Survey
* Your assessment is very important for improving the work of artificial intelligence, which forms the content of this project
Download Morphological determinants.
Document related concepts
no text concepts found
Transcript
FUNCTIONALLY SINGLE VENTRICLE MORPHOLOGICAL DETERMINANTS VI TRAN Institute of Cardiovascular Science, UCL, London EuroEcho, Budapest, 7th December 2011 DECLARATION OF CONFLICT OF INTEREST: I have nothing to declare FUNCTIONALLY SINGLE VENTRICLE What is the functionally single ventricle? The heart that is incapable of supporting both the pulmonary and systemic circulations Why should this be? Only one ventricle within ventricular mass Two ventricles, but one is incapable of supporting the entirety of either the systemic or the pulmonary circulation Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Ventricles unable to support circulations Normally formed, but too small Hypoplastic and rudimentary Obstructions to flows through inlet or outlet Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Anatomic substrates Tricuspid atresia Double inlet left ventricle Hypoplastic left heart Pulmonary atresia with intact ventricular septum Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Understanding and describing the anatomy Analysis of atrioventricular junctions Do atrial chambers connect to both ventricles or to only one? Description of ventricular morphology What is the morphologic nature of the pumping ventricle, and is there a second ventricle? Describe arrangement in logical fashion Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Logical analysis Analyse ventricles in tripartite fashion Use the “Morphological method” Van Praagh et al, 1980 Use most constant feature of any structure for definition Do not define any structure that is itself variable on the basis of another variable feature Institute of Cardiovascular Science, UCL, London Morphologically Right Ventricle Outlet Inlet Institute of Cardiovascular Science, UCL, London Apical trabecular component Morphologically Left Ventricle Outlet Apical trabecular component Inlet Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Tripartite ventricular description Normal ventricles each have three parts Morphology determined by apical component Abnormal ventricles have inlets and outlet components shared between apical parts One ventricle is dominant Other ventricle is rudimentary & incomplete Rudimentary RV is always antero-superior Rudimentary LV is always postero-inferior Hearts with one big and one small ventricle are functionally univentricular Institute of Cardiovascular Science, UCL, London Double Inlet Left Ventricle Dominant Left Ventricle Rudimentary Right Ventricle Institute of Cardiovascular Science, UCL, London “Classical” Tricuspid Atresia Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Are these entities single ventricles? Both have one big and one small chamber Big chamber is morphologically left ventricle Small chamber is the incomplete and hypoplastic morphologically right ventricle “Univentricular” only if we deny ventricular status to small chamber! Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Logical description Tricuspid atresia & DILV both have big left ventricle and small, incomplete, RV Neither is an example of “univentricular heart” or “single ventricle” We were wrong, in the past, to deny ventricular status to small RV It is the ATRIOVENTRICULAR CONNECTION which is truly univentricular! Hole between chambers is VSD Institute of Cardiovascular Science, UCL, London UNIVENTRICULAR AV CONNECTION Institute of Cardiovascular Science, UCL, London Double Inlet Right Ventricle Dominant Right Ventricle Rudimentary Left Ventricle Rudimentary LV rarely can be right-sided Institute of Cardiovascular Science, UCL, London Double Inlet Solitary Ventricle Solitary and Indeterminate Ventricle Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Hearts with Biventricular AV Connection can also be functionally univentricular! Unbalanced atrioventricular septal defect Imperforate atrioventricular valve Hypoplastic left heart Hypoplastic right heart (pulmonary atresia with intact ventricular septum) Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Unbalanced AV Septal Defect Institute of Cardiovascular Science, UCL, London Imperforate Tricuspid Valve Biventricular AV connections Right atrium Left atrium Hypoplastic RV Left ventricle Functionally Univentricular Heart Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Hypoplastic left heart Mitral atresia or stenosis Aortic atresia or stenosis Left ventricle not capable of supporting systemic circulation Irrespective of morphology, all examples are functionally univentricular Institute of Cardiovascular Science, UCL, London Hypoplastic left heart Univentricular AV Connection Institute of Cardiovascular Science, UCL, London Hypoplastic Left Heart Biventricular AV Connections Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Pulmonary atresia with intact vent. septum Spectrum of malformation Overgrowth of RV cavity All ventricles have three components Cavity can be tripartite, bipartite, or unipartite Institute of Cardiovascular Science, UCL, London Pulmonary Atresia with Intact VS Minimal overgrowth of apical component Functionally biventricular Institute of Cardiovascular Science, UCL, London Pulmonary Atresia with Intact VS Bipartite, suitable for 1 ½ ventricle repair? Institute of Cardiovascular Science, UCL, London Pulmonary Atresia with Intact VS Functionally univentricular Institute of Cardiovascular Science, UCL, London FUNCTIONALLY SINGLE VENTRICLE Conclusions Many hearts can be functionally univentricular Anatomically univentricular hearts are exceedingly rare Functionally single ventricles can have biventricular or univentricular AV connections Analyse separately ventricular morphology and AV junctional morphology Tripartite approach to ventricular morphology Institute of Cardiovascular Science, UCL, London
Related documents