Congenital key morphology Flashcards
Saturations in all limbs in: Normal cardiac anatomy and physiology
Right upper: normal
Left upper: normal
Right lower: normal
Left lower: normal
Saturations in all limbs in: Normal cardiac anatomy and persistent pulmonary hypertension in the newborn
Right upper: normal
Left upper: mildly low
Right lower: mildly low
Left lower: mildly low
(pHTN forces mixing of blue blood from pulmonary artery into aorta via pda)
Saturations in all limbs in: d-TGA anatomy with pulmonary hypertension
Right upper: very low
Left upper: mildly low
Right lower: mildly low
Left lower: mildly low
(pHTN forces mixing of red blood from closed pulmonary loop into aorta)
Saturations in all limbs in: d-TGA anatomy without pulmonary hypertension
Right upper: very low
Left upper: very low
Right lower: very low
Left lower: very low
(low pulmonary pressures mean that red blood from pulmonary closed loop does not mix into the aorta - full cyanosis)
Cynanotic congenital heart diseases
Tetralogy of fallot
TGA
Truncus arteriosus
TAPVR
Tricuspid or pumonary atresia
Acyanotic obstructive left-sided diseases
Aortic stenosis
CoA
Acyanotic non-obstructive shunt lesions, pre-tricuspid
ASD
Acyanotic non-obstructive shunt lesions, post-tricuspid
VSD
PDA
AP window
3 indications for prostin in neonates
Duct dependant systemic circulation (left sided duct dependent lesions)
Duct dependant pulmonary circulation (right sided duct dependant lesions)
TGA
Dosing of prostin
Depends on clinical presentation
Guided by echo and blood investigations
While on prostin, monitor for…
Apnoea (less likely with doses <15ng/kg/min, usually happen within 1 hr)/
Profound bradycardia
Severe hypotension
Consideration in critical care transfer of babies to Level 1 centres
Coordination with cardio / transport team
Consider elective intubation
Ensure good vascular access
Network guidelines
Univentricular circulation explanation
Mainly one ventricle supporting both pulmonary and systemic circulation - functionally univentricular
Examples of univentricular circulation
HLHS, tricuspid atresia, Unbalanced AVSD, DORV
Management principles for univentricular circulations
Duct open
Norwood / PA banding ->
BDGS ->
Fontan ->
Aetiology of congenital heart disease
8% genetic (5% chromosomal, 3% single gene)
3% environmental (drugs, toxins, infection, radiation, alcohol)
89% unknown (multifactorial, polygenic)
15% concordance in monozygotic twins
Properly describe normal cardiac morphology
Usual atrial arrangement
Concordant atrioventricular connections
Concordant ventriculoarterial connections
No associated lesions
Valve positioning in a structurally normal heart
Important to remember that the valves are in different planes in the normal heart
- Pulmonary valve is the most superior, and it is in a coronal plane (horizontal)
- Aortic and mitral valve face anteriorly and leftward, and are side to side with the aorta medial and mitral lateral
- Tricuspid valve is the most inferior, and also faces anteriorly and is more vertically orientated
Normal morphology of RA
o Triangular large atrial appendage
o Systemic vena cava come into the back of the right atrial chamber
o Pectinate muscles and terminal crest
o Fossa ovalis
o Coronary sinus
Normal morphology of LA
o Finger like LAA; narrow junction with atrial chamber
o Four pulmonary veins
o Smooth walled with narrow junction to LAA
Morphologic features of the RV
o Tricuspid valve
o Separated by muscle to pulmonary valve
o Characteristic septal leaflet with characteristic cordae attaching to septum
o Septal marginal traberculation (septal band)
o Moderator band comes off the septal marginal traberculation, crosses RV cavity
o RV has coarse apical traberculations when compared to those found on the left
Morphologic features of LV
o Fine apical traberculations
o Smooth septal aspect, no septal muscles or chordae
o Two groups of papillary muscles both attaching close to one another
o Aortic-mitral fibrous continuity
Morphologic features of intraventricular septum
o Small part that does not contain muscle – membranous septum
o The tricuspid valve septal leaflet attachment attaches directly to ventricular septum, the hinge line of this septal leaflet crosses the membranous septum in two portions – one above the hinge line in the RA, and one below in the RV
What can you see on short axis view of TTE
RV and LV
Can obtain one image capturing part of the TV and AV; MV just below this, can see the pulmonary trunk on the side
What can you see in parasternal long axis in TTE
LA to LV; LV to Ao; RV on the side
Might be able to capture the papillary muscles in transection
What can you see in apical view in TTE
o Visualizes membranous septum well – in 4ch plane where Ao is in the 5Ch
o We can see papillary muscle attachments / moderator band
What can you see in subcostal view in TTE
o Can obtain 4ch and 5ch: can get a view eith RA/RV and PT and AO side by side,then RA RV LA LV, then start to lose RA chamber
o Can obtain short axis images with Ao, TV, and LA, with PT posteriorly
o On either side can cross section through ventricles, of through the atrial septum (SCV to RA on one side, LA on the other side)
What can you see in suprasternal plane in TTE
o Aorta with PV running below them
What are the heart ‘segments’ for segmental sequential analysis?
o 1. Atrial
Right, left, isomerism
o 2. Ventricular
Right, left, solitary and indeterminate
o 3. Arterial
Aorta, pulmonary, common, solitary (absent intrapericardial pulmonary arteries)
What are the two ‘junctions’ for segmental sequential analysis?
o 1. Atrioventricular junctions
o 2. Ventriculoarterial junctions
What is sequential segmental analysis?
Systematic analysis of the heart according to morphology of each chamber and assessment of how chambers are connected to one another, then describing lesions identified
What are the two main components of sequential segmental analysis?
- Three segments
- Two junctions
Atrial types
Situs solitus (usual)
Situs inversis (mirror image)
Right isomerism
Left isomerism
Describe atrial situs solitus
o Spleen on left, liver in right, stomach curves to left
o Three right lung lobes, two left lung lobes, short right main stem bronchus, long left main stem bronchus
o IVC anterior and right, aorta posterior and left of the spine
Describe atrial situs inversus
o Spleen on right, liver on left, stomach curves to left
o Three left lung lobes, short left main bronchus, two right lung lobes, long right main bronchus
o IVC anterior and left, aorta posterior and right of the spine
Describe atrial right isomerism
o Midline liver, malrotated gut, asplenia
o Three lung lobes bilaterally, short main stem bronchus bilaterally
o IVC anterior and left, aorta posterior but left of the spint
Describe left atrial isomerism
o Midline liver, malrotated gut, multiple spleens
o Two lung lobes bilaterally, long main stem bronchus bilaterally
o Aorta anterior and left of the spine, azygos continuation of IVC posterior and further left from aorta
Atrioventricular junction types
Lateralised atria
Isomeric arrangement
Univentricular connection
Describe possible arrangements of lateralised atria
o Concordant atrioventricular connections; Usual atrial arrangement
o Concordant atrioventricular connection; Mirror atrial arrangement
o Discordant atrioventricular connection; Usual atrial arrangement
o Discordant atrioventricular connection; Mirror atrial arrangement
Describe possible arrangements in isomeric arrangement
o Right isomerism, right hand topology (=RV on the right side) == Isomeric RA; RV on right, LV on left
o Right isomerism, left hand topology (=RV on the wrong side) == Isomeric RA; LV on right, RV on left
o Left isomerism, right hand topology == Isomeric LA; RV on right, LV on left
o Left isomerism, left hand topology == Isomeric LA: LV on right, RV on left
Describe the changers in Right isomerism, right hand topology
Isomeric RA; RV on right, LV on left
Describe the changers in Right isomerism, left hand topology
Isomeric RA; LV on right, RV on left
Describe the changers in Left isomerism, right hand topology
Isomeric LA; RV on right, LV on left
Describe the changers in Left isomerism, left hand topology
Isomeric LA: LV on right, RV on left
Describe univentricular connections
- Absent right connection
Right atrial chamber is a dead end, only left atrium has connection - Double inlet
Both atrial chambers connect to ventricular chamber - Absent left connection
Left atrial chamber is a dead end, only right atrium has connection
The uni-ventricle can be due to a dominant LV with incomplete RV; solitary and indeterminate ventricle, or a dominant RV with incomplete LV
What is absent right connection
Right atrial chamber is a dead end, only left atrium has connection
What is double inlet
Both atrial chambers connect to ventricular chamberW
What is absent left connection
Left atrial chamber is a dead end, only right atrium has connection
Possible features of the atrioventricular valve
- Single valve
- Common valve
Subtypes of single atrioventricular valve
o Both patent
o One (right or left) AV valve is imperforate
o One (right or left) AV valve is stenotic
o One (right or left) AV valve is straddling (Chordae attach to the other side of interventricular septum)
o One (right or left) AV valve is overriding (Valve orifice lies over the top of the intraventricular septum)
o One (right or left) AV valve is straddling and overriding
Possible configurations of a single atrioventricular valve
o Half of the common valve might be imperforate
o Half of the common valve might be stenotic
Arterial segment types
- Aorta and pulmonary artery
- Single outlet ventriculo-arterial connection
Possible configurations of arterial segment - aorta and pulmonary artery
Concordant
Discordant
Possible configurations of single outlet ventriculoarterial connections
Common
Solitary - 3 subtypes (with pulmonary atresia, with aortic atresia, with no intrapericardial pulmonary arteries)
3 types of solitary single outlet ventrociloarterial connections
Solitary with pulmonary atresia
Solitary with aortic atresia
Solitary with no intrapericardial pulmonary arteries
Ventriculoarterial junction types
- Concordant
- Discordant
- Double outlet ventricles
Possible configurations of double outlet ventricles
o One ventricle supports outflow to both aortic and pulmonary
o Double outlet right, left, or single indeterminate ventricle
o Common or solitary arterial segments