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Flashcards in Pediatric surgery Deck (66)
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Fetal circulation

Blood from placenta via umbilical vein -> IVC -> right atrium. Venous return from body -> IVC, mixes with oxygenated blood from placenta. Most oxygenated blood shunted through foramen ovale to left heart -> aorta. Most oxygenated blood goes to brain. Distal to BCS origins, ductus arteriosus feeds deoxygenated blood from lungs into aorta -> mixed blood to body. Umbilical arteries return blood to placenta.


Changes in circulation at birth

SVR increases, PVR drops. Ductus arteriosus closes in first 24 hours. Foramen ovale closes in first month.


What drug keeps ductus arteriosus open?

Prostaglandin keeps open, indomethacin closes it.


Cyanotic congenital heart disease (right to left shunting)

Tetralogy of Fallot, pulmonary stenosis/atresia, tricuspid atresia, Ebstein's anomaly, transposition of great arteries (TGA)


Definition of Tetralogy of Fallot (ToF)

1. VSD 2. Pulmonary atresia 3. Overriding aorta 4. RVH (most common of the cyanotic heart defects). "boot shaped" heart


Pulmonary stenosis/atresia with intact ventricular septum

Stenosis is more common than atresia, which is total obstruction, more severe. Must have PFO or ASD to survive. Tx: stenosis use balloon dilation. Atresia do surgical correction such as valvulotomy PLUS systemic-pulmonary shunt.


Tricuspid atresia features

Tricuspid valve atresia PLUS 1) atresia/hypoplasia of right ventricle 2) ASD, PFO, or VSD. May have TGA or pulmonary stenosis. Most cases have a PDA as well.


Treatment of tricuspid atresia

Emergent systemic-pulmonary shunt or enlargement of PDA/PFO in the newborn for initial palliation. Later, do bidirectional Glenn shunt followed by modified Fontan.


Glenn shunt

SVC anastamosed to the pulmonary artery


Fontan procedure

see attached image


Ebstein's anomaly definition

Malformed and downwardly displaced septal and posterior leaflets of tricuspid valve, leading to obstruction. PLUS PFO or ostium secundum defect --> shunting, cyanosis. Arrhythmias common.


Risk factor for Ebsetin's anomaly

Maternal lithium use during pregnancy, esp first trimester


Transposition of Great Arteries (TGA)

Incompatible with life unless PDA, PFO, or VSD exists to allow mixing between the two separate circuits. If septum is intact, have to make emergent balloon septostomy of foramen ovale at birth.


Jatene procedure

Transection of great vessels just superior to valves, then transfer of vessels to their correct locations. Used for kids with TGA with VSD, within first 2 weeks of life.


Rastelli procedure

Aorta rerouted internally to left ventricle across VSD, pulmonary artery is attached to RV externally (see picture). Used for kids with TGA with VSD plus LVOT obstruction: do a palliative systemic-pulmonary shunt, then Rastelli at age 4-5.


What are the three types of (at least initially) left to right shunt heart defects?



Risk factors for PDA

Shorter gestational age, lower birth weight, female sex


Treatment of PDA

Indomethacin is successful in 90%. Surgery in refractory cases. Unless CHF develops, can do surgery electively anytime between 6 months and 2 years


What is the most common congenital heart defect overall?



Do VSDs close on their own?

90% if small defects close by age 8. Treatment is to medically manage congestive symptoms. surgical closure is indicated if CHF is not controlled medically, or if it does not close by 9 months and pulmonic pressure has reached 2/3 of systemic pressure.


ASD features

Usually asymptomatic until adult life unless large defect -> growth retardation, fatigue, recurrent PNA. Adults often present with arrhythmia or CHF.


Three types of ASD

Secundum type (middle or lower defect, most common type), sinus venosus (high type assoc with partial anomalous pulmonary venous return), and ostium primum type


Ostium primum type ASD

Endocardial cushion defect, cleft in mitral valve leaflet leading to mitral insufficiency. Uncommon except in Down syndrome


Eisenmenger syndrome

When you have a left to right shunt, increased pulmonary blood flow, PVR increases to the point that shunt becomes right to left -> cyanosis. Only treatment is heart and lung transplant.


What are the obstructive left sided congenital lesions?

Coarctation of the aorta, congenital aortic stenosis, hypoplastic left heart syndrome


Where is aortic coarctation usually located?

Within four cm distal to left subclavian take off


When is surgery done for aortic coarctation?

Neonates with CHF get it right away. Otherwise, ideal time is 3-4 years old


What causes rib notching on CXR in aortic stenosis?

Collateral circulation distal to the coarctation causes marked dilation of intercostal arteries


How do older children with aortic coarctation present?

Hypertensive but asymptomatic. Delayed femoral pulses


What's the deal with hypoplastic left heart syndrome?

Left side of heart is underdeveloped, with ineffective contractility and a valves less aortic remnant. Fetus survives because blood enters systemic circulation via PDA, so when that closes it is incompatible with life. Surgery is 80% survival.