Term
| incidence of congenital heart defects in the United States |
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Definition
| roughly 8 per 1,000 live births |
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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. |
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Definition
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Term
| ___ in ____ adults are expected to have some form of congenital heart disease. |
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Definition
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Term
| The goal of the definitive repair of CHD |
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Definition
| to eliminate cyanosis and the body’s compensatory response of polycythemia |
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Term
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Definition
| CHD is completely repaired to the extent it can be |
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Term
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Definition
| Means that the repair is being done in stages. |
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Term
| ___% of the neonate’s myocardial mass is comprised of contractile tissue |
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Definition
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Term
| ___% of mature myocardium is comprised of contractile tissue by age 3 |
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Definition
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Term
| The most important parameter that drives cardiac output in the newborn heart |
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Definition
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Term
| The newborn heart is dependent on the _____________ __________to initiate and sustain contraction. |
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Definition
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Term
| With newborns you want to check ____ blood level and avoid _______ medications. |
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Definition
| ionized calcium, and avoid calcium channel blocking |
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Term
| If you see a falling BP, the child is often ____ __________ |
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Definition
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Term
| Three considerations regarding the preterm infant’s heart: |
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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 |
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Term
| Preemies also have pulmonary vasculature that is more sensitive to vasoconstriction by: |
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Definition
1. Hypoxia
2. Acidosis
3. Hypercarbia
4. Hypothermia. |
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Term
| What kind of anesthetic can you use for a patient with CHD? |
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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 |
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Term
| When dose permanent closure of the foramen ovale occur? |
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Definition
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Term
| Functional closure of the foramen ovale occurs at birth as the result of what? |
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Definition
| increased left atrial pressures in excess of right atrial pressures |
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Term
| Permanent closure {(ANATOMICAL CLOSURE (FIBROSIS)} of the PDA occurs when? |
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Definition
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Term
| Functional closure of the PDA occurs when? |
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Definition
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Term
|
Definition
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Term
|
Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
1 “Simple” Left-to-Right Shunt
2 “Simple” Right-to-Left Shunt
3 Complex shunts
4 Obstructive Lesions |
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Term
| If systemic flow is greater than pulmonary flow, the ratio will be <1 (Qp / Qs <1), what kind of shunt is this? |
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Definition
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Term
| If pulmonary flow is greater than systemic flow, the ratio will be >1 (Qp / Qs >1), what kind of shunt is this? |
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Definition
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Term
| Types of simple left to right shunts |
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Definition
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Term
| What is the Most common CHD? |
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Definition
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Term
| VSD accounts for approximately __% of all CHD |
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Definition
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Term
| Atrial septal defect (ASD): accounts for approximately __% of all CHD |
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Definition
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Term
| Atrioventricular septal defect (AVSD): accounts for approximately __% of all CHD |
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Definition
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Term
| Patent ductus arteriosus: (PDA): accounts for approximately __% of all CHD |
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Definition
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Term
| PDA is more common in _____ ______ |
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Definition
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Term
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Definition
| endocardial cushion defect or AV canal defect |
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Term
| Left-to-Right shunt physiology |
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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 |
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Term
| Lung to Lung shunt is which type? |
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Definition
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Term
| Potential issues related to a left-to-right shunt |
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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 |
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Term
| Physical exam characteristics of VSD |
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Definition
Pansystolic murmur on left sternal border.
Moderate defects show pulm congestion on x-ray. |
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Term
| Large VSDs shows symptoms by what age, and what are the symptoms? |
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Definition
By 4 weeks.
Symptoms include tachypnea, pulmonary infections, CHF and failure to thrive. |
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Term
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Definition
| will have a pressure gradient between the two ventricles |
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Term
|
Definition
| the pressure between the two ventricles actually equalizes |
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Term
| Which is worse, non-restrictive of restrictive VSD? |
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Definition
Non-restrictive VSD
LV pressure = RV pressure is not a good thing! Nonrestrictive VSDs produce worse symptoms and outcomes than a restrictive VSD |
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Term
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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. |
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Term
| What kind of problem can occur after surgical closure of a VSD? |
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Definition
| A high AV block or 3rd degree heart block |
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Term
| ASD, physcical exam findings |
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Definition
| systolic murmur heard over the pulmonic valve. Also, the second heart sound can have a wide split |
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Term
|
Definition
PFO
Primum ASD
Secundum ASD
Sinus Venosus ASD
Coronary sinus ASD
Common Atrium |
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Term
| End outcomes of an untreated ASD |
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Definition
| increased RV work, pulmonary hypertension and RV failure |
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Term
| Surgery is for an ASD is indicated when the pulmonary blood flow is ____ the systolic blood flow |
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Definition
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Term
| Which ASDs are commonly closed percutaneously? |
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Definition
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Term
| Main anesthetic goal in managing a patient with an ASD |
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Definition
to DECREASE THE SVR which will improve the shunt,
Use volatiles, and peripheral vasodilating drugs for this. |
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Term
| What is your goal with PVR and ASD |
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Definition
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Term
| How can you maintain PVR with an ASD |
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Definition
| employ positive pressure ventilation which will increase the intrathoracic pressure. |
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Term
| What are two things you should sure as shit avoid in ASD? |
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Definition
-Avoid increasing SVR, it will worsen the shunt
-Avoid air bubbles in the IV and tubing. |
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Term
| AVSDs are commonly associated with which chromosomal disorder? and what other two conditions may AVSDs occur with? |
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Definition
| Commonly associated with trisomy 21 and may also occur with DiGeorge Syndrome and tetralogy of fallot |
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Term
| Describe the ventricles in a balanced AVSD |
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Definition
| ventricles are similar in size |
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Term
| Describe the ventricles in an unbalanced AVSD |
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Definition
| one dominant ventricle and an opposite hypoplastic ventricle. |
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Term
| Considerations for a patient undergoing AVSD repair |
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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 |
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Term
| PDA occurs in roughly __ in every _____ births |
|
Definition
|
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Term
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Definition
| an increase in systemic vascular resistance, decrease in pulmonary resistance and diminished prostaglandin supply |
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Term
| Will a lower PaO2 keep the DA open or close it? |
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Definition
|
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Term
| What does a PDA sound like on a physical exam? |
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Definition
| a loud systolic murmur that impinges on diastole, heard best over the mid to left sternal border |
|
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Term
| two unfavorable conditions that arise from PDA |
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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 |
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Term
| In surgery for PDA repair, those with urine output issues will have ______ as a first choice. |
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Definition
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Term
| In surgery for PDA repair, those with blood pressure issues only will generally get _____ |
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Definition
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Term
| How would dopamine improve the hemodynamic status of a babe with a PDA? |
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Definition
| dopamine will improve the inotropic property of the heart! |
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Term
| What effect will dobutamine have on the PVR? |
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Definition
| It will decrease PVR by dilating the pulmonary vasculature. |
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Term
| In what case is a PDA beneficial? |
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Definition
| In a large RV outflow problem, they will be on a prostaglandin drip to maintain the PDA |
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Term
| 4 options for medical management of a PDA |
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Definition
1 Indomethacin: inhibits cyclooxygenase which inhibits prostaglandin synthesis
2 Ibuprofen: inhibits cyclooxygenase which inhibits prostaglandin synthesis
3 Fluid restriction
4 Furosemide |
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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 |
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Term
| What problems will you see related to fluid resriction and furosemide in pts being medically managed for a PDA |
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Definition
| Hypovolemia and hypokalemia |
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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 |
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Term
| The most common surgical approach for infants for a PDA repair |
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Definition
| right lateral position to allow for a left-sided thoracotomy |
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Term
| Preop Prep for PDA repair |
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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) |
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Term
| A __ ml/kg infusion of cold blood will quickly produce hypothermia |
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Definition
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Term
| In a term infant, immediately after delivery, would you expect the SpO2 to be greater preductally or postductally??? Why??? |
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Definition
| Preductal (right hand) O2 sat is higher and postductal (left hand) O2 sat is lower because the PDA is still open. |
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Term
| Monitors for PDA repair should include what 3 things? |
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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). |
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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 |
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Term
| Three changes you will assess with PDA ligation |
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Definition
1) clear S1 and S2 and resolution of the murmur
2) increased DBP
3) Improved pulses |
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Term
| 4 Intraop considerations for PDA repair |
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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 |
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Term
| 5 Potential complications of PDA repair |
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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! |
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|
Term
| Most common right to left shunts |
|
Definition
1 Tetralogy of Fallot (most common right-to-left shunt)
2 Pulmonary Atresia
3 Tricuspid Atresia |
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Term
|
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 |
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Term
| Which shunt is a “body to body” shunt |
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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. |
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Term
| What is the major concern with a right to left shunt? |
|
Definition
|
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Term
|
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 |
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Term
| Children with tetralogy of fallot will often do what during a TET spell? |
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Definition
| squat in an attempt to increase the SVR and improve hypoxic spells |
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Term
| A severe cyanotic spell is treated with |
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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 |
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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. |
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Term
| The major goal in the management of right-to-left shunts is... |
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Definition
| to increase pulmonary blood flow, thereby improving oxygenation! |
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Term
| Two ways to increase pulmonary blood flow in right to left shunts |
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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? |
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Definition
| intensification of the right-to-left shunt and therefore the degree of hypoxemia producing hypercyanotic spells (TET spells). |
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Term
| The major goal in management of tetralogy of fallot |
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Definition
| is to maintain adequate tissue oxygenation!! |
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Term
| Management of Tetralogy of Fallot (Mnemonic DDo Some PPeople Kill AAngry Vegans?) |
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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 |
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|
Term
| Pulmonary atresia occurs in about ___ out of every _____ live births |
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Definition
| one out of every 10,000 live births |
|
|
Term
| What needs to be maintained in pulmonary atresia? |
|
Definition
|
|
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
|
|
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 |
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Definition
|
|
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
|
|
Term
| Is TGA associated with other anomalies? |
|
Definition
|
|
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
|
Definition
| Mustard, Senning and Rastelli procedures |
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|
Term
| Truncus arteriosus is a ___ form of CHD that accounts for approximately __ % of all CHD |
|
Definition
|
|
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. |
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|
Term
| Hypoplastic Left Heart Syndrome (HLHS) occurs roughly ____ in every _____ births |
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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. |
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|
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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