Pediatric CIS Dow Flashcards
Tools for Evaluation of the Pediatric Cardiovascular System
History
Physical
Routine studies:
EKG
CXR
Studies for the cardiac patient:
cardiac cath
echocardiogram
PRENATAL exposures
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.
TORCH:
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
Prenatal exposures- meds/ substances
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
Prenatal exposures- Maternal conditions
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
Buzz words of presentation for considering CV:
infant who presents with diaphoresis and tachypnea with feeding, cyanosis, FTT (feeding is all they do = exertional symptoms) –> consider heart failure
Family history of rheumatic fever
Frequently occurs in more than one family member: higher incidence among relatives of rheumatic children, unknown genetic component
abnormal facies implies
chromosomal syndrome
Cornelia de Lange
(thick eyebrows, distinct facial features): VSD, ASD
CHARGE
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
Holt Oram
(cardiac-limb): defects or absence of thumb or radius
ASD
VSD
acrocyanosis
newborn always has it – bluish hands and feet
BP norms in kids are based on
on age, gender, and height percentile
Auscultation of heart sounds
in kids
wide and fixed split S2 is abnormal
Murmurs in PE
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.
innocent murmurs
: 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.
Suspicious murmurs
high-pitched
harsh
S1 coincident (holosystolic)
diastolic murmurs
The Seven S’s: Key Features of Innocent Murmurs
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)
EKG in newborns
Right ventricular dominance in newborns
Manifested by right axis deviation
Several interval lengths vary with age
Examples of Cardiac defects in Genetic Disorders and Nonhereditary Syndromes:
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
most common congenital heart defect
VSD is the most common overall
Infant deaths due to CHDs often occur when the baby is
less than 28 days old (the neonatal period
Basic categories of congenital heart defects
Acyanotic (left to right)
Cyanotic (the T’s)
Malpositions
Obstructive and valvular disease
Left to Right Congenital Heart Disease
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
Cyanotic Congenital Heart Disease- Presentation
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
Cyanotic Congenital Heart Diseases
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
Transposition of Great Vessels
**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
Tetralogy of Fallot
VSD
- Overriding aorta
- RV outflow obstruction
- RV hypertrophy
Think of it as an Aortic Takeover!
Most common cyanotic CHD beyond infancy.
boot-shaped heart in TOF and tricuspid atresia
TAPVR
snowman x-ray
abnormal connection of pulmonary vein
Coarctation of the Aorta
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.
Acquired heart disease
Disorders of Myocardium: cardiomyopathy, myocarditis
Kawasaki
Rheumatic Heart Disease
Endocarditis
Sticky valves, wimpy bugs / Smooth valves, sticky bugs
Pericardial disease
Myocarditis
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
Hypertrophic cardiomyopathy (HCM)
Autosomal dominant genetic disorder that has a variable presentation and carries a high incidence of sudden death.
Kawasaki- diagnostic criteria
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
Kawasaki big sequela
and treatment
If untreated, 15-25% develop coronary artery aneurysms
IVIG and ASA– normally no aspirin for kids! But in this case yes.
Hypertension in pediatric patients
***The younger the child and the higher the BP, the more likely it is a secondary to an identifiable cause (such as renal disease)
Arrhythmias
Less common in children than in adults
common cause of syncope in kids
hypovolemia from not drinking enough
Chest pain in children
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
chest pain- when to refer
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
Sports PE screening Refer for:
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
Special Considerations: recent cath or cardiac surgery
Embolization or compression of adjacent structures
Can have MI
** Fever and chest pain worse when supine: postpericardiotomy syndrome***
