Thoracic Surgery Directors Association (TSDA) Curriculum for Congenital Heart Surgery January, 2011 Nomenclature and Classification of Congenital Heart Disease Jeffrey P Jacobs, M.D The Congenital Heart Institute of Florida (CHIF) All Children’s Hospital Children’s Hospital of Tampa University of South Florida Cardiac Surgical Associates of Florida (CSAoF) Saint Petersburg and Tampa, Florida, United States of America Cardiac Description the segmental anatomy of the heart the classification of the cardiac lesion the description of the cardiac lesion Left and Right For cardiac chambers: ‘‘left’’ refers to morphologically left and ‘‘right’’ refers to morphologically right Cardiac Relations and Cardiac Connections Cardiac relations describe the broad interspatial relations between the various cardiac structures Cardiac connections describe the specific mechanism by which two cardiac structures connect to each other regardless of their interspatial relations Segmental Approach Van Praagh & Vlad the sidedness of the atrial chambers, or, in other words, the atrial “situs”, the ventricular topology, in other words, the ventricular “loop”, and the relationships of the arterial trunks in space Atrial ‘‘situs’’ (the sidedness of the atrial chambers) • The viscera and atria: The types of visceroatrial situs – S solitus (otherwise known as normal arrangement) – I inversus (or the mirror-imaged arrangement) – A ambiguus ‘‘Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralized and thus have neither the usual, or normal, nor the mirror-imaged arrangements.’’ Visceral Situs William T Mahle, MD Associate Professor of Pediatrics Emory University School of Medicine Norman H Silverman Stanford University Lucile Packard Children’s Hospital Ventricular Topology (Ventricular Loop) • The viscera and atria: The types of visceroatrial situs – S solitus – I inversus – A ambiguus • The ventricular topology – D D-loop ventricles, – L L-loop ventricles Robert Anderson Morphologically Right Ventricle Atrioventricular Connections • • Normal atrioventricular connections (Concordant atrioventricular connections in biventricular heart) Discordant AV connections Mixed ("Ambiguous") AV connections (biventricular) • • • • • Absent left sided AV connection Absent right sided AV connection Double inlet AV connection Double inlet AV connection, Double inlet LV Double inlet AV connection, Double inlet RV • Concordant Atrioventricular Connections with Discordant Ventriculoarterial Connections (TGA) Robert H Anderson, Paul M Weinberg The clinical anatomy of transposition In: Jacobs JP, Wernovsky G, Gaynor JW, and Anderson RH (editors) 2005 Supplement to Cardiology in the Young: Controversies of the Ventriculo-Arterial Junctions and Other Topics, Cardiology in the Young, Volume 15, Supplement 1: - 198, February 2005 Ventriculoarterial Connections • • • • • • • • Normal ventriculoarterial connections (Concordant ventriculoarterial connections in biventricular heart) Discordant VA connections (TGA) Single outlet VA connection, Common arterial trunk Single outlet VA connection, Single outlet VA connection via aorta (pulmonary atresia) Single outlet VA connection, Single outlet VA connection via pulmonary trunk (aortic atresia) Double outlet VA connections, Double outlet LV Double outlet VA connections, Double outlet RV Concordant VA connections with parallel great arteries (anatomically corrected malposition) Jacobs JP, Anderson RH, Weinberg P, Henry L Walters III HL, Christo I Tchervenkov CI, Del Ducae D, Franklin RCG, Aiello VD, The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy Béland MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing Paediatric Cardiovascular Practitioners and their Patients, Jacobs JP, Wernovsky G, Gaynor JW, and Anderson RH, editors Cardiology in the Young, Volume 17, Supplement X: XXX - XXX, 2007, accepted for publication, in press, 2007 • Specification of the atrial arrangement, the atrioventricular connections, and the ventriculoarterial connections, does not specify nor imply the ventricular topology, or the relationships of the cardiac chambers and great arteries in space These variables are separately specified Cardiac Position Can be surgically important – for example – Pathway for extracardiac Fontan • Dextrocardia (right sided ventricular mass) • Levocardia (left sided ventricular mass) • Mesocardia (central/midline ventricular mass) • Dextroversion (ventricular apex points to the right) • Levoversion (ventricular apex points to the left) Cardiac Position Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G, editors Cardiology in the Young, Volume 17, Supplement 2, pages 1–28, doi: 10.1017/S1047951107001138, September 2007 “Heterotaxy is synonymous with “visceral heterotaxy” and “heterotaxy syndrome” Heterotaxy is defined as an abnormality where the internal thoracoabdominal organs demonstrate abnormal arrangement across the left-right axis of the body By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the leftright axis, also known as “situs solitus”, nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as “situs inversus”.” Segmental Anatomy of the Heart situs ventricular topology (in other words, or ventricular loop) atrioventricular connections ventriculoarterial connections the relationships of the arterial trunks in space the position of the heart and the orientation of its apex Cardiac Description the segmental anatomy of the heart the classification of the cardiac lesion the description of the cardiac lesion • • • • • • • • • • • • • • • • • • Ventricular septal defect Atrial septal defect Patent ductus arteriosus Aortic coarctation Congenital aortic stenosis Tetralogy of Fallot Pulmonary stenosis Transposition of the great arteries Pulmonary atresia – With intact ventricular septum – With ventricular septal defect Atrioventricular septal defect Tricuspid atresia Truncus arteriosus Total anomalous pulmonary venous connection Hypoplastic left heart syndrome Interrupted aortic arch Ebstein’s anomaly Mitral stenosis Aortopulmonary window ACYANOTIC • Ventricular septal defect • Atrial septal defect • Patent ductus arteriosus • Aortic coarctation • Congenital aortic stenosis • Atrioventricular septal defect • Aortopulmonary window • Interrupted aortic arch • Mitral stenosis CYANOTIC • Tetralogy of Fallot • Pulmonary stenosis • Transposition of the great arteries • Pulmonary atresia – With intact ventricular septum – With ventricular septal defect • • • • • Tricuspid atresia Truncus arteriosus Total anomalous pulmonary venous connection Hypoplastic left heart syndrome Ebstein’s anomaly Segmental System of Classification of Lesions of Congenital Heart Disease: Level I II III IV V VI Great veins Atria Atrioventricular junction Ventricles Ventriculoarterial junction Great arteries I GREAT VEINS Systemic veins Pulmonary veins II ATRIA Right atrium Atrial septum Left atrium III ATRIOVENTRICULAR JUNCTION Right atrioventricular valve Common atrioventricular valve Left atrioventricular valve IV VENTRICLES Right ventricle Ventricular septum Left ventricle Single ventricle V VENTRICULOARTERIAL JUNCTION Right ventriculoarterial valve Common ventriculoarterial valve Left ventriculoarterial valve Both ventriculoarterial valves VI GREAT ARTERIES Pulmonary artery Aorta Both great arteries Coronary arteries Cardiac Description the segmental anatomy of the heart the classification of the cardiac lesion the description of the cardiac lesion The International Paediatric and Congenital Cardiac Code (IPCCC) http://www.IPCCC.NET ... space the position of the heart and the orientation of its apex Cardiac Description the segmental anatomy of the heart the classification of the cardiac lesion the description of the cardiac lesion... anomalous pulmonary venous connection Hypoplastic left heart syndrome Ebstein’s anomaly Segmental System of Classification of Lesions of Congenital Heart Disease: Level I II III IV V VI Great veins Atria... segmental anatomy of the heart the classification of the cardiac lesion the description of the cardiac lesion Left and Right For cardiac chambers: ‘‘left’’ refers to morphologically left and ‘‘right’’