-
Atrial flutter
- rapid atrial rate (~300bpm) w/ varying ventricular rate, dependingon degree of block
- -sawtooth pattern
- -normal QRS
-
Atrial fibrillation
- very fast atrial rate (350-600bpm)
- -irregularly irregular ventricular response
- -no P waves; normal QRS
-
Ventricular tachycardia
- series of 3+++ PVCs w/ HR 120-200
- -wide, unusually shaped QRS complexes
- -T waves in opposite direction of QRS complex
-
Ventricular fibrillation
- very irregular QRS complexes
- -rate rapid & irregular
- -kills you - can't maintain effective circulation
-
Supraventricular tachycardia (SVT)
- -narrow QRS, no variability in R-R interval
- -uses Av node in re-entrant circuit
- -sudden onset & resolution
- -absent P waves
-
Right axis deviation (RAD)
causes: severe pulm stenosis w/ RVH, pulmonary HTN, conduction disturbances (RBBB)
-
Left axis deviation (LAD)
- w/RVH - suggests AV canal, consider esp w/ Downs
- -mild LAD w/ LVH - in cyanotic infant suggests tricuspid atresia
-
-
no narrow Q waves in inferior & leftward leads
suspect CHD with ventricular inversion
-
Q waves of new onset or increased duration of previous Q waves w/ or w/o notching of Q
may represent MI (uncommon)
-
ST elevation or prolonged WTc
supports MI
-
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
-
Deep, wide Q wave in aVL
marker for LV infarction; suspect anomalous origin of left coronary a., esp child < 2 mos
-
Causes of ST displacement
- pericarditis
- cor pulmonale
- pneumopericardium
- head injury
- pneumothorax
- early ventricular repolarization
- normal atrial repolarization
-
ST elevation
-ischemia or pericarditis
-
ST depression
consistent with subendocardial ischemia or effects of digoxin
-
Peaked, pointed T waves
- hyperkalemia
- LVH
- head injury
-
Flattened T waves
- hypokalemia
- hypothyroidism
-
Causes of right atrial enlargement
- cor pulmonale (pulm HTN, RVH)
- anomalous pulm venous connection
- lg ASD (uncommon)
- Ebstein's anomaly
-
Causes left atrial enlargement
- VSD
- PDA
- mitral stenosis
- show up as wide P waves
-
RVH causes
- ASD
- TAPVR
- pulmonary stenosis
- tetralogy of Fallot
- lrg VSD w/pulm HTN
- coarctation in newborn
-
LVH causes
- VSD
- PDA
- anemia
- complete AV block
- aortic stenosis
- systemic HTN
- obstructive & nonobstructive hypertrophic cardiomyopathies
-
decreased QRS voltage
- <5mm in limb leads
- pericardial effusion
- pericarditis
- hypothyroidism
-
Increased Pulmonary Vascular Markings in acyanotic child
ASD, VSD, PDA, endocardial cusion defect, or partial anomalous pulmonary venous return
-
Increased Pulmonary Vascular Markings in cyanotic child
transposition of great arteries, TAPVR, hypoplastic left heart syndrome, persistent truncus arteriosus, or single ventricle
-
Decreased Pulmonary Vascular Markings
- lung fields dark w/small vessels
- pulmonary stenosis & atresia
- tricuspid stenosis & atresia
- tetralogy of Fallot
-
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
-
Abnormal Cardiac Silhouettes
- 1. Tetralogy of Fallot
- 2. Transposition of Great Arteries
- 3. Total Anomalous Pulmonary Venous Return
-
Tetralogy of Fallot - on CXR
- cardiac silhouette boot-shaped w/ decreased pulm vascular markings (boot b/c hypoplastic main pulm a.)
- RVH
-
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
-
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
-
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
-
Kawasaki Disease definition/etiology/epidemiology
- aka mucocutaneous LN syndrome
- unknown etiology, believed to have infectious cause
- affects kids (80% <4yrs); Asians, males, winter/spring
-
Kawasaki Disease Signs/Symptoms
- sterile pyuria
- aseptic meningitis
- thrombocytosis
- desquamation fingers & toes
- elevated ESR or CRP
- most significant sequelae: coronary aneurysm, pericardial effusion, & CHF
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
Cyanotic Heart Defects
- T's
- Tetralogy of Fallot
- Transposition of the great vessels
- Truncus arteriosus
- Total anomalous pulmonary venous return (obstructive)
-
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
-
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
-
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
-
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
-
Tetralogy of Fallot - Dx
- CXR - boot-shaped heart
- decreased pulm vascular markings
- Right aortic arch (25%)
-
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
-
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
-
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
-
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
-
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
-
Truncus Arteriosus - pathophys
- valve has 2, 3, or 4 leaflets & usually poorly fxning
- truncus overrides a VSD
-
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
-
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
-
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
-
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
-
HLHS - Dx
- CXR: cardiomegaly w/ globular shaped heart; increased pulm vascular markings; pulm edema
- Echo: diagnostic
-
HLHS - Tx options
- 1 - no intervention
- 2 - 3 stage surgery
- 3 - heart transplant
-
HLHS - no intervention outcome
high mortality & complicated surgical course - ethical dilemma as to how far intervene
-
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
-
HLHS - heart transplant
primary intervention if organ available or after any other palliative surgeries provided maximal but ultimately insufficient benefit
-
Acyanotic Heart Defects
- L -> R shunt
- subendocardial cushion defect
- ASD
- VSD
- PDA
- PFO
-
subendocardial cushion defect - pathophys
- from abnormal development of AV canal (endocardial cushions) resulting in - VSD + an ostium primum ASD
- clefts in mitral & tricupsid valves
-
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
-
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
-
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
-
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)
-
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)
-
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
-
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)
-
VSD - epidemiology
- most common form of recognized CHD
- usually membranous as opposed to in muscular septum
- occurs 2/1000 live births
-
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
-
VSD - Dx
- ECG: lvh
- CXR: cardiomegaly
-
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
-
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)
-
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
-
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
-
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
-
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
-
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
-
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)
-
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
-
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
-
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)
-
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
-
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
-
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
-
Mitral Stenosis - Epidem
- rare in kids; usually sequela of acute rheumatic fever
- congenital forms generally severe
-
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
-
Mitral Stenosis - Tx
- balloon valvuloplasty
- surgical: commisurotomy, valve replacememt
-
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
-
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)
-
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)
-
Coarctation of Aorta - Epidem
- more common in males (M:F = 2:1)
- seen in 1/3 of pts w/Turner's syndrome
-
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)
-
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
-
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
-
Ebstein's Anomaly - Epidemiology
w/o intervention: CHF in 1st 6 mos; nearly 50% mortality
-
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
-
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
-
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
-
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
-
TAPVR - Epidem + S/Sx
- Epidem: much more common in males (M:F = 4:1)
- S/Sx: depends on if obstruction
-
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
-
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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