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Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Robert W. McDonald, RCS, RDCS, FASE Doernbecher Children’s Hospital Portland, Oregon Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Lesson Objectives: The participant will be able to: • Improve patient care through segmental approach. • Improve sonographer/MD communication. • Improve efficiency in congenital heart disease echo exam. • Increase congenital heart disease awareness amongst all sonographers. • Assure complete classification of cardiovascular morphology and physiology in any patient with congenital heart disease. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Why is using a segmental approach important? • More and more children with congenital heart disease are reaching adulthood; palliated, repaired and un-repaired. • Necessary in order to describe, visualize, document and communicate findings adequately. • Increases understanding of structural, hemodynamic, and functional aspects of congenital heart disease. • Improves patient care. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis What is it? • A methodical description of the anatomical and hemodynamic inter-relationship of cardiac structure, function and physiology. How is it applied? • Utilize multiple echo planes to visualize all cardiac structures and related viscera. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Who can do it? • All sonographers and physicians. When is a segmental approach necessary? • Anytime a patient with complex or substantial congenital heart disease lesions is being evaluated by echo. • Anytime when a standard approach is confusing due to abnormal and/or complex structural or surgical anatomy. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Transthoracic technique • If parasternal images are bizarre or confusing, move to the apical or subcostal views. • If known complex congenital heart disease exists, start from either the apical or subcostal view.. • Pay special attention to proper transducer orientation. • Utilize all available echo windows. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Back to the Basics • Cardiac anatomy • How the blood flows Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis • Determination of cardiac location and situs – Two major organ groups • The abdominal viscera – positions of liver, stomach, spleen, and abdominal great vessels (aorta and inferior vena cava) • The atria (cardiac situs) – Arrangement of the atria – Three possible positions of the organ groups • Solitus – normal position • Inversus – mirror image of normal • Ambiguous – complex, spatial arrangement of the organs Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Abdominal situs • Situs Solitus – Normal arrangements – Left stomach, left spleen, right liver, right tri-lobed lung • Situs Inversus – Inverted arrangements – Right stomach, right spleen, left liver, left tri-lobed lung Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Abdominal situs • Left Atrial Isomerism (Polysplenia) – Bilateral left-sidedness – Multiple spleens, often interrupted IVC. • Right Atrial Isomerism (Asplenia) – Bilateral right-sidedness – No spleen • Often PS/PA, dextrocardia, AVC defect, TAPVC, no coronary sinus. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Cardiac Location • Cardiac Position – Levoposition – Mesoposition – Dextroposition • Cardiac Orientation – Levocardia – Mesocardia – Dextrocardia Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis • Venous segment • Veno-atrial connection • Atrial segment • Atrioventricular connection • Ventricular segment • Ventricular-great arterial connection • Great arterial segment Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Venous Segment • Systemic veins – – – – Inferior vena cava Superior vena cava Coronary sinus Hepatic veins • Pulmonary veins – Right upper and lower veins – Left upper and lower veins Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Atrial Segment • Right Atrium – Large pyramidal appendage – Terminal crest • (crista terminalis) – Pectinate muscles – Receives caval veins and coronary sinus • Variable feature • Left Atrium – Small fingerlike appendage – No pectinate muscles – Receives pulmonary veins • Variable feature Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Atrioventricular Valves • Tricuspid Valve – Low septal annular attachment – Septal cordal attachments – Triangular orifice • (mid-leaflet level) – Three leaflets and commissures – Three papillary muscles – Empties into right ventricle Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Atrioventricular Valves • Mitral Valve – High septal annular attachment – No septal cordal attachments – Elliptical orifice • (mid-leaflet level) – Two leaflets and commissures – Two large papillary muscles – Empties into left ventricle Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Atrioventricular Connection • • • • Concordant Discordant Ambiguous Double inlet – Univentricular • Single inlet – Atresia • Common Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Atrioventricular Connection • AV valves follow the ventricle • Can be right and left straddling, overriding • Functional assessment – – – – Normal Regurgitation Hypoplasia (atresia) Obstruction/Stenosis Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Ventricular Segment • Right Ventricle – Tricuspid-pulmonary discontinuity – Muscular outflow tract – Septal and parietal bands – Large apical trabeculations – Coarse septal surface – Crescentic in crosssections – Thin free wall – Receives tricuspid valve Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Ventricular Segment • Left Ventricle – Mitral-aortic continuity – Muscular-valvular outflow tract – No septal or parietal band – Small apical trabeculations – Smooth upper septal surface – Circular in crosssection – Thick free wall – Receives mitral valve Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Semilunar Valves • Aortic Valve – Tri-leaflet valve – Empties into the ascending aorta – Coronary arteries • Pulmonary Valve – Tri-leaflet valve – Empties into the pulmonary trunk Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Ventriculoarterial Connection – Describes the junction of ventricular outflow into the great arteries • • • • • Concordant Discordant Double outlet Single outlet Common outlet Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Ventriculoarterial Connection 50% rule Fibrous continuity Visualize the amount of override Evaluate outlet orientation to each other and ventricle Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Great Arterial Segment • Solitus • Side-by-side • Transposed • refers to abnormal relation of the great arteries to each other – d-looped – l-looped – Anterior Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Great Arterial Segment – Describes the presence, absence, origin, size, position and anatomic deformities • Aorta – Ascending aorta – Aortic arch • Brachiocephalic • Left common carotid • Left subclavian – Descending aorta • Pulmonary Artery – Main and branch pulmonary arteries – Ductus arteriosus Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Blood Flow • Describe the blood flow into and out of the heart in a methodical manner. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Summary • Segmental approach provides a framework that can support the understanding of any congenital heart defect. • Can help the sonographer and physician define anatomy in simple terms, using segments and connections. – Use descriptive terms what you can identify and state what you cannot. • Guides the sonographer in obtaining all echocardiographic images to better understand congenital heart disease. • Improves patient care. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis References • • • • • Edwards WD. Cardiac anatomy and examination of cardiac specimens. In Moss and Adams Heart Disease in Infants, Children, and Adolescents. 6th ed., Allen HD, Gutgesell HP, Clark EB, Driscoll DJ (eds). Philadelphia, PA: Lippincott Williams and Wilkins, 2001, 80-117. Edwards WD. Classification and terminology of cardiovascular anomalies. In Moss and Adams Heart Disease in Infants, Children, and Adolescents. 6th ed., Allen HD, Gutgesell HP, Clark EB, Driscoll DJ (eds). Philadelphia, PA: Lippincott Williams and Wilkins, 2001, 118-142. Kimbal TR, Meyer RA. Echocardiography. In Moss and Adams Heart Disease in Infants, Children, and Adolescents. 6th ed., Allen HD, Gutgesell HP, Clark EB, Driscoll DJ (eds). Philadelphia, PA: Lippincott Williams and Wilkins, 2001, 204-233. O’Leary PW. The segmental approach to congenital heart disease. Pediatric Ultrasound Today 2005;10(5):105-132. Reller MD, McDonald RW, Gerlis LM and Thornburg KL: Basic cardiac embryology with clinical correlations to congenital cardiac defects. J Am Soc Echocardiogr 1991;4:519-532. Congenital Heart Disease for the Adult Sonographer: How Do I … Image with Segmental Analysis Web References • Anderson RH, Cook AC, Shirali GS. Atrioventricular septal malalignment. www.acc.org/community/pediatric/opinion_apr03.htm. • www.user.gru.net/clawrence/vccl/chpt1/fetal.HTM • www.pediheart.org/practitioners/anatomy/ventricles.htm • http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066 -782X1999001100005 • http://www.med.mun.ca/anatomyts/first/heart1.html • http://www.cicmd.com/images/cicmd/anatomy%20images/AN ATOMY.htm • http://www.guidant.com/