acebook, T witter, instagram, etc.)?

What do you do with your friends outside of school?

Past yr drug use (%) in Ontario

adolescents (2019): alcohol (41.7%).

cannabis (22.0%). tobacco cigarettes

(5.0%). electronic cigarettes(22.7%)

Drugs: Which seems to be more popular at your school, alcohol or drugs? How often do you drink/

smoke cannabis or cigarettes/take other drugs? Have you ever passed out or not been able to remember

what happened? Has anything bad ever happened to you while you were drunk orstoned?

• can organize as a CRAFFT screen Ask Part A, questions 1-3. if yes to any, then ask 6CRAFFT

questions. If no, then move on to Part B, question i

Part A:during the last 12 mo, did you:

1. Drink any alcohol?

2. Smoke any cannabis, vape,or inhale any other substance?

3. Use anything else to get high?

CRAFFT Screen

The CRAFFT is a well-validated

substance use screening tool lot

adolescents12-21 yr.See the CRAFFT

website:crafft.org

Consent and Close Age Exceptions

• The age of consent is16

• A youth 16 or 17 y/o cannot consent

if:the partner isin a position of

trust/authority (e.g.coach, teacher),

young person is dependent on the

partner (e.g.for care or support),

the relationship is exploitative (e.g.

prostitution or pornography)

• A 14 or15y/o can consent aslong as

the partner isless than 5yr older and

aslong asthere is no relationship

of trust authority, dependency, or

exploitation

• A12 or13 y/o can consent aslong as

the partner islessthan 2 yr older and

aslong asthere is no relationship

of trust authority, dependency, or

exploitation

• Part B:

1. Have you ever ridden in a car driven by someone (including yourself) who was high or

had been using drugs/alcohol?

2. Do you ever use drugs/alcohol to relax,feel better about yourself, or fit in?

3. Do you ever use drugs/alcohol when you are alone?

4. Do you ever forget things you did while using drugs/alcohol?

5. Do your family/ffiends ever tell you that you should cut down on your drinking or drug use?

6. Have you ever gotten into trouble while you were using drugs/alcohol?

• see Psychiatry, Substance-Related and Addictive Disorders, PS26

Sexuality: What are your preferred pronouns? Do you have a crush on anyone? Do you have a partner?

What does‘sex’mean to you? Have you ever had sex? Whether the answer is yes or no, the next question

is:What activities would you include in the term ‘having sex'

? What do you do to prevent getting a STI/

getting pregnant/getting someone pregnant? Has anyone ever given you money, drugs, or otherstuff in

exchange for sex? (seeGvnaecologv, Sexually Transmitted Infections,GY28)

Suicidality/Depression: How would you describe your mood most days? On a scale of 1 to 10, where

1 isso sad that you might kill yourself and 10 is the happiest you could be, where are you most days?

Have you lost interest in activities that you used to enjoy? Do you often have trouble sleeping (is there a

difference between school days and the weekend?)? Have you ever thoughtseriously aboutsuicide? Did

you make apian? (seePsychiatry. Depression/Suicide,PS12, PS5)

Safety/Violence: Do you ever get into a car with a driver who has been drinking or taking drugs? Do

you always wear a seatbelt/bicycle helmet? Are you being bullied atschool? Has anyone ever touched you

in an unwanted way?

See Disorders of Sexual Development, P35

Half of police-reported sexual offences

are against children and youth

n

i j

4.9% of Canadian youth (ages12-17)

have a mood disorder

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P18 Paediatrics Toronto Notes 2023

Table 14. Developmental Stages of Adolescence

Early Adolescence (10-13 yr) Middle Adolescence (14-16 Late Adolescence (17-19 y»J

yr)

Cognitive and Moral Concrete

Unable toperceive long-term

outcome olcurrent decisionmaking

Future oriented with sense ol

perspective

Idealism

Ability to think things through

independently

More stable body image

Attractiveness may still be ol

concern

Firmer identity

Emotional and physical separation

from family

Struggle for acceptance of greater Increased autonomy

autonomy

Emergence of abstract thought

Questioning more

SeIf-Concept/Identity Formation Preoccupied with changing body

Self-consciousness about

appearance and attractiveness

Concern withattractiveness

“Stereotypical adolescent'

Family Increasedneed lor privacy Conflicts over control and

nieoersence

Peers Seeks same-sex peer affiliation to Intense peer group involvement

counterinstability

Peer group and values recede ia

importance

Mmacy/possible commitment

takes precedence

Consolidation of sexual identity

Focus on intimacy andformation

of stable relationships

Sexual Preoccupationwith peers Testing ability toattractpartner

Initiation of relationships and

sexual activity

Questions of seiual orientation

Child Abuse and Neglect

Definition

• an act of commission (physical,sexual, or psychological abuse) or omission (neglect) by a caregiver

that results in harm to a child or potential for harm

Legal Duty to Report

• upon reasonable grounds to suspect abuse and/or neglect, physicians are required by legislation to

contact the CAS to personally disclose all information relevant to the child’

ssafety concern

• duty to report overrides patient confidentiality; physician is protected against liability

Ongoing Duty to Report

• if there are additional reasonable groundsto suspect abuse and/or neglect, a further report to theCAS

must be made

Risk Factors

• environmental factors:social isolation, poverty, domestic violence

• caregiver factors: personal history of abuse, psychiatric illness, postpartum depression,substance

misuse,single parent family, poor social and vocationalskills,below average intelligence

• child factors:difficult temperament,disability,special needs(e.g.developmental delay),premature

Management of Physical Abuse, Child Abuse, and Neglect

• do not take an abuse history from a young child; this must be done by trained personnel (e.g.during a

forensic interview)

• report all suspicions to CAS;request emergency visit if imminent risk to child or any siblingsin the

home

• acute medical care:hospitalize for medical evaluation or treatment of injuries if indicated

. arrange consultation from social work and appropriate follow-up

Physical Abuse

History

. history that is not compatible with physical findings or with child'

s developmental capabilities

• history not reproducible or changes dramatically over time

• delay in seeking medical attention that is unexplained by other factors

• assess previous trauma or hospitalizations

• ask f

'

Hx: bleeding disorder, bone disorder, metabolic conditions

• ask developmental history

Physical Exam

• physical findings not explained by underlying medical condition

• growth parameters including past recorded parameters (weight, height, head circumference)

• multiple injuries not explained by accidental injury or child’

s development level

• patterned skin injuries:linear,shapes, etc. that do not match provided history +

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P19 Paediatrics Toronto Notes 2023

•injury location:

bruises:on areas with abundant soft-tissue cushioning,such as abdomen, buttocks, genitalia,

fleshy part of cheek or on ears, neck or feet, bruises that do not fit described cause

fractures: posterior rib/metaphyseal/scapular/vertebral/sternal fractures

• immersion burns (e.g. hot water)

•altered mental status: head injury, poisoning

•eyes -retinal hemorrhages

•scalp - patchy hair loss from traumatic alopecia or severe malnutrition

•oral exam - check the frenula for tears

•head trauma is the leading cause of death in child maltreatment (e.g, acceleration-deceleration forces

(shaking), direct force application (blow or impact))

•consider " red herrings" (e.g.slate grey macule/congenital dermal melanocytosls vs, bruises)

Investigations

•document all injuries on a body diagram: type, location,size,shape, colour, pattern

photography of skin injuries is ideal (police or hospital photography preferred; do not use

physicians personal camera)

•rule out medical causes of bruising/fracture with appropriate investigations (e.g. blood disorders or

rickets):

• if fractures evident:CV\Mg-

,

’

, PO

^

,ALP, PTH, Vitamin D, albumin

• if bruising present:CBC, INR, PIT,von Willebrand factor,factors Vlll/IX

•screen for abdominal trauma

• transaminases and amylase if elevated: abdominal CT recommended

renal function - electrolytes, urinalysis

toxicology screen - overdose or poisoning

•skeletalsurvey in children <2 yr;select imaging based on history in children >5 yr

• neuroimaging:CT and/or MR1 - dilated eye examination by paediatric ophthalmologist to rule

out retinal hemorrhage ifsubdural hemorrhage detected on head imaging

Sexual Abuse

Epidemiology

• peak at 2-6 yr and 12-16 yr,most do not report until adulthood

• as adults: more likely to develop obesity,sexual problems,IBS, fibromyalgia,STI,substance use

disorder

• more likely to experience intimate partner violence and sexual assault

in decreasing order:family member, non-relative known to victim,stranger

History

• psychosocial:specific or generalized fears, depression, nightmares,social withdrawal, lack of trust,

low self-esteem,school failure,sexually aggressive behaviour, advanced sexual knowledge,sexual

preoccupation or play

Physical Exam

• recurrent UTls, pregnancy, STIs, vaginitis, vaginal bleeding, pain, genital injury, enuresis

anogenital exam performed along with head-to-toe physical for physical trauma

• instrumentation not required for anogenital exam,speculum contraindicated in prepubertal girls

most victims have normal anogenital exam - cannot rule out sexual abuse if exam is negative

Investigations

• depend on presentation, age,sex, and pubertal development of child

sexual assault examination kit within 24 h if prepubertal, within 72 h if pubertal

• rule out STI, UTI, pregnancy (consider STI prophylaxis or emergency contraception)

rule out other injuries (vaginal/anal/oral penetration, fractures, head trauma)

rule out drug and alcohol screen (e.g. Rohypnol, "Liquid G,‘etc.)

Medical Assessment of Bruising in

Suspected Child Maltreatment Cases

feediatr Child Health 2013;18(8):433-437

CPS Position Statement:While bruises

are most often due to minor accidental

injury,they may also signal underlying

medical illness or inflicted injury.

Knowing when to assess bruisesin

the context of maltreatment can be

challenging.The following are red flags

for inflicted injury in such bruising cases:

• Babies not yet cruising

• Present on ears. neck,feet, buttocks,

or torso

• Not on the front of the body and/or

overlying bone

• Unusually large or numerous

• Clustered or patterned

• Not fitting with the described causal

mechanism

Neglect

Definition

• omissions in care by parents or caregiver that leads to actual or potential harm

History

• from child and each caregiver separately (if possible)

Physical Exam

• head to toe (do not force),growth parameters, nutrition status

• dental care

• emotionalstate

Investigations

• blood tests to rule out medical conditions or nutritional deficiencies (e.g.thrombocytopenia or

coagulopathy)

Presentation of Neglect

• FIT.developmental delay

• Inadequate or dirty clothing, poor

hygiene

• Child exhibits poor attachment to

parents, no stranger anxiety

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P20 Paediatrics Toronto Notes 2023

Cardiology

Congenital Heart Disease

PRENATAL CIRCULATION

oxygenalod blood dooxygonatod blood

Iran placenta returns via SVC to R atrium -Aorta

Ductus Arteriosus

vf /j

Superior Vena Cava I i

1/3 of blood ordering

R atrium does not (low

through foramon ovala and

ductus venosus Hows to tho R ventricle

umbilical vein

I

Foramen Ovale i I

Pulmonary Trunk

IVC pulmonary atterios

I I Inferior Vena Cav&

R atrium ductus arteriosus

—Descending Aorta

Fetal circulation is designed so that

oxygenated blood is preferentially

delivered to the brain and myocardium

shunted through

foramen ovale

aorta

Liver

i systemic circulation Ductus Venosus

L atrium I

I placenta for reoxygenation

L ventricle

I

aorta

I

brairVmyocardiunV

upper extremities Placenta Umbilical Arteries

© Bonnie Tang 2012

Figure 1. Prenatal circulation

Before Birth

• shunting deoxygenated blood

ductus arteriosus:connection between pulmonary artery and aorta

• shunting oxygenated blood

• foramen ovale:connection between right and left atria

• ductus venosus: connection between umbilical vein and inferior vena cava

At Birth

• with first breath, lungs open up > pulmonary resistance decreases > pulmonic blood flow increases

• separation of low resistance placenta > systemic circulation becomes a high resistance system >

ductus venosus closure

• increased pulmonic flow > increased left atrial pressures > foramen ovale closure

• increased oxygen concentration in blood after first breath > decreased prostaglandins > ductus

arteriosus closure

• closure of fetal shunts and changes in vascular resistance > infant circulation assumes normal adult

flow

Epidemiology

• 8 in 1000 live births haveCHI), which may present as a heart murmur, heart failure, or cyanosis; VSD

is the most common lesion

Investigations

. echo, ECG, CXR

• pre- and postductal oxygen saturations, 4 limb BPs, hyperoxia test

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P21 Paediatrics Toronto Notes 2023

CYANOTIC VS. ACYANOTIC CONGENITAL HEART DISEASE

• cyanosis:blue mucous membranes, nail beds,and skin secondary to an absolute concentration of

deoxygenated hemoglobin of at least 30 g/dL

• acyanotic heart disease (i.e. L to R shunt,obstruction occurring beyond lungs): blood passes through

pulmonic circulation -> oxygenation takes place -» low levels of deoxygenated blood in systemic

circulation -> no cyanosis

• cyanotic heart disease (i.e. R to L shunt):blood bypasses the lungs-> no oxygenation occurs -» high

levels of deoxygenated hemoglobin enters the systemic circulation -» cyanosis

Congenital Heart Disease

I

i Characteristic CXR Findings in CHD

• Boot-shaped heart:tetralogy of

Fallot, tricuspid atresia

• Egg-shaped heart transposition of

great arteries

• “Snowman” heart total anomalous

pulmonary venous return

Acyanotic Cyanotic (5 “T" lesions)

I I

I 1 1

L -» R shunt Obstructive R -» L shunt

T 1 I I

ASD Coarctation of the aorta Tetralogy of Fallot Transposition of the great arteries

VSO Aortic stenosis Ebstein'

s anomaly

Pulmonic stenosis

Truncus arteriosus

PDA

Atrioventricular

septal defect

(endocardial

cushion defect)

Total anomalous

pulmonary venous return

Tricuspid atresia

Hypoplastic left

heartsyndrome

Figure 2. Common congenital heart diseases

Acyanotic Congenital Heart Disease

1. LEFT-TO-RIGHT SHUNT LESIONS

• extra blood is displaced through a communication from the left to the right side of the heart ->

increased pulmonary blood flow -> increased pulmonary pressures

• shunt volume is dependent upon three factors: (1) size of defect, (2) pressure gradient between

chambers or vessels, and (3) peripheral outflow resistance

• untreated shunts can result in pulmonary vascular disease, left ventricular dilatation and dysfunction,

right ventricular HTN and RVH, and ultimately R to L shunts

Atrial Septal Defect

• 3types:ostium primtim (common in DS, defect located at mitral or tricuspid valves), ostium secundum

(most common type,50-70%,defect located atseptum between left and right atria),sinus venosus

(defect located at entry ofsuperior vena cava into right atrium)

• epidemiology: 6-8% of congenital heart lesions, common in patients with certain congenital

disorders (e.g. DS, FAS)

• natural history

80-100% spontaneous closure rate if ASD diameter <8 mm

if remains patent,CHI'

and pulmonary HTN can develop in adult life

• clinical features

history:often asymptomatic in childhood

• physical exam:grade 2-3/6 pulmonic outflow murmur, widely split, and fixed S2

children with large ASDs may have signs of heart failure (tachypnea, FIT, hepatomegaly,

pulmonary rales/retractions)

• investigations

• ECG:RAD, mild RVH, RBBB (normal ECG does not rule out)

• CXR:increased pulmonary vasculature, cardiac enlargement (normal ECG does not rule out)

• echo:diagnostic

• management

elective surgical or catheter closure between 2-5 yr, though majority require no surgery

size <8 mm will likely spontaneously close

Ventricular Septal Defect

• most common congenital heart defect (30-50%)

• small VSD (majority)

clinical features

history: asymptomatic, normal growth, and development

physical exam:early systolic to holosystolic murmur, best heard at LLSB, thrill

• investigations: echo to confirm diagnosis (ECG and CXR are normal)

• management: most dose spontaneously

• moderate-to-large VSD

epidemiology:CHI'

by 2 mo;late secondary pulmonary HTN if left untreated

Moderate-to-large VSO

Size of VSD isinversely related to sound

of murmur (loud murmur - smaller hole)

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P22 Paediatrics Toronto Notes 2023

• clinical features

history: delayed growth, decreased exercise tolerance, recurrent U RT'

Is or “asthma"

episodes

physical exam:holosystolic murmur at LLSB, mid-diastolic rumble at apex,size of VSD is

inversely related to intensity of murmur, loss ofsplitting ofsecond heart sound and a loud P2

suggests pulmonary hypertension

investigations

ECG:LVH,LAH, RVH (normal ECG does not rule out)

CXR:increased pulmonary vasculature, cardiomegaly,CHI-

'

(normal CXR does not rule out)

echo:diagnostic

management: treatment of CHI-

'

and surgical closure by 1 yr, if surgery required

Patent Ductus Arteriosus

•patent vessel between descending aorta and left pulmonary artery ( normally, functional closure

within first 15 h of life, anatomical closure within first days oflife)

•epidemiology

• 5-10% of all congenital heart defects

delayed closure of ductusis common in premature infants (1/3 of infants <1750 g);thisis

different from PDA in term infants

•natural history:PDA is usually associated with immaturity in premature infants, but is typically

associated with a functional defect in term infants.Assuch,spontaneous closure isless common in

term infants

•clinicalfeatures

history: asymptomatic, or have apneic or bradycardic spells, poor feeding, accessory muscle use,

CHI-

'

• physical exam: tachycardia ± gallop rhythm, bounding pulses, hyperactive precordium, wide

pulse pressure, continuous “machinery" murmur best heard at left infradavicular area

•investigations

• ECG: may show left atrial enlargement, LVH, RVH

• echo is diagnostic

CXR: may show normal to mildly enlarged heart,increased pulmonary vasculature, prominent

pulmonary artery

•management

• indomethacin (Indocid*):antagonizes prostaglandin E2,which maintains ductus arteriosus

patency;only effective in premature infants

catheter orsurgical closure if PDA causes respirator)'compromise, FIT,or persists beyond 3rd

mo oflife

2. OBSTRUCTIVE LESIONS

•present with decreased urine output, pallor, cool extremities and poor pulses,shock, or sudden

collapse

Coarctation of the Aorta

•definition: narrowing of aorta (almost always at the level of the ductus arteriosus)

•epidemiology: commonly associated with bicuspid aortic valve (50%);Turner syndrome (35%)

•clinical features

history:often asymptomatic

physical exam

blood pressure discrepancy between upper and lower extremities (increased suspicion/

severity if >20 mmHg difference)

diminished or delayed femoral pulses relative to brachial pulses (i.e. brachial-femoral delay)

possible systolic murmur with late peak at apex,left axilla, and left back

if severe, presents with shock in the neonatal period when the ductus arteriosus closes

•investigations: EGG shows RVH early in infancy, LVH later in childhood; echo or MRI for diagnosis

•prognosis: can be complicated by HTN; if associated with other lesions (e.g. PDA, VSD) can lead to

CHE

•management:give prostaglandins to keep ductus arteriosus patent for stabilization and perform

surgical correction in neonates;for older infants and children balloon arterioplasty may be an

alternative to surgical correction

Aortic Stenosis

•4 types:valvular (75%),subvalvular (20%),supravalvular, and idiopathic hypertrophic subaortic

stenosis(5%)

•clinical features

history:often asymptomatic, but may be associated with CHE, exertional chest pain,syncope, or

sudden death

physical exam:SEM at RUSB with aortic ejection click at the apex (only for valvular stenosis)

•investigations: echo for diagnosis

•management: valvular stenosis is usually treated with balloon valvuloplasty, patients with

subvalvular or supravalvular stenosis require surgical repair, exercise restriction required

Figure 3. Patent ductus arteriosus

Ijl

Figure 4. Coarctation of the aorta

r i

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P23 Paediatrics Toronto Notes 2023

Pulmonary Stenosis

• 3 types:valvular (90%),subvalvular, orsupravalvular

• definition of critical pulmonary stenosis: inadequate pulmonary blood flow, dependent on ductus

arteriosus for oxygenation, progressive hypoxia and cyanosis

• natural history: may be part of other congenital heart lesions(e.g.Tetralogy of E

'

allot) or in

association with syndromes(e.g. congenital rubella, Noonan syndrome)

• clinical features

history:spectrum from asymptomatic to CHT

physical exam: wide split S2 on expiration, SEM at LUSB, pulmonary ejection dick (for valvular

lesions)

• investigations

- ECG findings: RVH

CXR:post-stenotic dilation of the main pulmonary artery (due to high velocity jet past stenotic

valve)

echo:diagnostic

• management:surgical repair if critically ill or if symptomatic in older infants/children

Cyanotic Congenital Heart Disease (§)

Causes of Cyanotic Heart Disease- 5Ts

•systemic venous return re-enterssystemic circulation directly

•most prominent feature is cyanosis (0 2 saturation <75%)

•hyperoxic test differentiates between cardiac and other causes of cyanosis

obtain preductal, right radial ABG in room air, then repeat after the child inspires 100% O 2

if PaOa improves to >150 mmHg, cyanosisless likely cardiac in origin

•pre-ductal and post-ductal pulse oximetry

• >5% difference suggests R to L shunt

Truncus arteriosus

Transposition of the great vessels

Tricuspid atresia

Tetralogy of Fallot

Total anomalous pulmonary venous

return

1. RIGHT-TO-LEFT SHUNT LESIONS

Tetralogy of Fallot

•epidemiology:10% of all CHD,most common cyanotic heart defect diagnosed beyond infancy with

peak incidence at 2-4 mo

•pathophysiology

• embryological defect due to anterior and superior deviation of the outlet septum leading to:VSD,

RVOTO (i.e. pulmonary stenosis ± subpulmonary valve stenosis), overriding aorta, and RVH

* infants may initially have a L -> R shunt (therefore no cyanosis); however, RVOTO is

progressive, leading to increasing R -> L shunting with hypoxemia and cyanosis

degree of RVOTO determines the direction and degree of shunt and, therefore,the extent of

clinical cyanosis and degree of RVH

Tetralogy of Fallot

1. VSD

2.RVOTO

3.Aortic root “overriding" VSD

4. RVH

•clinical features

history: hypoxic “tet” spells

during exertional states (crying, exercise) the increasing pulmonary vascular resistance and

decrease in systemic resistance causes an increase in right-to-left shunting

clinical features include paroxysms of rapid and deep breathing, irritability and crying,

increasing cyanosis, decreased intensity of murmur (decreased flow across RVOTO), patient

squatting for relief (increased peripheral resistance,decreased R to L shunting)

ifsevere, can lead to decreased level of consciousness,seizures, death

physical exam

single loud S2 due to severe pulmonary stenosis (i.e. RVOTO), SEM at LLSB

•investigations

. ECG:RAD,RVH

CXR:boot-shaped heart, decreased pulmonary vasculature,right aortic arch (in 25%)

echo:diagnostic

•management of spells: OT, knee-chest position, fluid bolus, morphine sulfate, propranolol,

phenylephrine

•treatment:surgical repair at 4-6 mo of age; earlier if marked cyanosis or “tet"spells

Figure 5.Tetralogy of Fallot

2 Pulmonary valve . OTHER CYANOTIC CONGENITAL HEART DISEASES

Transposition of the Great Arteries

•epidemiology:3-5% of all congenital cardiac lesions, most common cyanotic CHD in neonates

•pathophysiology: parallel pulmonary and systemic circulations

systemic:body -» RA -> RV -> aorta -> body

pulmonary:lungs -> LA -> LV pulmonary artery -> lungs

survival is dependent on mixing through PDA, ASD,or VSD

•physical exam

» neonates: ductus arteriosus closure causes rapidly progressive severe hypoxemia unresponsive to

oxygen therapy, acidosis, and death

VSD present: cyanosis is not prominent;CHE within first weeks of life

VSD absent: no murmur

Aortic valv

r "i

L J

z &

© Cassandra Collin 2014 oftor KM

Figure 6.Transposition of the great +

arteries

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!Paediatrics Toronto Notes 2023

• investigations

ECG:RAD, RVH,or may be normal

CXR:egg-shaped heart with narrow mediastinum (“egg on a string”)

echo:diagnostic

• management

• symptomatic neonates:prostaglandin E1 infusion to keep ductus open until balloon atrial

septostomy

surgical repair:arterialswitch performed in the first 2 wk in those without a VSD while LV

muscle is stillstrong

Total Anomalous Pulmonary Venous Return

• epidemiology: 1-2%ofCHD

• pathophysiology

• all pulmonary veins drain into right-sided circulation (systemic veins, RA)

no direct oxygenated pulmonary venous return to left atrium

often associated with obstruction at connection sites

ASD must be present for oxygenated blood to shunt into the LA and systemic circulation

• management:surgical repair in all cases and required urgently for severe cyanosis

Truncus Arteriosus

• pathophysiology

single great vessel gives rise to the aorta, pulmonary'

, and coronary arteries

truncal valve overlies a large VSD

potential for coronary ischemia with fall in pulmonary vascular resistance

• management:surgical repair within first 6 wk of life

Hypoplastic Left Heart Syndrome

• epidemiology: 1 -3% of CHD; most common cause of death from CHD in first mo of life

• pathophysiology: LV hypoplasia may include atretic or stenotic mitral and/or aortic valve,small

ascending aorta,and coarctation of the aorta with resultant systemic hypoperfusion

• systemic circulation is dependent on ductus patency'; upon closure of the ductus, infant presents with

circulatory shock and metabolic acidosis

• management

intubate and correct metabolic acidosis

IV infusion of prostaglandin El to keep ductus open

surgical palliation (overall survival 50% to late childhood) or heart transplant

Ebstein'sAnomaly

• Septal and posterior leaflets of

tricuspid valve are malformed and

displaced into the RV

• Potential for RV dysfunction,tricuspid

stenosis, tricuspid regurgitation, or

functional pulmonary atresia if RV

unable to open pulmonic valves

. Accessory conduction pathways(e.g.

WPW) arc often present

Etiology

. Unknown, heterogeneous genetic

predisposition, associated with

maternal lithium and benzodiazepine

use in 1st trimester

Treatment

• Newborns:consider closure of

tricuspid valve + aortopulmonary

shunt,or transplantation

• Older children:tricuspid valve repair

or valve replacement + ASD closure

4 Features of Hypoplastic Left Heart

Syndrome

• Hypoplastic LV

• Narrow mitral/aortic valves

• Small ascending aorta

• Coa rotation of the aorta

Congestive Heart Failure

•see Cardiology and Cardiac Surgery. C40

Etiology

•CHD

•cardiomyopathy (primary orsecondary)

•high output circulatory states (e.g. anemia, AVMs, cor pulmonale, hyperthyroidism)

•non-cardiac (e.g.sepsis,renal failure)

•pressure overload (e.g. aortic stenosis/coarctation, pulmonary stenosis, HTN)

•volume overload (e.g. L to R shunt, valve insufficiency)

Clinical Features

•infant: weak cry, irritability, feeding difficulties, early fatigability, diaphoresis while sleeping or eating,

respiratory distress,lethargy, FIT

•child: decreased exercise tolerance, fatigue, decreased appetite,respiratory distress,frequent URTls,

or “asthma" episodes

•orthopnea, paroxysmal nocturnal dyspnea, pedal/dependent edema are all uncommon in children

•physical findings:4 key features (tachycardia, tachypnea, cardiomegaly, hepatomegaly).Additionally,

FI T, alterationsin peripheral pulses, fourlimb blood pressures(in some CHDs), dysmorphic features

associated with congenital syndromes, gallop rhythm

Investigations

•CXR: cardiomegaly, pulmonary venous congestion

•E(Xi:sinus tachycardia,signs of underlying cause (heart block, atrial enlargement, hypertrophy,

ischemia/infarct)

•echo:structural and functional assessment

•blood work:CBC, electrolytes,BUN,Cr, LET'

S