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Segmental approach to normal and abnormal situs arrangement - Echocardiography Jan Marek Great Ormond Street Hospital & Institute of Cardiovascular Sciences, University College London No disclosures How the heart should be imaged? “European Chaos” •“Transatlantic” way LV LV •“Adult” way •“Dutch” way LV 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 ST IVC AO SP Mirror Imagery (Inversus) A R Right Isomerism (Asplenia) Liver Liver IVC AO A R Left Isomerism (Polysplenia) Liver Liver AO AZ A R Left Isomerism (Polysplenia) Fetus 32 WoG Isomerism (Polysplenia & Asplenia sy) Isomerism (Polysplenia & Asplenia sy) Is the symmetry always what we like to see? Courtesy Ian Sullivan Position of the Heart in the Chest •Levocardia •Dextrocardia •Mesocardia •Dextroversion •Levoversion •Dextroposition •Levoposition Position of the Heart in the Chest Levocardia with Apex pointing to the Left Dextrocardia with Apex pointing to the Right Mesocardia with Apex pointing to the Right Left Dextrocardia with Apex pointing to the Left Levocardia with Apex pointing to the Right • 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) - Atrial appendages - Fetus 20. WoG Right Isomerism (Asplenia) - Atrial appendages - Left Isomerism (Polysplenia) - Atrial appendages - Atrial appendages - Juxtaposition - • Abdominal situs • Atriums - Atrial situs (systemic and pulmonary venous connections) • AV connections • Ventricles • VA connections • Arterial trunk (AO arch and PA branches) Atrio-ventricular Connections •Concordant •Discordant •Univentricular AV connections Absent AV connection Common AV valve Double inlet connections Concordant Connections PV AO RA LA PV RV LV Offsetting Concordant AV connection Discordant AV connection Discordant Connections CCTGA DORV/VI PA/VSD/VI Univentricular AV Connections •Absent AV connection (right / left) Tricuspid / mitral atresia •Common AV valve With single ventricle •Double inlet ventricle Connection of both AV valve to the same ventricle: Anatomically left Anatomically right Undetermined (rudimentary chamber always present) Univentricular AV connections RA LA RA LA Absent right AV connection * * Absent left AV connection LA RA * Common AV valve RA RA * Double inlet RA Absent right AV connection Absent left AV connection Double inlet Common AV valve Univentricular AV connections Double inlet ventricle versus Ventricular septal defect LA DIV > 50% RV S LV • 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 (=normal) Ventricular septal defect, tetralogy of Fallot… • Discordant Transposition •Double outlet ventricle With subaortic VSD subpulmonary VSD doubly committed VSD non-committed VSD •Solitary arterial trunk Discordant AV / VA connections Congenitally Corrected TGA Overriding aortic valve: 50% rule VSD TOF DORV TOF or Double outlet right ventricle? RV TOF vs Double outlet right ventricle Ao Pa Ao RV TOF Pa Ao LV Double outlet right ventricle Courtesy RH Anderson & A. Cook Double conus = Double outlet right ventricle? Double conus does not determine DORV! 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 TGA Right/Left ventricle with two outlets AV discordance & VA discordance AV discordance & VA concordance DORV DOLV CCTGA CCMGA 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 Sequential Segmental Approach KISS Keep It Simple, Stupid