Pediatric CIS Dow

Question 1

Tools for Evaluation of the Pediatric Cardiovascular System

Answer 1

History
Physical

Routine studies:
EKG
CXR

Studies for the cardiac patient:
cardiac cath
echocardiogram

Question 2

PRENATAL exposures

Answer 2

Infections
Perinatal infections account for 2% to 3% of all congenital anomalies

TORCH and

Coxsackievirus B, CMV, HSV
In early pregnancy are teratogenic; later in pregnancy may cause myocarditis

***Active HSV lesions at time of delivery is an indication for cesarean section.

Question 3

TORCH:

Answer 3

are some of the most common infections associated with congenital anomalies (a lot of eye and skin findings in common) including cardiac defects
Most of the TORCH infections cause mild maternal morbidity, but have serious fetal consequences including fetal loss.

Toxoplasmosis
Other (syphilis, varicella-zoster, parvovirus B19, HIV)
Rubella (think “bluebella”)
congenital rubella syndrome includes cardiac manifestations: PDA, PS
Cytomegalovirus (CMV)
HSV

Question 4

Prenatal exposures- meds/ substances

Answer 4

Amphetamines–> VSD, PDA, ASD, TGA
Anticonvulsants
hydantoin: PS, AS, coarc, PDA
trimethadione: TGA, TOF, hypoplastic left heart
Alcohol –> FAS: in which VSD, PDA, ASD, and TOF are common

Question 5

Prenatal exposures- Maternal conditions

Answer 5

Of course, maternal congenital heart disease: increases risk of CHD from 1% to 15%.
Infant of diabetic mothers (IDM):
cardiomyopathy, TGA, VSD, PDA
Also hypoglycemia, hypocalcemia, polycythemia, ***LGA (large for gestational age) or SGA (small for gestational age)

**Maternal lupus/mixed connective tissue disease: congenital heart block

Question 6

Buzz words of presentation for considering CV:

Answer 6

infant who presents with diaphoresis and tachypnea with feeding, cyanosis, FTT (feeding is all they do = exertional symptoms) –> consider heart failure

Question 7

Family history of rheumatic fever

Answer 7

Frequently occurs in more than one family member: higher incidence among relatives of rheumatic children, unknown genetic component

Question 8

abnormal facies implies

Answer 8

chromosomal syndrome

Question 9

Cornelia de Lange

Answer 9

(thick eyebrows, distinct facial features): VSD, ASD

Question 10

CHARGE

Answer 10

Coloboma (normal tissue in or around the eye is missing from birth
) , Heart defects, choanal Atresia, growth/mental Retardation, GU anomalies and genital hypoplasia, Ear anomlies
conotruncal anomalies (truncus, TOF)
aortic arch anomalies

Question 11

Holt Oram

Answer 11

(cardiac-limb): defects or absence of thumb or radius
ASD
VSD

Question 12

acrocyanosis

Answer 12

newborn always has it – bluish hands and feet

Question 13

BP norms in kids are based on

Answer 13

on age, gender, and height percentile

Question 14

Auscultation of heart sounds

in kids

Answer 14

wide and fixed split S2 is abnormal

Question 15

Murmurs in PE

Answer 15

Not present in many severe forms of CHD, such as tricuspid atresia, coarctation of the aorta and transposition of the great vessels.

When a murmur is associated with a cardiac defect, the intensity of the murmur is unrelated to the severity of the abnormality.

Question 16

innocent murmurs

Answer 16

: in children, physiologic murmurs are more common than in adults

** Low pitched (non-turbulent, not high velocity)

** Still’s: low-pitched sounds heard at the lower left sternal area, “** musical.” These most commonly occur between age 3 and adolescence. Low pitched - best heard with the bell of the stethoscope. Can change with position alteration and then can decrease or disappear with the Valsalva maneuver. No clicks are present.

venous hum: low-pitched continuous murmurs made by blood returning from the great veins to the heart - bell

pulmonary flow murmur: upper left sternal border. High pitched - best heard with the diaphragm. Also known as benign peripheral pulmonary stenosis of the newborn. Also seen in adolescents or in children with pectus excavatum. Prominent in high-flow situations, such as when a child has a fever or is anemic.

Question 17

Suspicious murmurs

Answer 17

high-pitched
harsh
S1 coincident (holosystolic)
diastolic murmurs

Question 18

The Seven S’s: Key Features of Innocent Murmurs

Answer 18

Sensitive (changes with child’s position or with respiration)
Short duration (not holosystolic)
Single (no associated clicks or gallops)
Small (murmur limited to a small area and nonradiating)
Soft (low amplitude)
Sweet (not harsh sounding)
Systolic (occurs during and is limited to systole)

Question 19

EKG in newborns

Answer 19

Right ventricular dominance in newborns
Manifested by right axis deviation
Several interval lengths vary with age

Question 20

Examples of Cardiac defects in Genetic Disorders and Nonhereditary Syndromes:

Answer 20

Down: atrioventriculatr septal defects (AVSD) = aka endocardial cushion defect, VSD
Turner: coarc, AS, ASD
Noonan: PS, ASD, AVSD (partial), coarc, HCM

Williams: SVAS
Marfan syndrome: aortic root dilation, mitral valve prolapse
Fetal Alcohol Syndrome: VSD, PDA, ASD, TOF
DiGeorge: interrupted aortic arch, truncus arteriosus, VSD, PDA, TOF

Question 21

most common congenital heart defect

Answer 21

VSD is the most common overall

Question 22

Infant deaths due to CHDs often occur when the baby is

Answer 22

less than 28 days old (the neonatal period

Question 23

Basic categories of congenital heart defects

Answer 23

Acyanotic (left to right)
Cyanotic (the T’s)
Malpositions
Obstructive and valvular disease

Question 24

Left to Right Congenital Heart Disease

Answer 24

aka acyanotic

ASD
Often asymptomatic;

VSD
Size of defect dictates hemodynamic presentation.

In 30 to 40% of cases, spontaneous closure occurs within the first 6 months

PDA
In premature infants, spontaneous closure or indomethacin-induced closure may occur.

ASVD = atrioventricular septal defects
(also known as Endocardial cushion defects)

Partial TAPVR

Question 25

Cyanotic Congenital Heart Disease- Presentation

Answer 25

not always obvious at birth Clinical presentation of left heart obstructive disease may mimic sepsis Tachypnea, mottled gray skin, poor perfusion, decreased pulses Clinical clues that are critical are ***gallop rhythm and hepatomegaly or cardiomegaly***

Question 26

Cyanotic Congenital Heart Diseases

Answer 26

``` 5 T’s: Truncus arteriosus Transposition of the Great Arteries Tricuspid Atresia Tetralogy of Fallot: “Tet spells” in infancy TAPVR ``` ``` Hypoplastic Left Heart Ebstein Anomaly (right sided heart abnormality) Pulmonary Atresia Single ventricle Double Outlet Right Ventricle ```

Question 27

Transposition of Great Vessels

Answer 27

**TGV is associated with severe cyanosis in the first hours after birth (in other words, TGV is the mostly likely diagnosis in a severely cyanotic neonate who is just a few hours old) Ductus-dependent lesion (no mixing!) – keep ductus open with PGE Consider palliative balloon atrial septostomy, but definitive surgical switch of aorta and pulmonary artery required as soon as possible. *** Egg-shaped heart

Question 28

Tetralogy of Fallot

Answer 28

VSD 2. Overriding aorta 3. RV outflow obstruction 4. RV hypertrophy Think of it as an Aortic Takeover! Most common cyanotic CHD beyond infancy. boot-shaped heart in TOF and tricuspid atresia

Question 29

TAPVR

Answer 29

snowman x-ray | abnormal connection of pulmonary vein

Question 30

Coarctation of the Aorta

Answer 30

While the presentation in the first year of life can be rather dramatic (heart failure, circulatory collapse, death), later in life the presentation can be more insidious and can go overlooked or underappreciated.

Question 31

Acquired heart disease

Answer 31

Disorders of Myocardium: cardiomyopathy, myocarditis Kawasaki Rheumatic Heart Disease Endocarditis Sticky valves, wimpy bugs / Smooth valves, sticky bugs Pericardial disease

Question 32

Myocarditis

Answer 32

Inflammation of myocardial tissue *** Etiology: viral Coxsackievirus, (echovirus, poliomyelitis, mumps, rubella, CMV, HIV, arbovirus, adenovirus, influenza) ``` Sxs: nonspecific and inconsistent, depends on severity of disease Anorexia Lethargy Emesis Lightheadedness Cool extremities SOB ``` ** EKG- ST changes *** Labwork: PCR: used to find the viral genome in myocardial cells; it is rapid and sensitive, and it may become the test of choice for the diagnosis of viral myocarditis Treatment *** IVIG has been associated with improved left ventricular function and improved survival, though still controversial

Question 33

Hypertrophic cardiomyopathy (HCM)

Answer 33

Autosomal dominant genetic disorder that has a variable presentation and carries a high incidence of sudden death.

Question 34

Kawasaki- diagnostic criteria

Answer 34

High fever for FIVE days PLUS At least FOUR of the following (head to toe): Bilateral bulbar ***conjunctival injection, without exudate Erythematous mouth and pharynx, ***strawberry tongue, and/or red, cracked lips ***Cervical lymphadenopathy (> 1.5 cm in diameter), usually single and unilateral Polymorphous exanthem (morbilliform, maculopapular, or scarlatiniform) ***Swelling of hands and feet with erythema of palms and soles

Question 35

Kawasaki big sequela and treatment

Answer 35

If untreated, 15-25% develop coronary artery aneurysms IVIG and ASA-- normally no aspirin for kids! But in this case yes.

Question 36

Hypertension in pediatric patients

Answer 36

***The younger the child and the higher the BP, the more likely it is a secondary to an identifiable cause (such as renal disease)

Question 37

Arrhythmias

Answer 37

Less common in children than in adults

Question 38

common cause of syncope in kids

Answer 38

hypovolemia from not drinking enough

Question 39

Chest pain in children

Answer 39

Noncardiac causes (much more common than cardiac etiology)! Examples: ``` Musculoskeletal etiology very common Costochondritis Tietze syndrome (recent URI) – similar to costochondritis but often a single joint with associated warmth, swelling, tenderness Nonspecific Slipping rib syndrome Trauma, strain Precordial catch (Texidor twinge): posture or pinched nerve related Xiphodynia (reproducible) ``` GI (GERD) Psychogenic (anxiety) Zoster (superficial pain – before lesions appear) Spinal cord/nerve root compression in conjunction with scoliosis

Question 40

chest pain- when to refer

Answer 40

``` Abnormal cardiac findings * Exertional chest pain * Exertional syncope Chest pain with palpitations EKG abnormalities * Significant family history of arrhythmias, sudden death, or genetic disorders Heart transplant h/o Kawasaki First degree relative with familial hypercholesterolemia ```

Question 41

Sports PE screening Refer for:

Answer 41

Family h/o early sudden death Cardiac, seizure, one car accident, unknown Murmurs (HCM) Marfan’s stigmata Simultaneous palpation of radial and femoral pulses (undiagnosed coarctation) reveals unequal pulses Other PE findings consistent with cardiac disease

Question 42

Special Considerations: recent cath or cardiac surgery

Answer 42

Embolization or compression of adjacent structures Can have MI ** Fever and chest pain worse when supine: postpericardiotomy syndrome***