Therapeutics I Exam VI (Pediatric Cardiology) Flashcards
Congenital Heart Disease, Low Cardiac Output, Hypercholestermia
What are the two types of pediatric cardiopathophysiology?
Acquired heart disease and congenital heart disease
What is acquired heart disease in pediatrics?
This is heart disease that is developed anytime during childhood.
What are the most common acquired heart diseases seen in the pediatric population?
Arrhythmias, cardiomyopathies, kawasaki disease, rheumatic fever, familial hypercholesterolemia, and AIDs with myocarditis.
What is the most common cardiac pathophysiology seen in the pediatric population?
Congenital heart disease
T or F: Premature infants are at higher risk for congenital heart defects compared to normal birth time babies.
True
What are the 5 different possible causes of congenital heart disease in the pediatric population?
Chromosomal, genetic, maternal, environmental, and multifactoral
T or F: Illness and drug use in the mother can increase the risk for congenital heart disease in babies.
True
T or F: Those with trisomy 21 or 18 are less likely to develop congenital heart disease.
False.
T or F: Fetal alcohol syndrome does not contribute to the development of congenital heart disease.
False
What is the ductus venosus?
This is a part of fetal circulation. The ductus venosus is a temporary blood vessel that connects the umbilical vein to the inferior vena cava in the fetus. It carries oxygenated blood from the placenta into the inferior vena cava which leads to the right atrium.
What is the foramen ovale?
This is a hole that connects the right atrium and the left atrium. During fetal development, the foramen ovale allows blood to flow from the right atrium to the left atrium, bypassing the lungs. This is because the fetus does not breathe and its lungs are not yet functional.
What is the ductus arteriosus?
The ductus arteriosus (DA) is a temporary blood vessel that connects the aorta (the main artery that carries blood from the heart to the body) and the pulmonary artery (the artery that carries blood to the lungs) in the fetus. This is used if oxygenated blood from the right atrium enters the right ventricle, goes into the pulmonary artery, and through the ductus arteriosus into the descending aorta.
Once oxygenated blood passes from the placenta, through the ductus venosus, and into the right atrium, in what two ways can blood be moved once in the right atrium?
It can either go into the right ventricule, pumped into the pulmonary artery and then through the ductus arteriosus and into the descending aorta to oxygenate systemic tissues.
It could also, once in the right atrium, go through the foramen ovale between the right and left atrium and shunt into the left atrium, go into the left ventricle and get shot out of the ascending aorta to oxygenate systemic tissues.
What three anatomies used in fetal circulation should be closed a few days after birth?
Ductus venosus, foramen ovale, and ductus arteriosus
What is the normal transitional circulation seen in fresh babies?
Once a baby cries, the lungs open up and breathing with the lungs occurs normally. There will be cessation of right to left shunting (foramen ovale should close), placenta is removed, and pulmonary vascular resistance decreases allowing for the right ventricule to push blood into the pulmonary circuit and allows blood to enter the left atrium from the pulmonary circuit.
What are the 3 big things that happen in transitional circulation in the pediatric population?
- Cessation of right to left shunting via closure of the foramen ovale
- Placenta removal
- Decrease in pulmonary vascular resistance to blood can flow through pulmonary circuit
What are the 4 expected things to be seen in mature circulation?
- Closure of foramen ovale
- Closure of ductus arteriosus
- Left heart has greater oxygen saturation than right
- Pulmonary vascular resistance is less than systemic vascular resistance
What are the two categories of congenital heart disease?
Acyanotic and cyanotic
What categorizes acyanotic congenital heart disease?
- Left to right shunting (oxygenated blood is mixing with deoxygenated, but since it is going to the right heart which is blood about to oxygenated, there is not a large strain on oxygen saturation)
- Adequate or excessive pulmonary blood flow
- Compromised systemic blood flow (blood leaving left heart)
What categorizes cyanotic congenital heart disease?
- Right to left shunting (deoxygenated blood mixing with oxygenated about to be pumped into systemic circulation, this means a decent amount of deoxygenated blood is being given to the body which is bad)
- Compromised pulmonary blood flow
- Adequate or excessive systemic blood flow
What are the 4 most common acyanotic heart lesions (congenital heart defect)?
- Atrial septal defect (ASD)
- Ventricular septal defects (VSD)
- Patent ductus arteriosus (PDA)
- Coarctation of the aorta (CoA)
What is an atrial septal defect (ASD)?
This is an acyanotic heart lesion. This is when the foramen ovale (hole between right and left atrium) does not close.
What is ventricular septal defect (VSD)?
This is an acyanotic heart lesion. This is when there is a hole between the right and left ventricles.
What is patent ductus arteriosus (PDA)?
This is an acyanotic heart lesion. It is when the ductus arteriosus (connects pulmonary artery to aorta) does not close.
What is coarctation of the aorta (CoA)?
This is an acyanotic heart lesion. It is when the aorta is pinched.
What characterizes the blood flow in the heart in atrial septal defects (ASD)?
This is an acyanotic type of congenital heart disease which means blood shunt from the left atrium to the right atrium as the foramen ovale is still open.
What two things are happening to the lungs with acyanotic congenital heart defects like atrial septal defects (ASD)?
- More blood flow into lungs
- Higher oxygen content of blood entering the lungs
This makes sense because oxygenated blood from the left atrium is shunted to right atrium which will enter pulmonary circulation.
How are atrial septal defects (ASD) treated?
In 40-50% of patients the foramen ovale will close on its own within one year. In other patients, surgical closure is needed.
What are the two possible complications associated with atrial septal defects (ASD)?
- Right ventricle hypertrophy leading to heart failure
- Right atrial enlargement leading to arrhythmias
T or F: Pediatric patients with atrial septal defects are typically symptomatic.
False. Most patients with this are asymptomatic depending on the size of the foramen ovale. A murmur will likely always be present though when listening to heart sounds.
T or F: In normal fetal circulation there is a hole/foramen that connects the right and left ventricles.
False. There is only the foramen ovale that connects the right and left atrium. If there is a hole between the right and left ventricles, this is a congenital heart defect called ventricular septal defects (VSD).
What is happening to blood circulation in a pediatric patient with a ventricular septal defect (VSD)?
The left and right ventricles are connected so there is a lot of mixing of oxygenated and deoxygenated blood. Mainly seeing left ventricle blood mixing with deoxygenated right ventricle blood.
The signs and symptoms of ventricular septal defects are dependent on what 3 things?
- Location and size of the hole
- Pulmonary vascular resistance
- Degree of left to right shunting
What are the 3 complications associated with ventricular septal defects?
- Ventricular dysfunction leading to heart failure
- Pulmonary vascular obstructive disease leading to pulmonary hypertension
- Failure to thrive
What are the 3 things that treatment targets for ventricular septal defects (VSD)?
Treatment targets the complications associated with ventricular septal defects including calorically dense enteral formula for failure to thrive, diuretics and ACEi for heart failure, and surgical close.
For babies with ventricular septal defects that are failing to thrive, what is given?
Calorically dense enteral formula
For babies with ventricular septal defects that are experiencing heart failure, what is given?
Loop diuretics like furosemide (Lasix) and/or ACE inhibitors like enalapril or captopril.
What is the ultimate treatment for ventricular septal defects (VSD)?
Surgical closure
T or F: The acyanotic congenital heart defect, patent ductus arteriosus (PDA) is more common in babies with mothers who had gestational diabetes.
False. Congenital heart disease is not related to gestational diabetes. This type of acyanotic congenital heart defect is seen most commonly in premie babies.
In normal babies, what triggers the ductus arteriosus to close?
Increase in oxygen saturation and decreased circulating prostaglandins allow this natural hole between the pulmonary artery and aorta to close.
What is circulation like if a child has patent ductus arteriosus?
Due to the ductus arteriosus being opened, blood pumped into the aorta from the left ventricle actually goes into the pulmonary artery and into the lungs. This is not good as the body is not getting enough oxygen.
What are the 3 common complications associated with patent ductus arteriosus (PDA)?
- Calcification of ductus
- Heart failure
- Pulmonary hypertension
What two medications are commonly used in the pediatric population to manage heart failure?
Loop diuretics and ACE-inhibitors to reduce afterload
What medications should a child be receiving who is in heart failure due to patent ductus arteriosus (PDA)?
Loop diuretic like furosemide (Lasix) and/or Ace-inhibitor.
What is the treatment for patent ductus arteriosus (PDA)?
Closure of the ductus either via surgery or pharmaceutical agents like NSAIDs.
What class of medications are used to close the ductus arteriosus in patent ductus arteriosus (PDA)?
NSAIDs are used as they inhibit prostaglandin synthesis which is needed to close that hole. Specifically ibuprofen or indomethacin is used.
What needs to be monitored in a patient with patent ductus arteriosus that is under going pharmacological closure of the ductus arteriosus with NSAIDs?
Basic metabolic panel, BUN, SCr, guaiac-positive stools, enlarging ventricles, and thrombocytopenia
What are complications associated with closure of the ductus arteriosus with NSAIDs?
Transient decrease in GFR, increase risk for GI bleeds, Necrotizing enterocolitis (NEC), and platelet dysfunction.
If a pediatric patient can’t use NSAIDs for closure of the ductus arteriosus, what other medication can be used?
Acetaminophen.
What are the 4 contraindications for NSAID use in the pediatric piopulation?
GI bleeding or perforations, hyperbilirubinemia, renal failure, and/or thrombocytopenia
If NSAIDS fail and acetaminophen does not work to close to ductus arteriosus, what can be done?
Last resort is surgical ligation of the ductus arteriosus.
How is blood flow disrupted in a patient with coarctation of the aorta (CoA)?
There is left ventricular outflow obstruction due to the narrowing of the aorta. This decreases oxygen perfusion to tissues.
What are the two complications associated with the coarctation of the aorta (CoA)?
- Decrease peripheral perfusion
- Increased left atrial and left ventricle pressure
What are the 4 ways in which coarctation of the aorta can be treated?
- Improve peripheral perfusion
- Correct metabolic acidosis
- Maintain ductal arterious patency (keep open or reopen) with prostaglandin infusion
- Surgical correction (ultimate goal)
What is unique about the treatments/ short-term fixes for coarctation of the aorta (CoA)?
Part of the management of this condition until surgical correction can be done includes opening up the ductus arteriosus with prostaglandins.
What 2 things do pharmacological agents target when managing pulmonary volume overload and/or congestive heart failure in pediatric patients with congenital heart disease?
Decreasing preload and afterload
With coarctation of the aorta (CoA) and congenital heart disease in general, in what 3 ways is pulmonary volume overload and congestive heart failure managed?
- The goal is to augment cardiac output by decreasing preload with the use of diuretics including loop diuretics (furosemide or bumetanide) potassium sparing diuretics (spironolactone), or thiazide diuretics (chlorothiazide).
- Another goal is to augment cardiac output by decreasing afterload with the use of ACE-inhibitors like captopril, enalapril, or lisinopril
- Final goal is to augment cardiac output by increasing inotropy via the use of milrinone (inodilator) or low-dose epinephrine (inopressor)
What needs to be monitored in a patients with coarctation of the aorta / congenital heart disease in general being treated for pulmonary fluid overload and/or congestive heart failure with diuretics?
Electrolytes, fluid balance, basic metabolic panel, weights, and physical exam
Thinking about how cardiac output is directly related to heart rate and stroke volume, which of these do providers use and manipulate to optimize outcomes in pediatric patients?
Stroke volume as there are limitations with increasing heart rate in the pediatric population.
What needs to be monitored in a patients with coarctation of the aorta / congenital heart disease in general being treated for pulmonary fluid overload and/or congestive heart failure with ACE-inhibitors?
Blood pressure, renal function, and potassium levels.
When managing pulmonary volume overload and congestive heart failure in pediatrics with congenital heart diseases, one goal is to augment _______ _________ by decreasing _________ with the use of diuretics including loop diuretics (furosemide or bumetanide) potassium sparing diuretics (spironolactone), or thiazide diuretics (chlorothiazide).
Cardiac output
Preload with diuretics
When managing pulmonary volume overload and congestive heart failure in pediatrics with congenital heart diseases, another goal is to augment cardiac output by decreasing ________ with the use of ACE-inhibitors like captopril, enalapril, or lisinopril
Afterload
When managing pulmonary volume overload and congestive heart failure in pediatrics with congenital heart diseases, another goal is to augment cardiac output by increasing _______ via the use of milrinone (inodilator) or low-dose epinephrine (inopressor)
Inotropy
What is the MOA of milrinone?
This is an inodilator meaning it increases inotropy (contractility of the heart) while inducing vasodilation leading to a decrease in afterload and systemic vascular resistance.
What are the two adverse effects associated with milrinone?
Thrombocytopenia and accumulation in the kidneys during renal disease
What is the MOA of sodium nitroprusside?
This is a potent vasodilator that decreases preload and afterload.
What are the adverse effects associated with sodium nitroprusside?
Hypotension and cyanide toxicity in renal and liver dysfunction. Due to this, the use of this medication is only indicated for a few days.
At low doses of epinephrine given aid in pulmonary volume overload and congestive heart failure in children with congenital heart diseases, what receptors are activated?
Low dose epinephrine (<0.08mcg/kg/min) only acts on beta 1 receptors to increase inotropy and chronotropy of the heart.
When is calcium chloride utilized in children with congestive heart failure?
It is only used in infants who have naturally low calcium stores in their heart. It increase contractility of the heart.
What are the two most common/discussed cyanotic lesions/ congenital heart defects?
- transposition of the great arteries (TGA)
- tetralogy of fallot (includes ventricular septal defect, overriding aorta, right ventricular hypertrophy, and right ventricular outflow tract obstruction)
What is the cyanotic lesion congenital heart disease called the transposition of the great arteries (TGA)?
This is when the aorta and pulmonary artery are switched. The aorta is connected to the right ventricle while the pulmonary artery is connected to the left ventricle. This causes deoxygenated blood to go to the body and oxygenated blood to go to the lungs. It is two very closed circuits.
What is the management of transposition of the great arteries (TGA) until surgery can be performed?
It is essential to survival that the these patients have acyanotic heart lesions including patent ductus arteriosus (PDA), atrial septal defects (ASD), and ventricular septal defects (VSD). Prostaglandin infusions are given to keep these ducts open.
What is the cyanotic lesion/ congenital heart disease, tetralogy of fallot?
This is a type of cyanotic lesion that consists of four anomalies including ventricular septal defect (VSD), overriding aorta (RV and LV go to aorta), right ventricular hypertrophy, and right ventricular outflow tract obstruction (pulmonary stenosis).
If a patient have very severe right ventricular outflow tract obstruction in the tetralogy of fallot, what is essential to their survival?
The ductus arteriosus must be open for pulmonary flow
What is a TET spell?
This occurs when a child gets upset and is crying a lot and lung pressures begin to changes and deoxygenated blood begins to go towards systemic outflow. It is also called a hypercyanotic crisis. This is a common thing in those with tetralogy of fallot.
In what 3 ways is the tetralogy of fallot managed?
- Prostaglandins to keep ductus arteriosus open
- Systemic-pulmonary shunt
- Prophylactic antibiotics to prevent endocarditis
What is the pathophysiology surrounding TETs spells?
When a child cries, this triggers their sympathetic stimulation and catecholamines are released which contractions the right ventricular outflow tract which increases the right to left shunting of deoxygenated blood leading to deoxygenated blood being pumped into systemic circulation.
What are the 3 non-pharmacological ways to manage a TET spell?
- Knee to chest maneuver (decrease right to left shunt)
- Oxygen for the hypoxia
- Fluid bolus to increase systemic vascular resistance (increases left to right shunt)
What are the 4 pharmacological ways to manage a TET spell?
- Morphine (CNS depressant which decreases hyperpnea)
- Sodium bicarb (correct metabolic acidosis)
- Propranolol (decrease RVOT obstruction)
- Phenylephrine (increase systemic vascular resistance to increase left to right shunting)
A patient that is susceptible to TETs spell may be sent home on what medication?
Propranolol
Which of the following statements is false regarding the treatment of hypercyanotic spells (TET spells)?
A. A knee-to-chest maneuver can be used to increase SVR and decrease right to left shunting
B. Phenylephrine can be used to decrease SVR and decrease right to left shunting
C. Oxygen can be used to decrease PVR and decrease right to left shunting
D. Beta blockers can be used to decrease right ventricular myocardial spams and promote pulmonary blood flow
B. False. Phenylephrine increases SVR to promote left to right shunting
What is low cardiac output syndrome (LCOS)?
This is the inability of the heart to meet the oxygen demand from the body. There is decreased cardiac output, increased pulmonary vascular resistance, and increased systemic vascular resistance
What typically causes low cardiac output syndrome (LCOS)?
Typically due to post-operative cardiac surgery that is stimulated due to high levels of interleukins and inflammatory mediators causing myocardial stun.
What 4 types of surgical methods typically cause low cardiac output syndrome (LCOS)?
Cardiopulmonary bypass
Aortic cross clamp
Myocardial tissue incision
Cardioplegia solutions
What are the complications seen with low cardiac output syndrome (LCOS)?
Cyanotic, cool extremities, decreased organ perfusion, decreased UOP, metabolic acidosis, and elevated lactate.
How is low cardiac output syndrome (LCOS) managed?
- Optimize preload with diuretics (furosemide or thiazides)
- Decrease afterload with systemic vasodilators (milrinone, nitroprusside or nicardipine)
- Increase inotropy and contractility (milrinone or low dose epinephrine)
2-week old white male patient has just undergone a cardiac surgical procedure that required cardiopulmonary bypass. Which of the following is false?
A. The patient may have LCOS after the procedure
B. The severity and length of the LCOS will depend upon the length of the bypass, illness prior to surgery, and the invasiveness of the procedure.
C. Milrinone can be used to increase inotropy and increase afterload
D. Diuretics can be used to optimize the patient’s fluid status (preload)
E. Inotropic and vasopressor support must be titrated to individual patient endpoints.
C. Is false. Milrinone increases inotropy but decreases afterload as it is an inodilator.
When should lipid screenings be initiated in the pediatric population if the child is at risk?
2-8 years old
What are considered risk factors in children for hypercholesteralemia?
- Grandparent, parents, aunt/uncle or sibling with CAD before 55 years in males or 65 years in females
- Parent with TC 240 mg/dL or higher
- Parent with know dyslipidemia
- Patient has T2D, HTN, BMI 95th percentile or greater, or has exposure to cigarette smoke
- Patient has moderate to high risk medical condition with cardiovascular disease
Why are lipid screenings not done in the 9-16 age group?
This age group is likely experiencing puberty which can alter lipids levels.
When should all people have their lipids screened?
17-21 years
How is the diagnosis of heterozygous familial hypercholesterolemia made?
- Molecular genetic testing OR
- High levels of TC and LDL with at least one of the following: family history of FH, history of premature CAD in family, or physical exam should abnormal cholesterol deposits.
What is the first-line treatment for children with heterozygous familial hypercholesterolemia?
HMG CoA Reductase Inhibitors (Statins)
What statin is most commonly used in the pediatric population?
Atorvastatin
What things need to be monitored if a child is on a statin for HeFH?
Creatinine kinase, AST, and ALT at baseline, 1-3 months after initiation, and yearly.
What is one of the most important things when using anti-arrhythmics in pediatrics?
Safety!!
What is the overall function of class 1 anti-arrhythmic?
These are sodium channel blockers. It includes class 1A, 1B, and 1C
What is the one medication in class IA for anti-arrhythmics we need to know?
Procainamide
What is the one medication in class IB for anti-arrhythmics we need to know?
Lidocaine
What is the one medication in class IC for anti-arrhythmics we need to know?
Flecainide
What is the very general impact on depolarization with class IA medications like procainamide?
It slows depolarization and increases the QTc interval
What is the very general impact on depolarization with class IB medications like lidocaine?
It slows depolarization and shortens the QTc interval
What is the very general impact on depolarization with class IC medications like flecainide?
It slows depolarization and prolongs the QRS interval
What is the class of drugs that represent class II anti-arrhythmics?
Beta blockers like esmolol, propranolol, atenolol, and metoprolol
What is the very general impact on conduction with class II anti-arrthymics?
Slows AV nodal conduction
What are the two class III anti-arrhythmics we need to know?
Amiodarone and sotalol
What is the general impact of class III anti-arrhythmics on repolarization?
It slows repolarization and prolongs the QTc interval
What are the two drugs that are in the class IV anti-arrhythmics?
Non-DHP CCBs including diltiazem and verapamil
What is the general impact of class IV anti-arrhythmics on conduction?
Slows AV nodal conduction
In what pediatric population is verapamil not used in and why?
Verapamil is not to be used in those younger than 1 year old due to the risk of cardiovascular collapse and death.
In what pediatric population are beta-blockers, CCBs, disopyramide, flecainide, and propafenone not used and why?
BBs, CCBs, disopyramide, flecainide, and propafenone are not to be used in **cardiomyopathy and/or left ventricular dysfunction **due to the potential for negative inotropic effects.
In what populations should beta blockers and propafenone not be used in and why?
BBs and propafenone should not be used in those with severe asthma due to its beta blockade effects.
In what populations should digoxin, disopyramide, verapamil, and beta-blockers not be used and why?
Do not use these medications in those with Wolff-Parkinson-White Syndrome (WPW) due to the potential to induce ventricular fibrillation with or without bradycardia.
What populations should amiodarone, sotalol, procainamide, disopyramide, flecainide, and propafenone not be used and why?
Those with long QT syndrome or taking drugs know to prolong the QT as these medications can further prolong the QT.
What populations should not take flecainide and propafenone and why?
Do not use in those with structural heart disease as these medications may cause myocardial depression and pro arrhythmic effects.
T or F: Methadone does not prolong the QT inteval.
False. Methadone is a medication that can prolong the QTc interval.
What are two considerations/ monitoring parameters with digoxin use in children?
- Monitor electrolytes including K and Mg
- Toxicity levels in babies typically are at levels above 2 (however, digoxin levels in pediatrics are not well correlated to serum levels)
_______________ indicates digoxin toxicity.
Hyperkalemia
What are many considerations/ monitoring parameters with flecainide (class IC) use?
- Monitor electrolytes including Mg
- Goal 0.2-1 due to narrow TI
- Multiple concentrations available
- Interacts with MILK
How are feeding altered with a pediatric patient on formula or breast milk on flecainde?
We do not really change feedings but try to administer the flecainide in between the feedings to avoid its interaction with milk.
What are the many monitoring parameters/ considerations with procainamide (Class IA)?
- Get NAPA and Procainamide levels
- Serum goal for procainamide is 4-10
- serum goal between both NAPA and procainamide goal is 10-12
- Can cause hypotension and proarrythmogenic
What are the 3 considerations/ monitoring parameters for propranolol (Class II)?
- hypotension (β1 receptors in the juxtaglomerular cells of the kidney stimulate renin release. Blocking these = ↓ renin → ↓ angiotensin II → vasodilation and ↓ blood volume. That further drops BP.)
- hypoglycemia with initiation and dose increases
What are the 3 considerations/monitoring parameters for sotalol (Class III)?
- monitor electrolytes K and Mg
- Monitor renal function
- ECG on initiation
NAPA is the active metabolite of _______________.
Procainamide
Procainamide can result in a systemic _________ like reaction?
Systemic Lupus like reaction
What are the two most common side effects associated with lidocaine?
CNS excitability and seizures
What are the two most common side effects associated with flecainide?
Dose related headaches and dizziness
What is the major side effect of beta blockers?
Bronchspasms
What is a medication that interacts with basically all anti-arrhythmics?
Amiodarone
What is the most common pediatric symptomatic arrhythmia?
Supraventricular Tachycardia (SVT)
What is the definition of a supraventricular tachycardia (SVT)?
This is sustained tachycardia originating about the bundle of His.
T or F: Most supraventricular tachycardia occurs in structurally abnormal hearts.
False. Most with SVT have structurally normal hearts.
What are two things, though rare, that supraventricular tachycardia can lead to?
Cardiomyopathy or congestive heart failure
What is the typical heart rate presentation for infants with SVT?
220-280 bpm
What is the typical heart rate presentation for children older than 1 year old with SVT?
180-240 bpm
What are the sign and symptoms of SVT in those greater than 1 year old?
Poor feeding, vomiting, diaphoresis, increased sleeping and irritability. If the HR is prolonged about 280 bpm, they will be experiencing congestive heart failure which presents as pallor, cough, respiratory distress, and cyanosis.
What is the typical heart rate presentation for school-aged children and adolescents with SVT?
Greater than 150 bpm
What are the typical SVT presentation for school-aged children and adolescents with SVT?
Palpitations, chest pain, dizziness, SOB, pallor, pre-syncope, diaphoresis, and/or clammy skin
What are the non-pharm methods to get someone out of supraventricular ventricular tachycardia (SVT)?
If the patient is presenting as stable, use vagal maneuvers like putting face in ice, bearing down like you would poop, and other vagal maneuvers.
T or F: It is easier to identify patients with SVT as they get older.
True
What is the first line medication for the acute treatment of SVT in children?
Adenosine!
What is the dosing for adenosine use for acute SVT in the pediatric population?
0.1mg/kg/dose (max 6 mg)
Can repeat 2 more doses at 0.3mg/kg/dose (max 12mg)
If adenosine fails are 3 administrations for acute SVT in children, what happens next?
Hypothetically you could use calcium channel blockers, but in reality, most teams will immediately go for cardioversion
What is the half-life of adenosine?
Only seconds. This means when giving IV push, you want to administer it in the IV that is closest to the heart.
What are the 2 first line medications for chronic SVT in children?
Digoxin and beta blockers (propranolol). Beta blockers are safer than digoxin
What are the 3 second line medications for chronic SVT in children?
Amiodarone, flecainide, or sotalol
What are the most common 5 medications used for chronic SVT in those younger than 1 year old?
Propranolol (1st line)
Digoxin (1st line)
Amiodarone (2nd line)
Flecainide (2nd line)
Sotalol (2nd line)
What needs to be monitored in a pediatric patient taking digoxin?
Monitoring is not recommended in children less than 1 year old but just in case keep an eye out for renal dysfunction, drug interactions, and electrolyte imbalances (hyperkalemia indicates digoxin toxicity).
What two things need to be monitored for those less than 1 year old taking amiodarone?
Thyroid and liver function
What needs to be monitored in patients taking sotalol?
QTc prolongation monitoring
A 4 week old previously healthy infant is admitted to the ED with a narrow complex tachycardia on the EKG. The patient is pale with cool extremities but otherwise normal. What would NOT be a reasonable first line therapy for SVT?
A. Place and IV and administer IV adenosine
B. Place ice on forehead
C. Administer oral propranolol
D. Start and IV line and administer IV digoxin
Technically the answer is D because it should be oral digoxin and not IV digoxin.
Is oral or IV digoxin used in acute cases of SVT in children?
Oral
T or F: Neonates have lower levels of factors II, VIII, IX, X, protein C, protein S, and antithrombin III.
True! These factors do increase rapidly from birth to 6 months, but at the beginning these levels are very low.
T or F: There is not normally abnormal hemostasis in children with congenital and/or acquired heart disease.
False. There is normally abnormal hemostasis in these patients.
Hypoxemia in congenital heart disease leads to ________ damage. This promotes the activation of platelets and coagulation.
Vessel damage
Is hypoxemia more present in cyanotic or acyanotic congenital heart disease?
Hypoxemia is present more in cyanotic congenital heart disease
What are the 3 big things that may induce coagulation in children?
- Hypoxemia in congenital heart disease (more so with cyanotic)
- Central line placements
- Erythrocytosis (too many RBCs which leads to rigid shape and clotting)
What are the signs and symptoms of a thromboembolism?
Pain, discoloration, cyanosis, swelling, loss of distal pulse, loss of perfusion, cold extremities, catheter malfunction due to clot blocking it, and thrombocytopenia
T or F: The CHEST guidelines on anticoagulation for adults is adapted for children.
True
Which DOAC is the best and most commonly used in children?
Rivaroxaban (Xarelto)
What things/conditions can increase the risk for thrombosis in children with cardiac disease?
stents, shunts, single ventricle physiology (Fontan surgery), ventricular dysfunction, arrhythmias, and ventricular assist devices (VADs)
When a child is being treated prophylaxically for a blood clot, do they use treatment or prophylaxis anti-coagulation dosing?
Surprisingly, most children will get treatment dosing for prophylaxis but it is all dependent on the indication and agent being used.
Does unfractionated heparin has a long or short half-life?
Short half-life ranging from 60-90 minutes
T or F: Unfractionated heparin dosing is children is dependent on weight.
False. The dosing of UFH in children is based on age.
What two things absolutely need to be monitored for a patient on unfractionated heparin?
aPTT and anti-Xa/UFH levels
T or F: HIT from using heparin is incredibly common in children.
False. It is actually super uncommon in children.
T or F: Unfractionated heparin can be used as mechanical circulatory support.
True
T or F: Low molecular weight heparin can be used for mechanical circulatory support.
False. Only unfractionated heparin is used for that indication.
What are the big differences between unfractionated heparin and low-molecular weight heparin?
LMWH has a large volume and distribution and half life.
When and what should be monitored for a patient on LMWH?
Anti-Xa levels should be checked 4-6 hours after the second dose for initiation and when doses are changed. The goal is 0.5-1 unit/mL for anti-Xa levels on LMWH.
Where should LMWH be injected on infants and children?
Thighs. Young children do not have belly fat yet so the stomach is not preferred.
T or F: Caregiver education is crucial for children on LMWH as many children on this medication may weight less than 60 pounds which is the minimum weight for the prefilled syringes.
True
What are the 4 common indications for warfarin use in children?
- mechanical prosthetic heart valves
- heart disease- cardiomyopathy or fontan procedure
- pulmonary hypertension
- arrhythmias
Is warfarin bridged in children, or just adults?
Warfarin is bridged in children as well because initial administration of warfarin increases clotting risk.
What is the warfarin dosing in the majority of children?
0.2mg/kg/day (max 5-10mg)
If they have fontan procedure, liver disease, or drug interactions, the dose can be 0.1mg/kg/day
How is warfarin bridged in children?
It is typically bridged with LMWH or unfractionated heparin until therapeutic INR value or for a minimum of 5 days.
Warfarin binds to _______ _______ nutrition.
Liquid enteral nutrition
T or F: Infants on formula who are also taking warfarin, will require a higher dose of warfarin compared to those not on formula.
True. This is true because liquid enteral nutrition binds warfarin.
T or F: Vitamin K should be limited in children on warfarin.
False. It should not be limited buy consistent with the diet.
What are the 4 indications for aspirin use in the pediatric population?
- Prosthetic heart valves
- conduits
- stents
- BTT shunt
What are the two common adverse effects of aspirin?
Bleeding and reye syndrome
What is Reyes syndrome?
Non-inflammatory hepatice encephalopathy
What is the MOA of DOACs like rivaroxaban and apixaban?
DOACs directly inhibit factor Xa
Why might DOACs be a better option than warfarin in the pediatric population?
DOACs require less monitoring, have less interactions with food and medications, and more predictable pharmacokinetics
Which of the following is true regarding developmental anticoagulation in children?
A. Neonates require the same doses of anticoagulants as adults because they have a normal hemostatic system at birth
B. Children are at higher risk for adverse effects including bleeding due to immature development of their kidneys
C. Infants/Neonates require higher doses of unfractionated heparin and enoxaparin compared to older children because they have lower levels of endogenous anti-thrombin III
D. Children require lower doses of unfractionated heparin and enoxaparin compared to adults because they have a smaller body surface size and decreased clearance
C. Infants/Neonates require higher doses of unfractionated heparin and enoxaparin compared to older children because they have lower levels of endogenous anti-thrombin III
Remember that heparins bind to antithrombin and deactivate factor Xa (UFH also deactivates IIa).
What should be monitored if a patient is taking a DOAC?
Liver enzymes
What is the MOA of unfractionated heparin?
Binds to antithrombin and deactivates factor Xa and IIa (thrombin)
What is the MOA of LMWH like enoxaparin?
Binds antithrombin and inhibits factor Xa
What are the 2 types of pediatric heart failure?
Primary and secondary cardiomyopathies
T or F: The management of heart failure in children is extrapolated from the adult guidelines.
True.
T or F: Children are at higher risk for adverse effects including bleeding due to immature development of their kidneys.
False. Neonates are at a higher risk for these adverse effects but not children.
What are the 3 things that lead to heart failure in the pediatric population?
- structural heart disease
- sarcomeric mutations
- systemic disease
What are the 4 examples of primary cardiomyopathies in pediatric heart failure?
- dilated
- hypertrophic
- restrictive
- left ventricular non-compaction
What are some examples of secondary cardiomyopathies in pediatric heart failure?
Myocarditis, hypertension, kawasaki disease, MI, infections, arrhythmias, and more
What is the typical presentation of heart failure in an infant?
Feeding issues, failure to thrive, tachypnea, tachycardia, and diaphoresis
For all ages of children, what is the typical presentation of heart failure?
Resting tachycardia, tachypnea, cool or cyanotic extremities, gallop, hepatomegaly, pallor, weak pulse.
What is the definition of pediatric heart failure?
This includes pulmonary and systemic congestion, respiratory insufficiency, GI dysfunction, and adverse cardiac remodeling
What are we targeting when treating heart failure in the pediatric population?
-symptoms
-remodeling
-increase calories
-limit fluids
Diuretics reduce _________ and control symptoms of _______.
Preload
Edema
Thinking back to adult heart failure reduced ejection fraction, how are the medications given to children similar?
They are very similar. Think DABAS
Diuretics- loop
ARNI- entresto (could use ACE or ARB as well)
Beta blockers- metoprolol succinate, carvedilol
Aldosterone antagonist- spironolactone
SGLT2 inhibitor- dapaglafozin (farxiga)
Can also use digoxin and/or Ivabradine
What should be monitored in children on diuretics (loops) for heart failure?
Electrolytes, arrhythmias, and fluid status
What are the most commonly used ACEs and ARBs for heart failure in children?
Enalapril, captopril, or lisinopril
Losartan is the ARB
What needs to be monitored in children on ACEis or ARBs for heart failure?
Blood pressure, renal function, and potassium levels
What is the dosing motto for ACEis and ARBs in heart failure for children?
Initiate low and titrate up slow
What needs to be monitored if a child with heart failure is on an entresto?
Blood pressure, renal function, and possible washout period if patient was on an ACEi prior.
What needs to be monitored if a child is on a beta blocker like carvedilol for heart failure?
Bradycardia, hypoglycemia, bronchospasms
What is the dosing motto for beta blocker use in children?
Initiate low and titrate up slow
What two things need to be monitored when a child is taking an aldosterone antagonist like spironolactone?
Potassium levels and gynecomastia
If a children is placed on ivabradine for heart failure, what needs to be monitored?
Heart rate
If a children is placed on an SGLT2 inhibitor like dapaglafozin, what needs to be monitored?
Blood glucose, renal function, and possible changes in diuretic dosing
