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Tetralogy of Fallot Morphology Andrew C Cook Head of Cardiovascular Morphology & Education UCL Great Ormond Street Institute of Cardiovascular Science London, UK Tetralogy of Fallot ‣ Understanding Anatomy - What do we need to know ? - Structure of the normal RVOT - Change to components in Tetralogy - The spectrum of associated malformations Normal RV Outflow Tract WWW.CARDIACMORPHOLOGY.COM Components of the Structurally Normal RVOT Pulmonary valve VentriculoInfundibular Fold (VIF) Outlet Septum Membranous Septum Inferior branch of SMT Medial Papillary Muscle Tricuspid Valve Moderator Band INSTITUTE OF CARDIOVASCULAR SCIENCES / GOSH Infundibulum (conus) Superior branch of SMT Septo-parietal trabeculations Body of Septo-marginal trabeculation (SMT) Coarse Apical Trabeculations Where is the outlet septum ? STRUCTURE IN THE NORMAL HEART Infundibulum (‘conus’) Ventricular Infundibular fold Infundibulum (‘conus’) Resected outlet septum Outside heart SPTs Outlet septum SeptoMarginal Trabec (SMT) SMT Outlet septum in plane of ventric. septum SMT To left ventricle Supra ventricular crest Outlet septum is minuscule in the normal heart Components of the RVOT in Tetralogy of Fallot VentriculoInfundibular Fold (VIF) Overriding Aorta VSD Membranous Septum Inferior branch of SMT Medial Papillary Muscle Pulmonary valve Outlet Septum Infundibulum (conus) Sub-pulmonary narrowing Septo-Parietal Trabeculations Superior branch of SMT Body of SeptoMarginal Trabeculation Tricuspid Valve Moderator Band INSTITUTE OF CARDIOVASCULAR SCIENCES / GOSH Hypertrophy of Right Ventricle Outlet Septum in RVOT Short Axis View WWW.CARDIACMORPHOLOGY.COM Outlet Septum in RVOT Short Axis View Courtesy Jan Marek WWW.CARDIACMORPHOLOGY.COM Rotation and Displacement of Outlet Septum WWW.CARDIACMORPHOLOGY.COM Rotation and Displacement of Outlet Septum RVOT RVOT Parietal Septal Parietal Septal SMT SMT Antero-Cephalad Deviation INSTITUTE OF CARDIOVASCULAR SCIENCES / GOSH WWW.CARDIACMORPHOLOGY.COM Tetralogy of Fallot is a Spectrum of Disease WWW.CARDIACMORPHOLOGY.COM Tetralogy of Fallot ‣ Variability in Phenotype • • • • • • • Deviation of & structure of Outlet Septum Type of VSD - usually perimembranous Degree of aortic override Length of outlet septum Presence of RV hypertrophy Structure of pulmonary valve & pulm. arteries Other associated lesions... - AVSD, right aortic arch, absence of arterial duct Coronary branch crossing RVOT, Aortic dilation Tetralogy of Fallot SPECTRUM OF DISEASE - SPECTRUM OF RVOTO - HUMAN FETUS ‘Mexican’ Tetralogy WWW.CARDIACMORPHOLOGY.COM RVOTO with marked hypertrophy in Unrepaired Adult ToF WWW.CARDIACMORPHOLOGY.COM Tetralogy of Fallot ‣ Pulmonary valve & arteries • Usually thickened, stenotic, bicuspid - • Can also be rudimentary (absent pulm. valve) - • PA hypoplasia, duct typically present PA enlargement, duct sometimes present At severe end can be atretic - Severe PA hypoplasia, duct sometimes present Or presence of systemic to pulmonary collateral arteries (MAPCAS - major aortapulmonary collateral arteries) Tetralogy of Fallot SPECTRUM IN SIZE OF PULMONARY TRUNK Smaller Normal Atretic Small Tetralogy of Fallot 24 WEEK GESTATION FETUS Tetralogy of Fallot - Un-repaired Adult SEVERE PULMONARY VALVAR STENOSIS Tetralogy of Fallot - 25 week gestation fetus ABSENT PULMONARY VALVE, ABSENT ARTERIAL DUCT, HUGE LPA,RPA Tetralogy of Fallot ATRETIC PULMONARY VALVE - PULMONARY ARTERIES FED VIA DUCT Tetralogy of Fallot PULMONARY ATRESIA + ABSENT DUCT + SMALL COLLATERALS TO LUNGS (MAPCAS) Tetralogy of Fallot ‣ Summary • Key to understanding is the outlet septum • Change in position leads to features of Fallot’s Tetralogy Spectrum of disease : - Variation in features - Minimal obstruction & typical features - Severe end affecting entire RVOT, pulmonary arteries, valve and arterial duct