Pediatric First Aid: Cardiovascular Disease Flashcards
1
Q
Murmur Grading
Grade I
Grade II
Grade III
Grade IV
Grade V
Grade VI
A
I = very soft; need very careful auscultation II = soft and readily heard, but faint; equal to S1/S2 III = moderate intensity; louder than S1/S2
IV = loud murmur; palpable thrill intermittent V = loud murmur with palpable thrill VI = loud murmur with palpable thrill that can be heard when stethoscope is lifted slightly off chest
2
Q
What is a Still’s murmur?
What age range is it commonly seen in?
A
benign murmur due to turbulent flow in the left ventricular outflow tract
- typically seen between 3-6 yo; uncommon < 2 yo
LLSB w/musical or vibratory midsystolic accentuation
3
Q
How much time does each small box and large box on an ECG correspond to?
A
Small = 0.04 seconds (1 mm) Large = 0.2 seconds (5 mm)
4
Q
What is the most common cause of bradycardia in children?
A
Hypoxemia
5
Q
What is the normal QRS axis and which leads do you look at to help determine Axis on an ECG?
A
0 to +90 degrees
- check leads I and aVF (both should be upright if normal)
6
Q
How long is a normal P-wave on ECG of an infant vs children?
A
Infant = \< 0.08 seconds (2 small boxes) Children = \< 0.10 seconds (1/2 big box)
7
Q
What does a Premature Ventricular Contraction look like on ECG?
A
premature and wide QRS without P wave; T wave may be inverted
- evaluate further if runs of PVS or they occur regularly
8
Q
In what direction do the T-waves face on an ECG of a patient with Ventricular Tachycardia?
A
OPPOSITE direction of the QRS complex
9
Q
What is the QRS axis if Leads I and aVF are:
- I (+) and aVF (+)
- I (-) and aVF (+)
- I (+) and aVF (-)
- I (-) and aVF (-)
A
- normal axis
- LEFT axis deviation
- RIGHT axis deviation
- EXTREME axis deviation (direction based on Q-wave)
10
Q
What are 3 common pathologies that can result in Right Axis Deviation (RAD)?
A
severe pulmonary stenosis w/right ventricular hypertrophy
pulmonary hypertension
conduction disturbances (RBBB)
11
Q
Left Axis Deviation with Right Ventricular Hypertrophy (RVH) is highly suggestive of what, especially in children with Down Syndrome?
A
AV canal
12
Q
Mild LAD with Left Ventricular Hypertrophy (LVH) in a cyanotic infant suggests what condition?
A
Tricuspid Atresia
13
Q
What is the normal P axis and what leads are commonly used to determine it?
What does a P axis > +90 degrees indicate?
A
Normal deflection: Lead II (+), Lead aVR (-)
> +90 = atrial inversion or misplaced leads
14
Q
Peaked, pointed T waves occur due to what? (3)
Flattened T waves occur due to what? (2)
A
Peaked = HYPERkalemia, LVH, head injury
Flattened = HYPOkalemia, hypothyroidism
15
Q
What leads would you look at to determine Right Atrial Enlargement and Left Atrial Enlargement?
How does the P-wave look?
A
RAH = Peaked waves in Leads II and V1
LAH = notched Lead II, deep terminal inversion V1
16
Q
What is Wolff-Parkinson-White Syndrome?
A
ventricular preexcitation via accessory conduction pathway through Bundle of Kent
- conducts more rapidly than AV node but takes longer to recover
**DELTA WAVE**
17
Q
For infants and children, what echo is commonly used to evaluate the coronary arteries?
A
Transthoracic Echocardiogram
18
Q
Transesophageal Echocardiogram
A
transducer down esophagus for enhanced imaging during cardiac surgery or catherization
19
Q
2-D Echocardiography
A
cross-sectional images of the heart are seen in order to assess structures
Ex: inflow/outflow tracts, valves, ascending/descending aorta, pulmonary arteries/veins, ventricles, septa
20
Q
Color-Flow Doppler Echocardiography
What do the red and blue colors indicate?
A
blood flow and direction can be seen via this method
Red = blood flowing TOWARDS from transducer Blue = blood flowing AWAY from transducer
21
Q
Color-Flow Doppler Echocardiography
Difference between M-Mode Echo vs Fetal Echo?
A
M-Mode = info from one scan point is measured over time
- motion creates depth graph of structures
- determines chamber/valve dimensions and size
Fetal = prenatal diagnosis of congenital heart disease
- screen > 16 weeks
22
Q
What is a normal Cardiothoracic ratio (heart size) on CXR and how is it determined?
A
Normal ratio = < 0.6
measure largest width of heart and divide by largest diameter of the chest
**needs good inspiratory effort**
23
Q
Why are the hearts of patients with Tetralogy of Fallot commonly in the shape of a “boot?”
A
due to hypoplastic main pulmonary artery
24
Q
Why are the hearts of patients with Transposition of the Great Arteries commonly in the shape of an “egg?”
A
due to narrow superior aspect of cardiac silhouette
- absence of thymus and irregular relationship of great arteries
25
What is the name of the shape that can sometimes been seen on CXR of a patient with Total Anomalous Pulmonary Venous Return (TAPVR)?
"Snowman" shape
- left vertical vein, left innominate (brachiocephalic) vein, and dilated SVC create the head
26
Rheumatic Fever
What is it and how long does it take to show signs?
delayed immunologic sequela of previous Group A streptococcal infection of pharynx
- cutaneous infection precursor to glomerulonephritis but not rheumatic fever
- peaks in children 5-15 yo; follows pharyngitis by 1-5 weeks (average: 3 weeks)
27
Rheumatic Fever
Typically causing carditis in 50-70% of patients, what 3 valves (in order of dec. frequency) are most commonly affected?
Mitral Valve regurgitation
Aortic Valve regurgitation
Tricuspid Valve regurgitation
28
Rheumatic Fever
What is the most common first symptom of ARF?
Where is it most commonly found and what does it respond to?
First Symptom --\> Migratory Arthritis
- large joints \>\> spine/cranial joints
Tx: aspirin (usually lasts less than a month)
29
Rheumatic Fever
What is Chorea and when is it typically seen?
loss of motor coordination with spontaneous, purposeless movement and motor weakness
- emotional lability onset before motor symptoms (similar to ADHD or OCD behavior)
- longest latent period (vs other sxs), presenting 1-8 months post-infection and lasting for months
30
Rheumatic Fever
Erythema Marginatum looks like what and is most commonly found where on the body of a patient?
pink, erythematous, nonpruritic macular rash with clear center
- evanescent and migratory; disappears when cold/reappears when warm
TRUNK and PROXIMAL EXTREMITIES
31
Rheumatic Fever
Where are the subcutaneous nodules most commonly found at? (3)
BONY PROMINENCES over extensor surfaces (most commonly tendons of hand)
- also scalp and spine
32
Rheumatic Fever
What is required to make the diagnosis?
1. 2 Major criteria (JONES)
- joints (polyarthritis)
- heart (carditis)
- nodules (SubQ)
- Erythema Marginatum
- Sydenham's Chorea
2. 1 Major criteria and 2 minor criteria
33
Rheumatic Fever
What are 5 minor criteria that can help make the diagnosis of Rheumatic Fever?
arthralgia
fever
elevated ESR/CRP
prolonged PR interval
ASO titer (lab evidence of antecedent group A strep infection)
34
What diagnostic atrial myocardium finding can be found in patients with Rheumatic Fever?
Aschoff bodies
35
Where is Erythema Marginatum NEVER found?
the face
36
Rheumatic Fever
What is the approach to treatment once diagnosed? What medication is used if patient is allergic to first-line option?
Aspirin --\> reduce fever and arthritis symptoms
Oral PCN V x10 days OR PCN G IM, single dose
\*\*if PCN allergy --\> Azithromycin x5 days\*\*
37
Rheumatic Fever
What are the 3 options for patient prophylaxis?
1. Benzathine penicillin G IM every 3-4 weeks
2. PCN PO 3x/day
3. Sulfadiazine PO 1x/day
38
Endocarditis
What are the two most common organisms that cause infection?
If you get a culture-negative endocarditis, what two organisms should you keep in mind?
1. alpha hemolytic streptococci (70%)
- S. pneumoniae and S. viridans
2. Staph Aureus (20%)
culture (-)? = Coxiella burnetii or Bartonella
39
How does endocarditis develop?
due to turbulent blood flow across an abnormal valve or any cardiac defect
40
Embolic phenomena of Endocarditis
roth spots
splinter hemorrhages
Osler nodes
Janeway lesions (less common in children)
41
4 high risk predisposing conditions that can lead to endocarditis
Prosthetic cardiac valves
Previous bacterial endocarditis
CHD
Surgical pulmonary-systemic shunts (correct cyanotic heart disease)
42
What is Libman-Sacks valve?
NON-bacterial endocarditis associated with Lupus
43
How is the diagnosis of endocarditis made?
- at least 3 blood cultures over 48 hrs from different sites
(+) Cx, ESR, hematuria, anemia
- Echo showing vegetations or thrombi
44
How is endocarditis treated?
- 4-8 weeks of organism-specific IV antibiotics
- surgery for refractory cases or those with prosthetic valves, fungal endocarditis, or hemodynamic compromise
45
Endocarditis PPX meds
- needed prior to dental procedures in children with structural heart dz or predisposing conditions
Amoxicillin --\> Clindamycin (PCN allergy)
46
What are Janeway lesions and Osler nodes?
J = small, painless, erythematous lesions on palms/soles
- septic emboli causing microabscesses of the dermis
O = red, painful on hands/feet
- immune complex deposition
47
Myocarditis
What 3 viruses are common causes of disease?
Coxsackieviruses
Echoviruses
Adenoviruses
48
Myocarditis
Non-viral causes of disease
- immune-mediated diseases
- toxic ingestion (alcohol, amphetamines, anthracyclines, clozapine)
49
What long-term disease etiology can myocarditis lead to if left untreated?
DILATED cardiomyopathy and Heart Failure
50
Patient is tachycardic and tachypneic, and has a gallop on auscultation. What is the likely disease etiology?
Congestive Heart Failure (CHF)
51
Myocarditis
How is it treated?
First - treat underlying cause
- largely supportive (viral disease = rest/activity mod)
- possibly treat CHF (diuretics, inotropic agents); gamma globulins
52
Pericarditis
What is the most common cause and what 5 bacteria are commonly implicated?
most common: VIRAL
Bacteria: S. aureus, H. flu, N. meningitidis, Strep, TB
53
Pericarditis
What causes it and what can it lead to long-term?
- caused by inflammation of the pericardium
- can progress to pericardial effusion/cardiac tamponade if significant fluid accumulates around heart
54
What is Pulsus Paradoxus and what disease is it classically associated with?
decrease in SBP \> 10 mmHg on inspiration
- commonly associated with Pericarditis
\*\*due to reduced venous return because of increased intrathoracic or intracardiac pressure during inspiration\*\*
55
Child has pericarditis and a salmon-colored rash with joint pain. What should you think of?
Juvenile Rheumatoid Arthritis
56
What is Electrical Alternans and what disease is it classically associated with?
QRS alternates between larger and smaller voltages as heart swings with the pericardial effusion
- commonly associated with Cardiac Tamponade
57
Pericarditis
How does it classically present on exam? What about Cardiac Tamponade?
precordial pain (sudden chest pain that comes and goes) with radiation to the shoulder/neck
- pericardial friction rub on auscultation
CT: distant heart sounds, tachycardia, pulsus paradoxus, hepatomegaly/venous distension
58
How is Pericarditis diagnosed?
Echocardiograph
59
What are the 3 most common causes of Congestive Heart Failure in the first 6 months of life?
VSD, PDA, and endocardial cushion defects
60
Pericarditis
How is it treated?
- treat underlying disease process
- pericardiocentesis indicated if effusion present
- urgent drainage if signs/symptoms of tamponade
61
How long does a left-to-right shunt take to start putting significant stress on the left ventricle?
6 weeks
62
Which is better for treating pediatric CHF: salt/fluid restriction or diuretics?
Diuretics
63
Congestive Heart Failure
How do Digitalis and Diuretics help treat patients?
What are some common AFTERload-reducing agents that can help treat CHF?
Digitalis - improve ventricular function
- CI'd in complete heart block and HCM
Diuretics - dec. volume overload and pulmonary edema
- loop diuretics (Furosemide) most common
AL agents: ACEi, CCBs, nitroglycerin --\> dilate peripheral vasculature and dec. work on heart
64
Central Cyanosis vs Acrocyanosis
CC: mucus membrane, blue-tinged lips
- ALWAYS pathologic in newborns
- either cardiac or pulmonary disease
- \> 5 mg/dL of deoxyhemoglobin
A: distal extremities
- NORMAL in newborns
- dec. distal perfusion, exposure to cold
65
What are the 5 T's and 1 P of Cyanotic Heart Defects?
Truncus Arteriosus
Transposition of the Great Arteries
Tricuspid Atresia
Tetralogy of Fallot
Total Anomalous Venous Pulmonary Return (TAPVR)
Pulmonary Atresia
66
What is the most common form of cyanotic CHD in the postinfancy period?
Tetralogy of Fallot
67
What 4 anomalies constitute Tetralogy of Fallot?
Right Ventricular Outflow Obstruction (RVOTO)
- aka "Pulmonary Stenosis"
VSD
Overriding Aorta
Right Ventricular Hypertrophy (RVH)
68
What dictates the degree of shunting in ToF? What's the difference between minimal, mild, and severe obstruction?
severity of RVOT obstruction dictates shunting
Minimal - inc. flow with PVR dec; eventual CHF
Mild - hemodynamic balance pressure between ventricles equal = NO SHUNTING ("pink tet")
Severe - blood cannot exit through RVOT, pulmonary blood flow dec. --\> cyanosis
69
What is Conotruncal Facies seen in DiGeorge Syndrome and ToF?
hypertelorism (wide-set eyes)
lateral displacement of inner canthi
flat nasal bridge
narrow palpebral fissures
ear anomalies
70
How does squatting help children with ToF?
desaturated blood gets trapped in LEs and SVR increases
RVOT remains fixed but squatting decreases R --\> L shunting, increase pulmonary blood flow, and inc. arterial saturation
71
Why is there a single S2 sound in ToF?
Pulmonary Stenosis
72
What is a "Tet Spell"? What age is it most commonly observed at?
most common: 2-6 months of age
- morning or after nap (low SVR)
Precipitating factors: stress, drugs that dec. SVR, hot baths, fever, exercise
- inc. CO with fixed RVOTO = inc. R--\>L shunting with inc. cyanosis
73
How is ToF diagnosed? (4)
- CXR
- "boot-shaped" heart
- dec. pulmonary vascular markings
- right aortic arch (25%)
74
What shunt is commonly used to treat ToF with severe pulmonary stenosis?
Blalock-Taussig shunt
- increases pulmonary blood flow by grafting the Subclavian or Carotid artery to the pulmonary artery
75
What four things must be repaired in order to completely resolve ToF?
1. VSD closure
2. relief of RVOTO
3. ligation of shunts
4. ASD/PFO closure
76
Without repair of ToF, what is the mortality rate at 3, 20, and 30 years?
3 years = 50% mortality
20 years = 90% mortality
30 years = 95% mortality
77
What is the most common cyanotic heart lesion in the newborn period?
Transposition of the Great Arteries
78
How does Transposition of the Great Arteries develop?
What is ESSENTIAL for survival of these patients?
primitive heart loops to the LEFT instead of the RIGHT
aorta originates from RV and pulmonary trunk originates from LV
(**_A_**orta is **_A_**nterior, **_P_**ulmonary trunk is **_P_**osterior)
there MUST be a mixing lesion SOMEWHERE (i.e VSD/ASD/PDA)
79
How does TGA (Transposition of the Great Arteries) present?
How does it appear on CXR?
CHF in the first week (PDA alone is not sufficient enough to allow adequate mixing in extrauterine environment)
CXR: "egg-shaped" heart with narrow mediastinum and cardiomegaly with increased pulmonary markings
80
How is TGA with IVS (intact ventricular septum) treated vs TGA with VSD?
IVS? - "ductal dependant" = needs PGE to keep PDA open
- early balloon atrial septostomy (allows blood mixing)
- Arterial Switch procedure definitive
VSD? - PA band to control inc. pulmonary blood flow
- Arterial Switch procedure definitive
\*\*Arterial Switch = reattach great arteries to their appropriate ventricles\*\*
81
What syndrome is Truncus Arteriosus associated with?
DiGeorge Syndrome
82
What is the most common congenital heart lesions in children?
Bicuspid Aortic Valve
83
What is the difference between a Type I, II, and III Truncus Arteriosis?
Type I - short common pulmonary trunk from right side of common trunk, just above truncal valve
Type II - pulmonary arteries arise directly from ascending aorta, from posterior surface
Type III - similar to Type II; PAs arise more laterally and distant from semilunar valves
84
How does Truncus Arteriosus present clinically?
CHF/cyanosis in the first week
- initially L--\>R shunting
- 2nd heart sound prominent and single due to single semilunar valve
- strong, bounding peripheral pulses
- often, systolic ejection click
85
How is Truncus Arteriosus treated?
surgery BEFORE significant pulmonary vascular disease (3-4 months of age)
- VSD surgically closed (valve on LV side)
- pulmonary arteries freed from truncus and attached to valved conduit = new pulmonary trunk (RASTELLI procedure)
86
What 4 conditions make up Hypoplastic Left Heart Syndrome (HLHS)?
1. aortic valve hypoplasia, stenosis, atresia (with or without mitral valve involvement)
2. hypoplasia of ascending aorta
3. LV hypoplasia or agenesis
4. mitral valve stenosis or atresia
\*\*result? = single RV providing blood to pulmonary system, the systemic circulation (PDA), and coronary system via retrograde flow across PDA\*\*
87
How does HLHS differ in utero vs at birth?
In utero = all systemic flow is DUCT DEPENDENT
- pulm resistance \> systemic resistance
- normal perfusion pressure maintained with R--\>L shunt through PDA and pulmonary resistance
Birth = PDA closes and SVR \> PVR
- PDA closes = dec. CO and systemic perfusion = METABOLIC ACIDOSIS
- PDA dependent until intervention is undertaken
88
What is the second most common congenital heart defect, presenting in the first week of life (and most common cause of death from CHD in the first month)?
Hypoplastic Left Heart Syndrome
89
How is Hypoplastic Left Heart Syndrome diagnosed? (2)
CXR: cardiomegaly with globular-shaped heart; inc. pulmonary vascular markings, pulmonary edema
Echo: DIAGNOSTIC; typically detected in utero
90
How does Hypoplastic Left Heart Syndrome present?
- pulses ranging from normal to absent (depending on ductal patency)
- hyperdynamic RV impulse
- single S2 of INCREASING intensity
- nonspecific LSB murmur
- skin with **grayish pallor**
91
What is the 3 stage surgical treatment of Hypoplastic Left Heart Syndrome?
1. Norwood - pulmonary trunk reconstructs hypoplastic aorta, and RV becomes functional LV
- pulmonary arteries connected but separated from heart
- subclavian arteries used to establish blood flow to pulmonary arteries
2. Glenn - superior vena cava connected to right Pulmonary Artery, restoring partial venous return to lungs
3. Fontan - inferior vena cava anastomosed to PAs, resulting in complete venous diversion from systemic circulation to the lungs
92
What is a Subendocardial Cushion Defect and what 3 conditions does it cause?
abnormal development of AV canal (endocardial cushion)
- VSD
- ostium primum ASD
- clefts in the mitral and tricuspid valves
93
What 3 conditions are Subendocardial Cushion Defects associated with?
1. Down Syndrome (30%)
2. asplenia
3. polysplenia syndromes
94
What does the ECG of a patient with Subendocardial Cushion Defects look like? (3)
Superior QRS axis with RVH
RBBB and LVH
prolonged PR interval
95
What is the most common congenital heart disease recognized in adults?
ASD
96
What is the difference between Secundum, Primum, and Sinus Venous ASD defects?
Secundum - most common (50-70%) = CENTRAL portion of atrial septum
Primum - (30%) = atrial LOWER margin and associated with mitral/tricuspid valves
Sinus Venous - (10%) = UPPER portion of atrial septum and often extends into superior vena cava
97
Why is widely split and fixed S2 a common feature of ASDs?
right-sided volume overload causes delayed closing of pulmonic valves
murmurs more likely to occur as patient ages
98
When do symptoms of CHF and pulmonary HTN occur in adults with ASD?
CHF - second decade
Pulmonary HTN - third decade
99
How are ASDs treated?
- nearly 90% close spontaneously, and 100% will lose if \< 3 mm
- ASDs \> 8 mm are unlikely to close spontaneously (use surgical or catheter closure = clamshell or umbrella device)
100
What causes the closure of Foramen Ovale?
increasing Left Atrial pressure causes the septae to press against each other
101
What is the most common congenital heart disorder?
VSD
102
How often does VSD occur out of 1000 live births?
2
103
How often does spontaneous closure occur in VSDs?
30-50% of the time
104
How is VSD treated (if not closed)?
intervention based on development of CHF, pulmonary HTN, and growth failure
Initially: diuretics and digitalis
Medical therapy fails? = SURGERY
\*\***don't forget endocarditis prophylaxis**\*\*
105
How long does it typically take a PDA murmur to disappear in a newborn infant?
12 hours
106
What is the process by which the PDA closes? What pulmonary vascular pressure is required?
- ductus arteriosus primarily closes in response to **ductal PO2 \> 50 mmHg**
- PDA closes within the first 15 hours of life with complete closure by 3 weeks of life (Ligamentum Arteriosum)
107
What is the most common complication of PDA in late childhood?
Infective Endocarditis
- subacute bacterial endocarditis more common in small PDAs vs large PDAs
108
If Eisenmonger Syndrome develops in a patient with PDA, what kind of cyanosis would be seen?
cyanosis restricted to the **LOWER extremities** as oxygen poor blood mixes into aorta distal to subclavian arteries
109
What is an occasional complication of surgical ligation of PDAs?
recurrent **LARYNGEAL** nerve injury = HOARSENESS
110
What are 6 common PDA-dependent congenital heart abnormalities?
1. Tetralogy of Fallot
2. Tricuspid Atresia
3. TAPVR with obstruction
4. severe Aortic Coarctation
5. Pulmonic Atresia
6. Hypoplastic Left Heart Syndrome
111
CHD presenting in the first 2-3 weeks of life is usually due to what?
Ductal-DEPENDANT lesions
112
PGE1 administration
What dosage is used to reopen the ducts and to maintain ductal patency?
0.5-1.0 mg/kg/min = reopen ductus
0.01 mcg/kg/min = maintain ductal patency
113
What are the most common symptoms associated with PGE1 administration?
Apnea - endotracheal intubation prior to transport
fever, hypotension (vasodilator), and seizures
114
Name 5 congenital valvular defects
1. Tricuspid Atresia
2. Pulmonary Atresia
3. Aortic Insufficiency
4. Mitral Stenosis
5. Mitral Valve Prolapse
115
Tricuspid Atresia
Signs/Symptoms, Diagnosis, and Treatment
S/S = LV impulse displaced laterally
Dx: ECG = LVH, prominent LV forces (dec. RV voltages)
Tx: PGE1 (ductal patency), surgical intervention (modified Blalock-Taussig), Glenn then Fontan
116
Pulmonary Atresia (with intact ventricular septum; IVS)
How does it present?
cyanosis within hours of birth (PDA closing)
hypotension, tachycardia, acidosis
single S2 with holosystolic murmur (tricuspid regurgitation)
117
Pulmonary Atresia (with intact ventricular septum; IVS)
How is it diagnosed and treated?
Dx: ECG = dec. RV forces and occasional RVH, CXR = normal-enlarged RV with dec. pulmonary vascular markings
Tx: PGE1 (ductal patency), BAS (sometimes; balloon atrial septostomy), reconstruction of RVOT (transannular patch or pulmonary valvotomy), ASD left open
118
How many congenitally stenoic aortic valves are bicuspid?
85%
119
What kind of pain do older children with Aortic Stenosis complain of?
chest or stomach (epigastric) pain
120
What kind of sounds can be auscultated on a patient with Aortic Stenosis?
1. crescendo-decrescendo systolic murmur
2. systolic ejection click
\*\*severe Dz = paradoxical S2 splitting that narrows with inspiration\*\*
121
What treatment is available for Aortic Stenosis? When is Valvotomy typically indicated?
Tx: surgical/interventional balloon, valvotomy, valve replacement
- valvotomy is most common intervention; indicated if catheterization gradient is \> 50 mmHg
- valve replacement typically deferred until patient completes growth
122
How is Aortic Insufficiency diagnosed?
CXR showing LV enlargement and dilation of ascending aorta
123
How is Aortic Insufficiency treated?
surgery or balloon valvuloplasty to treat stenosis may WORSEN the insufficency
- only definitive therapy is aortic valve replacement
124
Signs and Symptoms of Aortic Insufficiency
- diastolic, decrescendo murmur at LUSB
- chest pain and CHF are ominous signs (presentation with symptoms indicates advanced disease)
125
Listening at the apex of the heart, what is the difference in etiology between an opening snap and a mid-systolic "click"?
Snap = mitral STENOSIS
Click = mitral PROLAPSE
126
What syndrome is Coarctation of the Aorta associated with?
Turner Syndrome
127
While rare in children, what is mitral stenosis typically a sequale of, especially if vaccination status is unknown?
Acute Rheumatic Fever
128
What is the most common symptom of Mitral Stenosis and what can be seen on CXR?
Sx: Dyspnea
CXR: interstital edema (pulmonary venous congestion)
\*\*hemoptysis can also be seen due to small bronchial vessel rupture\*\*
129
How is Mitral Stenosis treated? (2)
1. balloon valvuloplasty
2. surgery (commissurotomy/valve replacement)
130
What heart disease do nearly all patients with Marfan syndrome have?
Mitral Valve Prolapse
131
What is Mitral Valve Prolapse caused by and how is it commonly treated?
caused by thick and redundant valve leaflets that bulge into the mitral annulus
Tx: symptomatic (BB for chest pain)
132
How long does Failure to Thrive, Respiratory Distress, and CHF take to develop in a child with Coarctation of the Aorta?
What develops as the lower extremity receives insufficient blood supply?
2-3 months
**_acidosis_** may develop as lower extremity receives significantly less blood supply than upper extremity
133
In a symptomatic child with CoA, where is the murmur most commonly heard?
Left back
134
What CXR finding can help diagnose Coarctation of the Aorta?
"3 Sign" and dilated ascending aorta that displaces the SVC to the right
"3 Sign" --\> made at section that is narrowed
135
How is Coarctation of the Aorta treated?
Initial Tx: resection of coarctation with end-to-end anastomosis
Allograft augmentation can also be considered
Catheter balloon dilation can be used **_BUT_** has higher rate or restenosis and inc. risk of aortic aneurysms
136
What is Ebstein's Anomaly? (3 parts)
1. tricuspid valve displaced **_APICALLY_** in RV
2. valve leaflets redundant and plastered against ventricular wall = functional tricuspid atresia
3. RA frequently **_LARGEST_** structure
137
Without intervention, what is the outlook for patient's with Ebstein's Anomaly?
CHF in first 6 months with nearly 50% mortality rate
138
How does Ebstein's Anomaly present?
normal growth/development are possible, but older pts complain of dyspnea, cyanosis, and palpitations
widely split S1, fixed split S2, variable S3/S4
holosystolic LSB murmur
opening snap and cyanosis (R--\>L shunt)
139
What is seen on CXR and ECG for a patient with Ebstein's Anomaly?
What imaging is diagnostic for this disease?
CXR = cardiomegaly ("balloon-shaped") w/"wall-to-wall" heart in severely affected children
ECG = right-axis deviation, RA enlargement, RBBB (**_WPW in 20%_** due to accessory pathways developing **_due to increased surface area_**)
**ECHO is DIAGNOSTIC**
140
How is Ebstein's Anomaly treated?
Glenn procedure = inc. pulmonary blood flow (passive venous flow to pulmonary artery)
possible aortopulmonary shunt (severely affected)
tricuspid valve replacement/reconstruction
right atrial reduction surgery
ablation of accessory pathways
141
What is Totaly Anomalous Pulmonary Venous Return (TAPVR)?
pulmonary veins bring blood from lungs to the right atrium instead of the left atrium
NO CONNECTION exists between the pulmonary veins and left atrium and all PV's drain to a common vein
142
Where does the Common Vein in TAPVR most common drain to in order of most common to least? (4)
1. Right SVC (50%)
2. Coronary sinus or Right atrium (20%)
3. Portal vein or IVC (20%)
4. combination of above types (10%)
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What is the most important thing to insure survival in a patient with TAPVR?
ASD
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What is the difference between TAPVR with Obstruction vs TAPVR without Obstruction?
Obstruction = inc. pulmonary artery pressure (pulmonary edema) due to PVR in response to excessive volume being pumped into lungs from RV
No Obstruction = free communication between right atrium and left atrium (large R--\>L shunt)
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How does TAPVR with Obstruction present and how is it treated?
inc. pulmonary pressure = inc. RV and eventually RA pressures (R--\> shunt and cyanosis)
early/severe respiratory distress and cyanosis, no murmur, hepatomegaly
CXR = normal-size heart/pulmonary edema (ECHO diagnostic)
**Tx: BAS or immediate corrective surgery**
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How does TAPVR without Obstruction present and how is it treated?
presents later in life, with mild FTT, recurrent pulmonary infections, tachypnea, right heart failure, and rarely cyanosis
CXR = cardiomegaly, large PAs, inc. pulmonary vascular markings ("snowman" or "figure 8" sign) infants \> 4 months old
**Tx: surgical movement of pulmonary veins to left atrium**
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What is Hypertrophic Obstructive Cardiomyopathy and how is inherited?
asymmetrical septal hypertrophy or idiopathic hypertrophic subaortic stenosis (IHSS) is most common
Inheritance = AutoDom (60%) or sporadic (40%)
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What is the incidence of Sudden Death in pts with HOCM?
**4-6%**
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What affects the murmur in patients with HOCM and what is seen on ECG/ECHO?
**Murmur** = systolic ejection murmur best heard with maneuvers that ***_DECREASE PRELOAD (valsalva/squatting)_*** --\> dec. preload decreases ventricular volume = hypertrophic septum comes closer to aortic outflow tract = inc. turbulence
\*\*murmur DECREASES with INCREASED preload --\> larger ventricular volume expands outflow tract causing less turbulent flow\*\*
**ECG**: LVH, LA enlargement, large Q wave (septal hypertrophy)
**Echo**: asymmetrical septal hypertrophy, outflow obstruction
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What does a large Q wave indicate on ECG?
septal hypertrophy
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How is Hypertrophic Obstructive Cardiomyopathy treated? (3)
1. moderate physical activity restriction
2. BB or CCB to improve filling
3. endocarditis prophylaxis
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Henoch-Schonlein Purpura is caused by what?
What 3 areas does it commonly affect?
When does it most often occur?
immune-mediated vasculitis affecting **GI tract, joints, and kidneys** with characteristic rash
occurs most often in **WINTER** months, **_following Group A strep URI_**
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How does Henoch-Scholein Purpura affect the GI tract and kidneys?
How is it treated?
**GI** = vomiting, upper/lower GI bleeding, gut wall hematoma cause intussussception
**Renal** = glomerulonephritis with RBC casts --\> acute renal failure (1-2%); more commonly chronic proteinuria
**Tx**: supportive with full recovery in 4-6 weeks
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What is Kawasaki Disease and who does it most commonly affect?
acute vasculitis of MEDIUM-sized arteries of unknown etiology
mostly affects infants and children (\>80% under 4 yo), Asians, males
\*\*most common in Winter and Spring\*\*
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What is Kawasaki Disease also known as?
Mucocutaneous Lymph Node Syndrome
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What is the most common acquired heart disease in children?
Kawasaki Disease
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Diagnostic Critera for Kawasaki Disease
Fever for \> 5 days + 4 of the following 5 things
1. bilateral conjunctivitis (no exudate)
2. mucocutaneous lesions ("strawberry tongue", dry/cracked red lips, diffuse erythema of oral cavity)
3. erythema/edema of hands/feets
4. polymorphic rash (truncal)
5. cervical lymphadenopathy (\>1.5 cm diameter); usually unilateral
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What is the dosage for IVIG and ASA in children with Kawasaki Disease?
**IVIG** = usually 1 dose, 2g/kg over 10-12 hrs (dec. coronary artery dilation by \>90%)
**ASA** = high-dose (80-100 mg/kg/day QID) until 48-72 hrs after defervescence
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Difference in ASA use if coronary artery abnormalities present vs not being present?
**(+) coronary artery abnormalities** = continue indefinitly in consultation with pediatric cardiologist (reduced after afebril for 48 hrs)
**(-) coronary artery abnormalities** = low-dose ASA (3-5 mg/kg/day daily) for 6-8 weeks, or until platelet/ESR normal
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What is Polyarteritis Nodosa?
necrotizing inflammation of **_SMALL/MEDIUM_** muscular arteries
involves renal/visceral vessels and spares pulmonary circuation
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How does Polyarteritis Nodosa present and how is it diagnosed?
**Sxs**: prolonged fever, WL, malaise, subQ nodules on extremities; Livedo reticularis; waxes and wanes; distal gangrene (severe)
**Dx**: no Dx tests; p-ANCA (+), thrombocytosis/leukocytosis/proteinuria/hematuria; medium-sized vessel aneurysms; **_Echo of coronary artery aneurysms is DIAGNOSTIC_** with other clinical evidence
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How is Polyarteritis Nodosa treated? (2)
1. **corticosteroids** suppress clinical manifestations
2. **Cyclophosphamide/Azathioprine** induces remission
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What is Takayasu's Arteritis?
"aortoarteritis" --\> large vessel vasculitis of unknown cause involving aorta, aorta's branches, and pulmonary vasculature
lesions are segmental and obliterative; aneurysmal and saccular dilations occur
\*\***thoracoabdominal aorta** predominant site in children\*\*
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Who does Takayasu's Arteritis most commonly affect?
females age 4-45 yo
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What physical exam finding might help you determine a patient has Takayasu's Arteritis?
How is this diease treated?
check peripheral pulses = "pulseless disease" due to absent, decreased, or unequal peripheral arterial pulses
**Tx**: corticosteroids to induce remission
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