Sie sind auf Seite 1von 7

Pediatric EOR Study Guide - Cardiovascular

Patent Ductus Arteriosus (PDA) Coarctation of Aorta (CoA) Atrial Septal Defect (ASD)
Result of a failure to close after Pathologic narrowing of the aortic lumen that can occur anywhere along ASD is the 2nd MCC of CHD, after VSD
birth  persistent connection b/t its length. Usually noncyanotic. Patho: Opening in the atrial septum caused by excessive
thoracic aorta and pulmonary RF: M:F = 2:1. Suspect in child with 2ry hypertension, bilat. LE resorption around the foramen secundum or hypoplastic growth
trunk claudication of septum secundum.
RF: M:F = 1:3, premature infants Types: preductal (occurs in fetal life, CHF shortly after birth) and RF: M:F = 1:2
at higher risk, and frequently postductal (occurs postnatally), each type also has a type where the duct Types: ostium secundum (MC, 80%), ostium primum
associated with maternal rubella is open or closed = 4 types total (associated w/ mitral regurg), sinus venosus (at roof of atrium,
infection during 1st trimester. MC observed distal to the left subclavian artery d/t the ductus coronary sinus)
Can be life-threatening or life contraction pinching the aorta too. 70% also have a bicuspid aortic S&S: asx or minimal in childhood until >30y. In infants/young
saving. valve.* children see recurrent resperatory infections, FFT, exertional
Embryology: PDA closes Patho: increased LV afterload with SNS and RAAS  HTN, LVH, dyspnea. In Adolescents/adults see exertional dysnea, easy
normally d/t increase in O2 when CHF fatigability, palpations, atrial arrhythmias, HF. Can have
the lungs inflate on 1st breath, PE: systolic murmur that radiates to the back/scapula chest*. BP paroxysmal embolus  stroke. Most infants are aSx, detected
stim. by NSAIDs, inhibited by increased in the upper > lower extremities. bya a murmur when shool age, MC sx is palpitations.
PGs Delayed/weak femoral pulses d/t decreased flow distal to obstruction in PE: systolic ejection crescendo-decrescendo flow murmur
PE: small = aSx, large = large the LE at pulmonic area (left upper sternal border). Sounds like
L R shunt (noncyanotic) CXR: rib notching, increased collateral circulation via intercostal PS *functiona flow murmur). Widely split S2 that does not
resulting in CHF Sx in newborns, arteries. “3 sign” d/t narrowed aorta looks like notch of the #3 vary with resperations*. Loud S2, hyperdynamic RV
continuous machinery murmur* Angiogram: GS* CXR: cardiomegaly
Wide pulse pressure  bounding Tx: surgical, balloon angio + stent, PGE1 preoperatively reduces ECG: incomplete RBB and crochetage sign (notching of the R
peripheral pulses* symptoms and improves LE blood flow wave iin inferior leads
Imaging: CXR: normal or Echo: GS
cardiomegaly, ECH: LVH, left Tetralogy of Fallot Tx: sponataneous closure likely in 1st year, if not surg if
atrial enlargement, Echo: GS Cyanotic CHD (R  L shunt)* is the MC cyanotic CHD, “Blue Baby symptomatic about 2-4y
Tx: IV indomethacin 1st line to Syndrome”
close in infants (PG inhibitor) Group of malformations: RV outflow obstruction (pulmonary artery
stenosis), RVH, VSD (large unrestrictive), and overriding aorta = 3
Possible complication: structural defects and 1 acquired
Eisenmenger’s syndrome when S&S: exertional dyspnea and cyanosis that worsens w/ age. “tet-spells”
pulmonary HTN  L to R shunt paroxysms of cyanosis where older kids relieve spells by squatting.
switches and becomes a cyanotic Possible Eisenmenger’s syndrome
R to L shunt, will have normal Embryology: the aorta got greedy and went on top of the pulmonary
upper extremities with cyanotic trunk
lower extremities PE: harsh holosystolic murmur @ left upper sternal border*
(sounds like PS), right ventricular heave, digital clubbing
CXR: boot shaped heart (d/t prominent RV
ECH: Right ventricular hypertrophy* and right atrial enlargement
Echo: GS
TX: surgical repair in first 4-12m, PGE1 infusion prevents ductal
closure if patient is cyanotic prior to surg
Pediatric EOR Study Guide - Cardiovascular

The test:
 10% of total test
 Breakdown: 1 H&P, 1 Diagnostic Study, 2 Diagnosis, 1 Health Maintenance, 1 Clinical Intervention, 2 Clinical Therapeutics, 2 Scientific
Concepts

Ventricular Septal Defect (VSD)


MC defect , 30% of CHD
Abnormal opening in the ventricular septum.
Embryology: normally the ventricular septum grows up from bottom of ventricles to reach the atrial septum and fuse
Types:
1. Perimembranous: MC (80%). Hole in LV outflow near the tricuspid valve
2. Muscular: 5-20%. Usually multiple holes in a “swiss cheese” pattern
3. Inlet (posterior): 10%. Located posterior to the septal leaflet of the tricuspid valve
4. Supracristal (outlet): 5%. Beneath the pulmonic valve. +/- aortic valve insufficiency
Patho: L  R shunt
Clinical Manifestations:
1. Small (restrictive) VSD: asx or mild. MC found incidentally d/t murmur. May close w/ time spontaneously (80% muscular, 40%
perimembranous). Restrictive = normal pressure b/t ventricles is maintained* if defect is <0.5cm2
2. Moderate VSD: excessive sweating or fatigue especially during feeds (seen d/t increased SNS activity from decreased CO), fatigue with
feed, lack of adequate growth, frequent respiratory infections
3. Larger VSD: more severe symptoms than moderate. Non-restrictive = no pressure difference
4. Eisenmenger’s syndrome: in nonrestrictive the blood takes the path of least resistance (flows to R side). Over time the pulmonary pressure
becomes > systemic pressure and there is a R to L shunt. Asymptomatic at rest but +/- cyanosis, exertional dyspnea, chest pain, and syncope
PE: loud high-pitched harsh holosystolic murmur at LLSB*, classic isolated VSD is not usually associated with cyanosis.
Moderate: +/- thrill, diastolic rubble at the mitral area (d/t increased flow across the valve).
Large: signs of CHF (tachycardia, tachypnea, rales, cardiomegaly)

Dx: CXR: varies, may show left atrial enlargement, RVH. Echo is preferred over catheterization as it determines size and location of VSD. ECH:
LVH with mild to moderate VSD, normal with small VSD, +/- combined RVH/LVH (large equiphasic waves >50% in precordial leads  Katz
Wachtel phenomenon) LAE/RAE. MRI only if echo non diagnostic. Catheterization if all other tests are nondiagnostic or there is pulmonary HTN

Management: restrictive VSD (L>R sided pressure) is associated with good prognosis
1. Most small VSD will close spontaneously w/in 10y
2. Surgery: patch closure if sx infants or uncontrolled CHF, growth delay, recurrent respiratory infections. Larger shunts repaired by age of 2y to
prevent pulmonary HTN.
Pediatric EOR Study Guide - Cardiovascular

Hypertrophic cardiomyopathy
Inherited genetic disorder of inappropriate LV and/or RV hypertrophy (especially septal)

Patho: subaortic outflow obstruction  narrowed LB outflow tract 2/2/ 1. Hypertrophied septum and 2. Systolic anterior motion (SAM) of the mitral valve and
papillary muscle displacement. Increased SAM is seen with increased contractility (Digoxin, beta agonist, exercise) and decreased LV volume (decreased venous return,
dehydration, Valsalva). Also see diastolic dysfunction d/t stiff chamber  impaired ventricular relaxation/filling (bc thick walls lead to smaller LV volume and decreased
filling)

Clinical Manifestations: (often asymptomatic, first sx may be sudden cardiac death)


Pediatric EOR Study Guide - Cardiovascular

1. Dyspnea – MC initial complaint (90%). Fatigue.


2. Angina pectoris (chest pain) (75%) - Usually in the setting of a normal angiogram
3. Syncope – includes pre syncope and dizziness (d/t inadequate CO on exertion)
4. Arrhythmias – A fib; VT/VF (palpitations, syncope, sudden cardiac death)
5. Sudden cardiac death – especially see in adolescent/preadolescent children (especially during times of extreme
exertion). Usually d/t ventricular fibrillation*

PE: harsh systolic crescendo-decrescendo murmur best heard at LLSB (similar to AS)*
 Decrease murmur intensity: handgrip, increased venous return (squatting, lying supine) b/c increase LV volume
preserves outflow and increased fluid pushes septum out of the way and decreases SAM of mitral valve
 Increase murmur intensity: decreased venous return (Valsalva and standing)
 Usually no carotid radiation* May have loud S4, mitral regurgitation, S3 or pulsus bisferiens
Dx:
 Echo: asymmetrical wall thickness (esp septal) >/=15mm, systolic
anterior motion of mitral valve, small LV chamber size, dynamic
outflow obstruction,+/- mitral regurgitation
 ECG: LVH, atrial enlargement, anterolateral and inferior pseudo q
waves
 CXR: cardiomegaly

Management
 Focus on early detection, medical management, surgical and or ICD
placement
 Counseling to avoid dehydration and extreme exertion/exercise very
important
o Medical: beta-blockers are 1st line*, CCB (verapamil),
Disopyramide. All 3 are negative intropes that increase
ventricular diastolic filling time. Continuous use of Digoxin,
nitrates, and diuretics* (Digoxin increases contractility,
Ntrates and diuretics decrease LV volume)
o Surgical (Myomectomy)
o Alcohol Septal Ablation: ethanol destroys extra myocardial
tissue
Secondary HCM: d/t longstanding hypertension, aortic stenosis possible however that is not true HCM
Pediatric EOR Study Guide - Cardiovascular

Acute rheumatic fever


Acute autoimmune inflammatory multi-system illness mainly affecting children 5-15y*

Patho: symptomatic or asx infection w/ GABHS (strep pyogenes)* stimulates antibody production
to host tissues and damages organs diretly. The infection usually precedes the onset of rheumatic
fever by 2-6w

Complications: rheumatic valvular disease: mitral (75-80%), aortic (30%), tricuspid and pulmonic
(5)

Major criteria
1. Polyarthritis: (75%) 2 or more joints affected (simultaneous more diagnostic) or migratory
(lower  upper joints). Medium large joints MC affected (knees, hips, wrists, elbows,
shoulders). Heat, redness, swelling, severe joint tenderness must be present. Joint pain
(arthralgia) w/out other sx doesn’t classify as major. Usually lasts 3-4w.
2. Active Carditis: (40-60%) can affect valves (especially mitral and aortic), myocardium (myocarditis) and or pericardium (pericarditis).
Carditis confers greater morbidity and mortality.
3. Sydenham’s Chorea: (<10%) “Saint Vitus dance” may occur 1-8m after initial infection. Manifestations include sudden involuntary, jerky,
non-rhythmic, purposeless movements especially involving the head/arms. Usually resolves spontaneously. MC in females.
4. Erythema Marginatum: often accompanies carditis. Macular, erythematous, non-pruritic annular rash with rounded sharply demarcated
borders (may have central clearing). MC seen primarily on the trunk and extremities (not on the face). Crops last hours-days before
disappearing
5. Subcutaneous Nodules: (rare). Seen over joints (extensor surfaces), scalp and spinal column
6. Other not associated w/ Jones: abdominal pain, facial tics/grimaces, epistaxis.

Treatment:
 Anti-inflammatory: Asprin (2-6w w/
taper) +/- corticosteroids in severe cases
and carditis
 Pen G abx of choice (or Erythromycin if
PCN-allergic) both in acute phase and
after acute episode. Prevention is the most
important therapeutic course. Therefore all
patients (even if presenting with acute
rheumatic fever) should be treated with
abx)
Pediatric EOR Study Guide - Cardiovascular

Kawasaki disease
Pediatric EOR Study Guide - Cardiovascular

Syncope
Structural heart: ASD, CoA, myocarditis, hypertrophic
cardiomyopathy, aortic stenosis (3y survival)
Merck:
 Syncope results from global CNS dysfunction, usually from insufficient
cerebral blood flow.
 Most syncope results from benign causes.
 Some less common causes involve cardiac arrhythmia or outflow
obstruction and are serious or potentially fatal.
 Vasovagal syncope usually has an apparent trigger, warning symptoms, and
a few minutes or longer of postrecovery symptoms.
 Syncope due to cardiac arrhythmias typically occurs abruptly and with
quick recovery.
 Seizures have a prolonged (eg, hours) recovery period.
 If a benign etiology is not clear, driving and use of machinery should be
prohibited until the etiology is determined and treated—the next
manifestation of an unrecognized cardiac cause may be fatal.