Studied by 7 people
What is Eisenmenger’s syndrome?
complication of longstanding/uncorrected congenital heart anomalies that produce L→R shunting
What cardiac anomalies commonly lead to Eisenmenger’s syndrome?
VSD, ASD, Truncus Arteriosis, TGA
What is the tx for Eisenmenger’s syndrome?
supportive, heart/lung transplant, endocarditis prophylaxis
Is Pulm HTN more common in children or adults?
children
What are cardiac causes of Pulm HTN in children?
PDA, VSD, ASD, TGA
What causes Persistent Pulm HTN of the Newborn (PPHN)?
failure in the normal circulatory transition that is inborn in infacts → hypoexemia → R to L shunting
What are sx of PPHN?
tachypnea, resp. distress, cyanosis, poor cardiac funciton/perfusion, hypotension, shock, loud single S2
What causes Pediatric Pulm HTN?
idiopathic, cause & etiology unknown
How can Pulm HTN be diagnosed?
MAP > 20 mmHg at rest w/ normal pulmonary capillary wedge pressure
Pulm HTN in childhood may cause exercise-related hemodynamic abnormalities. What does this cause?
significant morbidity & mortality
What sx do younger children w/ Pulm HTN experience?
poor appetite, poor growth, N/V, lethargy, sweating, tachypnea, tachycardia
What sx do older children w/ Pulm HTN experience?
exertional dyspnea and CP
What is the GOLD standard for dx Pulm HTN?
cardiac cath
What is the tx for Pulm HTN?
no cure; can use medications to improve sx and life expectancy
*Warfarin, Digoxin, Vasodilators
What heart defects are associated w/ Downs Syndrome?
AV canal/septal defects, ASD, VSD
What heart defects are associated w/ Marfan Syndrome?
aortic root dilation/dissection, MVP
What heart defect is associated w/ maternal Lithium ingestion?
Ebstein’s Anomaly of the Tricuspid Valve
If a neonate is experiencing CHF w/in 4 min of age, what is the most likely cause?
Vein of Galen AV Malformation
If a neonate is experiencing CHF w/in 4 days of age, what is the most likely cause?
CoA or Left heart obstruction
If a neonate is experiencing CHF w/in 4 weeks of age, what is the most likely cause?
VSD
If a neonate is experiencing CHF w/in 4 years of age, what is the most likely cause?
Myocarditis or acquired condition
If a neonate is experiencing CHF w/in 1st 1-2 months of age, what is the most likely cause?
L → R shunts
What is Marfan Syndrome caused by?
mutation in FBN1 gene → effects fxn of fibrillin-1 in connective tissues
What tests need to be ordered to evaluate for CHD in pts w/ Marfan?
Echo, MRI (aorta), EKG
Which condition is similar to Marfan’s and has an aggressive vascular pathology?
Loeys-Dietz syndrome (LDS)
How does LDS present?
aortic root dilation, cleft palate, ± bifid/abnormal uvula
When do aortic dissection/rupture occure in LDS pts?
young age or at aortic root diameters not considered at risk in MFS
What is the tx for LDS (heart component)?
careful f/u & aggressive surgical tx
What percent of cardiac murmurs are benign or normal?
> 95%
When children are thin w/ thin chest walls, what is causing the ‘murmur’?
normal blood flow through the heart
Which heart murmurs are innocent?
Still’s, Pulm ejection, Pulm flow, Venous hum, Carotid bruit
What is a key characteristic of Still’s murmur?
systolic, vibratory or musical nature; usually 3-6 yo
What are some characteristics of a Pulm Ejection murmur?
low grade, blowing, dec w/ inspiration, sitting up, standing up; 8-14 yo
What are the characteristics of a Pulm Flow murmur in a newborn?
transmits to L&R chest, axillae & back
What is a Venous Hum caused by?
turbulence in the SVC
What are the characteristics of a Venous Hum murmur?
loudest @ infra-clavicular area (especially w/ sitting/standing), disappears while lying supine, intensity changes w/ rotation of head, disappears w/ jugular compression; 3-6 yo
What causes a Carotid bruit?
turbulence in carotid/subclavian artery
What are the characteristics of a Carotid bruit?
thrill over carotid, disappears w/ hyperextension of shoulders, low grade; any age
What are the 8 S’s of innocent murmurs?
Soft, Systolic, Short, Sounds (S1/2), Symptomless, Special tests normal, Standing/sitting variation, Sternal depression
What tachyarrhythmias are commonly encountered in children?
sinus tach, SVT, WPW, Atrial flutter, V tach
What causes sustained Sinus tachy in infancy?
CHF
What causes sustained Sinus tachy in older children?
dilated cardiomyopathy
What tachyarrhythmia is generally well tolerated in children?
SVT
What is the tx for sinus tach?
identify/tx underlying cause
What is the tx for SVT?
dx maneauvers, vagal tone, adenosine, supportive care
What key feature on an EKG indicates WPW?
delta wave
Is atrial flutter common in children?
no; rare in pts w/ no hx of heart surgery or CHD
What does Atrial flutter in older children usually indicate?
underlying atrial conduciton disease; ex: SSS or myocarditis
What is the tx for Atrial flutter?
Digoxin; Class 1 (procainamide, flecainide); Class 3 (amio, sotalol), DC cardioversion
What acute tx can be used to stop a tachyarrhythmia?
Vagal (ice to face, valsalva, gag reflex), IV adenosine or verapamil, DC cardioversion
When is okay to use verapamil?
pt > 1 yo & normal cardiac fxn
When should you utilize a cardioversion immediately?
if in shock from SVT (even if digoxin has been given)
If a tachyarrhythmia breaks and recurs what needs to be given before repeating a cardioversion?
antiarrhythmic
When can you use digoxin in a child?
> 1 yo w/ WPW
When might you see sinus bradycardia in children?
inc parasympathetic tone (athletes/sedation), hypoxemia, inc ICP (Cushing’s triad)
When do you usually see SA node dysfunction in children?
s/p atrial surgery
What causes Long QT syndrome in children?
ion channelopathy (K or Na)
What bradyarrhythmia can cause sudden Torsades or VT?
long QT syndrome
What are the three common type of Long QT syndrome?
LQTS1: emotional stess or exercise (swimming!!)
LQST2: extreme emotions
LQST3: bradycardia during sleep can trigger
What is the 1st line tx for Long QT syndrome?
B-blockers
*avoid abrupt discontinuation
What is the MC complication of CHD?
arrhythmia
What are the MC arrhythmias?
supraventricular
What is the MC of syncope in children?
vasocardiogenic (vasovagal)
What causes vasovagal syncope?
venous pooling → dec venous return → hypotension/bradycardia
What is the tx for vasovagal syncope?
inc fluid/salt intake
What should you be concerned for if a child presents w/ recurrent syncope (esp. w/ exertion)? What test should be done?
WPW, Long QT, HCM
EKG
What is the best tx for Dyslipidemia in children?
diet/lifestyle modifications
What ages should children receive a serum lipid screening?
9-11 yo; repeat 17-21 yo
When should children w/ cardiovascular RF be complete a serum lipid screening?
2-8 yo; repeat 12-16 yo
What is the preferred screening test for dyslipidemia in children?
fasting lipid profile
What RF inc a childs odds of developing dyslipidemia?
HTN, obesity, FHx of premature CV disease
What lifestyle modifications are recommended for children w/ dyslipidemia? (LDL >130)
healthy diet for all > 2 yo; low fat milk, wt management (tx of choice for overweight pts)
When do you refer a child to a lipid specialist?
LDL-C >/= 250
When should children w/ dyslipidemia be put on drug therapy to manage lipid levels?
> 8 yo w/ LDL > 190 (> 160 if +FMH; > 130 w/ DM), after 6-12 months of restricted diet
What is the goal lipid level in children?
minimal = <130
ideal = <110
What bile acid sequestrant can be used in children to tx dyslipidemia?
Cholestyramine or colestyramine
How is HTN in children defined?
BP > 95th percentile
*normal values vary based on age, sex, ht
What is the more likely cause of HTN in children?
secondary causes: MC -renal parenchymal disease & renovascular disease
Where should BP be measured in children?
all 4 extremities
What is the tx for HTN in children?
lifestyle changes
secondary: thiazide, ACE-I, ARBs, BBs, CCBs
What is HTN urgency?
significant elevation in BP w/o end-organ damage
What are sx of HTN urgency?
HA, blurred vision, nausea
What is a HTN emergency?
elevation of both systolic/diastolic BP w/ acute end-organ damage (cerebral infarction, pulm edema, renal failure)
What is the tx for HTN urgency?
lower MAP by 20% over 1 hr and return to baseline over 24-48 hrs; observe in ED for 4-6 hrs
What is the tx for HTN emergency?
IV/arterial line for continuous BP monitoring, cardio/nephro consult, GOAL = lower BP promptly but gradually
What are the 2 factors that help infective endocarditis develop?
bacteremia & structural abnormality of heart/great vessels w/ significant flow turbulence or pressure gradient (VSD, TOF, IHSS (HOCM))
What is the major Duke criteria for dx infective endocarditis?
1-blood cultures (+)
2-evidence of endocardial involvement
What is the minor Duke criteria for infective endocarditis?
predisposing factor, temp >38 C, vascular phenomena, immunologic phenomena, microbiologic evidence
What is the leading cause of acquired heart disease in children?
Kawasaki disease
What is one of the MC vasculitis in childhood?
Kawasaki disease
What age is most likely to get Kawasaki disease?
6 mo - 5 yo
*more frequent in those of Asian ancestry
T/F: Kawasaki is a contagious disease
False
What is the etiology of Kawasaki?
unknown, thought to be an immune rxn
Why do you need to act fast if Kawasaki disease is suspected?
affects the heart muscles and valves early
What are the sx of Kawasaki disease?
persistent high fever (> 5 days) + 2 or more of the following:
strawberry tongue, cherry red lips
changes in hands and feet
diffuse rash (worse in diaper area)
bloodshot eyes w/o d/c
swollen glands
*may not all be present at the same time
Pneumonic for remembering Kawasaki sx
FEARS ME
Fever x 5 days, Eyes -conjunctivitis, Arthralgia, Rash, Skin desquamation (hands/feet), Mucosal involvement (strawberry tongue, dry lips), Elevated ESR/Plts
What are the cardiac manifestations of Kawasaki disease?
coronary artery aneurysms, HF, depressed contractility, MI, arrhythmias, peripheral arterial occlusion
How are coronary artery aneurysms?
Echo & angiography
What percentage of pts w/ Kawasaki disease get coronary artery aneurysms?
20% of untreated pts - more common in young boys, high fever, & ESR
Where do coronary artery aneurysms commonly occur in Kawasaki pts?
proximal LCA/RCA
