Congenital Heart Disease Flashcards
Name the presenting sings of Heart Disease in Neonates (5)
- Cyanosis
- Shock
- Congestive Heart Failure
- Murmurs
- Arrhythmias
What fraction of Cyanotic CHD are found on U/S?
2/3
Name the clues to Cyanotic CHD
- Cyanosis w/normal lung exam, without RDS PCO2 is normal or low (w/compensation) but low SpO2’s
- Unresponsive to Oxygen (PO2 <150 on 100% FiO2)
- Murmurs (often present)
- Abnormal heart on CXR
What is the best site for a blood gas to evaluate for Cyanotic CHD?
Right wrist–Preductal
What are high-risk situations of Cyanotic CHD? hint-same as for any CHD at all
Chromosomal abnormalities
Multiple congenital anomalies
IDM’s
Family Hx of CHD
What is the % of CHD if a sibling has CHD?
3-4%
What is the % of CHD if a parent has CHD?
5-10%
What Cyanotic CHD is it?
An x-ray describes: Cardiothoracic ratio >0.6 Large, Boot-shaped heart, Apex tipped up Decreased lung vascularity Missing main pulmonary artery marking
Tetrology of Fallot
Where does the least oxygenated blood flow back to the heart from?
Head
What is often the first clue of a cyanotic CHD?
Is it responsive to giving O2?
Why or why not?
Cyanotic baby w/low SpO2’s
No
Some of the blue blood going back to the body is going out through the Aorta. Nothing you do to the lungs will help this.
Can you have absolutely normal PO2’s with Cyanotic CHD?
No
Is saturation a sensitive measure?
Why or why not?
No
W/Hgb F can have PO2 of 50 and Saturations high 90’s.
In lecture what PO2 level is used to determine a cyanotic lesion?
What can it depend on?
Can’t get PO2 >150 Torr (not perfect system)
Depends on degree of pulmonary blood flow
Can you get a venous stick to get a blood gas to detect cyanotic heart dz?
No, Must be Arterial (preferably right wrist)
Is a murmur definitive of cyanotic heart Dz?
No, not all w/Cyanotic CHD have murmurs and not all w/murmurs have Cyanotic CHD
In a TOF heart, why is the apex tipped up on CXR?
There’s a thick R heart and the Diaphragmatic surface of the heart is the R Ventricle
What diagnostic test can pick up CHD better than in-utero U/S?
Fetal Echocardiogram
Describe Fetal circulation starting with oxygenated blood from the placenta.
Placenta–>Umb. Vein–>Ductus Venosus & IVC–>R. Atrium (via FO)–>L. Atrium–>Mitral Valve–>L. Ventricle–>Aortic Artery–>Aortic Arch to head/coronary A’s or Descending Aorta to body–>Deox. blood from head via SVC–>R. Atrium–>Tri valve–>R. Ventricle–>Pulmonary Artery–>(almost all through) Ductus Arteriosis–>lower body via Descending aorta–>most to placenta via Umb. Arteries
What 2 organs require the most oxygeated blood in-Utero and post-natally?
Where does it come from?
- Coronary Arteries
- Head
Supplied by blood from LV/Aortic Artery
How much oxygenated blood flows across the Aortic Isthmus down the descending Aorta?
~10%
Where is the least oxygenated blood in the fetus?
That coming back from the upper body–it extracts the most oxygen.
What is his rudimentary definition of Cyanotic CHD?
Blue blood somehow comes from the Vena Cava through the Aorta & Red blood comes back (in the absence of lung dz, normal pulmonary venous saturation-close to 100%)
Why don’t you get much effect from supplemental oxygen in babies w/cyantoic CHD?
The cyanotic blood is going to the systemic circulation, not pulmonary–so in the absence of lung dz, extra O2 won’t affect their cyanosis.
In order for blue blood o get out to the Aorta, there needs to be what?
Is this sufficient to create cyanotic CHD?
A site of mixing
No–depends on the flow-most L–>R shunts don’t create cyanosis
What is required for Cyanotic CHD?
Something that makes it hard for systemic venous blood to go to the lungs
- Rt side obstruction
- Unfavorable streaming
- Complete mixing with decreased pulmonary flow
What is the ultimate condition of Unfavorable streaming of blood?
Transposition of the Great Vessels/Arteries
Name the Right-sided obstructive lesions (5).
- Tricuspid Atresia
- Ebstein’s Anomaly
- Tetrology of Fallot
- Pulmonary Atresia
- Pulmonic Stenosis w/Atrial shunting
Name the 3 complete mixing lesions w/decreased pulmonary blood flow.
- Atresia of any valve
- Single ventricle
- TAPVR
What does Atresia mean?
Complete absence of a connection
True/False:
Babies are designed to allow R–>L shunting?
True, via FO
With Tricuspid Atresia, all PULMONARY blood must cross either of what 2 things?
VSD
PDA
What is the tx for babies w/Tricuspid Atresia?
Palliation
Treated like a single ventricle defect, can’t fix it.
With Tricuspid Atresia with PDA, there will be more/less blood going out to the lungs?
Would baby be more/less symptomatic w/PDA?
More
Less
Obstruction at the Tricuspid valve makes it easier/more difficult for blue blood to get to the lungs?
More Difficult
Ebstein’s Anomaly causes the blood to do what at the Tricuspid valve?
Leak
What is Ebstein’s anomaly?
Anomaly of the Tricuspid valve. It fails to delaminate from the RV endocardium.
Ebstein’s is a/w _______ regurgitation & ____ sided Atrial enlargement
Tricuspid
Right
What type of shunt is there w/Ebstein’s?
Right–>Left Atrial
Tricuspid regurgitation may do what to forward flow of blood?
Impair forward flow
True/False: w/Ebstein’s there’s “Atrialized” Right ventricular tissue?
True
True/False: TOF is a very common form of cyanotic heart disease?
True
TOF is mixing at what level?
Why?
Mixing at the Ventricular level
Wall between Lt & Rt Ventricular outflow tract is underdeveloped and it get’s pulled over to the Rt
TOF consists of 4 things, what are they?
- Obstruction of the pulmonary outflow tract
- Over-riding Aorta
- VSD
- R.V. hypertrophy
True/False: w/TOF, there can be several levels of obstruction of blood flow to lungs?
True
True/False: With many types of Cyanotic heart disease, Opening the Ductus Arteriosus is a good thing to do, even when you don’t know what the cause is.
How?
True
Prostaglandins, PGE1
Pulmonary Atresia w/Intact Ventricular Septum is a mild/extreme form of TOF.
True, but it can also be w/o an intact ventricular septum
What are the only 2 places blood can go once in the Rt Ventricle with Pulmonary Atresia with intact Ventricular Septum?
What can sometimes happen w/this D/O?
Backward across the Tricuspid valve or backward flow in to the veins that normally drain from coronary arteries to RV (coronary sinusoids)
MI (d/t lack of blood flow to septum of the heart)
In Pulmonary Atresia w/Intact V Septum, all systemic venous flow must cross what?
Pulmonary blood flow arises mainly from?
Eventually will need what done?
If they can have a Balloon Pulmonary Valvuloplasty, this allows what?
Atrial Septal Defect
Ductus Arteriosus (sometimes will stent it open) Single ventricle palliation, Blallock-Taussig shunt
Right ventricle to develop normally
What is Critical Pulmonic Stenosis?
If Pulmonic stenosis is only mild, what might you have?
What kind of shunt?
Is often responsive to what?
Obstruction to pulmonary outflow at pulmonary valve
Only a murmur
R–>L across PFO
Balloon Valvuloplasty
There are 2 parallel circuit circulations in what anomaly?
Transposition of the Great Arteries
With transposition of the great arteries, you must have what?
Some form of communication (many are Atrial) but also need a PDA; otherwise they die
**must have a way to get blue and red blood to mix
With transposition, to get mixing you must have what kind of flow?
Is this an efficient form of blood flow?
Bi-directional flow
No
If you have to have a single site of mixing, which site is better, Atria (PFO) or Ductus Arteriosus?
Atrial shunt (PFO)
If a baby needs a PFO to allow mixing, what is the name of the procedure used to create it?
Balloon Atrial Septostomy
What 3 things determine the Arterial oxygen Saturation (SaO2)?
- How blue is the blue blood? (systemic venous saturation)
- How red is the red blood? (pulmonary venous saturation)
- How much is mixed?
In a complete mixing lesion with more Pulmonic flow versus Systemic flow there would be a higher/lower SaO2?
Lower
In a complete mixing lesion with more Systemic versus Pulmonic flow, there would be higher/lower SaO2?
Higher
With a complete mixing lesion and opening the ductus, you may see a baby go from profound cyanosis to no visual cyanosis.
True
If you have a lesion with unfavorable streaming (from TGA) or Rt-sided obstructive lesion, what will help the pulmonary flow of the baby?
Open the DA
What happens to the pulmonary venous connection with TAPVR?
The Pulmonary veins that should come back to the L Atrium, did not form that connection.
What happens to circulation in TAPVR?
Persistent fetal circulation (fetal connections of pulmonary veins to SVC or IVC persist).
There are 2 forms of TAPVR what are they? Which one is a surgical emergency?
Supracardiac TAPVR Infracardiac TAPVR (this one is emergent d/t flow of blood-can't get blood into or out of the lungs)
Supracardiac TAPVR is classified as what type of lesion?
Nearly complete mixing lesion. All blue and red blood comes back to R. Atrium
True/False, with supracardiac TAPVR, the only blood to the L. Atria is that from the R.A. across the PFO.
True
With what 2 cardiac lesions is it possible to have post-ductal saturations higher than pre-ductal?
Supracardiac TAPVR
Transposition of the Great Arteries
With Supracardiac TAPVR, most of the red blood travels where?
Most of the blue blood travels where?
If there’s a PDA, some of the red blood can travel where creating possibility of higher post-ductal saturations than pre-ductal?
SVC–>R.A.–>tricuspid valve–>R.V.
IVC–>R.A.–>PFO–>L.V.
To the descending Aorta
With TAPVR if the pulmonary veins are obstructed, there’s what?
If the pulmonary veins are unobstructed, there’s what?
Pulmonary Edema (lungs white-out) Decreased Pulmonary flow
Increased Pulmonary flow
Little Cyanosis
Congestive Heart Failure
True/False: In Infracardiac TAPVR is almost always a complete mixing lesion.
True, surgical emergency
In Tricuspid Atresia, when the DA closes, what happens?
What procedure can be done to help this baby?
Increased cyanosis (Less blood flow to lungs = less red blood to body)
Blalock-Taussig shunt (subclavian artery to pulmonary artery shunt to get some blood to lungs)
What shunt was the first one done to help babies w/CCHD?
Blalock-Taussig shunt
subclavian artery to pulm artery
True/False: We have good treatments for almost all forms of CHD.
What are some exceptions?
How long can single ventricle palliations last?
True
Single ventricle anomalies (no way to make a ventricle) i.e. Tricuspid Atresia
Decades
What is the goal of blood flow w/single ventricle anomaly?
Could you put in a Blalock-Taussig shunt at birth?
Why?
Get the venous return to go directly to the lungs.
No (can do bi-directional Glenn at 4 mos)
PVR is very high just after birth
What happens w/Bi-Directional Glenn?
- Blalock-Taussig shunt is disected.
- SVC is connected directly to Rt. Pulmonary artery (passive flow)
- Ligation, Dissection, or Banding of Pulmonary Artery off R.Ventricle
Can a Fontan procedure be done right away?
No, at age 2-3 y/o that creates a conduit from IVC to SVC (allows blue blood from IVC out directly to lungs)
What is a Fontan?
A connection done at 2-3 y/o
If you only have 1 ventricle, the goal of that blood flow is what?
Get blood to the systemic circulation
a connection can be surgically made to get blood to the lungs
What can be done for complete repair of TOF?
- Resection of valvular pulmonic stenosis area (no valve)
- Patch closure of VSD
- Patch augmentation of RV outflow tract (Pulm Artery)
What may be needed later in life in someone who has had a complete TOF repair?
A Pulmonary valve replacement
What were the first operations done for TGA?
Mustard or Senning operation
baffling blue blood inside the R. atrium and directed it to L. A. toward mitral valve–>lungs
What is the down-side of the Mustard or Senning operation?
R.V. has to do L.V. pumping workload
but people can live into 30’s with these procedures
What is the current option for TGA?
When is this done?
Down side of this procedure?
Arterial Switch procedure (w/closure of ASD/VSD if present)
Done at ~ 1 wk of age
Have to move the coronary arteries
What is shock?
Inadequate Systemic Perfusion
What is a late sign of shock?
Hypotension
True/False: Hypertension can be seen early in shock.
True
Shock can be difficult to detect, what is the best tool to recognize it?
Experienced bedside RN
What types of neonatal Heart dz can present with Shock?
Left-sided Obstructive lesions Sustained Tachycardia (SVT or Ventricular) Profound Bradycardia
Name Left-sided obstructive lesions
Coarctation, Critical Aortic Stenosis, Hypoplastic Left Heart Syndrome
Is shock instantaneous w/SVT?
No, ~48 hrs
With Left-sided obstructive lesions is the baby blue?
Will they have low saturations?
How do you know they have perfusion issues?
No
No
Low U.O. (low CO), Cap refill, Periph pulses
The CCHD test is designed to detect what lesion?
Is it perfect?
Coarctation of the Aorta
No
Where does Coarctation occur usually?
Adjacent to the Ductus (Juxtaductal)
Are babies w/Coarctation usually ok in the normal nursery?
Yes (due to Ductus)
When the ductus closes, which side closes first?
The Pulmonary Artery end
functional closure–first 24-48 hrs
When do babies w/Coarctation get into “trouble”?
Why?
Couple weeks of age
PDA finishes closing on Aortic Arterial side (Anatomic closure) and it becomes more difficult for the blood to get around the Coarct “shelf” ( the little diverticulum into the aorta)
What happens to blood flow with coarctation?
Less flow to descending Aorta–>Very high Afterload–>Decreases C.O.–>L. Ventricle can’t squeeze well–>Increased pressure on L. Atrium–>Pulmonary Edema & Decreased Perfusion to lower part of body (low U.O., increased edema, Renal failure)
Think about what lesion in every baby?
Do what at every opportunity?
Coarctation
Feel the Femoral vs. Brachial pulses
Do what if a baby comes back in w/Jaundice, Sepsis, Feeding difficulties?
Feel the Femoral Pulses, R/O Coarctation. It can be tough to pick up.
Are there good results from tx/repair of Coarctation?
Yes, very good results
With any baby in Shock, Assume they have what until proven otherwise?
Coarctation of the Aorta
-Many more babies die of Coarctation than Septic Shock
True/False: with Coarctation, there’s always a discrepancy of b/p from upper to lower extremities.
False, this time is fleeting
L.V. just can’t pump very well and eventually just have bad pulses everywhere
True/False: You can use the DA to maintain either Pulmonary or Aortic blood flow.
True. It can be used w/Coarct
What would you do 1st in baby w/Coartation?
What would you do 2nd?
Intubate (take away their WOB)
Start PGE ASAP
With Critical Aortic Stenosis are the saturations typcially normal?
How is this tx’d?
No
Is Ductal dependent, likely fail CCHD
PGE, balloon or surgical valvotomy, Norwood
Which babies likely fail CCHD?
Critical Aortic Stenosis
Hypoplastic Left Heart Syndrome is really the extreme form of?
Aortic Stenosis
What is often present with HLHS?
Aortic Atresia or Stenosis
Mitral Atresia or Stenosis
Which side is dependent upon to be the pumping chamber of the heart for the body with HLHS?
The Right Ventricle
Do babies w/HLHS have pulmonary edema?
Decreased systemic flow?
Are they ducal dependent?
Yes
Yes
Yes
Are most HLHS diagnosed prenatally?
Yes, most of the time.
What tx is there for HLHS?
PGE
Norwood
Transplant
Comfort Care
True/False: when the DA closes in a baby with HLHS, there will be increased pulmonary edema.
True
Babies w/HLHS are typically what color.
Why?
Grey (not blue)
D/t low C.O.
Why is a PDA necessary w/HLHS?
Depending blood flow from Ductus through Aorta for systemic blood flow —O2 sats may actually increase w/open DA
In what cardiac lesion do you need to be very judicious with O2 use?
Why?
HLHS
O2 is a very potent pulmonary vasodilator–> easier for blood to go to lungs–>R.V. may not be able to increase the output enough
What is the ratio aim for Pulmonary to Systemic blood flow in a baby with a complete mixing lesion?
1:1
If you have a baby w/HLHS with saturations in the 90’s, is this good or bad?
What is the cause?
Bad, want 75-85%
It is caused by increased pulmonary blood flow.
The tx of HLHS is to create a reliable connection from R.V. to the Aorta by first the Norwood, then the Glenn, lastly the Fontan. What is the Norwood Procedure?
Norwood Procedure:
- Anastamose proximal Pulmonary Artery to Ascending Aorta
- Enlarge Aortic Arch, dissect ductal tissue
- Shunt from Aorta to Distal Pulmonary Artery
- Enlarge ASD
- Mixing with controlled pulmonary flow
With HLHS when going from Norwood to Glenn, what does this do?
Creates bi-directional flow (connects SVC to Pulm Artery, & they get rid of Arterial shunt)
With HLHS what does the ultimate Fontan procedure do?
Allows the blue blood from the lower extremities go directly to the lungs
What is CHF?
Failure of the Heart to Pump Adequately
Some of the signs of CHF overlap with what signs? Plus?
Signs of Shock
+Sweating w/feeds
Enlarged Liver
Increased Respiratory Effort
What lesions can cause CHF?
Large L–>R shunts (ASD, VSD, Truncus, Single Ventricle w/o Pulmonary Stenosis)
Left-Sided obstructive lesions
Cardiomyopathy
Longstanding Tachycardia/Bradycardia
With a small VSD you will hear?
Harsh, High-Pitched Holosystolic Murmur
Will you always hear a murmur with a Large VSD?
Why?
No
Less Turbulence
With a VSD, Is it typical to have signs of CHF before 6 wks?
Why/why not?
No
see when PVR falls
Would you be more likely to have CHF with a large or small VSD?
Large
With VSD, A Diastolic Rumble heard at the Apex is better heard with Bell or Diaphragm?
Caused from?
Bell–easiest to hear when you press down first, then let up on the pressure
Turbulence across Mitral valve
Do muscular VSD’s get smaller?
Yes
What is an Atriovetricular Septal Defect?
A hole in the middle of the heart/septum both Atrially and Vetricularly
What babies have increased relative risk of Atrioventricular Septal Defects?
Babies w/Down Syndrome
With Atrioventricular Septal Defect, what kind of shunt do they have L-R or R-L?
L–>R
About how many babies w/Down Syndrome have CHD?
In Down Syndrome is AVSD more or less common than VSD?
50%
Less
Is Truncus Arteriosus a Cyanotic Lesion?
No, Almost never
What is Truncus Arteriosus?
A single Artery Valve that forms, giving rise to both the Pulmonary Artery and the Aorta.
Usually the blood goes to the correct Artery though.
Typically Truncus is R->L or L->R lesion?
L->R
Is there much cyanosis with truncus?
Almost never/minimal–sats near normal (90’s)
What is the major problem with Truncus?
Increased Pulmonary blood flow (both systole and diastole)
Decreased blood to coronary sinuses (easier for blood to go to pulm artery vs coronary arteries) ->MI, Ischemia & sudden death
When is repair of Truncus usually done?
Before Discharge
In repair of Truncus, the truncal valve stays connected to which ventricle?
LV (a conduit is connected RV to distal pulmonary artery)
W/single ventricle, if you have increased pulmonary blood flow w/o obstruction, you can have same physiology as what other lesion?
Large VSD/AVSD
What is the big problem w/single ventricle?
Excessive Pulmonary blood flow
What % of kids will have a mumur at some time?
80%
Murmurs are usually what?
May indicate what?
Benign
Structural Heart Dz
What grade murmur indicates an abnormaltiy of the heart?
3-6/6
What is the best part of your hand to feel a thrill?
The base of the fingers near the palms, feels like a cat purring.
“Innocent” murmurs are also called what?
What kinds are there?
Functional or Normal
- Peripheral Pulmonic Stenosis Murmur (most common)
- Pulmonary flow murmur (esp anemia of prematurity)
- Transitional murmur
An innocent murmur is a systolic/diastolic murmur?
Grade?
Systolic
Grade less than 4/6 (no thrill)
Where is a PPS murmur best heard?
What population has increased PPS murmurs?
Axillae (equally) & back (crescendo-decrescendo murmur)
Preemies–usually gone by 6 mos
What is a PPS murmur caused from?
Turbulence of flow at the Pulmonary Arteries
Where is a Pulmonary flow murmur heard?
Upper Left Sternal Border
What is a transitional murmur caused from? (2 types)
- Closing PDA (can hear it into Diastole)
2. Transient Tricuspid Regurigation
Where would you hear a transitional murmur from PDA?
Upper Left Sternal Boarder Systolic or continuous Louder as PDA gets smaller Occasionally Vibratory Typically heard 12-48 hrs of age
Where would you hear a transitional murmur from Transient Tricuspid Regurgitation?
Lower Left Sternal Border
Regurgitant, systolic (blowing, holosystolic)
Heard in Asphyxiated or those w/PPHN
Resolves over several days
What murmurs need evaluation?
Loud
Diatolic
Don’t fit into innocent murmur caetgory
Those w/other signs of CHD (Shock, CHF, Cyanosis)
What CHD presents as an Asymptomatic Murmur?
Septal Defects
Outflow Tract Obstruction
PDA
Where would you hear Aortic stenosis?
- Systolic, ejection
- Upper Right Sternal Border, Radiating to neck
- Systolic ejection click at apex (valvular sound)
- May have thrill in Suprasternal Notch
Babies w/anemia of prematurity often have what kind of murmur?
Pumonary flow murmurs
When babies are first born, RV and LV pressures are what?
Will you hear a VSD in the first day or so?
The same
No-it will increase as PVR falls. If it is large, may not have much turbulence->not much murmur heard
If a baby has a large VSD, they will have what signs?
Increased LA pressure
Increased pulmonary edema
Increased WOB
Poor growth
The murmur with an ASD is a/w pulmonary flow. Do you hear an Atrial murmur?
Is CHF seen with an ASD?
No, because the Atrial flow is low pressure.
No, not generally
Which Neonatal Arrhythmias are of concern?
- Too fast
- Too Slow
- Too Irregular (could become too fast or too slow)
Name the 3 Tahcyarrhytmias
- Sinus tachy
- Supraventricular tachy
- Ventricular tachy
What will you see with sinus tachy?
- Narrow QRS
- P waves visible
- Rate <230
- Gradual onset and termination
- A/W underlying cause (fever, hypovolemia)
How do you tx sinus tachy?
Tx underlying cause (fever, fluids, etc)
What would you see w/SVT?
- Narrow QRS
- P waves burried in T wave
- Rate >240, monotonous
- Sudden onset and termination
- Often result of re-entry
What is the most common form of SVT?
Wolff-Parkinson-White syndrome
-An accessory connection allowing conduction to go SA down the AV node or down the accessory conduction site-interfering w/normal rhythm causing pre-excitation
What do you do if a baby is in shock and has SVT?
Shock w/0.25-1 Joules/Kg
Synchronized DC cardioversion
Very unusual to need to do
If hemodynamically stable in SVT, what should you get before, during and after termination?
ECG
What are the usual tx’s of acute SVT?
Valsalva (ice to face)
Adenosine
Adenosine causes what?
Transient AV block
Slows Sinus Node
Adenosine has an extremely short/long duration of action
Short
True/False: RBC’s rapidly break down Adenosine.
True-so don’t pull blood back into med syringe.
Does Adenosine work on Atrial Flutter?
Accessory Connection-Mediated tachycardia?
No
Yes
Ventricular tachy has what signs?
Wide QRS (maybe only slightly)
AV dissociation in VT
Underlying cause
If needing to synchronize DC cardiovert, what must be done after every shock?
Re-synchronize after every shock
In sinus bradycardia, what is slow?
Both atrial and ventricular rate
in AV block what is slow?
Ventricular rate is slower than Atrial
Sinus bradycardia is almost always what?
Vagally mediated
Besides vagal maneuvers, how can Sinus bradycardia be tx’d?
Atropine
Isoproterenol
Pacing
What is the Etiology of AV block?
Congenital Surgical Infectious Vagal Drugs
How do you tx Acute AV block?
Tx underlying cause
Drugs (Atropine, Isoproterenol)
Pacing
PAC’s are very ________ & very ______
Normal & benign
normal QRS, P waves present (may be in T wave)
May be blocked or conducted aberrantly
Are PVC’s common?
Are they normal?
What do they look like?
What are they from?
Yes
Yes
Wide QRS, no P-wave, may not be a pulse from PVC
Metabolic, Drugs, Mechanical stim (CVP cath inventricle), idopathic
