congenital heart disease - students
TRANSCRIPT
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Congenital Heart Disease
Cindy Chan, MD
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Tetralogy of Fallot
Coarctation of the aorta
Hypoplastic left heart syndrome
D-transposition of the great arteries
Total anomalous pulmonary venous connection
Atrial septal defect Ventricular septal defect
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In utero, placenta (with low resistance) providesoxygenation, and sends oxygenated blood (only 10% tolungs) to the body, bypassing lungs via patent foramenovale (PFO) and ductus arteriosis (DA)
At birth, lungs mechanically expand and baby takes firstbreath (vessels mechanically not compressed & O2 potentpulm vasodilator - so pulm vascular resistance decreases (ittakes 6-8 weeks for pulm resistence to go down to adultlevels))
When placenta taken out, systemic vascular resistenceincreases. (pulm and systemic resistance about same attime of birth). DA closes first 24-48 hrs, FO only reallyfunctionally closes (2-3 months to close, if at all)
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Cyanosis
in adults - blue lips & nailbeds
in babies - also blue around mouth & eyes
in dark skinned - check tongue & mucous
membranes
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No respiratory distress
pOa2 in utero is 25, but newborn pOa2 is 30
(adults are in the 80s)
there is enough oxygenation to meet tissue
demands
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Single S2
pulm valve abnormal (only hear aortic
closure)
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Hypoxemia
normal pH, pCO2 normal, pOa2 20-40s
(discomfort if down to teens)
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Pulmonarystenosis
Ventricular
septal defect
R ventricular
hypertrophy
Overridingaorta
Tetralogy of Fallot
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Physiology
Malaligned VSDblocked RV outflowtract and anteriorly displaced aortapulm
stenosishigh pressure in RV (where RVpressure = LV pressure) RVhypertrophy
Blood takes path of least resistance
Blue blood from RV to LV
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Tetralogy of Fallot
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Medical Management
PDA VERYimportant to move blood from L side (aorta)to the R side (pulmonary artery)
Prostaglandin E1 (PGE1) maintains or restores patency of
the ductus arteriosus Treatment of hypercyanotic spells focuses on decreasing
pulmonary vascular resistance (administration of oxygen,morphine) and increasing systemic vascular resistance (toincrease blood flow to lungs)
Decrease pulm resistance (O2, morphine)
Increase systemic resistance (knee-chest position, volumeexpansion, IV phenylephrine)
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Surgical Management - definitive
Closing the ventricular septal defect with a
patch (made of Daykron)
Enlarging the right ventricular outflow tract
incision across the pulmonary valve annulus
and placement of a patch of synthetic material
with resulting pulmonary regurgitation (butwell-tolerated if TV competent)
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Gray color - poor circulation to body
Tachypnea - increased blood volume in lungs
Differential pulses - disease like coartaction ofaorta
Single S2 - abnormal AV (sharp & distinct c/w
normal muffled S2 in newborns)
RV heave - extra RV work
ABG abnormalities - low pH, pO2 in 40s
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Coarctation of Aorta
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almost always located just beyond the
origin of the left subclavian artery and
across from the insertion of the ductusarteriosus
approximately 80% of patients with
coarctation have a bicuspid aortic valve
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Physiology
Coarctation of the aortaincreased LV
afterload of the left ventricleLV
hypertrophyCCF Proximal to coarctationincrease in BP
Distal to coarctationdecrease in BP
diminished and delayed femoral pulses
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Coarctation of the Aorta
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Post-stenotic
dilitation
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Medical Management
Also, inotropes for LV dysfunction and
diuretics for congestion
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Surgical Management
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Hypoplastic Left Heart Syndrome
aortic valve - hypoplastic, stenotic or atretic
mitral valve - hypoplastic, stenotic, or atretic
left ventricle - hypoplastic or absent
ascending aorta - typically hypoplastic
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Physiology
Nonfunctional LV pulm venous return
passes across ASD or PFO into RA
blood mixes with systemic venous returnRV pumps mixed blood to both lungs and
body (via PDA)
Flow across PDA dependent on pulmresistance vs. systemic resistance
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1. Hypoplastic ascending aorta and aortic arch.
2. Hypoplastic left ventricle.
3. Large patent ductus arteriosus supplying the only source of blood
flow to the body.4. Atrial septal defect allowing blood returning from lungs to reach
the single ventricle.
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Medical Management
PGE1
Increase pulmonary vascular resistance (viacarbon dioxide, inhaled nitrogen)
Decrease systemic vascular resitance (viaNitroprusside)
Avoid agents that decrease pulmonary vascularresistance (supplemental oxygen,
hyperventilation) Avoid agents that increase systemic vascular
resistance (pressors)
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Surgical Management
Fatal without surgical intervention
3 separate surgical procedures
Right ventricle and pulmonary artery are
utilized to pump to the systemic circulation
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Single S2 - position of great vessels (softer,
parallel/posterior pulm sound)
Hypoxemia - p02 in teens to 20s
D T iti f G t A t i
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D-Transposition of Great Arteries
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Physiology
2 parallel circulations
Blue blood (desaturated) from SVC/IVCto RAto RVto aortaeventually, SVC/IVC
Red blood (oxygenated) from lungsto pulmveinsto LAto LVto pulm artery
back to lungs
Mixing can occur through an atrial defect (ASD orPFO), a VSD or a PDA (best source is atrial defectbi-directional mixing)
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Transposition of Great Arteries
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Medical Management
The use of PGE1 is controversialshunting
in PDA unidirectional (aorta to pulm artery)
Also, extra blood flow going to the lungsresults in more blood flow returning to the left
atrium and distention of LA, which can close
foramen ovale
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Surgical Management
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Surgical Management
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Total Anomalous Pulmonary Venous Connection
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Total Anomalous Pulmonary Venous Connection
Supracardiac TAPVC
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Supracardiac TAPVC
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Physiology
obstruction to pulmonary venous flowbackpressure to pulmonary capillary bedpulmonary edema
no blood flow entering left atrium directly
all flow arrives in the left heart via right to leftshunting across the atrial communicationmarked hypoxemia
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Medical Management
Very little can be done medically
Positive pressure ventilation
Diuretics
PGE1 contraindicated
Widened PDA would increase pulmonary blood
flow and exacerbate the pulmonary venouscongestion
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Surgical Management
anastamose the common pulmonary vein tothe to the back of the left atrium with the
widest possible anastomosis to prevent anyongoing obstruction to pulmonary venousreturn
atrial communication and PDA then closed
veins with extremely high tendency to formscar tissue, with later complications
S i l M t
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Surgical Management
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ASD
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VSD
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PDA