Peds E1 -Cardio pt. 2

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