Segmental approach to normal and abnormal situs arrangement - Echocardiography - Jan Marek Great Ormond Street Hospital & Institute of Cardiovascular Sciences, University College London No disclosures
SEQUENTIAL SEGMENTAL ANALYSIS ATRIUMS ARTERIAL TRUNKS ATRIOVENTRICULAR CONNECTIONS VENTRICULO-ARTERIAL CONNECTIONS VENTRICLES ANALYSE SEGMENTS Courtesy RH Anderson & A. Cook
Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
Usual arrangement (Solitus) A R Liver IVC AO ST SP
Mirror imagery (Inversus) A R
Right isomerism (Asplenia) Liver Liver AO IVC A R
Left isomerism (Polysplenia) Liver Liver AO AZ A R
Fetus 32 WoG Left isomerism (Polysplenia)
Isomerism: Polysplenia & Asplenia (Heterotaxy syndrom)
Right atrial isomerism: LSCV + left Hemiazygos vein
Position of the heart in the chest Levocardia Dextrocardia Mesocardia Dextroversion Levoversion Dextroposition Levoposition
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
Imaging mirror imagery dextrocardia Subcostal views important Left mark always on pt`s left side Never flip R/L side!
Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
Usual atrial arrangement (Solitus)
Mirror imagery dextrocardia
Right atrial isomerism Bilateral right atrial appendages
Left atrial isomerism Bilateral left atrial appendages
Juxtaposition of atrial appendages
Atrial appendages: TOE
Abdominal situs Atriums - Atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
Abdominal situs Atriums - Atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
Atrio-ventricular connections Concordant Discordant Mixed (R/L Isomerism) Univentricular AV connections Absent AV connection Common AV valve Double inlet connections
Concordant AV connections PV AO RA LA PV RV LV Offsetting
Concordant AV connection Discordant AV connection
Discordant AV connections
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)
Absent right AV connection Absent left AV connection Double inlet (2 AV valves) Double inlet (Common AV valve)
Univentricular AV connections Double inlet ventricle vs Ventricular septal defect DIV > 50%
Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
Abdominal situs Atriums - atrial situs (systemic and pulmonary venous connections) AV connections Ventricles VA connections Arterial trunks (AO arch and PA branches)
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
TOF vs Double outlet right ventricle Ao Ao Pa Pa Ao RV LV TOF Double outlet right ventricle Courtesy RH Anderson & A. Cook
Overriding aortic valve: 50% rule VSD TOF DORV
Complex Double outlet right ventricle
Discordant AV / VA connections Congenitally Corrected TGA
Overriding aortic valve: 50% rule Double outlet left ventricle
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
AV concordance & VA concordance with malposition of the great arteries
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!
Sequential Segmental Approach KISS rule Keep It Simple, Stupid
Double conus = Double outlet right ventricle? Double conus does not determine DORV!
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
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)