Peds Cardio

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kboyne3
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116607
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Peds Cardio
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2011-11-15 22:53:35
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cardiology
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peds cardiology rhythms, etc
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  1. Atrial flutter
    • rapid atrial rate (~300bpm) w/ varying ventricular rate, dependingon degree of block
    • -sawtooth pattern
    • -normal QRS
  2. Atrial fibrillation
    • very fast atrial rate (350-600bpm)
    • -irregularly irregular ventricular response
    • -no P waves; normal QRS
  3. Ventricular tachycardia
    • series of 3+++ PVCs w/ HR 120-200
    • -wide, unusually shaped QRS complexes
    • -T waves in opposite direction of QRS complex
  4. Ventricular fibrillation
    • very irregular QRS complexes
    • -rate rapid & irregular
    • -kills you - can't maintain effective circulation
  5. Supraventricular tachycardia (SVT)
    • -narrow QRS, no variability in R-R interval
    • -uses Av node in re-entrant circuit
    • -sudden onset & resolution
    • -absent P waves
  6. Right axis deviation (RAD)
    causes: severe pulm stenosis w/ RVH, pulmonary HTN, conduction disturbances (RBBB)
  7. Left axis deviation (LAD)
    • w/RVH - suggests AV canal, consider esp w/ Downs
    • -mild LAD w/ LVH - in cyanotic infant suggests tricuspid atresia
  8. No
  9. no narrow Q waves in inferior & leftward leads
    suspect CHD with ventricular inversion
  10. Q waves of new onset or increased duration of previous Q waves w/ or w/o notching of Q
    may represent MI (uncommon)
  11. ST elevation or prolonged WTc
    supports MI
  12. causes of ischemia & infarction
    • -anomalous origin of left coronary a from pulm a
    • -coronary artery aneurysm & thrombosis in Kawasaki's
    • -asphyxia
    • -cardiomyopathy
    • -severe aortic stenosis
    • -myocarditis
    • -cocaine use
  13. Deep, wide Q wave in aVL
    marker for LV infarction; suspect anomalous origin of left coronary a., esp child < 2 mos
  14. Causes of ST displacement
    • pericarditis
    • cor pulmonale
    • pneumopericardium
    • head injury
    • pneumothorax
    • early ventricular repolarization
    • normal atrial repolarization
  15. ST elevation
    -ischemia or pericarditis
  16. ST depression
    consistent with subendocardial ischemia or effects of digoxin
  17. Peaked, pointed T waves
    • hyperkalemia
    • LVH
    • head injury
  18. Flattened T waves
    • hypokalemia
    • hypothyroidism
  19. Causes of right atrial enlargement
    • cor pulmonale (pulm HTN, RVH)
    • anomalous pulm venous connection
    • lg ASD (uncommon)
    • Ebstein's anomaly
  20. Causes left atrial enlargement
    • VSD
    • PDA
    • mitral stenosis
    • show up as wide P waves
  21. RVH causes
    • ASD
    • TAPVR
    • pulmonary stenosis
    • tetralogy of Fallot
    • lrg VSD w/pulm HTN
    • coarctation in newborn
  22. LVH causes
    • VSD
    • PDA
    • anemia
    • complete AV block
    • aortic stenosis
    • systemic HTN
    • obstructive & nonobstructive hypertrophic cardiomyopathies
  23. decreased QRS voltage
    • <5mm in limb leads
    • pericardial effusion
    • pericarditis
    • hypothyroidism
  24. Increased Pulmonary Vascular Markings in acyanotic child
    ASD, VSD, PDA, endocardial cusion defect, or partial anomalous pulmonary venous return
  25. Increased Pulmonary Vascular Markings in cyanotic child
    transposition of great arteries, TAPVR, hypoplastic left heart syndrome, persistent truncus arteriosus, or single ventricle
  26. Decreased Pulmonary Vascular Markings
    • lung fields dark w/small vessels
    • pulmonary stenosis & atresia
    • tricuspid stenosis & atresia
    • tetralogy of Fallot
  27. Pulmonary Venous Congestion
    • hazy lung fields
    • Kerley B lines
    • Causes: LV failure or obstruction of pulm veins
    • mitral stenosis
    • TAPVR
    • cor triatriatum
    • hypoplastic left heart syndrome
    • any left-sided obstructive lesion w/ heart failure
  28. Abnormal Cardiac Silhouettes
    • 1. Tetralogy of Fallot
    • 2. Transposition of Great Arteries
    • 3. Total Anomalous Pulmonary Venous Return
  29. Tetralogy of Fallot - on CXR
    • cardiac silhouette boot-shaped w/ decreased pulm vascular markings (boot b/c hypoplastic main pulm a.)
    • RVH
  30. Transposition of Great Arteries - on CXR
    • cardiac silhouette 'egg-shaped'
    • narrow superior aspect of cardiac silhouette b/c absent thymus & irregular relation of great aa's
  31. Total Anomalous Pulmonary Venous Return - on CXR
    • cardiac silhouette 'snowman' shaped
    • left vertical vein, left innominate vein, & dilated superior vena cava create 'snowman's' head
  32. Henoch-Schonlein Purpura
    • immune-mediated vasculitis
    • affects GI tract, joints, & kidneys
    • rash
    • winter, fllws Grp A strep URI
    • glomerulonephritis can progress to acute renal failure (chronic proteinuria)
    • Tx = supportive, full recovery 4-6 wks
  33. Kawasaki Disease definition/etiology/epidemiology
    • aka mucocutaneous LN syndrome
    • unknown etiology, believed to have infectious cause
    • affects kids (80% <4yrs); Asians, males, winter/spring
  34. Kawasaki Disease Signs/Symptoms
    • sterile pyuria
    • aseptic meningitis
    • thrombocytosis
    • desquamation fingers & toes
    • elevated ESR or CRP
    • most significant sequelae: coronary aneurysm, pericardial effusion, & CHF
  35. Kawasaki Disease Dx
    • Fever for >5 days + more than 4 of following:
    • 1. b/l conjunctivitis w/o exudate
    • 2. mucocutaneous lesions ('strawberry' tongue; dry, red, cracked lips; diffuse erythema oral cavity)
    • 3. changes UE & LE (erythema &/or edema of hands/feet)
    • 4. polymorphic rash (usually truncal)
    • 5. cervical LAD (>1.5cm diameter); usually u/l
    • Echo: baseline study; evaluate for early coronary aneurysms; f/u to establish presence or absence
  36. Kawasaki Disease Tx
    • IVIG - usually 1 dose
    • high dose aspirin (80-100 mg/kg/day)
    • aspirin reduced after afebrile x 48 hrs
    • steroids controversial, reserved for refractory to repeat IVIG
  37. Polyarteritis Nodosa - definition + S/Sx
    • necrotizing inflammation of small & medium-sized muscular arteries
    • prolonged fever
    • wt. loss
    • malaise
    • subcutaneous nodules on extremities
    • HTN & abd pain can be imp clues
    • various rashes
    • resp sx: rhinorrhea, congestion
    • waxes & wanes
    • gangrene of distal extremities in severe disease
  38. Polyarteritis Nodosa - Dx/ Tx
    • no diagnostic tests; abnormal cell counts (thrombocytosis, leukocytosis), abnormal UA, elevated acute-phase reactants, p-ANCA
    • *conclusive w/ med-sized artery aneurysms
    • Echo evidence of coronary a. aneurysms dx'ic w/ clinical evidence
    • Tx: corticosteroids suppress clinical manifestations
    • cyclophosphamide or azathioprine to induce remission
  39. Takayasu's Arteritis - definition/pathophys/epidemiol
    • aka aortoarteritis or 'pulseless disease'
    • chronic inflammatory disease involving: aorta, arterial branches from aorta, pulm vasculature
    • Pathophys: lesions segmental, often obliterative; aneurysmal & saccular dilation; thoracoabdominal aorta predominantly affected site in peds
    • Epidem: mostly females age 4-45 yrs
  40. Takayasu's Arteritis - S/Sx + Tx
    • S/Sx: sig # pts w/LV dysfunction + CHF (even w/o coronary a. involvement, HTN, or valve abnormalities)
    • lymphocytic infiltration consistent w/myocarditis in 50%
    • fever, polyarthralgias, polyarhtritis, & loss radial pulsations

    Tx: corticosteroids may induce remission
  41. Wegener's Granulomatosis - define/epidem/SSx
    • Def: rare vasculitis both aas & vvs leading to widespread necrotizing granulomas
    • Epidem: most common adults; described in kids
    • S/Sx: rhinorrhea, nasal mucosa ulcers, sinusitis; hematuria; cough, hemoptysis, pleuritis; heart involvement - granulomatous inflammation cardiac mm causing arrhythmias
  42. Wegener's Granulomatosis - Dx + Tx
    • Dx: c-ANCA present; ESR sig elevated; organ bx (kidney &/or lung) may be needed to establish eary dx
    • Tx: corticosteroids alone may be unsuccessful; cyclophosphamide or azathioprine recommended
  43. Cyanotic Heart Defects
    • T's
    • Tetralogy of Fallot
    • Transposition of the great vessels
    • Truncus arteriosus
    • Total anomalous pulmonary venous return (obstructive)
  44. Tetralogy of Fallot - define / etiology
    • cyanotic heart defect w/4 anomalies:
    • 1. right ventricular outflow tract obstruction
    • 2. VSD
    • 3. aortic override
    • 4. RVH
    • Etiology: prenatal factors incl maternal rubella or viral illness
  45. Tetralogy of Fallot Patohphys
    • RVOTO dictates degree of shunting:
    • minimal obstruction: increased pulm blood flow as PVR decreases, leading to CHF
    • mild obstruct: hemodynamic balance pressure btw right & left ventricles equal, so no net shunting
    • severe obstruction: decreased pulm blood flow leading to cyanosis
  46. Tetralogy of Fallot - Epidem/ S/Sx
    • -most common cyanotic heart defect in kids who survive infancy
    • S/Sx: FTT; conotruncal facies; variable cyanosis (clubbing later if unrepaired); right ventricular impulse, occasional thrill, single S2 systolic ejection murmur ULSB w/ or w/o ejection click
    • squatting is common posture in older, unoperated kids - often after exercise; traps desaturated bood in LEs & increases systemic vascular resistance while RVOTO remains fixed --> decreased R->L shunting, increases pulm blood flow, increases arterial saturation
  47. Tetralogy of Fallot- "Tet spells"
    • most common 2-6 mos
    • occur in morning or after nap when SVR low
    • precipitating factors: stress, drugs decreasing SVR, hot baths, fever, exercise
    • Mech: likely due to increased CO w/ fixed RVOT, leading to increased R->L shunting, which increases cyanosis
    • if prolonged or severe: syncope, seizures, cardiac arrest
  48. Tetralogy of Fallot - Dx
    • CXR - boot-shaped heart
    • decreased pulm vascular markings
    • Right aortic arch (25%)
  49. Tetralogy of Fallot Tx
    • clinical status may prevent definitive repair initially
    • shunting - when pulm stenosis severe & alternate route for blood to reach lungs is needed
    • complete repair: VSD closure, relief of RVOTO, ligation of shunts, ASD/patent foramen ovale closure
  50. Transposition of Great Vessels - Pathophysiology
    • "big blue baby"
    • -primitive heart loops to left instead of R
    • aort originates from RV
    • pulm a. originates from LV (aorta anterior, pulm trunk posterior)
    • R & L hearts in parallel (pulm venous return goes to pulm a via LV; systemic venous return goes to aorta via RV)
    • ASD &/or VSD essential to allow mixing
    • PDA alone usually not sufficient to allw adeq mixing in extrauterine environ
  51. Transposition of Great Vessels - S/Sx + Dx
    • Intact Ventricular Septum: no valve abnormality
    • early cyanosis, single S2, no murmur
    • intact atrial septum or very restrictive PFO is emergency
    • Dx: ECG normal 1st, then RVH by 1 month
    • CXR - "egg on a string"
    • w/VSD (lg VSD allws adeq mixing):
    • sx related to increased pulm blood flow w/ CHF sometimes early
    • may have little cyanosis
    • Dx: ECG - Rt or biventricular hypertrophy
  52. Transposition of Great Vessels - Tx
    • Intact Ventricular Septum:
    • 'ductal dependent,' require PGE1 to keep PDA patent
    • early balloon atrial septostomy (BAS) needed to allw mixing of oxygenated + deoxygenated blood
    • arterial switch procedure - definitive
    • w/VSD
    • BAS if not adequate mixing
    • PA band to ctrl increased pulm blood flow
    • arterial switch procedure w/VSD closure - definitive
  53. Truncus Arteriosus - defined
    • single arterial trunk emerges from ventricles, supplying coronary, pulmonary, & systemic circulations
    • Types:
    • I - short common pulm trunk arising from R side common trunk, just above truncal valve
    • II - pulm aa's arise directly from ascending aorta, from post. surface
    • III - similar to II, w/ pulm aa's arising more laterally & more distant from semilunar valves
  54. Truncus Arteriosus - pathophys
    • valve has 2, 3, or 4 leaflets & usually poorly fxning
    • truncus overrides a VSD
  55. Truncus Arteriosus - S/Sx
    • usually w/i 1st few weeks of life
    • intial L -> R shunt sx: dyspnea, frequent resp infections , FTT
    • if pulm vascular resistance increases, cyanosis increases
    • 2nd heart sound prominent & single due to single semilunar valve
    • peripheral pulses strong, often bounding
    • often systolic ejection click
  56. Truncus Arteriosus - Dx / Tx
    • Dx: CXR w/ cardiomeagly & increased pulm vascular markings
    • Tx: surgery must occur before sig pulm vascular disease (3-4mos)
    • VSD surgically closed, leaving valve on LV side
    • pulm aa's freed from truncus & connected to valved conduit (Rastelli procedure), which serves as new pulm trunk
  57. Hypoplastic Left Heart Syndrome - Definition
    • aortic valve hypoplasia, stenosis, or atresia w or w/o mitral valve stenosis or atresia
    • hypoplasia of ascending aorta
    • LV hypoplasia or agenesis
    • mitral valve stenosis or atresia
    • Result: single (rt) ventricel that provides blood to pulm system, systemic circul via PDA, & coronary system via retrograde flow after crossing PDA
    • ductal dependent requiring patency until intervene
  58. HLHS - S/Sx
    • pulses range normal to absent (depends on ductal patency)
    • hyperdynamic RV impulse
    • single S2 of increased intensity
    • gallop at apex due if there is heart failure
    • nonspecific systolic murmur at left sternal border
    • skin may have characteristic grayish pallor
  59. HLHS - Dx
    • CXR: cardiomegaly w/ globular shaped heart; increased pulm vascular markings; pulm edema
    • Echo: diagnostic
  60. HLHS - Tx options
    • 1 - no intervention
    • 2 - 3 stage surgery
    • 3 - heart transplant
  61. HLHS - no intervention outcome
    high mortality & complicated surgical course - ethical dilemma as to how far intervene
  62. HLHS - 3 stage surgery
    • 1 - Norwood procedure: pulm trunk used to reconstruct hypoplastic aorta; RV becomes fxnal LV; leaves pulm aa's connected but separated from the heart; pulm blood flow then reestablished via systemic to pulm conduits from subclavian arteries to pulm aa's
    • 2 - Glenn procedure: SVC connected to right PA, restoring partial venous return to lungs
    • 3 - Fontan procedure: IVC anastamosed to PA's resulting in complete venous diversion from systemic circulation to lungs
  63. HLHS - heart transplant
    primary intervention if organ available or after any other palliative surgeries provided maximal but ultimately insufficient benefit
  64. Acyanotic Heart Defects
    • L -> R shunt
    • subendocardial cushion defect
    • ASD
    • VSD
    • PDA
    • PFO
  65. subendocardial cushion defect - pathophys
    • from abnormal development of AV canal (endocardial cushions) resulting in - VSD + an ostium primum ASD
    • clefts in mitral & tricupsid valves
  66. Subendocardial Cushion Defect - epidemiology + S/Sx
    • epidem: 30% of these pts have trisomy 21; also frequently found w/ asplenia & polysplenia syndromes
    • S/Sx: often from sp. components of accumulated defects -
    • holosystolic murmur from VSD if restrictive
    • systolic murmur from mitral & tricuspid valve insufficiency
    • high risk of developing Eisenmenger's
  67. Subendocardial Cushion Defect - Tx
    • surgical correction sometimes only option when pt has unbalanced AV canal - high risk surg
    • some benefit from PA banding if shunting predominantly at ventricular level (rare)
    • kids w/trisomy 21 often have more favorable anatomy for surg
  68. ASD - define 3 types
    • 1 - Secumdum (50-70%): in central portion atrial septum
    • 2 - Primum (30%): at atrial lower margin; assoc'd w/ abnormalities of mitral & tricuspid valves
    • 3 - sinus venosus (10%): at upper portion atrial septum & often extends into SVC
    • ASD is most common congenital heart lesion recognized in adults
  69. ASD - epidemiology
    • common 'co-conspirator' in CHD
    • as many as 50% of pts w/congenital heart defects have ASD as one of the defects
    • more common in females (M:F = 1:2)
  70. ASD - S/Sx
    • typically asymptomatic
    • widely split & fixed S2; murmurs uncommon, but may occur as age (is secondary pulm flow murmur)
    • Sx of CHF & pulm HTN occur in adults (2nd-3rd decades)
  71. ASD - dx/tx
    • Dx: ECG - L->R shunt may produce rt atrial enlargement & RVH
    • Tx: nearly 90% close spontaneously; 100% if <3mm; ASDs>8mm unlikely to close spontaneously
    • surgical or catheter closure (via 'clamshell' or 'umbrella' device) used when indicated
  72. PFO
    • foramen ovale used prenatally to provide oxygenated blood from placenta to LA
    • normally fxnally closes when increased LA pressue causes septa to press against each other (many stay 'probe-patent into adulthood)
    • in some kids, tissue of formaen ovale insufficient to cover foramen (insuff growth or stretched from increased pressure or vol)
    • some CHDs require PFO for pt survival afer birth (e.g. tricuspid & mitral atresia, TAPVR)
  73. VSD - epidemiology
    • most common form of recognized CHD
    • usually membranous as opposed to in muscular septum
    • occurs 2/1000 live births
  74. VSD - S/Sx
    • dependent on defect size
    • Small: usually asymptomatic; norm growth & development, high-pitched holosystolic murmur; no ECG or CXR changes
    • Large: can lead to CHF & pulm HTN; may have FTT; lwr pitched murmur, intensity dependent on degree of shunting
  75. VSD - Dx
    • ECG: lvh
    • CXR: cardiomegaly
  76. VSD - Treatment
    • spontaneous closure - muscular defects most likely to close (up to 50%) occuring during 1st yr of life; inlet & infundibular defects don't reduce in size or close
    • intervention based on development of CHF, pulm HTN, & growth failure
    • initial management w/ diuretics & digitalis
    • surg closure indicated when therapy fails
    • catheter-induced closure devices less commonly used w/ VSDs than ASDs
    • endocarditis prophylaxis
  77. PDA - pathophys
    • most often prob in premature neonates: L->R shunts handled poorly by premature infants, many develop idiopathic respiratory distress syndrome; some progress to develop LV failure
    • failure of spontaneous closure = premature infants due to ineffective response to oxygen tension; mature infants due to structural abnormality of ductal smooth m.
    • (in norml neonate, ductus closes primarily in response to ductal PO2>50mmHg)
  78. PDA - epidemiology
    • more common in females (M:F = 1:3)
    • incidence higher at higher altitudes due to lower atmospheric O2 tension
    • maternal rubella in 1st trimester been implicated in PDA
  79. PDA - S/Sx
    • smll - usually asymptomatic
    • lg - increase incidence of lower resp tract infections & CHF
    • machinery like murmur
    • bounding peripheral pulses & wide pulse pressure
    • if eisenmenger's syndrome -> p may have cyanosis restricted to LEs
    • *infective endocarditis most common complication of PDA in late childhood
  80. PDA -Tx
    • Indomethacin - in premature infants, inhibits PG synthesis leading to closure
    • Catheter closure via devices such as dbl-umbrella devices & coils in older kids
    • Surgical ligation & division via left lateral thoracotomy (occasional complication - recurrent laryngeal n. injury-> hoarseness); Eisenmenger's = contraindication to surgery
  81. Eisenmenger's Syndrome
    • can occure in unrepaired L-> R shunts that cause increased pressure load on pulm vasculature (which can result in irreversible changes in arterioles)
    • --> pulm vascular obstructive disease, usually over # yrs
    • pulm HTN reduces L=>R shunt & previous LVH often resolves
    • persistent HTN maintains enlarged RV & can dilate main pulm segment (becomes evident on CXR)
    • avoidance of this condition via surgical correction of CHD essential as it causes irreversible changes
  82. Tricuspid Atresia - Definition, Epidem, S/Sx, Dx, Tx
    • RV inlet absent or near absent
    • Epidem: ASD &/or VSD usually present; 75% present w/ cyanosis w/i 1st week
    • S/Sx: LV impulse displaced laterally
    • Dx: LVH, prominent LV forces (b/c decreased RV voltages)
    • Tx: PGE1 to maintain ductal patency; surgery; modified BT shunt; Glenn procedure fllw'd by Fontan procedure
  83. Pulmonary Atresia (w/Intact Ventricular Septum) - S/Sx
    • cyanosis w/i hrs of birth (PDA closing)
    • hypotension, tachypnea, acidosis
    • single S2, w/ holosystolic murmur (tricuspid regurg)
  84. Pulmonary Atresia (w/Intact Ventricular Septum) - Dx/Tx
    • Dx: ECG - decreased RV forces & occasionally RVH; CXR - normal to enlarged RV w/ decreased pulm vascular markings
    • Tx: PGE1 to maintain ductal patency; balloon atrial septostomy (sometimes); reconstruction of RVOTw/ transannular patch or pulmonary valvotomy; ASD left open to prevent systemic venous HTN
  85. Case: 4 yo boy w/ recurrent episodes syncope while playing has harsh systolic murmur radiating to carotids, diminished cardiac pulses, & sever LVH
    Think: congenital aortic stenosis
  86. Aortic Stenosis - Epidemiology + S/Sx
    • Epidem: 85% congenitally stenotic aortic valves are bicuspid
    • S/Sx:
    • severe stenosis generally presents shortly after birth
    • older kids may complain of chest or stomach pain (epigastric)
    • pts w/untreated severe AS at risk for syncope & sudden death
    • murmur: crescendo-decrescendo systolic murmur; systolic ejection click common (esp if bicuspid aortic valve); if severe, paradoxical splitting of S2 (split narrows w/inspiration)
  87. Aortic Stenosis - Dx/ Tx
    • Dx: clinical findings including on ECG, can be deceiving
    • Echo or cath to evaluate pressure diff btw aorta & LV needed
    • Tx: surgical or interventional balloon
    • valvotomy most common (indication usually if measured catheterization gradient is >50mmHg); high incidence recurrent stenosis
    • valve replacement - deferred, when possible, until pt completes growth
  88. Aortic Insufficiency - Epidemiology + S/Sx
    • Epidem: uncommon, usually assoc'd w/ mitral valvae disease or aortic stenosis
    • S/Sx: diastolic decrescendo murmur at LUSB; Sx indicate advanced disease; CP & CHF ominous signs
  89. Aortic Insufficiency - Dx/Tx
    • Dx: CXR - LV enlargement, dilated ascending aorta
    • Tx: surgery or balloon valvuloplasty to tx aortic stenosis may worse insufficiency; aortic valve replacement is only definitive therapy
  90. Mitral Stenosis - Epidem
    • rare in kids; usually sequela of acute rheumatic fever
    • congenital forms generally severe
  91. Mitral Stenosis - S/Sx
    • when symptomatic, dyspnea most common
    • weak peripheral pulses w/ narrow pulse pressure
    • opening snap heard on auscultation; also, presystolic murmur may be heard
    • pulmonary venous congestion --> CXR evidence of interstitial edema + hemoptysis from smll bronchial vessel rupture
  92. Mitral Stenosis - Tx
    • balloon valvuloplasty
    • surgical: commisurotomy, valve replacememt
  93. Mitral Valve Prolapse - Pathophys + Epidem
    • Pathophys: caused by thick & redundant valve leaflets that bulge into mitral annulus
    • Epidem: usually in older kids & adolescents; familial component AD; nearly all pts w/ Marfans have this
  94. Mitral Valve Prolapse - S/Sx + Tx
    • S/Sx: ausculatation - midsystolic lcick & late systolic murmur; often asymptomatic w/ some hx of palpitations & CP
    • Tx: management symptomatic (e.g. beta-blocker for CP)
  95. Coarctation of Aorta - Pathophys
    • most commonly in juxtaductal position (where ductus arteriosus joins aorta)
    • development of sx may correspond to closure of ductus arteriosus (patent ductus provides additional room for blood to reach postductal aorta)
  96. Coarctation of Aorta - Epidem
    • more common in males (M:F = 2:1)
    • seen in 1/3 of pts w/Turner's syndrome
  97. Coarctation of the Aorta - S/Sx
    • Clinical Presentation of Symptomatic Infants:
    • FTT, resp distress, & CHF in 1st 2-3 mos life
    • LE changes: decreased pulses
    • acidosis may develop as lower body receives insufficient blood
    • usually murmur heard over left back
    • Clinical Presentation of Asymptomatic Infants/Children
    • normal growth/development
    • occasional complain leg weakness/pain after exertion
    • decreased pulses in LE
    • UE HTN (or at least greater than in LE)
  98. Coarctaiton of Aorta Dx + Tx
    • Dx: CXR '3 sign' dilated ascending aorta that displaces SVC to right
    • Tx: resect coarctation segment w/ end-to-end anastamosis initially; allograft patch augmentation can be used
    • can use catheter balloon dilation - higher restenosis rate than surg, increased risk aortic aneurysm, more frequently used when stenosis occurs at surgical site of primary reanastamosis
  99. Ebstein's Anomaly - Define
    • tricuspid valve displaced apically in RV
    • valve leaflets redundant & plastered against ventricular wall, often causing fxnal tricuspid atresia
    • RA frequently largest structure
  100. Ebstein's Anomaly - Epidemiology
    w/o intervention: CHF in 1st 6 mos; nearly 50% mortality
  101. Ebstein's Anomaly - S/Sx
    • growth & development can be normal depending on severity
    • older pts usually complain dyspnea, cyanosis, & palpitations
    • widely split S1, fixed split S2, variable S3 & S4 (characteristic triple or quadruple rhythm)
    • holosystolic murmur at LLSB
    • opening snap
    • cyanosis from atrial R=>L shunt
  102. Ebstein's Anomaly - Dx
    • ECG: Rt axis deviation, RA enlargement, RBBB; WPW in 20%
    • CXR: cardiomegaly (balloon-shaped); 'wall-to-wall heart' in severely affected infants
    • Echo: diagnostic
  103. Ebstein's Anomaly - Tx
    • 87% do well
    • Glenn procedure to increase pulmonary blood flow
    • severely affected infants may require aortopulmonary shunt
    • Tricuspid valve replacement or reconstruction
    • RA reduction surgery
    • ablation of accessory conduction pathways
  104. TAPVR - Pathophys
    • no communication btw pulmonary vv's & LA
    • all pulm vv's drain to a common v. & common v. drains into: R SVC (50%), CS or RA (20%), portal v. or IVC (20%), or combination
    • ASD needed for survival
  105. TAPVR - Epidem + S/Sx
    • Epidem: much more common in males (M:F = 4:1)
    • S/Sx: depends on if obstruction
  106. TAPVR w/ obstruction
    • increased pulm a. pressure (& subsequent pulm edema) that increases pulm, then RA & ventricular pressures; causes R=>L shunt & resultant cyanosis
    • presents w/ early, severe resp distress & cyanosis, no murmur, hepatomegaly
    • CXR: normal size heart, pulm edema
    • Echo: diagnostic
    • Management: balloon atrial septostomy or immediate corrective surgery
  107. TAPVR w/o Obstruction
    • free communication btw RA & LA
    • lg R=>L shunt (lg ASD)
    • presents later during 1st yr of life w/ mild FTT, recurrent pulmonary infections, tachypnea, right heart failure, & rarely cyanosis
    • CXR: cardiomegaly, lg. PAs; increase pulm vascular markings ('snowman' or 'figure eight' sign)
    • Management: surgical movement of pulm vv's to LA

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