Class 10: CHD and Kawasaki disease

Major cuase of death in the ifrst year of life

Congenital heart disease

More severe, needs intervention eaarly in life

Critical CHD (CCHD)

Classifiications of CHD

Atrial septal defect

Ventricular septal defect 

Patent ductus areriosus 

Coarctationn of aorta 

Tetralogy of fallot 

Transposition of the great arteries 

Heart blood flow

Wants to flow fro high pressure to low pressure

Blood flow woth cardiac abnormality

Left to right shunt

Blood flow with conditions that can cause cyanosis

Right to left shunt

Because of increased vascular resitance or obstruction to blood flow through pulmonic valve and/or pulmonary artery

When should New born Pulse oximetry screening be done 

Within first 24 hours of life 

Newborn pulse ox screening

1 probe goes on right hand (pre ductal)

1 probe on either foot (post ductal)

Pulse ox abnormal result meaning

5.5 times more likely to have a CHD

Pulse ox results

>95%: normal

90-94%: borderline value, repeat and consult provider if still <95%

first sign of a CHD

Hypoxemia

Acynotic CHD: increased pulmonary blood flow

Atrial septal defect

Ventricular septal defect

Patent ductus arteriosus

Acyanotic CHD: )bstruction of blood flow from ventricles

Coarctation of artoa 

Cyanotic CHD: decreased pulmonary blood flow

Tetralogy of fallot

Transposition of the great arteries

Cyanotic CHD: Mixed blood flow

Trasnposition of great arteries

Total anomalous pulmonary venous return 

Truncus arteriosus 

Hypoplastic left heart syndrome 

Atrial septial defect (ASD)

Abnormal opening between left and right atria

Blood flows from higher pressure left atrium to lower pressure right atrium

Get increased oxygeneration on right side of the heart

Characteristuics  of ASD

Right atrium tolerates extra blood volume well, may be asymptomatic until heart failure develops

Characteristic systolic murmur with a fixed split second sound

May also have audible diastolic murmur 

What are patients with ASD also at risk for

Atrial dysryhthmias

Pulmonary vascular obstructive disease

Emboli formation

Ventricular septal defect

Abnromal opening between left and right ventricle

Blood flows from high pressure eft ventricle to low pressure right ventric;e 

Increased oxygenation on right side of heart 

20-60% close spontaneously

VSD size

Can vary from pinhole to absence of ventricular septum

What is VSD often associated with

Other congential heart defects such as:

Pulmonary stenosis 

Transposition of great arteries 

Patent ductus ateriosus 

Atrial defects 

Coarctation of aorta 

VSD and the pulonary artery

Increased blood flow enters the pulmonary artery and then the lungs

This can cause increased pulmonary vascular resistance, increasd pressure in the right ventricle and right ventricular hypertrophy

Right atrium can also be enlarged if right ventricle cant handle it/if compensating 

Characteristics of VSD

Heart failure is common

loud holosystolic murmur is best heard at left sternal border

What does VSD increase your risk of getting

Bacterial endocarditis and pulomnary vascular obstructive disease

Surgical procedures fro VSD

Band around pulmonary artery t decrease pulmonary blood flow, can be perofmred in infants with multiple vascular VSDs or complex anatomy

Sutures for small defects 

Knitted dacron patch for large defects 

Cardiopulomary bypass

Patent ductus ateriosus

Faikure of fetal ductus arteriosis (connects aorta with pulmonary artery) to closew wothin first few weeks of life

COntinued patecny of DA allows blood to flow from higher pressure aorta to pulmonary artery '

Left to right shunt 

PDA additional blood flow

recirculated through lungs and returned to ;eft atrium and ventricle

causes increased workload on l;eft side of heart, increased pulmonary cingestion and potentially increased risk of right ventricular hypertrophy 

PDA symptoms

May be asymptomatic or show signs of heart failure

Characteritic machine like murmur

Boudning pulses and widene pressure 

What might happen if an infant has a small PDA and is asymptomatic 

May be deischarged home witha referal to pediatric cardiology as outpatient 

FOllow up for PDA closure 

PDA closure med

Indomethacin IV

Indomethacin IV consideration

Significant side effects

Dont use umbilical line as it can akter cerebral blood flow (risk of stroke, MI, necrotizing enterocolitis and decreased urine output)

If NEC occurs: emergency abdokinal surgery to remove necrotic bowel tissue 

Complications from PDA surgery

Typically low risk, but can get

Injury to pharyngeal nerve

Paralysis pof left hemi diaphrpagm 

Injury ton thoracic duct

Coarctation of the aorta

Localozed narrowing near insertion of ductus arteriosus

Increased pressure proximal to defect (head and upper extremities) and decreased pressure distal to obstruction (body and lower extremities)

Characteristics of coarction of aorta

High BP and bounding pulse inarms

coarction of aorta in infants

Always check femoral pulses

Signs of heart failure: can deteriorate rapidly with severe acidosis and hypotension

Mechanical ventilation and isotropes are often needed before surgery (may be in ICU)

coarction of aorta in older children

May experience dizziness, headaches, and fainting from hypotension

What are [atiensts with coarction of aorta also at risk for

Hypertesnion

Ruptures aorta

aortic aneurysm

stroke

First choice of treatment in infants (<6 months) and patients with long segment stenosis or complex anatomy with coarction of aorta

Surgical repair

can be oerformed on alll patients

Why is cardiopulmonary bypass not needed for coarction of aorta

becayse defect is outside of the heart and pericardium

Thoractomy incision sed instead 

coarction of aorta surgical repair: post op treatment

IV sodium nitroprusside, esmolol or milrinone followed by 

Oral meds (ACE inhibitors or beta blockers)

When is lective surgery advised for coarction of aorta

Within first 2 years of life

Prevents hypertension at rest and exercise proboked systenic hypertension

Recurrence rate for individuals with a previous COA repair 

15-30%

Treatment of choice for children over 2 with COA

Balloon angioplasty

Baloon angioplasty risks

Femoral injury in infants

mortality in less than 5 % of patients (increases with other cardiac conditiosn)

Defects with decreased pulmonary blood flow traits

Onbstruction ot pulmonary blood flow and defect ASD or VSD between right and left sideof heart 

Blood has difciculty exiting right side of heart via pulmonary artery, so pressure on right side increase and is greater then left sided pressure 

Desat blood shunts from right side to left side (desat left side and systemic circulation)

Patients are typically hypoxemic and cyanotic (Pay attention to colour)

Tetralogy of fallot occurance 

occurs in 5-10% of all CHD

Most common cyanotic lesion

ToF defects

Ventricular septal defect

Pulmonic stenosis 

overriding aorta 

Right ventricular aorta

Right ventricular hypertrophy

ToF blood shunt direction

Depends on difference between pulmonary and syetmic vascular reistance

Pukonary reistance>systemic reistance: shunit is right to left

Systemic>pulmonary reistance: Shunt is left to right

ToF and pulmonary blood flow

Decreases blood flow to lungs, so therefore decreases amount of oxygenated blood returned to left atrium

ToF in infants

Some infants may be acutely cyanotic at birth, others have mild cyanosis over first year if life as pulmonary stenosis worsens

Characteristic systolic murmur 

Aciute epidoses of cyanosis and hypozia (blue/Tet spells)

Anoxic spells: infants oxygen requirements exceed blood supply (crying/feeding)

How to relieve Tet spells

Position childs knees to chest to reduce venous return from legs and increase syetmic vascular resistance, diverting more blood to pulomary artery (parents may report squatting)

Adminster 100% blow by oxygen 

SQ or IV morphine (repeat as needed)

IV fluid replacement

Why do children with Tet and cyanosis need to be well hydrated

to keep Hct and blood viscosity within acceptable limits and avoid CVA (stroke, seizures, death, emboli)

ToF surgucal repair post Tet spell

Shunt may be placed in infants who are unabke to undergo a complete repair

Elective repair performed in first year of life 

Mixed defects 

Called that because survival in post natal period depends on mixing of blood from pulmonary and susyemic circulation within heart chambers 

Transposition of great arteries

2 seperate pathways with no intersection between pulmonary and systemic circulation

TGA associated defecits

Septal defects or paten ductus ateriiosus must be present to permit blood to neter the systemic circulation or pulomnary circulation for mixing of sat and desat blood 

VSD may be present 

most common defecit associated with TGA

patent foramen ovale

at birth:nalos patenty ductus ateriosus (usually closes after newborn period)

Presentaiton of newborns who have mixing of dest and sat blood at birth

Severely cyanotic and depressed function

Cardiomegaly usually eveident within a few weeks of birth

Babies with TGA and a large ductus arteriosus or large septal defects

Jay be less cyanotic but still exhibit symptoms of heart failure

TGA med

IV prostaglandin E1

Progag;andin E1

Med for TGA, given IV

Maintaines patent ductus arteriosus

Permits o2 sat of 75% or more to maintain cardiac output

What is the aim of giving prostaglandin E1 forTGA

Maintain patent ductus ateriosus untik baby can recive emegercny surgery

Prostaglandin E1 important side effect

Apnea

particularly in babies with a birth weight <2kg

Prostglandin E1 half life

Very short, so adminsiter via continous IV infusion

Important note for prostaglandin E1 dosing

Know birth weight and daily weight

Potential long term complications of TGA

Suprapulmonic stenosis and regurgitation

Can also get coronary artery obstruction

Heart failure in children 

Normally secondary to structural abnormaltity 

Tachycardia,decreased urine output, sweating, decompensating during feeding

apnic, cyanotic, weight loss, may need NG for feeds

clinical consequences of CHD

Heart failure eart muscle beciomes damaged if untreated

Diagnosis of heart failure

Based on clincial symptoms

Therapietic management goals of heart failure

KImprove cardiac funciton

Remove accumuated fluid and sodium 

Decrease cardiac demands 

Imrpove tissue oxygenation and decrease oxygen consumption 

heart failure nursing care

Assist in measures to improve cardiac funciton

Neutral thermal environment toi prevent cold stress

Semi fowlers to decrease work of breathing

Reduce environmental stimuli

ANticpate needs to limit crying 

oxygen 

digoxin 

Digoxin in pediatrics 

Provider miust specify heart range that necessitates holding the dose 

Propr to admin: feel apical pulse for 1 miniute 

Monitor for dognoxin toxicity 

Signs of digoxin toxicity 

Nausea 

vomiting 

Poor weight gain/anorexia

Bradycardia 

disyrhythmia 

Heart failure and respiratory distress

Report resp distress asaps as it can indiacate heart failure

Assessment: colpour

Carefully note cokour as the slihghtes hint of pallor can be the only indication of heart failure and at risk of imminent death

Note about heart failure presentation in children

Vitasl and Spo2 may be stable and show no outward signs of respmdistress

Need to know baseline

Nursing care for heart failure

Maintain nutritional status

Assist measures to promote fluid loss (Lassie, monitor lyte levels)

Support

Signs and symptoms of hypozemia

Polycythemia (increased number of RBCs can lead to iron not being as readily availableto form gab and increased blood viscoisity)

Clubbing of nails 

Pallor 

Hypercyanotic spels 

Children in ICU with heart failure

Some may have arterial lines to monitor arterial O2 sat

Do not ever connect IV fluids ot meds to arterial line!!!

kawasaki disease 

Most common acquired heart disease in children in developed countries 

Systematic vasculitis of unknown cause lasting 6-8 weeks (may follow infxn)

KD cases

Majority are children <5

Peak incidence in toddlers and those born wigh male genitalia 

When are KD outbreaks more common

Late winter and early sprin, fall

What happens if KD isnt treated

20-25% of children will develop coronary artery dilation or aneurysm formation

KD in children <1 year and >5

Have greatest risk of heart involvement

KD: acute phase

May require cardiac CT or MRI to better estimate extent of coronary damage

1/3 of patients get temporary arthritis beginning in small joints

Abrupt onset of persistent, high fever thats unresponsive to antipyretics and ABX

Edema and erythema of palms and soles

Bilateral conjuctival inflammation/infxn without exudate

Erythema in oral membranes (red, cracked lips, orpharyngeal redness, srawberry tongue)

Desquamta egroin

Rash in perineal area that spreads to trunk, extremities and face

Cervical lymphadenopathy present in at least 1 lymph node (palpate lymph nodes)

Irritability and inconsolable

KD echocardiogram

Necessary to assess coronary artery dilation and monitor cardiac function

Done at dx then repeat at 4-6 weeks

If inital echo idetifies coronary artery dilation or aneursym fornation,

KD phases

Acute

Subacute

Convalescent 

KD: subacute phase

Continues untik all synptoms resolve

May see coronary aneurysm during illness

Temporary arthritis during [hase and can affect larger weight bearing joints

Fever resolves

desquamnation of hands and feet (peeling) observed

KD: Convalescent phase 

Symptoms resolve, but lab values dont return to normal for 6-8 weks 

End of phase: bakc to baseline appetite, temperament and energy level

KD treatment

High dose intervals of IVIG and salicylate therapy have been effective

Aspirin

Clopidogrel (plavix), lovenox or warfarin

KD: aspirin

(80-100mg/kg/day) Q6 to control fever and inflammaiton

(fever resides: go down to 3-5 mg/kg/day)

take for 6-8 weeks untul platelet counts returj to normal if no coronary abnornality 

If coronary abnormality: take indefintely 

KD: IVIG

Reduces incidence of cronary artery abnormalities whne given within ifrst 10 days of treatment (ideally within 7 days)

KD treatment: Clopidogrel (placid), lovenox, warfarin

Indicated in children with medium to large coronary artery aneurysms

KD prognosis

Excellent prgnosis for thpse without coronary artery damage

KD nursing care

Continous cardiac monitering

Daily weight on same scale at 8 am

Strict intake and output (IV fluids may be required iof dehydrated or unable to eat, reduced rate if mypcarditis is present)

monitor for sogns of heart failure (tachycardia, decreased urinary output, respiratory distress)

Cool cloths, no scents, soft, loose clothing

Ckear liquids, soft and non spicy foods

Ointment for lips, soft toothbrushed and toothettes

Defer live immunizations for 11 months after IVG dose

Nursing care during IVIG adminsiatraion

Blood product so:

Baseline vitals

Frequent vital signs

follow policy