Pediatric Cardiology Flashcards
What does heart failure look like in an infant
Respiratory distress, especially with feeding
poor feeding
poor growth
hepatomegaly and rales
What is the foramen ovale
Right atrium to left atrium hole
what is ductus arteriosus
pulmonary artery to aorta
What is ductus venosis
umbilical verin to IVC (bypasses liver)
what happens to lead to the closure of ductus arteriosus
decreased level of prostaglandin E2
What is the leading cause of birth-defect related perinatal and infant death and disease
Congenital heart disease
What are the types of congenital heart disease
Acyanotic heart disease
obstructive lesions
cyanotic heart disease
What is the most common heart defect
Bicuspid aortic valve
often unknown until adulthood
What are risk factors for CHD
prematurity: 2-3x higher
family history: 3 fold increase if first degree relative
genetic syndrome
maternal factors: diabetes, HTN, obesity, PKU, thyroid, smoking, alcohol use
in utero infection: TORCH
What are TORCH infections
in utero infection
rubella, flu-like illness, CMV, HHV6, HSV, parvovirus, toxoplasmosis
What is Acyanotic heart disease
L>R shunts
Patent ductus arteriosus (PDA)
Ventricular septal defect (VSD)
Atrial septal defect (ASD)
What is the classic murmur description of PDA
continuous machine-like murmur LUSB or left infra-clavicular area
What is a PDA
failure of DA to close
more common in babies born < 30 weeks
how are PDA’s diagnosed
clinical findings (murmur)
ECHO
What is the management of a PDA
mod-severe or signs of CHF/PHN: surgical or percutaneous (preferred) closure
what is the management of premature infants with PDA
prostaglandin inhibitors tried first
-indomethacin, ibuprofen
(does not work in term neonates and older infants)
What is a VSD
ventricular septal defect
common congenital heart disease - 50% of all congenital heart disease have VSD as a component
what are symptoms of VSD
diaphoresis, particularly with feeds
difficulty feeding
failure to thrive
CHF
irreversible pulmonary vascular changes within 6-12 months
What is the classic sign of VSD
holosystolic murmur heard best a the mid to LLSB
what confirms the diagnosis of a VSD
ECHO - size and location confirmation
What is the management of VSD
35% will close spontaneously
small defects - will close by age 2
moderate or large: asymptomatic: regular follow ups, symptomatic depends
what is the treatment for moderate or severe symptomatic VSD
mild: oral diuretics (lasix), neutritional support
Moderate: lasiz +/- aldactone; maximize caloric intake
Severe: inpatient, IV diuretics, max nutritional suppport, stabilize condition for surgery
What are the types of ASD
Primum
Secundum
Sinus venosus
What is primum ASD
septrum primum doesnt fuse with the endocardial cushions
usually associated iwth other abnormalities
What is secundum ASD
hole near the foramen ovale
females> males
usu. isolated; can be associated with genetic disorders
what is sinus venosus ASD
malposition of insertion of vena cava
What is the clinical presentation of ASD
usu no symptomatic early, normally come to attention on PE
impact depends on size/location
infants with larger ASD can present with CHF, FTT
What is the classic findings with ASD
wide, fixed split S2 (vs only on inspiration)
mid-systolic ejection murmur
how is ASD diagnosed
clinical suspicion with murmur
EKG may show RBBB
ECHO confirms diagnosis
MRI can be helpful
what is the managment of ASD
many spontaneously close in infancy/early childhood
untreated, with persistent shunt: R sided heart failure afe 30-40
repair if R heart enlargement
surgical
What are Acyanotic Obstructive defects
Coarctation of the Aorta (COA)
Aortic Stenosis (AS)
Pulmonary stenosis (PS)
What is Coarctation of the Aorta
narrowin of descending oarta, distal to LSCA
symptoms depend on degree of obstruction
what are the classic findings with COA
hypertension in UEs, low-unobtainable BPs in LE
decreased/delayed femoral pulses
possible murmur caused by collateral blood flow
how is COA diagnosed
prenatal difficulties due to low CO via aorta
ECHO
what is the management of COA
imperative to identify critical COA in neonates: rapid initiation of prostaglandin E > once stable, surgery
balloon angioplasty preferred
What are signs of Critical aortic stenosis (AS)
severe heart failure, central cyanosis and shcok
-ductal dependent
what is the murmur with AS
harsh systolic ejection murmur
systloic ejection click
what is the diagnosis of AS
PE and ECHO; cardiac catheterization
What is the treatment of AS
prostaglandin if critical
baloon valvuloplasty
may need surgical management (valve replacement rare)
What are the signs of of severe/critical PS
right-to-left shunting; survival dependent on maintaining a PDA and a PFO
What is the murmur heard with PS
normal 1st heart sound followed by click
systolic ejection murmur at 2nd LICS
how is PS diagnosed
PE and ECHO
what is the treatment of PS
if severe: balloon valvuloplasty
What are the Cyanotic heart diseases
5 T’s: 1-5
* Truncus arteriosus
* transposition of great vessel
* tricuspid atresia
* tetralogy of fallot
* total anomalous pulmonary venous return
6th T: tons of other things, i.e. hypoplastic left heart (HLH)
how are cyanotic heart diseases diagnosed
cofirmed by ECHO
most diagnosed by fetal echo
What is the treatment of Cyanoic heart diseases
ALL require surgical repair at a pediatric cardiac surgery-capable center
- not always emergently or urgently
- stabilization first!
What is Truncus arteriosus
single great cessel (common arterial trunk)
ALWAYS has associated VSD
what are the symptoms of Truncus arteriosis
cyanosis (~mild)
respiratory distress / signs of CHF
+/- murmur: systolic ejection murmur with click
S2 is loud and single
develop heart failure over days to weeks
What is transpotition of the Great vessels
arota and pulmonary artery are switched
presents with cyanosis: mild to severe
what happens if transposion of great vessels is untreated
most die within first year of life
how do we treat/manage transposition of great vessels
prostaglandin E to keep PDA intact
balloon atrial spetostomy
surgical repaire: arterial switch
What is Tricuspid atresia
3rd most common cyanotic heart condition
abscent tricsupid valve
no communication between R atrium and ventricle
survival is dependent on Right>left flow
what is the presentation of Tricuspid atresia
severly cyanotic plus murmur
failed oximetry screen
what is the treatment of tricuspid atresia
if diagnosed prenatally - transfer for delivery to heart center
immediate initiation of prostaglandin
complex, staged surgery
How quickly does tetralogy of fallot require correction
wihtin first year of life
what is tetralogy of fallot
results from septation of truncus arteriosus into the aorta and pulmonary artery that occurs early in gestation (3-4 weeks)
what are the components of tetrology of fallot
4
- ventricular septal defect
- pulmonary valve stenosis
- overriding arota
- right ventricular hypertrophy
what is a tet spell
intermittent
sudden increase in cyanosis
restless, agitated, inconsolable
prolonged spells can lead to syncope, convulsions, stroke, cardiac arrest/death
what is the murmur heard with tetralogy of fallot
usually due to pulmonary stenosis not the VSD
crescendo-decrescendo systolic murmur heard best in the left mid to upper sternal corder
Single S2
what is seen on XR with tetralogy of fallot
boot-shaped heart
what is the treatment of TOF
surger (maybe temporary shunt)
What is a Total Anomalous Pulmonary Venous Return (TAPVR)
none of the pulonary veins connect to the left atrium; all return via sysetmic venous circulation
port of the blood shunts across ASD or PDA
What murmus is heart with TAPVR
systolic ejection murmur
widely split S2
What is the treatment of TAPVR
prostaglandin, other medical stabilization
may require ECMO and palliative septostomy prior to surgery
What is Hypoplastic left heart syndrome (HLHS)
underdeveloped left heart, normal great vessels, varying degress of valvular abnormalities
R ventricle supports both pulmonary AND systemic circulation
what is the presentation of HLHS
often detected by prenatal US
if baby does NOT have an ASD: severe respiratory distress, cyanosis, cardiogenic shock at birth
CYANOSIS(sometimes described as grey)
what is the initial management of HLHS
prostaglandin E2 to keep patient ductus open
if no ASD or a restrictive ASD: atrial septostomy
what is the definitive management of HLHS
complex, staged surgical rare
when are murmurs always pathologyic
Grade IV or more
Diastolic
at what grade murmur do we refer / evaluate murmurs
Grade III : no palpable thrill yet
What are the 7 S’s
key features of innocent murmurs
* sensitive
* short duration
* single
* small
* soft
* sweet
* systolic
what are 4 criteria for innocent murmurs
normal PE except for murmur
negative ROS
no hx that includes risk factors for structural heart disease
murmur has the characteristic features of a specific benign murmur
What is Still’s murmur, vibratory murmur
systolic ejection murmur
best heard at LLSB
vibratory - musical quality
grade 1 or 2
intensity decreases in upright position
What are red flags with murmurs
loud murmur, grade III or higher
harsh murmur
holo/pansystolic murmur
diastolic murmur
lound or single second heart sound
gallop rhythm
friction rub
Failure to thrive
What are asymptomatic arrhythmias
sinus arrhythmias
PVCs
PACs
What are the types of symptomatic arrhythmias
Atrial: m/c seen in children with CHD who have had cardiac surgery
SVT
Ventricular (uncommon without underlying heart disease)
sinus node dysfunction
heart block
What are warning signs of SCA/SCD
chest pain
fatigue/stamina issues
syncope or near-syncope
dizziness
palpitations
dyspnea
activity limitations
what is an autosomal dominant inherited cardiomyopathy
hypertrophic cardiomyopathy (HCM)
how is HCM diagnosed
EKG is abnormal
LVH, T-wave abnormalities
ECHO
what are presenting symptoms of HCM
chest pain
syncope/presyncope
palpitations
heart failure
sudden cardiac arrest/death
What is the first line for HCM management
Beta-blockers
what is second line for HCM management
calcium channel blockers
