Congenital Heart Disease Flashcards
Atrial septal defect (ASD) — forms ?
Ostium primum defect (ASD1)
Ostium secundum defect (ASD2)
Sinus venosus defect (superior and inferior)
Persistent foramen ovale (PFO)
Ostium primum defect (ASD1) — facts ?
absent fusion of the septum primum with the endocardial cushion
occurs in the 5th week of pregnancy
~20% of all ASD
located just next to the AV-valve plane
often associated with AV-valve anomalies e.g. mitral cleft
Ostium secundum defect (ASD2) — facts ?
failure of the development of the septum secundum
occurs in the 6th week of pregnancy
~ 70% of all ASD
located in the oval fossa
associated with other congenital anomalies, e.g. ~25% PAPVR
Sinus venosus defect — facts ?
~ 3-10% of all ASD
~93% associated with PAPVR
usually right upper pulmonary vein with superior sinus venosus defect
scimitar syndrome with inferior sinus venosus defect
Persistent foramen ovale (PFO) — facts ?
normal variant
~25-30%, f>m 2:1
can lead to paradoxical emboli during rise of right atrial pressure (valsalva maneuver)
atrial septal defect (ASD) — MRI protocol ?
Cine-SSFP (2ch, 3ch, 4ch, SA stack, atrial stack, LVOT, RVOT)
PCI (Ao, Pu, ASD Flow)
MRA
Atrial septal defect (ASD) — MRI report ?
LV and RV function including EDV, ESV, SV, EF and RWMAs ASD type, location, size Qp:Qs — Pa flow : Ao flow Pulmonary venous connection associated findings
Atrial septal defect (ASD) — red flags ?
significant RV dilatation or dysfunction
Qp:Qs > 1.8:1
associated abnormalities e.g. PAPVR
pulmonary hypertension
Atrial septal defect (ASD) — therapy and complications ?
intervention surgical closure (sinus venosus defect, some ASD1) Complications: residual ASD
Atrial septal defect — therapeutic indications ?
Qp:Qs > 1.5:1
ASD size > 10 mm
pulmonary hypertension
Ventricular septal defects (VSD) — forms ?
Membranous / perimembranous VSD
Inlet VSD
Outlet VSD (doubly committed)
Muscular VSD (singular, multiple — Swiss cheese)
Ventricular septal defects (VSD) — MRI protocol ?
Cine-SSFP (2ch, 4ch, LV Stack, RV Stack, LVOT, RVOT, AoV)
PCI (PA Flow, Ao Flow, VSD Flow)
Ventricular septal defect (VSD) — MRI report ?
LV and RV function including EDV, ESV, SV, EF and RWMAs
VSD type, location, size
Qp:Qs — Pa Flow : Ao Flow
associated findings
Ventricular septal defect (VSD) — red flags ?
non-restrictive
dilated LV +/- RV
Qp:Qs > 1.8:1
associated valvular dysfunction
Ventricular septal defect (VSD) — therapy and complications ?
Surgical closure
Percutaneous device
Complications: residual VSD, RVOT obstruction, AV-insufficiency
Ventricular septal defect (VSD) — therapeutic indications ?
Qp:Qs > 1,5:1
middle size and large defects (>0,5 cm2/m2 BSA)
pulmonary hypertension
volume overload of the left atrium and left ventricle
absent tendency of spontaneous closure
Atrioventricular septal defect (AVSD) — facts ?
defect of the atrial septum primum and inlet ventricular septum
presence of a common atrioventricular valve
associated findings: ASD, PDA, aortic coarctation (CoA), anomalous pulmonary venous return (APVR), mitral valve anomalies e.g. parachute MV, double orifice MV
Atrioventricular septal defect (AVSD) — MRI protocol ?
Anatomy
Cine-SSFP (2ch, 4ch, LV Stack, RV Stack, LVOT, RVOT)
PCI (PAFlow, AoFlow, VSD-Flow, ASD-Flow)
Atrioventricular septal defect (AVSD) — MRI report ?
LV and RV function including EDV, ESV, SV, EF and RWMAs presence of ASD and VSD and size Qp:Qs — PaFlow:AoFlow valve stenosis / regurgitation associated findings
Atrioventricular septal defect (ASVD) — key issues ?
Spectrum of defects: ranges from ASD1 with mitral cleft to complete AVSD
Partial AVSD: AV-valves have separate orifices, usually small VSD
Complete AVSD: common AV-valve and orifice (4-7 leaflets), large VSD
Atrioventricular septal defect (AVSD) — therapy ?
PA banding as staged approach in pulmonary overcirculation
Surgical closure with atrial and ventricular septal patch
Atrioventricular valve repair
PDA ligation
Atrioventricular septal defect (AVSD) — post-OP complications ?
Residual ASD and/or VSD
Residual atrioventricular valve insufficiency or stenosis
LV-Outflow obstruction
Atrioventricular septal defect (AVSD) — forms ?
Rastelli Type (A-C)
Position of the bridging leaflets (central, ventricular, atrial) — position-dependant atrial/ventricular only shunt or both shunts (central)
Ventricular imbalance with right/left heart hypoplasia
Double chambered right ventricle (DCRV) — facts ?
Anomalous muscular sub-pulmonary band dividing the RV-cavity into two different chambers
RV-hypertrophy (may contribute to sub-pulmonary stenosis)
VSD (not always present, often perimembranous, may involve the high or low pressure chamber)
Double chambered right ventricle (DCRV) - MRI protocol ?
Anatomical stack
Cine-SSFP (2ch, 4ch, LV+RV stacks, RVOT, LVOT)
PCI (PAFlow, AoFlow)
RV inflow/outflow, VSD-Flow, TV-Flow, coronal stack
Double chambered right ventricle (DCRV) - MRI report ?
LV / RV size and function inc. EDV, ESV, SV, EF, RWMA
RV muscular band: location (high/low) and severity of stenosis (may be difficult to assess adequately with MRI)
RVOT or LVOT obstruction
hypertrophy of the proximal / inflow RV chamber
VSD type, size, location, Qp(PAFlow):Qs(AoFlow)
Presence of tricuspid regurgitation (TR)
Double chambered right ventricle (DCRV), anomalous muscular bundle - key issues ?
usually associated with a VSD, consider spontaneous closure
divides RV into a prestenotic high pressure inflow chamber and a low pressure infundibular outflow chamber
may occur anywhere in the RV from adjacent to the PV down to the apex
best visible on RV inflow/outflow plane, maybe missed on 4ch
Double chambered right ventricle (DCRV), VSD - key issues ?
most commonly peri-membranous
may communicate with either proximal or distal chamber
shunts in the proximal chamber can be underestimated because of high-pressure status
Double chambered right ventricle (DCRV) - key issues ?
Anomalous muscle bundle
- usually associated with a VSD, consider spontaneous closure
- divides RV into a prestenotic high pressure inflow chamber and a low pressure infundibular outflow chamber
- may occur anywhere in the RV from adjacent to PV down to apex
- best visible on RV inflow/outflow plane, maybe missed on 4ch
VSD
- most commonly peri-membranous
- may communicate with either proximal or distal chamber
- shunts in the proximal chamber can be underestimated because of high-pressure status
RVOT obstruction
- due to progressive hypertrophy of RV and muscle bundles
- may lead to RV failure
Tricuspid regurgitation (TR)
- high tricuspid regurgitation jet velocity can be mistaken as pulmonary hypertension
Double chambered right ventricle (DCRV) - therapy and complications ?
Surgical resection
VSD closure
Post-operative complications: intra-ventricular restenosis
Patent ductus arteriosus (PDA) - facts and findings ?
- LV / LA dilatation and dysfunction
- dilated pulmonary veins and ascending aorta in large PDA
- can lead to pulmonary hypertension
- right PDA is associated with other congenital anomalies
associated findings: occasionally aortic coarctation (CoA)
Patent ductus arteriosus (PDA) - MRI protocol ?
Anatomy stack
Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT)
PAs, AoArch, PDA stack
PCI (mPAFlow, branchPAFlow, AoFlow, in-plane PAFlow)
pre-/post-PDA aortic flow
MRA, 3D-whole heart
Patient ductus arteriosus (PDA) - MRI report ?
LV and RV function including EDV, ESV, SV, EF and RWMAs
PDA length, diameter, form (conical, window, tubular)
Qp(PAFlow):Qs(AoFlow)
Patent ductus arteriosus (PDA) - shunt volume and excess pulmonary blood flow ?
Shunt volume = mPAFlow SV - AoFlow SV
Magnitude excess pulmonary blood flow depends on
- diameter and length of PDA
- systemic and pulmonary vascular resistance
Patent ductus arteriosus (PDA) - therapy and complications ?
Occluder device
Coil embolization
Surgical ligation
Complication: residual shunt
Blalock Taussig Shunt - definition and facts ?
Classic: subclavian artery to pulmonary artery
Modified: GoreTex tube from subclavian artery to pulmonary artery
Blalock-Taussig Shunt - complications and late interventions ?
Complications:
- BT-shunt stenosis
- aneurysm formation
- PA dilatation
- pulmonary hypertension if large excess shunting
- subclavian steal syndrome
Late interventions: Blalock-Taussig Stent
Blalock-Taussig Shunt - MRI protocol ?
Anatomy stack
Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT)
Shunt Cines and Shunt Flow
PCI (AoFlow, mPAFlow, PAFlow distal to shunt)
MRA, 3D-whole heart
Blalock-Taussig Shunt - MRI report ?
LV and RV function including EDV, ESV, SV, EF and RWMAs
BT-shunt patency, dimensions and flow
presence of aneurysm formation
Qp(PAFlow):Qs(AoFlow)
Blalock-Taussig Shunt - key issues ?
- palliative Intervention in cyanotic heart disease
- may be used as a bridge to Glenn- / Fontan-circulation
- shunts may be small and best seen in MRA (timed to aorta)
- hemodynamic complication: subclavian steal syndrome
Blalock-Taussig Shunt - alternative shunts ?
Waterstone shunt (ascending aorta to RPA) Potts shunt (descending aorta to LPA) Central shunt (ascending aorta to mPA) Cooley shunt (ascending aorta to RPA, intrapericardial)
Aortic valve disease - findings ?
- aortic stenosis (AS) / aortic regurgitation (AR)
- LV hypertrophy with aortic stenosis (AS)
- LV dilatation and eccentric hypertrophy with aortic regurgitation (AR)
- LV dilatation and impaired systolic function in late stage AS or AR
Aortic valve disease - associated abnormalities ?
- aortic coarctation (CoA)
- sub-valvular or supra-valvular stenosis
- VSD in sub-aortic stenosis
- Shone complex: parachute MV, MS, bicuspid AV, CoA
Aortic valve disease - MRI protocol ?
Basic protocol (CHD)
AoV, PAV
Aortic arch
AoFlow, PAFlow
Aortic valve disease - MRI report ?
LV+RV dimensions, mass and function
AS/AR quantification (Vmax, AVA, RF% etc.)
Aortic dimensions (annulus, SoV, STJ, Ao.asc/arch/desc)
Aneurysm formation
post-OP complications
associated findings
Aortic valve disease - therapy ?
Aortic valve repair
Aortic valve replacement +/- aortic root/ascending aorta
Ross procedure
Ross procedure - definition ?
- replacement of the aortic valve (AV) with patients own pulmonary valve (PV) - autograft
- implantation of the coronary arteries
- RV-to-PA homograft conduit
Aortic valve repair/replacement - post-operativ complications ?
- valve dysfunction
- para-valvular regurgitation / leakage
- pseudo-aneurysm formation
- para-valvular abscess
Ross procedure - complications ?
- aortic insufficiency (AI/AR)
- aortic autograft / aortic root dilatation / dehiscence
- RVOT obstruction
- pulmonary allograft stenosis or regurgitation
- coronary artery stenosis
Aortic valve disease - key issues of cardiac MRI ?
lower temporal and spatial resolution than ultrasound
but more reproducible
alternative especially in poor echo image quality
comprehensive valve assessment necessary
Cardiac MRI - comprehensive valve assessment ?
- LV / RV dimensions, mass, fibrosis and function
- forward and regurgitant flow / fraction
- mean / peak velocity - will underestimate
- jet detection, direction and origin
- valve area by direct planimetry
Ross procedure - advantages / disadvantages ?
Advantages
- longevity of allograft is superior to bio-prosthesis
- favourable haemodynamics
- no need for anticoagulation
- valve grows as patient grows
Disadvantages
- single valve disease (aortic) treated with a two valve procedure (aortic and pulmonary)
Aortic coarctation (CoA) - findings ?
- aortic narrowing in the region of the ligamentum arteriosum, the arch or the isthmus
- collaterals (e.g. 3rd and 4th intercostal arteries)
- +/- hypertrophic LV
Aortic coarctation (CoA) - associated abnormalities ?
- bicuspid AV and dilated ascending aorta
- sub-aortic stenosis
- arch hypoplasia
- VSD
- mitral valve abnormalities e.g. parachute mitral valve
Aortic coarctation (CoA) - MRI protocol ?
Anatomical stack Cine-SSFP (2ch, 3ch, 4ch, SA, LVOT, AoV) Aortic arch PCI (AoFlow, pre-stenotic Flow, post-stenotic Flow incl. Vmax) MRA aorta AoFlow diaphragm ?
Aortic coarctation (CoA) - report ?
LV dimensions, mass and function Aortic dimensions (LVOT, annulus, SoV, STJ, Ao.asc/arch/desc) Severity of stenosis: - minimal dimensions - post-stenotic peak flow - presence and degree of collateral flow Aneurysm formation associated findings
Aortic coarctation (CoA) - initial therapy ?
- stent
- end-to-end anastomosis
- interposition graft
- patch augmentation
- bypass graft
- subclavian flap repair
Aortic coarctation (CoA) - postoperative complications ?
- aneurysm
- restenosis
- collaterals
Aortic coarctation (CoA) - key issues ?
Aortic dimensions:
- be aware of caveats of aortic measurements
- diastolic measurements from cine images are preferred
- cardiac phase, orientation and sequence need to be reported
Severity of coarctation:
- peak systolic flow is often underestimated in CMR (echo better)
- diastolic forward flow prolongation is a sign of significant coarctation
Collateral flow:
- a decrease of <10% is expected
- an increase implies collateral flow joining the post-stenotic aorta
- abundant collaterals may reduce the gradient and mask severity
Associated findings:
- ~10% cerebral aneurysms in the circle of Willis (CoW)
- ~70-80% bicuspid aortic valve
Aortic coarctation (CoA) - caveats of aortic measurements ?
Trans-axial: overestimation due to non-orthogonal plane
Sagittal-oblique: underestimation due to non-central or non-perpendicular plane
Black-blood: overestimation possible due to inclusion of aortic wall
MRA: not cardiac cycle specific / motion artefacts
3D-whole heart: low spatial resolution / motion artefacts
Tetralogy of Fallot (ToF) - definition / findings ?
- VSD
- aortic override
- RVOT obstruction / pulmonary stenosis
- RV hypertrophy
Tetralogy of Fallot (ToF) - associated anomalies ?
- ASD (Pentalogy of Fallot)
- muscular VSD, AVSD
- PDA
- right sided aortic arch
- anomalous coronary arteries / pulmonary venous return
Tetralogy of Fallot (ToF) - MRI protocol ?
Anatomy stack Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT) PAs, AoArch PCI (mPAFlow, branchPAFlow, AoFlow) LGE (2ch, 3ch, 4ch, SA) MRA, 3D-whole heart
Tetralogy of Fallot (ToF) - MRI report ?
LV+RV dimensions, mass and function
RVOT obstruction: subvalvular / valvular / supravalvular
mPA and bPA obstruction and flows
pulmonary regurgitation volume + fraction
presence and severity of tricuspid regurgitation
residual shunting (ASD, VSD, APCs) Qp:Qs
relation to coronaries
aortic root and ascAo dimensions
AV regurgitation
associated findings
Tetralogy of Fallot (ToF) - initial therapy ?
palliative Blalock-Taussig shunt or RVOT stent - if cyanosed ++
definitive repair: VSD patch, RVOT patch, conduit
Tetralogy of Fallot (ToF) - late interventions ?
- pulmonary valve replacement (allograft, bio-prosthesis)
- redo conduit
- pulmonary artery stent
Tetralogy of Fallot (ToF) - postoperative complications ?
- RVOT +/- PA stenosis
- pulmonary and tricuspid valve regurgitation
- RV dilatation and dysfunction, LV dysfunction
- myocardial fibrosis / scarring
- residual VSD / ASD
- aortic root and ascending aorta dilatation
- aortic regurgitation
Tetralogy of Fallot (ToF) - key issues ?
Pulmonary regurgitation / insufficiency
- common after repair of ToF
- may be tolerated without symptoms
- RF% typically 35-45%
Unilateral branch-PA stenosis
- compare LPA and RPA-Flow
Regurgitant fraction (RF%) may exceed 50% if:
- RV is unusually large and compliant
- mPA and/or bPA are large and compliant
- elevated pulmonary vascular resistance
Late diastolic antegrade flow in mPA
- indicates restrictive RV
Timing of PV-replacement remains controversial:
- allograft replacement may function for ~15-20 years
- preOP RV-EDVI > 160-170 ml/m2 and RV-ESVI > 82-85 ml/m2 fail to recover to normal range after operation
Percutaneous Intervention of RVOT / branch-PA obstruction:
- consider 3D-whole heart to identify close relation to coronaries
Pulmonary atresia - findings ?
Underdeveloped RVOT and pulmonary valve - membranous pulmonary valve (PV) - hypoplastic pulmonary arteries (PAs) - atretic PAs and MAPCAs (major aorto-pulmonary collateral arteries) VSD / PDA Pulmonary collaterals RV / RA dilatation and hypertrophy
Pulmonary atresia - associated anomalies ?
- PFO / ASD
- tricuspid atresia / stenosis
- dTGA, ccTGA
Pulmonary atresia - MRI protocol ?
Anatomy stack Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT, PAs) PCI (mPAFlow, branchPAFlow) MRA PAs + MRA Ao 3D-whole heart
Pulmonary atresia - MRI report ?
LV+RV dimensions, mass and function
PA stenosis / hypoplasia - extent and severity
MAPCAs (major aorto-pulmonary collateral arteries)
pulmonary regurgitation volume + fraction
presence and severity of VSD, PDA and/or ASD
associated findings also depending on initial interventions
Pulmonary atresia - initial interventions ?
Radiofrequency perforation of the membranous pulmonary valve
Blalock-Taussig Shunt
Definitive repair:
- PV valvulotomy or conduit if suitable right ventricle (RV)
- atrial septostomy and Glenn -> Fontan, if RV small or coronaries depend on RV
Pulmonary atresia - late interventions ?
PV valvuloplasty PV replacement TV repair / replacement Conduit replacement MAPCA stenting, occlusion, unifocalization
Pulmonary atresia - complications ?
Dependant on special procedure
BT-shunt complications
ToF, single ventricle physiology and operations
Pulmonary atresia - key issues ?
Pulmonary atresia vs ToF with pulmonary atresia
- PAs in ToF are usually normal in size with normal arborisation
- systemic to pulmonary collaterals are less developed in ToF
Complete surgical repair if
- central PAs are present
- sufficient PA blood supply to the lungs
- a single PA is normal in size and reaches all lung segments
Complete surgical repair is contraindicated if
- intact ventricular septum and hypoplastic right ventricle
- hypoplastic or absent central PAs
- inadequate peripheral arborisation of PAs
Palliative procedures
- Blalock-Taussig Shunt
- Waterstone Shunt
MAPCAs
- are best visualised on an aortic MRA
- strict removal of air bubbles if MRA performed
- consider CT to visualise small MAPCAs
Dextro-transposition of the great arteries (dTGA) - findings ?
- VA discordance (morphological RV to aorta, LV to mPA)
- parallel great arteries
Dextro-transposition of the great arteries (dTGA) - associated anomalies ?
- VSD / pulmonary stenosis
- LVOT obstruction sub-pulmonary stenosis
- PDA / ASD
- aortic coarctation (CoA)
- coronary origin anomalies
Dextro-transposition of the great arteries (dTGA) - common therapies ?
- arterial switch
- Rastelli (if associated with VSD, RVOT obstruction or PS)
- atrial switch - historical (Senning / Mustard)
Arterial switch - early interventions ?
- switch of aortic and pulmonary root
- anterior positioning of distal mPA / bPAs (LeCompte manoeuvre)
- translocation of coronaries from aorta to neo-aortic root
Arterial switch - late interventions ?
- AV replacement
- aortic root replacement
- RVOT enlargement
- PA stenting
- PDA closure
Arterial switch - postoperative complications ?
- RVOT obstruction / mPA and bPA stenosis
- LVOT obstruction
- neo-aortic root dilatation
- neo-aortic valve regurgitation
- neo-pulmonary valve regurgitation
- coronary artery stenosis
- systemic RV dysfunction
Arterial switch - MRI protocol ?
Anatomy stack Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT, PAs) PCI (mPAFlow, AoFlow, branchPAFlow) 3D-whole heart MRA PAs stress +/- rest perfusion LGE
Arterial switch - MRI report ?
LV+RV dimensions, mass and function
RVOT / LVOT obstruction
mPA and bPA patency and flows
PR and AR volume + fraction
aortic dimensions
coronary arteries origin, proximal course and flow
myocardial ischaemia and / or scar tissue
Arterial switch - key issues ?
Arterial switch procedure:
- operation of choice in dTGA
- usually performed in the first two weeks of life
- favourable long term outcome
Most progressive postoperative complications:
- neo-aortic regurgitation
- neo pulmonary stenosis
- coronary obstruction
MRI sequences in case of coronary obstruction
- 3D whole heart to assess coronary origin, prod. course and patency
- stress perfusion to assess ischaemia
- LGE to assess myocardial scar
Rastelli - early and late interventions ?
Early interventions: - conduit RV-mPA - intraventricular baffle: VSD closure, redirection of left ventricular outflow to anterior aortic valve Late interventions: - re-operation conduit - VSD closure device
Rastelli - postoperative complications ?
- conduit or conduit valve stenosis / obstruction
- LVOT obstruction
- residual VSD
- residual ASD
- branch PA stenosis
Rastelli - MRI protocol ?
Anatomy stack Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT, PAs) PCI (mPAFlow, AoFlow, branchPAFlow) Conduit cross-cuts 3D-whole heart coronal cine stack
Rastelli - MRI report ?
LV+RV dimensions, mass and function
conduit patency and proximity to sternum
LVOT obstruction
mPA and bPA patency and flows
residual ASD / VSD, Qp(mPAFlow):Qs(AoFlow)
aortic dimensions
coronary arteries origin, proximal course and likelihood of compression in case of intervention
Rastelli - key issues ?
usually performed at age 1-2years with BT-shunt in the meantime
allows for correction of a combination of congenital defects:
- dTGA / double outlet right ventricle and
- VSD and
- RVOT obstruction, pulmonary atresia, (sub-)pulmonary stenosis
maintains systemic left ventricle
- at the cost of possible LVOT obstruction and inevitable conduit interventions (surgical or percutaneous)
obstruction of RV-PA conduit
- the conduit runs very anteriorly mostly directly beyond the sternum and therefore is prone to obstruction
Mustard / Senning - initial interventions ?
Systemic venous baffle: directing systemic venous blood to MV
Pulmonary venous baffle: directing pulmonary venous blood to TV
Mustard / Senning - late interventions ?
- Baffle dilatation / stenting
- closure devices
- surgical baffle revision
- Pacemaker
Mustard / Senning - postoperative complications ?
- systemic and pulmonary venous baffle obstruction
- systemic and pulmonary venous baffle leak
- systemic RV dysfunction
- tricuspid regurgitation
- sub-pulmonary obstruction
Mustard / Senning - MRI protocol ?
Anatomy stack Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT, PAs) Baffle cine stacks in axial and SA plane PCI (mPAFlow, AoFlow) 3D-whole heart, MRA PAs coronal cine stack
Mustard / Senning - MRI report ?
LV+RV dimensions, mass and function
Baffle obstruction
Baffle leak / shunt, Qp(mPAFlow):Qs(AoFlow)
Presence (and severity) of RVOT obstruction
Presence (and severity) of TR
Mustard / Senning - key issues ?
Systemic venous baffle stenosis
- in 5-15% of patients, SVC>IVC
- SVC channel patency required for transvenous pacing
- IVC baffle stenosis less-well tolerated as SVC baffle stenosis
-> alternative blood drainage through azygos veins system
-> elevated venous pressure on the liver
Pulmonary venous baffle stenosis
- physiological similar to mitral stenosis in a normal heart
- consider stenosis in patients with pulmonary hypertension
Systemic right ventricle
- MRI allows routine follow up and change in function
- in RV dilatation with severe systemic TR -> valve replacement
- in TR due to systemic RV failure and annular dilatation -> no options
if baffle stenosis consider
- 3D whole heart, MRA, transaxial flow to assess flow reversal in azygous veins system if SVC baffle limb is stenosed
Congenitally corrected transposition of the great arteries (ccTGA) - findings ?
- L-TGA: AV and VA discordance
- Systemic RV
- Parallel great arteries
Congenitally corrected transposition of the great arteries (ccTGA) - associated anomalies ?
- VSD
- Ebstein-like malformation of the TV
- (sub-)pulmonary stenosis
- aortic coarctation (CoA)
- abnormal situs
Congenitally corrected transposition of the great arteries (ccTGA) - MRI protocol ?
Anatomy stack Cine-SSFP (2ch, 4ch, LV+RV stacks, LVOT, RVOT, PAs) PCI (mPAFlow, AoFlow, branchPAFlow) 3D-whole heart Coronal stack TRFlow
Congenitally corrected transposition of the great arteries (ccTGA) - MRI report ?
LV+RV dimensions, mass and function
Presence type, size and location of VSD, jet velocity Qp:Qs
Presence (and severity) of TR ?
Presence (and severity) of (sub-)pulmonary stenosis
Associated diagnosis
Congenitally corrected transposition of the great arteries (ccTGA) - interventions ?
depends on associated findings
Congenitally corrected transposition of the great arteries (ccTGA) - key issues ?
ccTGA
- usually associated with other congenital anomalies
- most common associated anomaly: perimembranous VSD
- prognosis depends on associated anomalies
- may present late in life
Coronary arteries
- mirror image location
Systemic right ventricle
- multiple coarse trabeculations including moderator band
- best visible of RV stack (or LV stack)
- prone to dysfunction
- AV valve goes with ventricle: TV with RV, MV with LV
