Download Tetralogy of Fallot Morphology

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