogenic Increased prevalence of PFO and ASA in cryptogenic

stroke; less clear for ASD.stroke; less clear for ASD. The role of defect closure vs. medical therapy for The role of defect closure vs. medical therapy for

prevention of recurrent stroke is not well defined.prevention of recurrent stroke is not well defined. Aspirin is often used in setting of PFO or an Aspirin is often used in setting of PFO or an

isolated atrial septal aneurysm, and especially if isolated atrial septal aneurysm, and especially if PFO + ASA. Role of coumadin is not as clear – PFO + ASA. Role of coumadin is not as clear – coumadin recommended if patient has a coumadin recommended if patient has a documented DVT/PE. Less data available for documented DVT/PE. Less data available for ASDs.ASDs.

Surgical excision of an atrial septal aneurysm Surgical excision of an atrial septal aneurysm (without PFO or ASD) may be considered if (without PFO or ASD) may be considered if aspirin or coumadin fail to prevent a recurrent aspirin or coumadin fail to prevent a recurrent embolic event.embolic event.

Endocarditis Endocarditis Prophylaxis?Prophylaxis?

The 2007 AHA guidelines do The 2007 AHA guidelines do notnot recommend recommend antibiotic prophylaxis of endocarditis in antibiotic prophylaxis of endocarditis in isolated ASD, with the following exceptions:isolated ASD, with the following exceptions: Recently repaired ASD, whether by prosthetic Recently repaired ASD, whether by prosthetic

material or device, during the first 6 months after material or device, during the first 6 months after repair (to allow for sufficient endothelialization).repair (to allow for sufficient endothelialization).

Repaired ASD with a residual defect at the site or Repaired ASD with a residual defect at the site or adjacent to the site of a prosthetic device.adjacent to the site of a prosthetic device.

The updated guidelines no longer include The updated guidelines no longer include associated mitral regurgitation as an associated mitral regurgitation as an indication for prophylaxis.indication for prophylaxis.

Prophylaxis is no longer recommended for GI Prophylaxis is no longer recommended for GI or GU procedures; above criteria apply to or GU procedures; above criteria apply to dental procedures or respiratory procedures dental procedures or respiratory procedures that require biopsy of mucosa.that require biopsy of mucosa.

Wilson W, et al. Circulation 2007; 151: 1-19.

Thank You!Thank You!

Eli GelfandEli Gelfand Jason RyanJason Ryan

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Atrial Septal Defects Ali Mahajerin Non-Invasive Cardiology Conference December 12, 2007