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Congenital Heart Disease
Numbers and such
113
Nursing
Graduate
04/04/2015

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Term
incidence of congenital heart defects in the United States
Definition
roughly 8 per 1,000 live births
Term
An estimated minimum of _______ infants are expected to be affected each year in the United States. ____ %of these will require invasive treatment in the first year of life.
Definition
32,000
25%
Term
___ in ____ adults are expected to have some form of congenital heart disease.
Definition
1 in 150
Term
The goal of the definitive repair of CHD
Definition
to eliminate cyanosis and the body’s compensatory response of polycythemia
Term
Definitive repair of CHD
Definition
CHD is completely repaired to the extent it can be
Term
Palliative repair of CHD
Definition
Means that the repair is being done in stages.
Term
___% of the neonate’s myocardial mass is comprised of contractile tissue
Definition
30%
Term
___% of mature myocardium is comprised of contractile tissue by age 3
Definition
60%
Term
The most important parameter that drives cardiac output in the newborn heart
Definition
HEART RATE.
Term
The newborn heart is dependent on the _____________ __________to initiate and sustain contraction.
Definition
extracellular calcium
Term
With newborns you want to check ____ blood level and avoid _______ medications.
Definition
ionized calcium, and avoid calcium channel blocking
Term
If you see a falling BP, the child is often ____ __________
Definition
very hypovolemic
Term
Three considerations regarding the preterm infant’s heart:
Definition
1. There is an increased sensitivity to depressant effects of inhalation agents
2. There is also a decreased response to catecholamines
3. A relatively high PVR persists that will decrease as PaO2 increases over hours to days
Term
Preemies also have pulmonary vasculature that is more sensitive to vasoconstriction by:
Definition
1. Hypoxia
2. Acidosis
3. Hypercarbia
4. Hypothermia.
Term
What kind of anesthetic can you use for a patient with CHD?
Definition
Almost ANY anesthetic technique may be used in any CHD patient if the anesthetist understands……
1.Pathophysiology of the lesion
And 2. Pharmacology of the drugs used
Term
When dose permanent closure of the foramen ovale occur?
Definition
2-3 months
Term
Functional closure of the foramen ovale occurs at birth as the result of what?
Definition
increased left atrial pressures in excess of right atrial pressures
Term
Permanent closure {(ANATOMICAL CLOSURE (FIBROSIS)} of the PDA occurs when?
Definition
1-4 months
Term
Functional closure of the PDA occurs when?
Definition
1-8 days
Term
RV Pressure
Definition
30/3
Term
LV Pressure
Definition
80/5
Term
PA Pressure
Definition
30/12
Term
Aorta Pressure
Definition
80/50
Term
4 classifications of CHD
Definition
1 “Simple” Left-to-Right Shunt
2 “Simple” Right-to-Left Shunt
3 Complex shunts
4 Obstructive Lesions
Term
If systemic flow is greater than pulmonary flow, the ratio will be <1 (Qp / Qs <1), what kind of shunt is this?
Definition
right-to-left shunt
Term
If pulmonary flow is greater than systemic flow, the ratio will be >1 (Qp / Qs >1), what kind of shunt is this?
Definition
left-to-right shunt
Term
Types of simple left to right shunts
Definition
VSD
ASD
AVSD
PDA
Term
What is the Most common CHD?
Definition
VSD
Term
VSD accounts for approximately __% of all CHD
Definition
20%
Term
Atrial septal defect (ASD): accounts for approximately __% of all CHD
Definition
10%
Term
Atrioventricular septal defect (AVSD): accounts for approximately __% of all CHD
Definition
3%
Term
Patent ductus arteriosus: (PDA): accounts for approximately __% of all CHD
Definition
10%
Term
PDA is more common in _____ ______
Definition
premature infants
Term
AVSD is also known as
Definition
endocardial cushion defect or AV canal defect
Term
Left-to-Right shunt physiology
Definition
blood that is returning to the heart from the lungs is partially recirculated back to the lungs without going to the rest of the body, essentially creating a lung to lung shunt
Term
Lung to Lung shunt is which type?
Definition
left to right shunt
Term
Potential issues related to a left-to-right shunt
Definition
1. There may be an overload of the pulmonary circulation
2. Extravascular water can accumulate in alveolar spaces. This extra water increases the distance for gas exchange, therefore creating poor oxygen exchange.
1. The large LA preload can cause LA hypertrophy
2. LV hypertrophy can produce a poor LV function and poor EF/CO
3. The end organs suffer due to hypoperfusion
Term
Physical exam characteristics of VSD
Definition
Pansystolic murmur on left sternal border.
Moderate defects show pulm congestion on x-ray.
Term
Large VSDs shows symptoms by what age, and what are the symptoms?
Definition
By 4 weeks.
Symptoms include tachypnea, pulmonary infections, CHF and failure to thrive.
Term
Restrictive VSD
Definition
will have a pressure gradient between the two ventricles
Term
Non-Restrictive VSD
Definition
the pressure between the two ventricles actually equalizes
Term
Which is worse, non-restrictive of restrictive VSD?
Definition
Non-restrictive VSD

LV pressure = RV pressure is not a good thing! Nonrestrictive VSDs produce worse symptoms and outcomes than a restrictive VSD
Term
Eisenmenger syndrome
Definition
progresses over time due to prolonged pulmonary hypertension from a left to right shunt (ASD, VSD, AVSD, PDA), eventually the pressure in the right heart is greater than the left, causing a right to left shunt. Symptoms include cyanosis and severe hypoxia.
Term
What kind of problem can occur after surgical closure of a VSD?
Definition
A high AV block or 3rd degree heart block
Term
ASD, physcical exam findings
Definition
systolic murmur heard over the pulmonic valve. Also, the second heart sound can have a wide split
Term
Types of ASDs
Definition
PFO
Primum ASD
Secundum ASD
Sinus Venosus ASD
Coronary sinus ASD
Common Atrium
Term
End outcomes of an untreated ASD
Definition
increased RV work, pulmonary hypertension and RV failure
Term
Surgery is for an ASD is indicated when the pulmonary blood flow is ____ the systolic blood flow
Definition
twice
Term
Which ASDs are commonly closed percutaneously?
Definition
PFO and secundum ASDs
Term
Main anesthetic goal in managing a patient with an ASD
Definition
to DECREASE THE SVR which will improve the shunt,

Use volatiles, and peripheral vasodilating drugs for this.
Term
What is your goal with PVR and ASD
Definition
Maintain PVR
Term
How can you maintain PVR with an ASD
Definition
employ positive pressure ventilation which will increase the intrathoracic pressure.
Term
What are two things you should sure as shit avoid in ASD?
Definition
-Avoid increasing SVR, it will worsen the shunt
-Avoid air bubbles in the IV and tubing.
Term
AVSDs are commonly associated with which chromosomal disorder? and what other two conditions may AVSDs occur with?
Definition
Commonly associated with trisomy 21 and may also occur with DiGeorge Syndrome and tetralogy of fallot
Term
Describe the ventricles in a balanced AVSD
Definition
ventricles are similar in size
Term
Describe the ventricles in an unbalanced AVSD
Definition
one dominant ventricle and an opposite hypoplastic ventricle.
Term
Considerations for a patient undergoing AVSD repair
Definition
1 Any special care related to an associated syndrome (trisomy 21 or DiGeorge most common)

2 Inotropes are frequently required

3 Postoperative pulmonary HTN and heart block are both common occurrences
Term
PDA occurs in roughly __ in every _____ births
Definition
1 in every 2,500 births
Term
the DA closes due to...
Definition
an increase in systemic vascular resistance, decrease in pulmonary resistance and diminished prostaglandin supply
Term
Will a lower PaO2 keep the DA open or close it?
Definition
It will keep it open.
Term
What does a PDA sound like on a physical exam?
Definition
a loud systolic murmur that impinges on diastole, heard best over the mid to left sternal border
Term
two unfavorable conditions that arise from PDA
Definition
1 The pulmonary over circulation increases pulmonary vascular congestion and increases extravascular lung water that interferes with gas exchange. There is a SIGNIFICANT increase in the LA and LV volume resulting in a HIGH OUTPUT FAILURE!
2 The shunting can be so pronounced that it causes a backflow from the aorta during diastole. This deprives the lower body of oxygen and increases the risk for necrotizing enterocolitis (NEC) due to splanchnic hypoperfusion! NEC can be a medical and surgical emergency. Dead bowel can quickly cause overwhelming sepsis in an infant/preemie and can often lead to death
Term
In surgery for PDA repair, those with urine output issues will have ______ as a first choice.
Definition
dopamine
Term
In surgery for PDA repair, those with blood pressure issues only will generally get _____
Definition
epinephrine
Term
How would dopamine improve the hemodynamic status of a babe with a PDA?
Definition
dopamine will improve the inotropic property of the heart!
Term
What effect will dobutamine have on the PVR?
Definition
It will decrease PVR by dilating the pulmonary vasculature.
Term
In what case is a PDA beneficial?
Definition
In a large RV outflow problem, they will be on a prostaglandin drip to maintain the PDA
Term
4 options for medical management of a PDA
Definition
1 Indomethacin: inhibits cyclooxygenase which inhibits prostaglandin synthesis
2 Ibuprofen: inhibits cyclooxygenase which inhibits prostaglandin synthesis
3 Fluid restriction
4 Furosemide
Term
What problems will you see regarding inhibition of prostaglandin in pts being medically managed for a PDA
Definition
-Kidneys are affected, decreased renal blood flow
-Thromboxane A2 is inhibited for 48 hrs, so there are concerns with clotting factors
Term
What problems will you see related to fluid resriction and furosemide in pts being medically managed for a PDA
Definition
Hypovolemia and hypokalemia
Term
What 3 things do you want close to normal before doing a PDA repair?
Definition
1 potassium
2 renal function
3 fluid volume status
Term
The most common surgical approach for infants for a PDA repair
Definition
right lateral position to allow for a left-sided thoracotomy
Term
Preop Prep for PDA repair
Definition
-T & C 1 unit and have in the room
-Have a method available to warm blood
-some advocate a unit of plasma in the room
- Albumin 5% should also be in the room (dosed at 5-10ml/kg)
Term
A __ ml/kg infusion of cold blood will quickly produce hypothermia
Definition
10 ml/kg
Term
In a term infant, immediately after delivery, would you expect the SpO2 to be greater preductally or postductally??? Why???
Definition
Preductal (right hand) O2 sat is higher and postductal (left hand) O2 sat is lower because the PDA is still open.
Term
Monitors for PDA repair should include what 3 things?
Definition
1 The blood pressure cuff on the right arm.
2 A saturation monitor on the right arm/hand (Preductal).
3 A saturation monitor on the left arm/hand or one of the lower extremities (Postductal).
Term
List 5 considerations for a PDA repair outside the OR
Definition
1) monitor failure can occur with cautery
2) no ETCO2 available, bring your own
3) you can't manipulate under the drape without disturbing the field
4) 2nd BP cuff on the leg
5) Have a dedicated medication line with an extension and push drugs as close as possible to avoid a fluid bolus
Term
Three changes you will assess with PDA ligation
Definition
1) clear S1 and S2 and resolution of the murmur
2) increased DBP
3) Improved pulses
Term
4 Intraop considerations for PDA repair
Definition
1) General is for open PDA repair, perc cna use high dose fentanyl and deep sedation. Sevo can be helpful with a good EF d/t decrease in SVR, sevo is harmful with a poor EF

2) Keep pts warm, warm room, isolette

3) lung compression can occur, if bradycardia develops then tell the surgeon to stop.

4) Hypotension due to blood/evaporative loss or compression of the chest decreasing venous return
Term
5 Potential complications of PDA repair
Definition
1 Recurrent laryngeal nerve damage
2 Tearing of PDA
3 Clamping of the aorta, pulmonary artery or carotid!
4 Destruction of the thoracic duct
5 Clamping of a bronchus!
Term
Most common right to left shunts
Definition
1 Tetralogy of Fallot (most common right-to-left shunt)
2 Pulmonary Atresia
3 Tricuspid Atresia
Term
Right-to-left shunts
Definition
produced in defects between the right and left heart where there is resistance to pulmonary blood flow. Or to say it another way, the defect produces an increase in pulmonary vascular resistance. The pulmonary vascular resistance would be GREATER THAN the systemic vascular resistance. Reduced pulmonary blood flow leads to cyanosis and hypoxemia
Term
Which shunt is a “body to body” shunt
Definition
right to left shunt
Blood that is returning to the heart from the body is partially recirculated back to the body without first going to the lungs to be oxygenated.
Term
What is the major concern with a right to left shunt?
Definition
hypoxemia
Term
TET spells
Definition
sudden episodes of SEVERE cyanosis with rapid breathing. They may even lose consciousness. During exercise, older children may become short of breath and faint
Term
Children with tetralogy of fallot will often do what during a TET spell?
Definition
squat in an attempt to increase the SVR and improve hypoxic spells
Term
A severe cyanotic spell is treated with
Definition
1) 100% oxygen: this will not only provide additional oxygen, but will also help improve the pulmonary vascular resistance (if it contributes to the problem)
2) Knee to chest position: increases the SVR
3) Morphine: relaxes the outflow tract to improve pulmonary blood flow
Term
Persistent and severe cyanotic spells can be treated with...
Definition
crystalloids, phenylephrine and beta blockers to slow the heart rate. Why would this work???

Slowing the heart rate (beta blockers) will increase filling time which gives more time for blood to flow through a fixed lesion. Phenylephrine and fluids will also help increase SVR.
Term
The major goal in the management of right-to-left shunts is...
Definition
to increase pulmonary blood flow, thereby improving oxygenation!
Term
Two ways to increase pulmonary blood flow in right to left shunts
Definition
Maintaining a patent ductus arteriosus: this increases pulmonary blood flow and can be accomplished by intravenous prostaglandin administration (PGE1)

Palliative shunts: This can be accomplished via interventional cardiology or surgically. Interventional cardiology can create an ASD / PFO to improve pulmonary blood flow, improve hypoxemia and stimulate growth in the pulmonary artery. Surgical creation of a shunt is another option. This will also aid in technical feasibility of the future repair. (See next slide)
Term
Four distinct anatomical features of tetralogy of fallot
Definition
VSD
RV outflow tract obstruction
Over riding aorta
RV hypertrophy
Term
In tetralogy of fallot, what will happen if SVR is reduced?
Definition
intensification of the right-to-left shunt and therefore the degree of hypoxemia producing hypercyanotic spells (TET spells).
Term
The major goal in management of tetralogy of fallot
Definition
is to maintain adequate tissue oxygenation!!
Term
Management of Tetralogy of Fallot (Mnemonic DDo Some PPeople Kill AAngry Vegans?)
Definition
-Avoid increased O2 Demand
-Avoid dehydration
-SVR and BP maintained
-Premed to reduce anxiety and potential increase in PVR
-PVR minimized
-Ketamine to maintain SVR and bronchodilate
-Antibiotics
-No Air Bubbles
-Venilator, long insp. and short exp times will increase intrathoracic pressure and reduce RV outflow, Avoid PEEP, high rate and low volumes are good
Term
Pulmonary atresia occurs in about ___ out of every _____ live births
Definition
one out of every 10,000 live births
Term
What needs to be maintained in pulmonary atresia?
Definition
the foramen ovale or PDA
Term
Surgical correction for pulmonary atresia
Definition
Hemi-Fontan procedure, a Fontan procedure, RV outflow reconstruction, and valve replacement.
Term
Tricuspid atresia is the _____ most common cyanotic CHD
Definition
third
Term
What must be present in tricuspid atresia?
Definition
foramen ovale or an ASD is ESSENTIAL! Moreover, a patent PDA or VSD MUST be present for blood to flow from left to right into the right ventricle to enter the pulmonary circulation
Term
The most common surgery for tricuspid atresia
Definition
the Fontan procedure
Term
4 types of complex shunts
Definition
1) Transposition of the Great Arteries (TGA)
2) Truncus Arteriosus
3) Double-outlet Right Ventricle (DORV)
4) Hypoplastic Left Heart Syndrome (HLHS)
Term
Transposition of the Great Arteries (TGA) accounts for ___ % of all CHD
Definition
6%
Term
Is TGA associated with other anomalies?
Definition
No
Term
In TGA, survival is dependent on
Definition
oxygenated blood mixing with deoxygenated blood through the foramen ovale and PDA. If there is a VSD, this is even better as the hypoxemia will be less
Term
Surgeries to correct TGA
Definition
Mustard, Senning and Rastelli procedures
Term
Truncus arteriosus is a ___ form of CHD that accounts for approximately __ % of all CHD
Definition
rare, 1%
Term
Truncus Arteriosus is associated with what syndrome? and what are the implications?
Definition
DiGeorge syndrome

-irradiated blood products should be used and calcium levels should be monitored
Term
Early postoperative mortality for truncus arteriosus is
Definition
between 5 and 25% and factors that greatly impact mortality are truncal valve stenosis, coronary abnormalities and low birth weight.
Term
Hypoplastic Left Heart Syndrome (HLHS) occurs roughly ____ in every _____ births
Definition
twice in every 10,000 births
Term
5 Anatomical features of hypoplastic left heart syndrome
Definition
1 Hypoplastic Left Ventricle
2 Mitral Stenosis or Atresia
3 Aortic Stenosis or Atresia
4 Hypoplastic Aortic Arch
5 Ductal-dependent circulation
Term
Hypoplastic left heart syndrome is repaired by what procedure
Definition
Norwood (Stages 1, 2, and 3)
Term
Transport and Handover to ICU
Definition
1) Emergency drugs and equipment
2) Monitoring
3) Careful management of lines and gtts – small movements can make big changes!
4) RT for transport vent: it is a good idea to have RT meet you in the OR to take over ventilation for transport. It never hurts to have another pair of hands and someone else to help monitor during transport. This also reduces the risk of barotrauma that an be caused by providing manual ventilations on route to the ICU.
5) Thorough report is essential to both the RN taking care of the infant as well as the intensivist and RT.
Term
General Principles for Management of all CHD includes the following considerations
Definition
1) Presence of tracheal stenosis/shortening: especially in children with trisomy 21
2) Embolic events in all right-to-left shunts: this can result from air emboli or clots due to polycythemic patients. Be VIGILANT in maintenance of your IV lines and be a stickler for air!
3) Chronic hypoxemia: the compensation here is that your patient will be polycythemic to increase oxygen carrying capacity
4)Polycythemia: this can actually present with Hcts as high as 65 or more! Considerations for you include increased blood viscosity, venous thrombosus potential, increased SVR and PVR and possibility of stroke and cardiac ischemia!
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