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Adult Congenital Heart

Disease. Part 2

2012Individual Lesions in a Nutshell

Guideline Recommendations

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The Atrial Septum Formation

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Question 1

Which of the following statements is true regarding shunts

that occur before the tricuspid valve?

1. If left unrepaired, they invariably lead to

pulmonary hypertension.

2. There are now clear data that percutaneous closure of a

PFO in a patient

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PFO and TIA or Stroke

Association of PFO and Stroke-controversial

Mandkem M et al NEJM 2007;357:2268

Risk of recurrent stroke

Cryptogenic and otherwise

PFO in Cryptogenic Stroke Study

Homma S et al Circ 2002;105:2625.

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PFO and TIA or Stroke

Kent DM et al Stroke 2010;41:52

Issues:

? Whether PFO related to event-

20-25% of population have PFO

? Hypercoagulable state

? Advantage of PFO closure to prevent recurrences

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Results of CLOSURE 1 Trial

Furlan HJ et al NEJM 2012;366:991

Primary endpoint:Composite of CVA or TIA at 2 years,

30 day mortality or 2 year neuro mortality

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PFO and TIA or Stroke

No advantage yet established for PFO closure incryptogenic stroke

CLOSURE 1* trial (mostly Starflex device) safe but

no difference between closure and medical therapy

Ongoing Trials include: RESPECT- Amplatzer PFO device

PC-TRIAL- Amplatzer PFO device

REDUCE- Helex PFO device

CLOSE- Any device

AHA/ACC advisory position: Await completion of

trials**

*Furlan A, et al. NEJM 2012;366:991. **OGara P et al. Circulation 2009;119:2743

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Types of ASDs

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Question 2

Which of the following is true regarding the affectedstructural enlargement one expects in a patient with a

shunt due to an secundum atrial septal defect?

1. The RA, RV are the only structures enlarged.2. The RA, RV and PA are the structures enlarged.

3. The RA, RV, PA and LA are structures enlarged.

4. The RA, RV, PA, LA and LV are enlarged.

5. The RA, RV, PA, LA and Aorta are enlarged.

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Expected Chamber Enlargement

in Cardiac Shunting

RA RV PA LA LV Aorta

+ + + - - -

- + + + + -

- - + + + +

ASD

VSD

PDA

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Secundum ASD

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Secundum Atrial Septal Defect

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Secundum Atrial Septal Defect Clinical Pearls

About 30% have an associated malformation. 60% are female. Enlarged RA, RV, PA not LA

ECG with RIVCD. Crochetage in inferior leads.

Fixed splitting of A2and P2

Direction of shunt dependent on RA/LA compliance

Small shunts increase with age as LA compliance worsens

If right heart failure, JVP may not go up but pt may turn cyanotic

Only 10-15% develop pulmonary hypertension

Atrial arrhythmias common

Echo diagnostic. MRI for localization. Multiple fenestrations at times. Redundant septal tissue can

rarely be an issue for percutaneous closure

Intervene whenever RA and RV are enlarged regardless of

symptoms. Percutaneous closure if feasible.

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Secundum Atrial Septal Defect

Percutaneous closure preferred option iffeasible. Surgical results excellent if required.

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Percutaneous ASD Closure

BEFORE CLOSURE AFTER CLOSURE

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Secundum ASD Follow-up

Atrial arrhythmias remain common after

closure

Pregnancy well tolerated

Rare patients develop pulmonary

hypertension independent of ASD closure

(but generally in those closed late)

Chest pain after percutaneous closure

may imply device erosion

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Sinus Venosus ASD

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Sinus Venosus ASD

Bubble Study TEE

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Sinus Venosus ASD

Clinical Pearls About 10% of all atrial shunts

Defect in vena cava/LA junction

Association with SVC >>>IVC Anomalous RUPVC almost universal

SA node dysfunction with ectopic atrial rhythm

common on ECG

May be missed by chest wall echo. TEE or MRIbetter.

Operate when RA and RV enlarged. Surgical closure

only.

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Traditional Sinus Venosus Repair

Yale Website

RULPVs

Russel JL et al. Asian CV Thorac Ann 2002:10:231

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Warden Procedure to Repair Sinus Venosus ASD

SVCPericardial Patch

Sewn on top of Prox SVC

RA

STEP 1 STEP 2

SVC

SVC

RA

STEP 3

SVC

SVC

Baffling of Anom.Pulm Vein

Through ASD to LA

SVC SVC

RA

STEP 4

Stewart RD, et al. Ann Thorac Surg 2007;84:1651

Reattachment of SVC

To RA

Opening of RA

Prox SVC closed

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Sinus Venosus ASD Followup

SVC obstruction (esp from prior surgical

procedures)

SA node dysfunction

Atrial arrhythmias

Residual defects

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Sites of

Anomalous Pulmonary Venous Return

RUPV

May be Assoc. with

Sinus Venosus ASD

RUPV

To

RA

RLPV to IVC

Scimitar

LUPV

To Persistent LSVC

Vertical Vein

RVRA

RSVC

IVC

LSVC

INNOMINATE

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Classic Chest X-rays

Snowman*Vertical

Vein

*Ferguson EC et al. Radiographics 2007;27:1323, **Zylak CJ et al. Radiographics 2002;22:525

Scimitar Syndrome**

RLPV

Hypoplasia Right Lung

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Anomalous Pulmonary Venous Connection

IVC

PV

SCIMITAR

RLPV CONNECTION

SVC

RA

Vertical Vein

Lung

VERTICAL VEIN

LUPV CONNECTION

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Anomalous Pulmonary Venous Return

Clinical Pearls

Have high index of suspicion if RA and RV

size out of proportion to other clinical findings

Easiest to surgically correct is RUPV acrossSinus Venosus. Rest are more of a challenge

Pulmonary HBP rare (10-15%)

May be missed during routine echo orcatheterization unless sought

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Ostium Primum ASD

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AV Septal Defects

Complete and Partial

Primum ASD VSDBoth ASD and VSD

Shinebourne EA et al., Gatzoulis MA, Webb GD, Daubeney PEF. eds. Diagnosis and Management of Adult Congenital HD. Second Ed. page 196

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AV Septal Defects

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AV Septal Defect

Gooseneck Deformity on LV Angiogram

Outlet is longer than inletHo SY et al .Atlas of Congenital HD; Mosby, 1995, p 69.

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AV Septal Defects

Clinical Pearls The AV valves are divided into 5 leaflets with variouschordal attachments to each ventricle or top of

septum. Some of this results in cleft appearance.

If complete AVSD, 70-80% have Downs syndrome ECG- first degree AV block, with RBBB, LAFB. CHB

not uncommon.

Eisenmengers usual if unrepaired complete AVSD

Decision to repair may be difficult based on socialsituation and mental status. Generally done if only

primum ASD and cleft valves, but more extensive

surgery requires clinical judgment as to quality of life.

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Atrial Septal Defect Closure Guidelines

Closure of an ASD either percutaneously or surgically is

indicated for right atrial and RV enlargement with or

without symptoms. (LOE B)

A sinus venosus, coronary sinus, or primum ASD should

be repaired surgically rather than by percutaneous

closure. (LOE B)

Surgeons with training and expertise in CHD should

perform operations for various ASD closures. (LOE C)

Class 1

Warnes CA et al JACC 2008;52:1890-1947

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Surgical closure of secundum ASD is reasonable when

concomitant surgical repair/replacement of a tricuspid

valveis considered or when the anatomy of the defect

precludes the use of a percutaneous device. (LOE C)

Closure of an ASD, either percutaneously or surgically is

reasonable in the presence of:

a) Paradoxical embolism (LOE C)

b) Documented platypnea-orthodeoxia(LOE B)

Atrial Septal Defect Closure Guidelines

Class 2a

Warnes CA et al JACC 2008;52:1890-1947

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Closure of anASD in PULMONARY HYPERTENSION if left-

to-right shuntand

PA pressure < 2/3 Systemic pressure or

PVR

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Question 3A 20 year old is referred to you because of a murmur. He

appears healthy, and he denies any symptoms. Hisexamination is normal except for a harsh holosystolic

murmur along the LSB associated with a palpable thrill.

You order an echocardiogram and the results are

consistent with a muscular VSD in the mid ventricularseptum.

Based on these data alone, your next step would be to:

1. Refer him for percutaneous VSD closure

2. Refer him for surgical closure

3. Follow him with yearly echocardiograms and physical

examinations

4. Intitiate afterload reduction therapy

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Ventricular Septal Defects

Normal Anatomy VSD Locations

Uebing A et al., Gatzoulis MA, Webb GD, Daubeney PEF. eds. Diagnosis and Management of Adult Congenital HD. Second Ed. page 189

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Ventricular Septal Defect

Perimembranous VSD

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VSD with Large Windsock Deformity

Windsock VSD with No Shunt Windsock with Bicuspid Valve

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Ventricular Septal Defects

Small Muscular VSD Large VSD

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Ventricular Septal Defect

Clinical Pearls Generally, the smaller the VSD the louder the murmur and themore gradient between the LV and RV

VSDs often spontaneously close in childhood as the heart grows

Chamber enlargement includes RV, PA, LA and LV

Sudden AR may be due to aortic leaflet prolapse intoperimembranous or outlet VSD

Windsock deformity in perimembranous VSD is due to tricuspid

valve tissue

VSDs are restrictive(Qp/Qs 75% aorta)

Moderate or large VSDs result in pulmonary hypertension and

should be closed before that occurs

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Ventricular Septal Defect

Percutaneous Closure

Surgical Closure for most

Muscular VSD

Occluders

Perimembranous VSD

Occluder

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Surgical Ventricular Septal Defect Closure

Surgeons with training and expertise in CHD should

perform VSD closure operations (LOE C)

Closure of a VSD is indicated when there is a Qp/Qs

(pulmonary-to-systemic blood flow ratio) of 2.0or more

andclinical evidence of LV volume overload (LOE B)

Closure of a VSD is indicated when the patient has ahistory of IE (LOE C)

Class 1

Warnes CA et al JACC 2008;52:1890-1947

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VSD with Endocarditis

Colorflow

LV

VSD with vegs

LV

LA

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Surgical Ventricular Septal Defect Closure

Closure of a VSD is reasonable when

Net left-to-right shunting is present at a Qp/Qs >1.5

and PA pressure

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Device closure of a muscular VSD may be

considered, especially if the VSD is remote fromthe tricuspid valve and the aorta, if the VSD is

associated with severe left-sided heart chamber

enlargement or if there is PAH (LOE C)

Catheter-based Closure of VSD

Class 2b

Warnes CA et al JACC 2008;52:1890-1947

Question 4

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Question 4

Observe the hemodynamic tracing above.

Based on the tracing, you would conclude which of the following is

correct?

1. There is severe pulmonary stenosisand no regurgitation2. There is severe pulmonary stenosisand mild regurgitation

3. There is mild pulmonary stenosis and severe regurgitation

4. There is mild pulmonary stenosis and mild regurgitation

PA

RV

50

25

0

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The Problem of Low Pressure Pulmonary Regurgitation

PA

PA

RV

RV

Low Pressure PR High Pressure PR

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Tetralogy of Fallot

Babu-Narayan et al., Gatzoulis MA, Webb GD, Daubeney PEF. eds. Diagnosis and Management of Adult Congenital HD. Second Ed. page 316

VSD- usually large. 80% perimembranous

Subpulmonic stenosis- significant in most casesPulmonary valve- bicuspid. May or may not be stenotic

Over-riding aorta- if >50% over the RV, called double outlet RV

LAD from RCA crossing RV outflow in 3-7%

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Initial Palliative Procedures in TOF

Bashore TM. Circulation 2007;115:1933-1947

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Tetralogy Repair

Bashore TM. Circulation 2007;115:1933-1947

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Tetralogy of Fallot Clinical Pearls

Most common form of cyanotic congenitalheart disease (10% of all)

Most adults have had repair

After repair- pulmonary regurg, aortic regurg, LV

dysfunction, RV dysfunction, pulmonary branch

stenosis (native and acquired)

Arrhythmias- atrial and ventricular

Ventricular arrhythmias from RV outflow related to VSD

patch, tricuspid annulus and RVOT patch

Greatest risk for ICD shocks is an elevated LVEDP*

Decision regarding when pulmonary valve

replacement required usually major issue

* Khairy P et al. Circ 2008;117:363

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Prognosis and QRS Width

Gatzoulis MA et al Circulation 1995;92:231

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Pulmonary Valve Replacement After Tet Repair

Class 1- Severe PR and

Symptoms (LOE B)

Class 2a- Severe PR and

Moderate to severe RV dysfunction or RV enlargement or TR

(Upper limit of RV EDV= 140 ml/m2* to 170 ml/m2**)

Symptomatic or sustained atrial or ventricular arrhythmias Surgery also reasonable if any of following:

Residual RVOT gradient >50 mmHg

RV/LV systolic pressure ratio >0.7

Residual RVOT gradient with progressive RV enlargement of dysfunction

Residual VSD with Qp/Qs >1.5 Severe AR with sx or LV dysfunction

A combination of residual lesions leading to RV dysfunction

*Frigola A, et al. Circ 2008;118:S182-S190

** Canadian Guidelines 2010 Warnes CA et al JACC 2008;52:1890-1947

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Anomalous Coronary Arteries

Occur in about 1% of coronary angiograms Only a few are of importance

LCX from RCA occurs in up to 0.5%. Essentially always

posterior to aorta

LAD from RCA coursing anteriorly occurs in 3-7% oftetralogy of Fallot

Most important anomaly is LM or LAD from RCA

coursing between PA and aorta

CT or MRI emerging as excellent complement to cardiaccatheterization angiography and preferred to confirm

diagnosis. Frequently first found incidentally at coronary

angiography.

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Origin of LM or RCA from PA

Digital subtraction

LM from PA. RCA injectionRCA from PA

LM injection

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LM or LAD between PA and Ao

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LM or LAD Between PA and Ao

LVgram (RAO) PA injection and Ao Levophase

With LM injection each time

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Coronary Fistulae

Preclosure Post-closure

RA

Coils

Occluder device

LAD

Fistulae

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Potentially Clinically Important Coronary Anomalies

Anomalies of Coronary OriginAnomalous coronary origin from opposite coronary sinus with course between

aorta and PA (Left main from right cusp more likely a cause of ischemia than

RCA from left).

Associated with supravalvular aortic stenosis

D-transposition of the Great Vessels following arterial switch procedure

Anomalous origin of LM from the pulmonary artery (ALCAPA orBland-Garland-White)

Rarely, single coronary from right cusp

Anomalies of Coronary Course

Myocardial bridging with documented ischemia

Anomalies of the vessel wall

Congenital ostial stenosis

Coronary ectasia or aneurysm of large size

Anomalies of coronary destinat ion

Large coronary fistula

LM b t PA d A

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LM between PA and Ao

Surgical Repair Options

Gulati R et al. J Thorac CV Surg 2007;134:1171

Reimplantation Unroofing

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Anomalous Cors Guideline Recommendations

Anomalous OriginClass 1

Surgical coronary revascularization should be performed in patients with the following

indications:

Anomalous left main coronary artery coursing between the aorta and pulmonary artery (LOE B)

Documented ischemia when either coronary courses between the great vessels or in an

intramural fashion (LOE B)

Class IIa

Surgical coronary revascularization can be beneficial in the setting of documented vascular wallhypoplasia, coronary compression or obstruction to coronary flow, regardless of documented

ischemia(LOE C)

Class IIb

Surgical revascularization may be reasonable in patients with anomalous LAD coursing between

the aorta and pulmonary artery (LOE C)

Coronary FistulaClass I

A large fistula should be closed, regardless of symptomatology, via either a transcatheter or

surgical approach if feasible (LOE=C)

A small or moderate fistula should be closed if there is evidence for myocardial ischemia,

arrhythmia or otherwise unexplained systolic or diastolic dysfunction or enlargement, or

endarteritis, if feasible (LOE=C)

Warnes CA et al JACC 2008;52:1890-1947

Question 5

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Question 5

You are seeing a 50 y.o patient with a bicuspid aortic valve and known

coarctation of the aorta. His echo/Doppler suggests only mild aortic

stenosis and regurgitation. His peak coarctation gradient is estimatedat 30 mmHg. He has mild systemic hypertension that is currently

medically controlled.

Which of the following statements is correct regarding repair of his

coarctation of the aorta?

1. After age 30, the repair should be surgical and not

not percutaneous

2. The coarctation gradient suggests he should proceed to an

intervention at this time

3. Repair of his coarctation should await need for

simultaneous AVR

4. Repair of his coarctation will ensure he no longer needs

any anti-hypertensive medications

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Coarctation of the Aorta

Coarctation of the Aorta Pseudo-coarctation

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Coarctation of the Aorta

Native Coarct Pre Native Coarct Post Stent

C t ti f th A t

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Coarctation of the Aorta Clinical Pearls and Guidelines

Bicuspid aortic valve in up to 85%. Often associated with other

malformations.

Multiple surgical approaches as infant- resection and end-to-end anast.,

interposition graft or patch used most commonly

Figure 3 aortic shadow on CXR with rib notching

In adults follow for systemic HBP, bicuspid valve, recoarctation, aortic

aneurysm, CAD, possible berry aneurysm Stenting now preferred for restenosis (often for primary lesion)

BP may not fall after repair of aortic coarctation in adults. At times

rebound HBP after surgery.

Indication for intervention:

Class 1- peak gradient >20 mmHg. Stenting for discrete lesions,surgery for long lesions. Collaterals may be huge at times.

Class 2b- surgery preferred for long lesions, but stenting can be

considered if anatomy reasonable.

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Discrete Subaortic Stenosis

Subaortic Membrane Associated AR

Turbulence Below Ao Valve

Di t S b ti St i

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Discrete Subaortic Stenosis Clinical Pearls and Guidelines

Spectrum from membrane to tunnel. Occasionally due toaccessory mitral chordae or post-op

Shone Syndrome- multiple levels of obstruction (coarct, bicuspid

aortic valve, subaortic stenosis, parachute mitral, mitral ring)

Thought to be acquired over time. Not seen in neonates.

Progressive lesion. Often recurs after surgical resection.

Jet lesion results in AR in many patients

Percutaneous balloon dilatation not effective

At times LV outflow tract opening required (Konno procedure)

Surgical indications:Class 1- peak gradient of 50 mmHg with mean of 30 mmHg.

May do if less gradient if AR severe or LVESD>5.0 cm or EF

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Pulmonary Valve Stenosis

RV

PA

Doming

Pulm ValveRV

PA

Doming

Pulm Valve

AP VIEW LATERAL VIEW

P l V l St i

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Pulmonary Valve Stenosis Clinical Pearls and Guidelines

Most common RVOT obstructive lesion. Fusion of cusps. Severity from mild to atresia. Most have doming valve- some with

dysplasia. Dysplasia more common in Noonans phenotype.

In mild to moderate severity,-well tolerated for a long time

RVH can be significant. If cyanotic, likely due to opening of PFO

Pulmonic click is only right sided auscultatory event that decreases withinspiration. Decrease due to premature valve opening.

Preferential flow to left PA and left lung occurs.

Balloon valvuloplasty very effective in doming valve PS and preferred.

Intervene:

Class 1-Asymptomatic- peak echo gradient >60 mmHg and mean >40mmHg. Symptomatic- peak >50 mmHg and mean>30 mmHg.

Class 1- Surgery if associated PR, subvalvular or supravalvular stenosis.

Also for most dysplastic valves.

Class 2b- balloon valvuloplasty in rare patients with dysplastic valve

Class 3- if severe PR or asymptomatic with mean gradient

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Sinus of Valsalva Aneurysm

LV

Worm-like

Sinus of Valsalva

Aneurysm

RA

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Sinus of Valsalva Aneurysm

Clinical Pearls and Guidelines Almost never affects left coronary aortic cusp. 65-85% right, 10-30%

noncoronary

Rupture essentially always into right heart structure, rarely the LA. RV

in 90%.

Rupture may follow trauma. Clinically dramatic. Loud to and fro murmur when ruptures

Unruptured-can lead to RVOT obstruction, coronary ostial obstruction,

ventricular arrhythmias, TIA, AV block

Ruptured-CHF, tamponade, aortic or tricuspid regurg, acute coronary

disruption

All ruptured leaking should be repaired surgically. Transcatheter

closure has been described, but no long term data.

Therapy for nonruptured aneurysms unclear and left to clinical judgment

regarding size, etc. All need serial assessment.

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Question 6

Wald R, Crean A . Canadian MAJ 2010;182:E380

PDA with Pulmonary HBP

Not clubbed

Clubbed

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Patent Ductus Arteriosus

Pre Ductal Closure Post Ductal Closure

Ao AoPDA

Ductal Occluder

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Patent Ductus Arteriosus Clinical Pearls and Guidelines

Usually an isolated lesion in the adult

Size generally based on physical exam and consequences:

Silent-tiny PDA. Usually found on echo.

Small-audible PDA but no hemodynamic evidence for volume overload

Moderate-audible and dynamic pulses. LA, LV, RV and PA are enlarged.

Some degree of pulmonary hypertension frequent Large-Eisenmengers present. Pulmonary regurg. Reversed shunt (pink

hands, blue clubbed toes)

Repaired ductus as child usually without major sequela as adult

Percutaneous occlusion preferred if feasible. In older adult calcium

makes surgical repair greater risk as well.

Unrepaired guidelines: Class 1- Closure indicated if audible and evidence

for LA or LV enlargement or PAH or prior endarteritis. Surgical closure if

too large or anatomically unfavorable for device. Class 2a- reasonable to

close small PDA in asymptomatic or in those with PAH if net left-to-right

shunt. Class 3- PAH with net right-to-left shunt

Warnes CA et al JACC 2008;52:1890-1947

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Ebsteins Anomaly

Tricuspid Valve

Displacement

ASD

Mild Ebsteins

Associated ASD

Severe Ebsteins

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Ebsteins Anomaly Clinical Pearls and Guidelines

Displacment of septal and posterior leaflets, not anterior.

Anterior leaflet large. When closes a sail sound may be heard.

TV displacement into the RV by 20 mm (8 mm/m2) compared to MV plane

Atrialized RV is thin and dilated. ASD occurs in 50%.

Maternal lithium has been implicated in fetal cases

Cause of AV valve regurg in congenitally corrected TGV (systemic RV).

Right sided WPW pathway present in 15%. May need ablation.

Clinical survival dependent on severity of TR and how much good RV

remains. Some have assoc. TS.

Exercise cyanosis not uncommon with associated PFO or ASD

Surgical repair may be difficult- ASD closure, plication and RA atrioplasty,

creation of monocuspid valve with ant. leaflet, cone reconstruction, TVR

Surgical intervention*: Class 1- symptoms. Can be related to ASD , TR/TS,

right heart failure, bioprosthetic TV replacement (LOE B)

*Warnes CA et al JACC 2008;52:1890-1947

Gerbode Defect

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Gerbode Defect

LV to RA

LV

RA

L Transposition

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L-Transposition

(Congenitally Corrected Transposition of the GA)

Pulmonary

LV Systemic

RV Ao

Systemic RV

LA

L Transposition

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L-Transposition

(Congenitally Corrected Tranposition of the Great Arteries)

Clinical Pearls and Guidelines L means the RV is on the left side of the LV (actually anterior)

Circuit OK except that RV supports systemic circulation. Often picked

up incidentally as adult (if no other malformations)

VSD and PS common in children. TV (systemic AV valve) resembles

Ebstein-like malformation in 50% and prone to regurg. AR often seen aswell. In 2-5% an accessory pathway present.

Pulmonary ventricle is anatomic LV. 50% have outflow obstruct. to PA.

Dual AV nodes common. Complete heart block rate about 2% per year

In children, consideration for atrial and arterial dual switch procedures

In adults, options usually revolve around CHF therapy, CRT, pacing,TVR and cardiac transplantation. Surgical correction usually not an

option. No clear guidelines.

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D-Transposition of the Great Arteries

From www.yorksandhumberhearts.nhs.uk/template/page.aspx?id=415

D T iti E h

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D-Transposition Echo

Systemic RV Pulmonary LV

Ao

PA

Systemic RV

RV is on Right Side

Supports Systemic Circulation

Aorta Anterior

D-Transposition

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D Transposition

Surgical Procedures

www.yorksandhumberhearts.nhs.uk/template/page.aspx?id=415

Atrial Switch (Mustard) Arterial Switch (Jalene)

Gaca AM et al. Radiology 2008;247:617

D T i i f h G A i

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D-Transposition of the Great Arteries Clinical Pearls and Guidelines

Atria connected to correct ventricles, but ventricles connected to wrongarteries. RA to RV to AO; LA to LV to PA. Need VSD or ASD to

survive. Atrial septostomy required shortly after birth if no ASD or VSD

VSD in 40-45%. LVOT obstruction in 25% (usually in those with VSD)

Atrial switch procedure (Mustard or Senning) baffles SVC and IVC to

mitral valve. Pulmonary flow around baffles to tricuspid valve. Arterial switch procedure now preferred. Chest pain can be due to

coronary ostial stenosis.

In atrial switch procedures, RV remains systemic ventricle and will

eventually fail. Other problems include tachy and bradyarrythmias,

baffle obstruction (and often with associated baffle leaks), systemic AV

valve regurgitation. Rastelli (RV to PA conduit) used if VSD plus severe PS.

Guideline recommendations complex and dependent upon surgical

procedure. For many the only long-term option is transplantation.

At i l S it h C li ti

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Atrial Switch Complication

Azygous

Occluded

Superior

Baffle Stented

Superior

Baffle

Inferior Baffle

Superior Baffle Occlusion Stenting of Baffle

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Bonnet D. et al.; J Thorac Cardiovasc Surg 1999;117:352-357

LM lesion afterarterial switch

Surgical Repair

Arterial Switch Complication

LAD

LCX

LCX

RCA

S f B d

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Summary for Boards

Focus on broad concepts Understand anatomy of the most common

lesions and the associated surgery to

repair them Be aware of clinical quirks for each lesion.

These make good test questions

Be aware of guideline recommendationsfor surgery or for intervention in the more

common lesions