Pediatric Cardiology

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jknell
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226173
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Pediatric Cardiology
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2013-07-08 00:10:24
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Pediatrics
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Pediatric Cardiology
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  1. Important Physical Exam
    • -weight and height (growth)
    • -vital signs
    • -cyanosis
    • -clubbing
    • -pulses compared in upper and lower extremeties
    • -palpate chest
    • -auscultate
  2. Concerning Physical Exam Findings
    • -heaves, thrills
    • -brachiofemoral delay
    • -abnormal S1 or S2
    • -abnormal splitting
    • -extra heart sounds
    • -ejection click
    • -opening snap
    • -pericardial rub
    • -murmurs (harsh/loud/blowing, doesn't change intensity relative to patient position)
  3. Cyanosis in a Neonate
    -Hb concentration
    -conditions that exacerbate cyanosis
    -acrocyanosis
    -origins of cyanosis
    -not clinically evident until absolute concentration of deoxygenated hemoglobin is at least 3.5g/dL

    • Evident Sooner Under Certain Conditions:
    • -high Hb concentration
    • -decreased pH
    • -increased PCO2
    • -increased temperature
    • -increased ratio of adult:fetal Hb

    • Acrocyanosis:
    • -blueness of extremities only
    • -caused by peripheral vasoconstriction (normal during 24-48hrs)

    • Origins of Cyanosis:
    • -cardiac
    • -pulmonary
    • -neurologic
    • -hematologic
  4. Preductal vs Postductal Oxygen Saturation
    -differential cyanosis
    -reverse differential cyanosis
    • Preductal Saturation:
    • -right upper extremity

    • Postductal Saturation:
    • -lower extremity

    • Differential Cyanosis:
    • -preductal saturation > postductal saturation
    • -Persistent pulmonary HTN of newborn (PPHN)
    • -LV outflow tract obstructive lesions (coarctation of the aorta, aortic stenosis)

    • Reverse Differential Cyanosis:
    • -preductal saturation < postductal saturation
    • -transposition of great arteries with PPHN or coarctation of the aorta
  5. Hyperoxia Test
    • -baseline preductal ABG on room air (FIO2 = 0.21)
    • -repeat on 100% O2 (FIO2 = 1.00)

    -PaO2 > 250 mmHg on 100% O2 essentially rules out cardiac disease (→ pulmonary cause of stenosis)
  6. Cyanotic Congenital Heart Disease
    • DUCTAL-INDEPENDENT MIXING LESIONS
    •      1. Truncus Arteriosis
    •      2. D-Transposition of Great Arteries
    •      3. Total Anomalous Pulmonary Venous Connection (TAPVR)

    • LESIONS WITH DUCTAL-DEPENDENT PULMONARY BLOOD FLOW:
    •      1. Tricuspid atresia
    •      2. Tetralogy of fallot
    •      3. Ebstein anomaly

    • LESIONS WITH DUCTAL-DEPENDENT SYSTEMIC BLOOD FLOW:
    •      1. Hypoplastic Left Heart Syndrome
    •      2. Interrupted Aortic Arch
  7. Truncus Arteriosus
    • EPIDEMIOLOGY:
    • -rare
    • -associated with 22q11 microdeletion (DiGeorge)

    • PATHOPHYSIOLOGY:
    • -single arterial vessel arising from the base of the heart
    • -number of valve leaflets varies from 2-6
    • -valve may be insufficient or stenotic
    • -VSD always present
    • *complete mixing of systemic and pulmonary venous blood

    • CLINICAL MANIFESTATION:
    • -varies depending on the amount of pulmonary blood flow
    • -non-specific murmur/minimal cyanosis may be present at birth
    • -CHF develops in weeks (PVR falls and pulmonary blood flow increases at the expense of systemic blood flow)
    • -Murmur: SEM at LSB, loud ejection click and single heart sound S2
    • -widened pulse pressure
    • -bounding arterial pulses
    • -CXR: mild cardiomegaly, increased pulmonary vasculature, 30% R aortic arch
    • -EKG: biventricular hypertrophy

    • TREATMENT:
    • -surgery in neonatal period
  8. D-Transposition of Great Arteries
    • EPIDEMIOLOGY:
    • -5% of CHD
    • -most common form of CHD presenting in first 24 HOL
    • -3:1 male predominance

    • PATHOPHYSIOLOGY:
    • -aorta arises from RV, PA arises from LV
    • -pulmonary and systemic circuits in parallel
    • -required PFO

    • CLINICAL MANIFESTATION:
    • -cyanosis present from birth
    • -tachypnea
    • -Murmur: loud single S2, systolic murmur (VSD)
    • -CXR: mild cardiomegaly ("egg-shaped"), increased pulmonary vascular markings
    • -EKG: RAD, RVH

    • TREATMENT:
    • -PGE1
    • -balloon atrial septostomy (Rashkind procedure)
    • -arterial switch procedure during first week of life
  9. Total Anomalous Pulmonary Venous Connection (TAPVR)
    • EPIDEMIOLOGY:
    • -rare (1-2% of CHD)

    • PATHOPHYSIOLOGY:
    • -pulmonary veins not connected to LA (confluence behind LA then drain into RA)
    • 1. Supracardiac (SVC or innominate vien)
    • 2. Cardiac (coronary sinus or RA)
    • 3. Infradiaphragmatic (portal or hepatic vein)
    • 4. Mixed
    • -with obstruction when vein enters at acute angle
    • *ASD or PFO is required

    • CLINICAL MANIFESTATIONS W/O OBSTRUCTION:
    • -RV heave
    • -wide, fixed split S2 with loud pulmonary component
    • -Murmur: systolic ejection murmur at LUSB
    • -CXR: cardiomegaly ("snowman"), increased pulmonary vascularity
    • -EKG: RAD, RVH

    • CLINICAL MANIFESTATIONS W/ OBSTRUCTION:
    • -marked cyanosis and respiratory distress, tachypnea
    • -loud, single S2
    • -CXR: normal heart size, markedly increased pulmonary vascularity, diffuse PE
    • -EKG: RVH

    • TREATMENT:
    • -surgery as newborn if obstruction is present
  10. Tricuspid Atresia
    • EPIDEMIOLOGY:
    • -rare (<1% CHD)

    • PATHOPHYSIOLOGY:
    • -complete atresia of tricuspid valve
    • -leads to severe hypoplasia or absence of RV
    • 1. With normally related great arteries (NRGA)
    • 2. With transposition of great arteries (TGA)
    • -90% have VSD
    • -L heart handles both systemic and pulmonary venous return
    • -NRGA usually have pulmonary stenosis

    • CLINICAL MANIFESTATIONS:
    • -depend on degree of pulmonary stenosis (most have significant)
    • -progressive cyanosis, poor feeding, tachypnea (in first 2 weeks)
    • -Murmur: holosystolic (VSD), continuous (PDA)
    • -CXR: normal heart size, decreased pulmonary vascular markings
    • -EKG: LAD, RAH, LVH
    • *with TGA: shock when DA closes

    • TREATMENT:
    • -PGE1 to maintain PDA
    • -Blalock-Taussig shunt (maintain pulmonary blood flow)
    • -hemi-Fontan/bidirectional Glenn (cavopulmonary anastomosis)
    • -Fontan: IVC and hepatic vein flow into pulmonary circulation
  11. Tetralogy of Fallot
    • EPIDEMIOLOGY:
    • -most common CHD in childhood (10%)
    • -22q11 microdeletion

    • PATHOPHYSIOLOGY:
    • 1. VSD
    • 2. Pulmonary valve stenosis
    • 3. RVH
    • 4. "overriding" aorta
    • -R to L shunt across  VSD → cyanosis

    • CLINICAL MANIFESTATION:
    • -degree of pulmonic stenosis determines the timing and severity of cyanosis (hours after birth to later infancy)
    • -RV heave
    • -Murmur: loud systolic ejection murmur at LUSB
    • -CXR: normal heart size ("bootshaped"), decreased pulmonary vascular markings
    • -EKG: RAD, RVH

    • "tet spells":
    • -cyanosis, rapid breathing, agitation
    • -caused by increased RV outflow resistance
    • -last mins to hrs
    • -may resolve spontaneously or lead to hypoxia/metabolic acidosis/death

    • TREATMENT:
    • 1. Increase SVR
    • 2. Decrease PVR
    • 3. Increase Preload (squatting)
    • -calm patient, vagal manuevers
    • -supplemental O2 (pulmonary vasodilator)
    • -morphine sulfate (minimize O2 consumption)
    • -volume expansion
    • -vasoconstrictors
    • -β-blockers (decrease infudibular spasm)
    • -sodium bicarb (reduce acidosis, decrease PVR)
    • -surgery: 3-6 months or after first tet spell
  12. Ebstein Anomaly
    • EPIDEMIOLOGY:
    • -extremely rare
    • -associated with maternal Li use

    • PATHOPHYSIOLOGY:
    • -septal leaflet of tricuspid valve displaced inferiorly into RV, and anterior leaflet is "sail-like" and redundant
    • -RV into RA → functional hypoplasia of RV and tricuspid regurgitation
    • *majority of pulmonary blood flow from PDA
    • *PFO in 80% (R to L shunt at atrial level)
    • -dilated RA may lead to SVT (WPW)


    • CLINICAL MANIFESTATIONS:
    • -cyanosis and CHF in first few DOL
    • -widely fixed split S2, gallop rhythm
    • -Murmur: holosystolic murmur at LLSB
    • -CXR: extreme cardiomegaly with RAH, decreased pulmonary vascular markings
    • -EKG: RBBB, RAH

    • TREATMENT:
    • -PGE1 infusion
    • -avoid surgical intervention (tricuspid surgery has poor results)
    • -heart transplant
  13. Hypoplastic Left Heart Syndrome
    • EPIDEMIOLOGY:
    • -second most common CHD presenting in first week of life
    • -most common cause of death from CHD in first month of life

    • PATHOPHYSIOLOGY:
    • -hypoplasia of LV
    • -aortic valve stenosis or atresia
    • -mitral valve stenosis or atresia
    • -hypoplasia of ascending aorta
    • *Atrial defect
    • -systemic blood flow is completely ductal dependent

    • CLINICAL MANIFESTATIONS:
    • -shock when DA closes
    • -RV heave
    • -single S2
    • -Murmur: continuous (PDA)
    • -CXR: pulmonary edema and progressive cardiac enlargement
    • -EKG: RVH, poor R wave progression across precordial lead

    • TREATMENT:
    • -PGE1
    • -palliative procedure only, no corrective surgery
    • -Stage I: Norwood (combine PA and Ao, atrial septectomy, Blalock-Taussig shunt)
    • -Stage II: Bidirectional Glenn/Hemi-Fontan (cavopulmonary anastomosis, at 3-6 mos)
    • -Stage III: modified Fontan (2-5 yrs)
    • -Heart transplant
  14. Interrupted Aortic Arch
    • EPIDEMIOLOGY:
    • -associated with 22q11 microdeletion

    • PATHOPHYSIOLOGY:
    • -extreme form of coarctation of aorta
    • -Type A: after L subclavian artery
    • -Type B: between L subclavian and L common carotid
    • -Type C: between L common carotid and brachiocephalic artery
    • -systemic flow depends on PDA

    • CLINICAL MANIFESTATION:
    • -circulatory collapse as the ductus closes (similar to HLHS)

    • TREATMENT:
    • -PGE1 therapy
    • -Surgical end-to-end anastomosis of the aortic segments
  15. Acyanotic Congenital Heart Disease
    • INCREASED PULMONARY BLOOD FLOW (L to R)
    •      1. ASD
    •      2. VSD
    •      3. PDA
    •      4. Common AV canal

    • PULMONARY VENOUS HTN
    •      1. Coarctation of aorta
    •      2. Aortic valve stenosis

    • NORMAL/DECREASED PULMONARY BLOOD FLOW:
    •      1. Pulmonary valve stenosis
  16. Atrial Septal Defects
    • EPIDEMIOLOGY:
    • -8% of CHD
    • -2:1 female predominance

    • PATHOPHYSIOLOGY:
    • 1. Ostium Secundum (2°, midportion, most common)
    • 2. Ostium Primum (1°, lower portion)
    • 3. Sinus Venosus (junction of RA and SVC or IVC)
    • -degree of shunting depends on size of ASD and compliance of ventricles in diastole
    • -L to R shunting → R heart enlargement and increased pulmonary blood flow

    • CLINICAL MANIFESTATIONS:
    • -usually asymptomatic
    • -exercise intolerance
    • -paradoxical embolism
    • -SVT (atrial enlargement)
    • -RV heave
    • -loud S1
    • -widely "fixed" S2 split
    • -Murmur: systolic ejection murmur at LUSB, Mid-diastolic rumble at LRSB
    • -CXR: enlarged heart and main PA, increased pulmonary vasculature
    • -EKG: Secundum (RAD), Primum (extreme LAD)

    • TREATMENT:
    • -spontaneous closure of small secundum ASDs often occurs in first year
    • -transcatheter device closure (after 2 yrs)
    • -ostium primum and sinus venosus ASDs require surgery
    • -pericardial patch or suture closure
    • -Subacute bacterial endocarditis prophylaxis
  17. Ventricular Septal Defects
    • EPIDEMIOLOGY:
    • -most common congenital heart defect (25% of CHD)

    • PATHOPHYSIOLOGY:
    • 1. Muscular
    •      -most common
    •      -single or multiple
    • 2. Inlet
    •      -endocardial cushion defect
    •      -beneath septal leaflets of TC valve
    • 3. Conoseptal Hypoplasia
    •      -at outflow tract of RV beneath PV
    •      -membranous ventricular septum
    • 4. Conoventricular
    •      -most common type with muscular
    • 5. Malalignment
    •      -malalignment of infundibular septum
    •      -anterior malalignment → TOF
    •      -posterior malalignment → AS
    • -Restrictive (small): L to R flow, Normal PVR
    • -Nonrestrictive (large): LV = RV pressure, PVR and SVR determine shunt flow
    • -may lead to Eisenmenger syndrome

    • CLINICAL MANIFESTATIONS:
    • -depends on the size of the shunt
    • -large shunt: CHF, growth failure
    • -the smaller the defect the louder the murmur
    • -Murmur: harsh systolic murmur at mid to Lower LSB
    • -Eisenmenger: RV heave, PV ejection click, SEM, diastolic murmur, loud single S2

    • TREATMENT:
    • -most small VSDs close without intervention (40% by 3 years, 75% by 10 years)
    • -surgery unnecessary in small VSDs
    • -VSDs with large shunts require surgical closure before pulmonary vascular changes are irreversible
    • -Dacron patch closure
    • -transcatheter device placement
    • -SBE prophylaxis
  18. Common Atrioventricular Canal Defect
    • EPIDEMIOLOGY:
    • -5% of all CHD
    • -most commonly seen in Down Syndrome

    • PATHOPHYSIOLOGY:
    • -deficiency of the endocardial cushions
    • -ostium priumum ASD and inlet VSD with lack of septation of mitral and TC valves

    • 1. Incomplete CAVCD
    •      -CAVV leaflets attach to top of muscular ventricular septum
    •      -mitral valve is cleft (regurgitation)

    • 2. Complete CAVCD
    •      -CAVV not attached to muscular ventricular septum
    •      -large inlet VSD
    •      -L to R shunting at ASD and VSD
    •      -Pulmonary HTN develop over time

    • CLINICAL MANIFESTATIONS:
    • -same as ASD
    • -Murmur: blowing systolic murmur at LLSB and apex
    • -S2 is widely split and fixed
    • -CXR: cardiac enlargement, increased pulmonary vascularity
    • -EKG: superior axis

    • TREATMENT:
    • -CHF tx with digoxin, diuretics, ACEI
    • -complete usually surgically repaired during infancy (ASD, VSD repaired and CAVV divided into R and L)
    • -complete heart block occurs in 5% and residual mitral insufficiency is common
  19. Patent Ductus Arteriosus
    • EPIDEMIOLOGY:
    • -10% of CHD
    • -higher in premature neonates

    • PATHOPHYSIOLOGY:
    • -connects the underside of the aorta and the left PA distal to L subclavian
    • -direction of flow depends on SVR and PVR
    • -nonrestrictive (large): L to R shunt

    • CLINICAL MANIFESTATION:
    • -small PDA: no sx
    • -CHF
    • -FTT
    • -bounding pulses
    • -continuous murmur
    • -CXR: cardiomegaly, LA and LV enlargement, increased pulmonary vascular
    • -EKG: L or biventricular hypertrophy

    • TREATMENT:
    • -Indomethacin (closes PDA in preemie)
    • -coil embolization
    • -device closure
    • -surgical ligation
  20. Coarctation of Aorta
    • EPIDEMIOLOGY:
    • -8% of CHD
    • -2:1 male predominance
    • -in female consider Turner syndrome

    • PATHOPHYSIOLOGY:
    • -obstruction usually at the descending aorta at the insertion of the ductus arteriosus
    • -leads to increased LV afterload

    • CLINICAL MANIFESTATION:
    • -asymptomatic in 50%
    • -irritability, difficulty feeding, FTT
    • -may present with circulatory collapse when PDA closes
    • -weak femoral pulses
    • -upper extremity HTN
    • -murmur: non specific ejection murmur at apex
    • -CXR: enlarged aortic knob and cardiomegaly
    • -EKG: RVH in neonate, LVH in older pt

    • TREATMENT:
    • -PGE1
    • -surgery: end to end anastomosis, patch aortoplasty
    • -balloon dilation angioplasty
    • -restenosis commonly needed
    • -β-blockers in older children for persistent HTN
  21. Aortic Stenosis
    • PATHOPHYSIOLOGY:
    • -thickened valvular tissue, rigid, domed in systole
    • -commonly bicuspid valve
    • -LVH

    • CLINICAL MANIFESTATIONS:
    • -asymptomatic
    • -critical AS can have circulatory collapse if ductus closes
    • -Murmur: harsh systolic ejection murmur at RUSB, preceded by ejection click
    • *murmur increases with increased stenosis
    • *murmur softens when ventricular function is compromised
    • -CXR: cardiomegaly, pulmonary edema
    • -EKG: LVH, ischemic changes (ST depression, inverted T waves)

    • TREATMENT:
    • -PGE1 if ductal dependent
    • -balloon valvuloplasty (may result in progressive aortic regurgitation that requires valve replacement)
  22. Pulmonic Stenosis
    • EPIDEMIOLOGY:
    • -5-8% of CHD

    • PATHOPHYSIOLOGY:
    • - domed valve with small central opening
    • -poststenotic dilatation of the main pulmonary artery
    • -RVH occurs over time

    Critical Pulmonic Stenosis: decrease in compliance of RV decreases RA pressure and may open FO → R to L shunt

    • CLINICAL MANIFESTATIONS:
    • -most asymptomatic
    • -severe may cause dyspnea on exertion and angina
    • -Murmur: ejection click that varies with inspiration, harsh systolic ejection murmur at LUSB
    • -thrill and RV heave
    • -CXR: pulmonary artery segment is enlarged
    • -EKG: RVH and RAD
    •  
    • TREATMENT:
    • -PGE1 in critical stenosis
    • -surgery/catheter based intervention
  23. Findings of the 10 most common congenital heart lesions

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