Paeds_CVS

Question 1

**Congenital **
Cyanotic Heart Defects
The 5 Ts

Answer 1

ToF - Tetralogy of Fallot
ToGV - Transposition of Great Vessels
TA - Tricuspid Atresia
PTA - Persistent Truncus Arteriosus
TAPVR - Total Anomalous Pulmonary Venous Return (TAPVR)

Question 2

ToF - Tetralogy of Fallot

Question 3

What are the types of acyanotic congenital heart defects?

Answer 3

The main types of acyanotic heart defects are:

• Ventricular septal defect (VSD)
• Atrial septal defect (ASD)
• Patent ductus arteriosus (PDA)
• Coarctation of the aorta (CoA)

Question 4

Acyanotic congenital heart defects
acronynms

Answer 4

CoA
PDA
ASD
VSD

Question 5

VSD

Ventricular Septal Defect

Answer 5

there’s a gap in the ventricular septum, which separates the right and left ventricles

Question 6

ASD

Atrial Septal Defect

Answer 6

the gap is in the atrial septum, which separates the right and left atria

Question 7

PDA

Patent ductus arteriosus

Answer 7

when the ductus arteriosus fails to close after birth

Question 8

CoA

coarctation is a narrowing of the aorta

Answer 8

occurs below the origin of the left subclavian artery at the origin of the ductus arteriosus.

Question 9

Left to Right shunting most commonly occur in

** acyanotic/ cyanotic** heart defect?

Right to Left Shunting –> Eisenmenger Syndrome

Answer 9

Acyanotic

Due to the high pressure of the left side of the heart, the blood goes from the left to the right side of the heart and as a result, into the pulmonary circulation, and that is called left-to-right shunting.

Because this extra blood is already oxygenated, there’s no issue of cyanosis, but it does increase the pulmonary flow and that leads to pulmonary hypertension, which may progress into heart failure.

Question 10

Eisenmenger Syndrome

Answer 10

In some cases, over a long period of time, pulmonary hypertension becomes so severe that the pulmonary pressure exceeds the systemic pressure causing a reversal of blood flow from the right to the left side of the heart, called right-to-left shunting. This reversal is called Eisenmenger syndrome, and at that point, the deoxygenated right-sided pulmonary blood gets shunted to the left-sided systemic circulation, causing cyanosis.

Question 11

Complications of Acyanotic heart defects

Answer 11

arrhythmias,
embolism,
infective endocarditis.

Question 12

Why are patients with actanotic heart defects typically asymptomatic?

Answer 12

Because this extra blood is already oxygenated, there’s no issue of cyanosis, but it does increase the pulmonary flow and that leads to pulmonary hypertension, which may progress into heart failure.

Question 13

What are some of the signs and symptoms that they will typically experience ?

Answer 13

but some clients can develop tachypnea, tachycardia, and activity intolerance.

Some clients can also experience feeding problems, which can cause poor weight gain, and failure or difficulty to thrive.

Question 14

Difference in physical examination (i.e. auscultation) between each of the acyanotic heart defect?

Answer 14

VSD: Pansystolic murmur
ASD: Soft midsystolic murmur aka swishing sound
PDA: Cont. systolic murmur that extends into diastole
CoA: Systolic murmur

Question 15

Most significant Characteristic finding for acyanotic heart defect?

Answer 15

the most characteristic finding is a difference between upper and lower limb blood pressure,

with** higher BP** in the upper limbs and** pulses in the lower extremities** will be decreased or absent.

Question 16

Diagnosis of Acyanotic Heart Defect

Answer 16

1) Mother’s history during pregnancy

2) Physical assessment of the client (Auscultation, Vitals, ECG to check for arrhythmias)

3) Echocardiography to visualize the defect.

4) Most defects can also be detected prenatally by standard obstetric ultrasound examination.

5) Chest x-ray, CT, and MRI, might also be performed.

6) Cardiac catheterization is sometimes performed to assess the extent of the defect.

Question 17

Treatment of Acyanotic Heart defects

Answer 17

Most acyanotic heart defects close on their own during the First year of life
(Self-limiting)

Should heart failure develops:
various medication treatment follows according to the type of defect

Question 18

General Medication for Heart failure caused by acyanotic heart defects

Answer 18

• Digoxin
• Diuretics
• Potassium supplements
• Prophylactic antibiotics** should also be prescribed to prevent infective endocarditis.

Question 19

Add-on
Special medications for PDA

Patent ductus arteriosus

Answer 19

Indomethacin to close the PDA

Question 20

Add-on
Special medications for CoA

Coarctation of the aorta

Answer 20

prostaglandin E1 infusion, which can keep the ductus arteriosus open but also seems to relax the tissue of the coarctation segment.

This helps increase blood flow to the body past the area of coarctation.

Question 21

What will be the recommendation should all medications fail to treat patients with acyanotic heart defect

Answer 21

Heart Transplant

Question 22

Nursing Care for Acyanotic Heart Defect

ASD, VSD, PDA, CoA

Answer 22

** for an infant with a VSD **

Priority:
1) Maintain Adequate cardiac output (CO)
2) Provide nutritional support
3) Check VS (Auscultation: heart & lung), palpating peripheral pulses
4) Report signs of decr. CO & pulmonary congesion
5) Administer meds
6) Assess G&D
7) Ask caregivers on feeding history

Question 23

What are the signs of decreased CO and pulmonary congestion?

Answer 23

Tachycardia
decreased peripheral pulses Tachypnea
Pulmonary crackles
Intercostal retractions
Nasal flaring

Question 24

Family education - Nutritional needs for patients with acyanotic heart defects

Answer 24

infants with VSD often tire easily with feedings, and at the same time, infants with VSD will also have increased nutritional needs due to their increased cardiovascular demands.

So in addition to encouraging continued breastfeeding, you’ll need to **supplement the infant’s diet by administering the prescribed high-calorie formula, **

by orogastric or nasogastric tube.

Question 25

Ways to improve nutritional needs of acyanotic heart defect patients?

Answer 25

1) Continue breastfeeding

2) Supplement with high-calorie formula by orogastric or nasogastric tube

Question 26

Discharge planning:
Advice to caregivers
(Acyanotic heart defect)

Answer 26

1) Adherence to medication
2) Attend scheduled tests and appointments strictly
3) Educate caregivers, if the VSD does not close on its own or if their baby’s symptoms can’t be managed with medications, that surgery to patch the opening in the heart will be needed.

4) Highlight the importance of scheduling frequent feedings with breastmilk and the prescribed high-calorie formula, but for no > 20 mins each time, as tolerated, to conserve their baby’s energy.

5) Track baby’s** intake & wet/soiled diapers**, and how to weigh their baby.

6) Prevent **infection **by incl. frequent HH, get the recom. vaccines, and keeping their baby’s environment **smoke-free. **

7) Dental care will be an important part of their baby’s future health care, and instruct them how to clean their baby’s gums or any new teeth after each feeding with a soft cloth.

8) Contact HCP right away if their baby is not eating well or gaining weight. Seek emergency medical attention if their baby becomes lethargic, or is having difficulty breathing.

Question 27

ToF

Answer 27

tetralogy of Fallot (ToF)

** four heart abnormalities: **
1) Pulmonary stenosis, which is narrowing of the pulmonary valve;

2) Right ventricular hypertrophy, which is enlargement of the right ventricle;

3) VSD
4) in addition to overriding aorta where the aorta is shifted and sits above the VSD

So the result is oxygen rich blood and oxygen poor blood in the right and left ventricle mix due to the VSD, and then it’s pumped out of the overriding aorta.

Question 28

ToGV

Answer 28

transposition of great vessels

where the aorta and the pulmonary trunk swap locations. When this occurs, oxygen poor blood returning to the right side of the heart is pumped into the aorta and the rest of the body instead of the pulmonary artery and the lungs.

Question 29

TA

Answer 29

tricuspid atresia

the tricuspid valve that normally prevents blood from returning into the right atrium when the right ventricle contracts, is malformed or fails to develop entirely.

Because of this, oxygen poor blood returning to the right atrium can not enter the right ventricle, so an ASD is needed to mix the blood in the right and left atrium, and a VSD is needed for the blood to mix in the right and left ventricle.

Question 30

PTA

Answer 30

persistent truncus arteriosus

where the truncus arteriosus doesn’t split properly into the aorta and pulmonary artery during fetal development.

So this extra large vessel sits above both ventricles and allows deoxygenated blood and oxygenated blood to mix before getting pumped to the lungs and the rest of the body.

Question 31

TAPVR

Answer 31

total anomalous pulmonary venous return

all four pulmonary veins form abnormal connections.

So instead of returning blood from the lungs to the left atrium, they connect to the right atrium, the superior vena cava or inferior vena cava.

The result is that oxygen rich blood from the lungs returns to the right side of the heart, where it’s pumped back to the lungs.

The condition is only compatible with life if there’s an atrial septal defect that allows some of the blood in the right atrium to flow into the left atrium where it could eventually be pumped into systemic circulation.

Question 32

Risk factors that interfere with the development of the heart

Answer 32

Risk factors include chromosomal abnormalities of the fetus, in addition to maternal infections, chronic illnesses. and exposure to teratogens.

The list of teratogens is long, and it includes things like medications such as isotretinoin, alcohol, **recreational drugs like cocaine, tobacco smoke, and heavy metals like mercury. **

Question 33

Pathology of cyanotic congenital heart defects

Answer 33

cardiac structure fails to form or close properly

Question 34

Complications of Cyanotic Heart Defect

Answer 34

, the persistence of deoxygenated blood in systemic circulation can progress to chronic hypoxia, to which the body responds by producing more red blood cells, called polycythemia. The heart can also fail to pump enough oxygenated blood to the tissues, resulting in heart failure. At the same time, if the oxygen supply to the brain gets so low, it may result in a cerebrovascular accident, or CVA for short.

Other complications of cyanotic heart defects include arrhythmias, embolism, and infective endocarditis, brain abscess formation, pulmonary vascular disease, and even death.

Question 35

Clinical Manifestation of Cyanotic Heart Defects

Answer 35

mainly include cyanosis, which is typically present at birth or within the first few weeks of life. This is often joined by lethargy, tachycardia, tachypnea, activity intolerance, as well as clubbing of fingers and toes. Some clients may also experience feeding problems, which may lead to poor weight gain, and failure or difficulty to thrive.

Question 36

WHat’s special about ToF ? What are the additional acute signs to look out for?

Answer 36

It is the most common cyanotic heart defect as compared to the others.

Look out for Tet Spells (Acute & Severe cyanotic episodes)

Question 37

Tet Spells

Answer 37

“Tet spells” = acute & severe cyanotic episodes.

Activities like feeding, exercise, or crying, cause spasm of the infundibular septum which worsens the stenosis and increases the obstruction to pulmonary blood flow.

This is called right ventricular outflow tract obstruction and results in increased right to left shunting, eventually decreasing the blood flow through the lungs, causing a fall in arterial oxygen saturation.

The lack of oxygen causes tachypnea or rapid abnormal breathing; and increases the activity of the sympathetic nervous system, eventually increasing heart contractility and even more obstruction of the right ventricular outflow tract. As a result, cyanosis occurs.

Additionally, clients undergoing a tet spell **can be seen squatting **or assuming a fetal position.

This position increases the peripheral vascular resistance by kinking the femoral artery, which improves the pulmonary blood flow and relieves the client.

During auscultation of the heart, large defects can be heard as murmurs, from blood moving through the defect.

Question 38

Diagnosis of Cyanotic Heart Defects

Answer 38

The diagnosis of cyanotic heart defects starts with:

1) Mother’s history during pregnancy
2) Physical assessment & ECG to detect arrhythmias
3) Echocardiography to visualize the defect

4) Most defects can also be detected prenatally by standard obstetric ultrasound examination

5) Other tests include angiography & pulse oximetry.

6) Echocardiograms, chest X-ray and MRI can identify structural abnormalities;

in Tetralogy of Fallot a classic sign found on X-ray is an enlarged, boot-shaped heart.

7) Cardiac catheterization is sometimes performed to assess the extent of the defect.

Question 39

Treatment for A

Answer 39

Treatment of cyanotic heart defects depends on the type of defect, but starts with:

1) Oxygen supplementation.

2) If heart failure has developed, medications are needed, including digoxin and diuretics. Prophylactic antibiotics should also be prescribed to prevent infective endocarditis.

Some clients will also benefit from prostaglandin E1 infusion, which can keep the ductus arteriosus open, allowing blood from the pulmonary artery and aorta to mix, which increases the oxygen level of the blood going to the rest of the body.

Finally, definitive treatment consists of surgical correction of the defect, particularly in early infancy or during the first year of life. Alternatively, a palliative shunt might be performed, which is a surgical procedure in which the aorta is connected to the pulmonary trunk, in order to increase the pulmonary blood flow. In severe cases, heart transplantation might be required.

Question 40

Nursing Care for Patients with Cyanotic Heart Defect

Answer 40

Your priority nursing goal is to assist in:

1) **maintaining adequate oxygenation. **

2) Begin by assessing the infant’s oxygenation by initiating pulse oximetry, and assessing their vital signs, respiratory effort, and skin color.

3) Escalate immediately for R.D., such as tachypnea, nasal flaring, retractions, grunting, decreased oxygen saturation, and cyanosis.

4) Provide supplemental oxygen, which will help dilate the pulmonary vasculature; establish IV access and infuse the ordered fluids to increase right ventricular filling and pulmonary blood flow; and prostaglandin E1 to maintain ductal patency and promote pulmonary blood flow.

**Be sure to take steps to decrease the risk of hypercyanotic episodes, or tet spells, **by maintaining a calm, therapeutic environment to prevent agitation. If a tet spell occurs, place the infant into a knee-chest position, which will increase systemic vascular resistance and promote systemic venous return to the right side of the heart and into the pulmonary circulation.

Continue to monitor them closely, and immediately report if the tet spell persists.

Then, administer the prescribed medications, including morphine, which will provide sedation, as well as beta blockers, to and relax the outflow tract of the right ventricle and promote pulmonary blood flow. If these interventions don’t resolve the cyanosis, administer the prescribed phenylephrine which will improve outflow from the right ventricle to the lungs. When the infant is stable, assist with preparing them for complete repair of the heart defect.

Question 41

**For patients with cyanotic heart defect, what are the Nursing management to decrease the risk of hyper-cyanotic episodes

Answer 41

1) by maintaining a calm, therapeutic environment to prevent agitation.

2) If a tet spell occurs, place the infant into a knee-chest position, which will increase systemic vascular resistance and promote systemic venous return to the R side of the heart & into the pulmonary circulation.

3) Continuous monitoring, escalate if tet spell persists.

4) Prescribed medications, including morphine, which will provide sedation, as well as beta blockers, to and relax the outflow tract of the right ventricle and promote pulmonary blood flow.

5) If these interventions don’t resolve the cyanosis, administer the prescribed phenylephrine which will improve outflow from the right ventricle to the lungs.

When the infant is stable, assist with preparing them for complete repair of the heart defect.

Question 42

Discharge planning:
Education for caregivers with Cyanotic Heart defect

Answer 42

When their baby is ready to be discharged, teach them how to provide care at home after surgical repair. Talk to them about the importance of keeping the incision clean and dry, and to monitor the incision site for signs of infection.

Tell them to seek medical attention right away if they notice signs of an infection, such as a fever, excessive drainage, redness, increased pain, or swelling at the incision site.

Next, remind them that their baby will need **lifelong monitoring and care, **stress the importance of keeping all their baby’s regular appointments with their pediatrician and cardiologist.

As their baby grows, they will need routine tests like **ECGs to monitor the heart rhythm, echocardiograms to monitor the circulation around the heart, as well as cardiac magnetic resonance imaging **to get a close look at the heart chambers, and additional testing to measure right and left ventricular function.

Lastly, let them know that monitoring of their baby’s motor and neurocognitive development is an important part of their baby’s ongoing care. Be sure to connect the family to community resources and support groups for others who are parenting a child with a congenital heart defect.

Finally, recommend that the caregivers** learn how to provide infant CPR i**n case of emergency. Instruct them to seek emergency medical attention right away if their baby become lethargic, cyanotic, feeds poorly, or has trouble breathing.

Question 43

Signs to report for Respiratory Distress

Answer 43

1) Decr. oxygen saturation
2) Tachypnea
3) Retractions
4) Grunting
5) Cyanosis
6) Nasal Flaring

Question 44

A pediatric inpatient nurse observes several children with congenital heart defects as they play. Which child is most likely experiencing a hypercyanotic spell?

1) The child who has flushed cheeks
2) The child who pauses to squat down
3) The child who has mild shortness of breath
4) The child trips & falls.

Answer 44

The child who pauses to squat down.

Pausing to squat is a sign of a hypercyanotic spell, or “Tet” spell.

The squatting position increases peripheral vascular resistance by kinking the femoral artery, which improves pulmonary blood flow and relieves the client.

Question 45

A nurse is caring for a client diagnosed with tetralogy of Fallot (ToF).

When reviewing the results of the client’s echocardiogram, which cardiac abnormalities does the nurse expect to observe related to this diagnosis? Select all that apply.

1) Tricuspid valve regurgitation
2) Ventricular septal defect
3) Pulmonary stenosis
4) Overriding aorta
5) Right ventricular hypertrophy
6) Patent Foramen ovale

Answer 45

2,3,4,5

• Pulmonary stenosis
• Right ventricular hypertrophy
• VSD
• Overriding aorta

Answer 46

Question 47

List the Cyanotic and
Acyanotic Heart Defects

Answer 47

Acyanotic
1) ASD
2) VSD
3) CoA
4) PDA - Patent Ductus Arteriosus

Cyanotic
1) Tof - Tetralogy of Fallot
2) TA - Trecuspid Atresia
3) ToGV - Transposition of Great Vessels
4)P TA - Persistent Truncus Arteriosus
5) TAPVR - Total Anomalous Pulmonary Venous Return

Question 48

Risk factors of Kawasaki Disease

Answer 48

• Male
• Asian
• 6months - 5 years

Question 49

Kawasaki disease is a condition associated with acute inflammation of ……. arteries; and it’s typically seen in children between the ages of……… and ………. years.

Answer 49

Medium arteries,

6months and 5 years

Question 50

Kawasaki disease

Answer 50

• male
• asian
• 6 months to 5 yrs
• medium arteries
• autoimmune infection

Question 51

Kawasaki Disease
CRASH & BURN

Answer 51

** “C” ** Conjunctival hyperemia or eye redness that can be associated with photophobia, meaning increased sensitivity of the eyes to light.

**“R” **- Rash, polymorphous exanthem;

“A” - Adenopathy, more specifically lymphadenopathy of cervical lymph nodes.

“S” - Strawberry tongue, which refers to a red tongue that can be associated with dry and cracking lips.

“H” - Hand & foot changes, which include edema, erythema, as well as desquamation of the skin on tips of fingers and toes;

**BURN **refers to fever.

Other clinical manifestations include malaise, joint pain, diarrhea, thrombocytosis, and cardiac manifestations such as cardiac arrhythmias.

Question 52

Diagnosis for Kawasaki Disease

Answer 52

**Diagnosis is based on:
- medical history
- physical examination

A client must have:
1) a fever that last > 5 days (BURN)
AND
2) at least 4/5 clinical features of Kawasaki disease (CRASH)

Additional diagnostic methods that can help include CBC, CRP, ESR, serum transaminase levels, as well as echocardiography, to assess degree of cardiac involvement.

Question 53

Treatment for Kawasaki Disease

Answer 53

Treatment includes supportive care and minimization of the risk of the** coronary aneurysm**.

Clients should receive IVIG, as well as high-dose aspirin, to prevent thrombosis.

Normally, aspirin should be avoided in children because it can cause Reye syndrome, which is a condition characterized by liver failure and rapidly progressive encephalopathy; however, with Kawasaki disease, aspirin is permitted because its anti-thrombotic effects outweigh the risk of Reye syndrome.

Question 54

Nursing Care for pt with Kawasaki Disease

Answer 54

Promote comfort and healing, & to monitor for complications.

1) Create a calm environment by decreasing external stimulation. Also dim the lights in their room to ease the photophobia and discomfort caused by their conjunctivitis.

2) Apply lip balm to their chapped lips and a mild lubricant to areas of rash and peeling skin.

3) For edematous legs and feet, elevate them slightly, and perform gentle passive ROM exercises on each of the child’s edematous extremities.

4) Lastly, monitor their pain level, administer the prescribed analgesics, and collaborate with the Child Life Specialist for nonpharmacologic pain management strategies like distraction and quiet age-appropriate activities.

5) Promote healing by initiating IV access and infusing the prescribed IVIG. During the infusion, watch for reactions to the medications, and immediately report to the healthcare provider

6) if your client experiences dizziness, flushing, headache, diaphoresis, nausea and vomiting, or upper abdominal pain.

Stop the infusion, administer antihistamines or other prescribed treatments, and restart the infusion at a lower rate once symptoms have resolved.

7) Also, ensure your client receives adequate fluid and nutrition. Promote hydration by offering cool fluids, gelatin, or ice pops, and keep a close eye on their hydration status by monitoring their intake and output, as well as their daily weight. Promote nutrition by providing small, frequent, nourishing meals consisting of soft, bland foods.

Be sure to watch closely for potential cardiac complications. Listen to heart sounds, initiate ECG monitoring as ordered, and institute bed rest to decrease cardiac workload. Immediately report the presence of arrhythmias or abnormal heart sounds to the health care provider.

Finally, keep a close eye on your client’s vital signs, paying particular attention to their temperature. Institute seizure precautions, administer the prescribed PO aspirin, and institute cooling measures, as needed, such as cool compresses or tepid sponge baths. Immediately report to the healthcare provider if fever continues despite cooling measures.

Question 55

Client & Family teaching

Answer 55

Begin by explaining that Kawasaki disease is a condition that causes inflammation of the walls of the blood vessels, rash, and sore mucus membranes like the mouth, lips, and tongue.

Review the treatment plan, and each of their child’s prescribed medications; and remind them to delay live vaccinations such as measles and varicella until at least 11 months after the final dose of IVIG. Lastly, stress the importance of keeping all scheduled follow-up appointments, including ECGs and echocardiograms, for continued monitoring and care.

Next, teach them how to monitor their child’s temperature and recommend that they keep a log of each temperature reading. Instruct them to give acetaminophen for fever, and remind them not to use NSAIDs like ibuprofen while their child is on aspirin therapy. Advise them to notify their healthcare provider immediately for a fever of 100.4˚F or 38˚C or above.

Also, provide them with teaching related to their child’s activities. Talk to them about the importance of keeping their child well hydrated and to offer small frequent meals and snacks. Advise them to provide their child with plenty of opportunities for rest, and to encourage quiet activities like coloring, reading, or playing with puzzles. Stress the importance of keeping their child from engaging in any activity that could cause injury while their child is on aspirin therapy, due to the increased risk of bleeding. Instruct them to notify the healthcare provider immediately if they notice** unusual bleeding **such as bruising, nose bleeds, or blood in the urine, stool or vomit.

Finally teach them to recognize S/S of cardiac problems, and to immediately seek medical care if their child is more tired than usual; if they are not eating and seem to have a decreased appetite; or if their child is having trouble breathing or is experiencing chest pain.