Segmental approach to normal and abnormal situs arrangement - Echocardiography -

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1 Segmental approach to normal and abnormal situs arrangement - Echocardiography - Jan Marek Great Ormond Street Hospital & Institute of Cardiovascular Sciences, University College London No disclosures

2 SEQUENTIAL SEGMENTAL ANALYSIS ATRIUMS ARTERIAL TRUNKS ATRIOVENTRICULAR CONNECTIONS VENTRICULO-ARTERIAL CONNECTIONS VENTRICLES ANALYSE SEGMENTS Courtesy RH Anderson & A. Cook

3 Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

4 Usual arrangement (Solitus) A R Liver IVC AO ST SP

5 Mirror imagery (Inversus) A R

6 Right isomerism (Asplenia) Liver Liver AO IVC A R

7 Left isomerism (Polysplenia) Liver Liver AO AZ A R

8 Fetus 32 WoG Left isomerism (Polysplenia)

9 Isomerism: Polysplenia & Asplenia (Heterotaxy syndrom)

10

11

12 Right atrial isomerism: LSCV + left Hemiazygos vein

13 Position of the heart in the chest Levocardia Dextrocardia Mesocardia Dextroversion Levoversion Dextroposition Levoposition

14 Position of the heart in the chest Dextrocardia with Apex pointing to the Right Levocardia with Apex pointing to the Left Midline Mesocardia with Apex pointing to the Right Left Dextrocardia with Apex pointing to the Left Levocardia with Apex pointing to the Right

15 Imaging mirror imagery dextrocardia Subcostal views important Left mark always on pt`s left side Never flip R/L side!

16 Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

17 Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

18 Usual atrial arrangement (Solitus)

19 Mirror imagery dextrocardia

20 Right atrial isomerism Bilateral right atrial appendages

21 Left atrial isomerism Bilateral left atrial appendages

22 Juxtaposition of atrial appendages

23 Atrial appendages: TOE

24 Abdominal situs Atriums - Atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

25 Abdominal situs Atriums - Atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

26 Atrio-ventricular connections Concordant Discordant Mixed (R/L Isomerism) Univentricular AV connections Absent AV connection Common AV valve Double inlet connections

27 Concordant AV connections PV AO RA LA PV RV LV Offsetting

28 Concordant AV connection Discordant AV connection

29 Discordant AV connections

30 Univentricular AV connections Absent AV connection (right / left) Tricuspid / mitral atresia Double inlet ventricle Two separate valves Common AV valve Anatomically left Anatomically right Undetermined (rudimentary almost chamber always present)

31 Absent right AV connection Absent left AV connection Double inlet (2 AV valves) Double inlet (Common AV valve)

32 Univentricular AV connections Double inlet ventricle vs Ventricular septal defect DIV > 50%

33 Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

34 Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)

35 Ventriculo-arterial connections Concordant (ie.usual) Ventricular septal defect, tetralogy of Fallot Discordant Transposition Double outlet ventricle With subaortic VSD subpulmonary VSD doubly committed VSD non-committed VSD Single outlet

36 TOF vs Double outlet right ventricle Ao Ao Pa Pa Ao RV LV TOF Double outlet right ventricle Courtesy RH Anderson & A. Cook

37 Overriding aortic valve: 50% rule VSD TOF DORV

38 Complex Double outlet right ventricle

39 Discordant AV / VA connections Congenitally Corrected TGA

40 Overriding aortic valve: 50% rule Double outlet left ventricle

41 How to diagnose... Correct description of disease? Transposition? Congenitally Corrected Transposition? Malposition? Corrected malposition? L- Transposition? Ventricular inversion? Right/Left? D- Transposition AV concordance & VA discordance Right/Left ventricle with two outlets AV discordance & VA discordance AV discordance & VA concordance TGA DORV CCTGA CCMGA DOLV

42 AV concordance & VA concordance with malposition of the great arteries

43

44 Segmental approach Important to understand situs arrangement Provides complete coverage of lesion Minimises potential errors Easy for reading and interpretation First comprehensive scan most important on virgin heart most important!

45 Sequential Segmental Approach KISS rule Keep It Simple, Stupid

46

47 Double conus = Double outlet right ventricle? Double conus does not determine DORV!

48 Univentricular AV connections * RA LA Absent right AV connection RA LA * Absent left AV connection RA RA LA RA RA * * Common AV valve Double inlet

49 Univentricular AV connections Absent AV connection (right / left) Tricuspid / mitral atresia Common AV valve With morphologically single ventricle Double inlet ventricle Connection of both AV valve to the same ventricle: Anatomically left Anatomically right Undetermined (rudimentary almost chamber always present)

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