Congenital Heart Disease Cardiac Malpositions Approach to Diagnosis

Congenital Heart Disease Malpositions : Diagnosis Identify situs Identify chambers and vessels Arrange chambers and vessels Identify additional anomalies Define route of blood flow Diagnosis = 1+ 2 + 3 + 4 + 5

Congenital Heart Disease Situs Solitus usual normal Inversus mirror image normal Ambiguous Indeterminate Aplenia, Polysplenia, Palpation, Percussion, X-ray

Congenital Heart Disease Malpositions : Asplenia Absent spleen Malrotation of bowels Symmetrical liver Complex CHD (Bilateral – right sidedness)

Congenital Heart Disease Malposition : Polysplenia Multiple spleens Malrotation of bowels Symmetrical liver Complex CHD (Bilateral left sidedness)

Atria RA LA Ventricles RV LV Great arteries PA AO

Congenital Heart Disease Sequential Chamber Localisation Define situs Visceroatrial situs rule Tracheal bifurcation ECG ECHO Angiogram

Congenital Heart Disease Atrial Situs Morphologically right atrium Receives IVC Broad appendage Crista terminalis Fossa ovalis

Congenital Heart Disease Atrial situs Morphologically left atrium Finger like appendage with narrow neck Valve of fossa ovalis

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

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

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

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%

Congenital Heart Disease Atrial situs : situs rule 3 lobed lung RA 2 lobes lung LA Accuracy 72%

Congenital Heart Disease Atrial situs : Tracheal bifurcation RA follows Rt bronchus LA follows Lt bronchus Asymmetrical division Symmetrical – asplenia, polysplenia

Congenital Heart Disease Atrial situs : bronchial morphology LA follows left bronchus – Hyparterial, horizontal, longer RA follows right bronchus – Eparterial, vertical, shorter

Bronchial length (cm) 6 Left Right 5 4 3 2 1 2 4 6 8 10 12 14 16 Age (Yrs.)

Congenital Heart Disease Malpositions : Atrial situs Bronchial anatomy : Length Left bronchus :  2 cms Right bronchus :  2 cms (overlap below age 2 yrs.)

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

Congenital Heart Disease Atrial situs : ECG P  in 1, II, aVF Normal  in aVR P  in I, II aVF Inversus  in aVR

Congenital Heart Disease Atrial situs ‘P’ wave specificity poor i Sinus node – left side ii Ectopic atrial rhythm iii Bilateral sinus node (asplenia, polysplenia)

* S V C LA RA IVC LIVER

LA RA 34

RA CS LV RV 35

LAA LA RAA LV RA 36

Liver

Liver

Liver

Congenital Heart Disease 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 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 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

LV

RV LV

LA RA RV LV

RV LV

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)

Congenital Heart Disease Establish Atrio-ventricular relationship

LA RA V 58

LA RA LV RV

RA LA RV LV

Congenital Heart Disease Great vessel relationship Normal Transposition Malposition Single artery

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

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

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

Congenital Heart Disease Great arteries : Single vessel Persistent truncus arteriosus Pulm. atresia Aortic atresia

P L R A

P R L A

P R L A

Congenital Heart Disease Great arteries : Echo Single Two GV : i) One circle the other wrap around – normal. ii) Two circles – transposition & malposition

Normally related Great Vessels Great Vessels in Situs Inversus PA Ao PA Ao

a b

a b

D Malposition L Malposition Ao PA LA RA Ao PA

PA Ao TR RV RA

RV RPA LV TR TR LA Ao RA

A L R P

A R L P

A L R P

Congenital Heart Disease Establish : Ventriculo–arterial relationship

MPA RPA Ao Ao LV RA RV RA 81

PA Ao RA LV LV RV 82

RV Ao PA PA RV RA LV RA LV

Congenital Heart Disease Malpositions : Diagnosis Identify situs Identify chambers and vessels Arrange chambers and vessels Identify additional anomalies Define route of blood flow Diagnosis = 1+ 2 + 3 + 4 + 5

RA LA RV LV PA Ao

LA RA RV LV Ao PA

RA LA LV RV Ao PA

Thank you

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

Congenital Heart Disease Polysplenia syndrome : Symmetrical liver (less common) Malrotation of bowels Bilateral left bronchus Bilateral SVC, Inferior vena cava - azygos or hemiazygos continuation PAPVC, TAPVC Bilateral LA, ASD, ECD, Single A VSD, Single V. (DORV, TGA with PS rare)

Congenital Heart Disease Malposition : S. Ambiguous Abd. Viscera Bronchial anatomy Peripheral smear – Howell Jolly bodies ECG Splenic scintigraphy US Abdomen Multiple spleen Absent spleen Intrahepatic IVC

Congenital Heart Disease Malposition : Asplenia Blood picture Polycythemia Howell Jolly bodies Siderocystosis Target cells Heinz bodies

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