Acyanotic Congenital Heart Defects Flashcards
What is cyanosis? 2
- A blue, gray, or dark purple discolouration of the mucous membranes caused by low blood oxygen (hypoxia)
- Blood with low oxygen levels circulating to the body
What is cyanosis caused by?
Oxygenated blood mixing in with unoxygenated blood
Cyanosis defects does not allow for what?
Adequate oxygenation, therefore is more serious
With acyanotic, babies still receive adequate oxygen despite what?
The defect therefore is less serious
Determine the two hearts?
Left: complete AVSD
Right: Incomplete AVSD
Label the VSD
What is this image represent?
Inside a heart with patent ductus arteriosus
The label is a connection (patent ductus arteriosus)
What does this image represent?
PDA and Desc. Ao
What does this spectral doppler proove? (This represents flow from the Ao to PA)
Due to low PA pressure, there is continuous flow from Ao to PA. Peaks at mid- end systole
What does this image represent?
CCTGA L-TGA
What would we see in PSAX with CCTGA?
We would see a AV with 2 leaflet tips and a PV with 3 leaflets
What does this image represent?
Cor triatriatum
What does this image represent?
Cor Triatriatum Dexter
What does this image represent?
Partial anomalous pulmonary venous return
What does this image represent?
Ebstein anomaly
What does this image demonstrate?
Persistent Left SVC
If there is a persistent L-SVC, there is a good chance that the LSVC shunts blood into the RA, this would cause what?
RA dilation and coronary sinus dilation
What does this image represent?
Dilated coronary sinus
What does this image represent?
Persistent LSVC
What are 3 types of acyanotic heart defects?
- Shunt-related defects
- Obstructive Defects
- Other Acyanotic defects
What are 5 shunt related defects?
- Atrial septal Defects (ASD)
- Ventricular Septal defect (VSD)
- Atrioventricular septal defects (AVSD)
- Patent ductus Arteriosus (PDA)
- Congenitally corrected transposition of the great arteries
What are obstructive acyanotic heart defects? 2
- All involve a narrowing of a valve or vessel
- Non-shunt related acyanotic heart defects
What are 3 types of obstructive defects
- Congenital Aortic stenosis:(AS)/Bicuspid AV
- Pulmonary Stenosis:
- Coarctation of the aorta (COA)
Besides Shunt related and obstructive defects of acyanosis what are other defects? 4
- Cor triatriatum
- Partiall anomalus pulmonary venous return (PAPVR)
- Ebstein anomaly
- Persistent left superior vena cava
What is patent ductus arteriosus?
A connection between the descending aorta and origin of the left pulmonary artery
What is PDA caused by?
Failure of the ducts arteriosus to close at birth
PDA shunts move LT»_space;> RT why?
Because of higher LT sided pressure, therefore AO»_space;> PA
What is blood flow like through the PDA?
Continuous
What does PDAs sound like?
Machinery murmur due to the continuous blood flow
Depending on the size of the shunt what might happen to the PDA? 2
- PDA may be asymptomatic
- May cause LV volume overload
If the PDA causes LV volume overload, what happens? 2
- Hyperdynamic LV
- Dilatation of the LA and LV
How might he PDA close or get repaired? 3
- Pharmacologically - premature infants - indomethacin
- Surgically
- Percutaneously
PDA is best imaged from what window?
PSAX - AV/PV Level (base level AV/PV focus)
With the PSAX AV/PV view what does the colour doppler look like?
Continuous red high velocity jet in the MPA or its branches, which is best seen during diastole
In terms of PDAs: Qp and Qs is measured at what level? 2
- Qp is measured at the level of LVOT
- Qs is measured at the level of the RVOT
The magnitude of the PDA shunt is calculated as a ratio between what?
Qp and Qs
In terms of shunts, if there is increase flow, which side does a normal shunt increase and which side does a PDA increase? (Of the heart)
Normal shunts increase on the RT side of the heart and the PDA increases flow on the LT side of the heart.
Why is the QP:QS ratios switched for PDA?
Because of the flow directions of the shunts.
Congenitally corrected TGA (L-TGA) is also called or referred to as what? 4
- Corrected transposition
- L-transposition
- L-TGA
- CC-TGA
CC-TGA is characterized by what? 2
- Leftward looping of the ventricles
- Transposition of the great vessels
What’s an easy way to determine CC-TGAs?
Both the ventricles and the great arteries are switched
What is something we see with CC-TGAs which is abnormal with the Ao?
Typically the Ao is anterior and to the left of the PA but in this situation it is switched around
What is double discordance (AV + VA)? 2
- When the Morphological RV now pumps out to the aorta
- When the Morphological LV now pumps out to the pulmonary artery
With CCTGA/ L-TGA, The TV often leaks because of what? What does this result in?2
- The RV was not built to handle systemic arterial pressures
- This results in TV annulus dilating leading to TR
What are symptoms of CCTGA/ L-TGA?2
Depends on
1. Degree of TR (systemic AV valve), VSD
2. Severity of outflow tract obstruction
Patients may be asymptomatic
What are some associated lesions/anomalies seen with CCTGA/ L-TGA? 4
- VSD
- Tricuspid valve anomalies
- Pulmonary outflow tract obstruction
- Conduction defects
What is Cor Triatriatum?
A perforated membrane that partitions to the left or right atrium into two chambers
The size of the perforation of Cor Triatriatum determines what?3
- Severity of obstruction
- Symptoms
- Age of presentation
In terms of Cor Triatriatum, what does a small orifice mean?2
- Less flow
- More symptoms
In terms of Cor Triatriatum, what does large orifice mean?
- More flow
- Less symptoms
What is Cor Triatriatum sinister? What is it characterized by?
- Divided left atrium
- Characterized by a perforated membrane in the left atrium
Where are the pulmonary veins in Cor triatriatum sinister?2
- The pulmonary veins come together posteriorly
- Membrane between the PV confluence and the mitral valve
What are symptoms of Cor Triatriatum sinister? (What is it similar to?)
Similar to valvular mitral stenosis
What are some echo findings of Cor Triatriatum sinister?3
- Membranes across the LA
- PVs may be connected superiorly and inferiorly to the membrane
- If 1 or 2 PVs connect inferiorly to the septation, there are less symptoms
Cor triatriatum sinister is assocaited with what?
ASD
What is Cor Triatriatum dexter?
- Divided right atium
- Characterized by a perforate membrane in the RA
How common is Cor Triatriatum?
Extremely rare
What are echo features of Cor Triatriatum dexter?2
- Suspected when a linear structure is visualized in the right atrium that does not resemble a Eustachian valve
- Assess membrane/ orifice with colour and spectral doppler
What does PAPVR stand for?
Partial Anomalous pulmonary venous return
What happens with PAPVR?4
1-3 of the PVS are connected to
1. systemic vein (SVC/IVC)
2. RA
3. Coronary sinus
4. LT innominate vein
If there is a single PV connected to the SVC, this is associated with what?
Sinus venosus ASD
What are some configurations of PAPVR?3
- LT sided PVs may connect to the coronary sinus and/or the left innominate vein
- RT sided PVs may connect to the RA, SVC/ IVC
- Single PV connected to the SVC
PAPVR features are similar to those found in patients with an ASD and include what?3 (what we see with PAPVR)
- Hypertrophy and dilation of the RA and RV
- Dilatation of the PA
- RVVO
What does Atrialization of RV cause?3
- May cause PFO/ASD
- May become cyanotic if shunt reverses
- TR
What are echo features of Ebstein anomaly?4
- Apical displacement of one or all TV leaflets
- Leaflets tethered to myocardium
- Atrialization of RV
- Small RV, Large RA
What is Ebsteins anomaly associated with?5
- ASD/ VSD/ PFO
- PDA
- PS/MS
- Tetraology of fallot
- D-TGA
What is a persistent left SVC?
The L- SVC is formed by the confluence of the left jugular and subclavian veins and descends inferiorly parallel to the right SVC in most cases
In terms of Persistent Left SVC, The L-SVC commonly enters where? and it rarely enters where?
- Coronary sinus
- LA
Approximately how often does a persistent Left SVC occur?
0.3% of the population and is higher in patients with other CHDs
What is persistent left SVC associated with?
Any type of ASD but the coronary sinus ASD is most common
Does Persistent Left SVC affect physiology of the heart?
Nope it remains the same
What are some echo features of persistent L-SVC? 2
- The flow of L-SVC blood into the coronary sinus results in dilation of the coronary sinus
- Best imaged in the A4C view with posterior angulation which shows the coronary sinus nicely