1 Congenital Heart Disease Cardiac Malpositions Approach to Diagnosis

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Congenital Heart Disease

Cardiac Malpositions

Approach to Diagnosis

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Congenital Heart Disease

Malpositions : Diagnosis

1. Identify situs

2. Identify chambers and vessels

3. Arrange chambers and vessels

4. Identify additional anomalies

5. Define route of blood flow

Diagnosis = 1+ 2 + 3 + 4 + 5

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Congenital Heart Disease

Situs

Solitus usual normal

Inversus mirror image normal

Ambiguous Indeterminate

Aplenia, Polysplenia,

Palpation, Percussion, X-ray

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Congenital Heart Disease

Malpositions : Asplenia

Absent spleen

Malrotation of bowels

Symmetrical liver

Complex CHD

(Bilateral – right sidedness)

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Congenital Heart Disease

Malposition : Polysplenia

Multiple spleens

Malrotation of bowels

Symmetrical liver

Complex CHD

(Bilateral left sidedness)

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RA LA

RV LV

Atria

Ventricles

PA AOGreat arteries

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Congenital Heart Disease

Sequential Chamber Localisation

Define situs

1. Visceroatrial situs rule

2. Tracheal bifurcation

3. ECG

4. ECHO

5. Angiogram

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Congenital Heart Disease

Atrial Situs

Morphologically right atrium

Receives IVC

Broad appendage

Crista terminalis

Fossa ovalis

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Congenital Heart Disease

Atrial situs

Morphologically left atrium

Finger like appendage with narrow neck

Valve of fossa ovalis

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Congenital Heart Disease

Atrial situs

Visceroatrial situs rule

ECG – unreliable

Thoracic roentgenogram – bronchial morphology

Echo – IVC to RA

Femoral vein – contrast inj.

Angiogram – shape of appendage

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Congenital Heart Disease

Atrial situs : Viscero atrial situs rule

Stomach, spleen and LA

Liver & RA Same side

Not applicable with

Situs ambiguus

Asplenia - bilateral RA

Polysplenia - bilateral LA

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Congenital Heart Disease

Situs rule : fallacies

Thoracoabd. discordance 7.2%(No splenic abn.)

Solitus or Inversus predicted 3.6%as ambiguus

Midline liver Do no predict 30%Absent spleen S. ambiguus

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Congenital Heart Disease

Atrial situs : Situs rule

87 cases : Levo, meso, dextrocardia

Position of liver (central = ambiguus) 37%

Liver & Howell Jolly bodies 45%

Rt or Lt sided liver (no polysplenia) 59%

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Congenital Heart Disease

Atrial situs : situs rule

3 lobed lung RA

2 lobes lung LA

Accuracy 72%

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Congenital Heart Disease

Atrial situs : Tracheal bifurcation

RA follows Rt bronchus

LA follows Lt bronchus

Asymmetrical division

Symmetrical – asplenia, polysplenia

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Congenital Heart Disease

Atrial situs : bronchial morphology

LA follows left bronchus – Hyparterial, horizontal, longer

RA follows right bronchus – Eparterial, vertical, shorter

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LeftRight

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4 6 8 10 12 14 1620

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Bronchial length (cm)

Age (Yrs.)

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Congenital Heart Disease

Malpositions : Atrial situs

Bronchial anatomy : Length

Left bronchus : 2 cms

Right bronchus : 2 cms

(overlap below age 2 yrs.)

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Congenital Heart Disease

Atrial situs : bronchial anatomy

Lt / Rt bronchus

2.0 or Normal or Inversus

1.5 to 2.0 Tomography

1.5 or less Atrial isomerism

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Congenital Heart Disease

Atrial situs : ECG

P in 1, II, aVF Normal

in aVR

P in I, II aVF Inversus

in aVR

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Atrial situs

‘P’ wave specificity poor

i Sinus node – left side

ii Ectopic atrial rhythm

iii Bilateral sinus node

(asplenia, polysplenia)

Congenital Heart Disease

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RAIVC

LACV

S

LIVER

*

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LA

RA

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RA

RVLV

CS

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LAA

LA

RALV

RAA

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Liver

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Liver

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Liver

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Ventricular relationship

d loop RV Right side

LV Left side

l loop RV Left side

LV Right side

Indeterminate : Sup. Inferior vent.

Congenital Heart Disease

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Ventricular identification – ECG

QRS morphology RV : LV

rS, Rs 2 : 1

rsR, rSR, rRs 8 : 1

qRS, qR 1 : 3

QRs, QR, Qr 1 : 5

Congenital Heart Disease

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Ventricular identification : Echo• Short axis – mid LV level• 4 chamber – apex – coarse trabeculation & moderator band - RV• Papillary muscle orientation of LV• MV / TV – attachment (4 chamber)• Short axis – fish mouth – MV

Congenital Heart Disease

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LV

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LV

RV

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LV

RA LA

RV

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LV

RV

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Congenital Heart Disease

Chamber localization : Angiogram

• IVC RA

• RA : Broad appendage

• LA : Finger like appendage

• RV : Coarse trabeculation

• LV : Fine trabeculation

(two views : AP/Lat, RAO / LAO)

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Congenital Heart Disease

Establish

Atrio-ventricular relationship

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V

RA

LA

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LA

RV

RA

LV

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RA

RVLV

LA

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Congenital Heart Disease

Great vessel relationship

Normal

Transposition

Malposition

Single artery

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Congenital Heart Disease

• Great Vessels : Normal– Atrioventricular concordance– Both ventricles present– PA from RV – ant & to left– Ao from LV – post & to right

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Congenital Heart Disease

Great arteries : Transposition• PA from LV, Ao from RV• Vent. septum present• d loop : RV Ao

Transposition

LV PA• l loop RV Ao CCTGA

LV PA

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Congenital Heart Disease

Great arteries : Malposition– All GV relations in SV– D.O. RV– D.O. LV– d loop - abn. relation– l loop - abn. relation

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Congenital Heart Disease

Great arteries : Single vessel

Persistent truncus arteriosus

Pulm. atresia

Aortic atresia

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A

P

R L

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P

A

R L

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A

P

R L

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Congenital Heart Disease

Great arteries : Echo

Single

Two GV :

i) One circle the other wrap

around – normal.

ii) Two circles – transposition &

malposition

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PAAo

PAAo

Normally related Great Vessels Great Vessels in Situs Inversus

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a

b

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a

b

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Ao

PA

LARA

Ao

PA

D Malposition L Malposition

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RARV

PAAo

TR

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LV

RV

TR

Ao

RPATR

LA

RA

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A

P

RL

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A

P

R L

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A

P

R L

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Congenital Heart Disease

Establish :

Ventriculo–arterial relationship

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LVRA

AoRPAMPA

Ao

RARV

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RV

Ao

LVLVRA

PA

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PARV

RA

LV

PA

LVRA

RV

Ao

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Congenital Heart Disease

Malpositions : Diagnosis

1. Identify situs

2. Identify chambers and vessels

3. Arrange chambers and vessels

4. Identify additional anomalies

5. Define route of blood flow

Diagnosis = 1+ 2 + 3 + 4 + 5

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RA

LV

PA

LA

RV

Ao

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RA

RV

Ao

LA

LV

PA

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LARA

RV

Ao

LV

PA

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Congenital Heart Disease

Asplenia Syndrome

Symmetrical liver

Stomach : L, R, Midline

Bilateral Rt. Bronchus

Bilateral SVC, single atrium

IVC, Ao same side in Abd.

Common AVC, Single Vent

TGA & Pulm. Atresia, TAPVC

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Congenital Heart Disease

Polysplenia syndrome :Symmetrical liver (less common)Malrotation of bowelsBilateral left bronchusBilateral SVC, Inferior vena cava - azygos or

hemiazygos continuationPAPVC, TAPVCBilateral LA, ASD, ECD, Single AVSD, Single V.(DORV, TGA with PS rare)

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Congenital Heart Disease

Malposition : S. AmbiguousAbd. VisceraBronchial anatomyPeripheral smear – Howell Jolly bodiesECGSplenic scintigraphyUS Abdomen Multiple spleen

Absent spleenIntrahepatic IVC

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Congenital Heart Disease

Malposition : Asplenia

Blood picture

• Polycythemia

• Howell Jolly bodies

• Siderocystosis

• Target cells

• Heinz bodies

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Congenital Heart Disease

Malposition Asplenia (21)PS or Pulm. Atresia 18Heterotaxy 17ASD / Single A. 19VSD / Single V. 20Bilateral SVC 13Howell Jolly bodies 13Bilateral 3 lobed lung 12ECD 14

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