6000 CHD - Dr. Jensen

development of the heart:

1. begins in ___ week of pregnancy

2. complete at week ___

3. pregnancy test usually positive from week ___ onwards

3rd; 7; 5

fetal circulation:

1. O2 blood from ________ using the _______ _______ bypasses ________ using the ________ _________

2. dumps into ____ to RA, uses ____ to get to LA

3. blood that goes to RV and out PA gets sent to ______ via the ________ ________

4. small amount of blood goes to _____

5. blood returns to ________ using the ________ _________

placenta; umbilical vein; liver; ductus venosus; IVC; PFO; aorta; ductus arteriosus; lungs; placenta; umbilical artery

babies have a very ____ O2 saturation; around the ____s

low; 50s

birth and at first breath:

1. blood vessels in ______ will _______

2. pulmonary resistance drastically _________

3. pressures in the ___ will _____

4. the two septal “______” are pressed against each other and the _______ _______ will ______

lungs; dilate; plummets; RA; drop; leaflets; foramen ovale; close

at birth - cutting of umbilical cord:

1. ___-resistance ________ disappears

2. _______ _______ resistance _________

3. pressure becomes _______ in aorta than PA

4. the rapid increase in ____ of aortic blood triggers _________ of the smooth muscles of _______ _______ and ______ closes within ___ hours

low; placenta; peripheral vascular; increases; higher; O2; contraction; vessel wall; ductus; 48

at birth - ductus venosus and umbilical vessels:

1. functional closure of DV occurs within _________

2. structural closure of DV occurs within ___-___ ______

3. umbilical vessels _________

minutes; 3-7 days; degenerate

overview of heart development:

1. develops from a _____

2. initially ____ ________

3. great vessels are ____ _____

tube; one chamber; one tube

fetal heart rate CTG graph:

1. fetuses have an underdeveloped ________ NS, so the ____ and ____ are constantly battling causing extreme ___ _______ (this is ________)

2. if HR is NOT _______, then that is a problem

autonomic; PNS and SNS; HR variability; normal; variable;

incidence of CHD:

1. 1:___ in USA/Europe are born with a congenital cardiac defect (this is the _____ _____ birth defect)

2. _____ babies born annually in USA

3. _____ babies born with a complex defect

4. about ___% require intervention

5. about ___% require OHS

100; most common; 40,000; 10,000; 50; 25

diagnosis:

1. during pregnancy:

   1. at ___ week _______ US: chance of detecting a major structural defect is ___-___% depending on skill of facility

   2. heart size is ___cm/___in (the size of a _______)

2. at or soon after birth:

   1. _________ (____ baby)

   2. ____

   3. _________ _______

3. in childhood/adolescence/adulthood:

   1. hear heart ______, feel _______ or ____, lack of _____ (“failure to _____”), tendency for frequent ________, and ______

4. in autopsy:

   1. ______ death

   2. _________ finding

20; structural; 50-90; 2; 0.8; walnut; cyanosis; blue; SOB; cardiovascular shock; murmurs; fatigued or SOB; growth; thrive; infections; swelling; sudden; coincidental

types of defects

1. ______ or ______

2. defects that _________ BF

3. the ______ _______

4. ________ defects

holes or shunts; obstruct; great vessels; complex

holes and shunts:

1. ____ about ___-___% of defects

2. ____ about ___-___% of general population

3. ____ (___-___% of all CHD in children; ___-___% of defects diagnosed in adults)

4. ____ (___-___ of every 1000 live births; accounts for more than ___% of all CHD)

5. _____ (1 every 2,120 [about _____ babies per year]; commonly associated with ______ _______)

PDA; 5-10; PFO; 10-35; ASD; 7-10; 30-33; VSD; 2-6; 20; AVSD; 2,000; down syndrome

hole/shunt treatment:

1. about ___% of these defects are suitable for __________ ________ closure

2. rest are closed ________ with a ______

3. however, _____ ALWAYS requires surgery

80; transcatheter device; surgically; patch; AVSD

obstructive defects:

1. ____ (___% of all congenital cardiac abnormalities; ____ develops)

2. ______ _______/_______ ____ (often due to _______ ___, about ___-___% of all CHD; about ___% of general population; ________ form is very common in elderly)

3. TV:

   1. both _____ and _______ occur; rarely _________ anomalies; _________ anomaly; TV _______

4. MV:

   1. congenital anomalies are _____ _____ (___% of defects) and usually never occur __________; ______ and ______ possible; mitral _______

PS; 9; RVH; aortic stenosis/bicuspid AV; bicuspid AV; 3-5; 1; acquired; regurg and stenosis; independent; Epstein’s; atresia; very rare; 0.2; independently; regurg and stenosis; atresia

obstructive defect treatment:

1. _________ valve repair - involves a _______ ________ _________

2. _______ valve repair

3. valve ________

   1. ______ valves - either ________ (from donor) or __________ (porcine/bovine)

   2. _________ valves - rarely suitable for patients under ___ yo; problems with _________ and _____/_____

percutaneous; balloon dilation valvuloplasty; surgical; replacement; tissue; homograft; bioprosthesis; mechanical; 5; anticoagulation and size/growth

great vessel defects:

1. ________ ________ - both great vessels arising from a common _____; severe _________ problems as blood mixes uncontrollably

2. _________ of the ______ - a _______; wide range of _______ and _______

3. ________ of the ______ ______ - Ao and PA are _______; about _____ born each year in the US; ______ if left untreated; surgery within first ______ of life (can do an ______ ______ procedure)

truncus arteriosus; trunk; circulatory; coarctation; aorta; narrowing; severity and location; transposition; great arteries; flipped; 1,901; lethal; weeks; artery switch

complex defects:

1. ____ - four components: 1) ___, 2) large ____, 3) _________ aorta, 4) ____ (thick because of ___); _______ always needed to correct it; timing depends on _______ of the ___; patients are usually left with ________ ______; pulmonary valve often needs to be _______ in adolescence; ______ ______ is a gold medal Olympian with this

2. ________ _____ _____ _________ - the whole ____ side of the heart is critically __________; “_____ a _____”; about ____ babies born each year in US; need a ___ stage surgery that results in a heart with a ______ _______ ________ and both _____ ________ connected to it; end up with ________ circulation

TOF; PS; VSD; overriding; RVH; PS; surgery; severity; PS; pulmonary regurg; replaced; Shaun White; hypoplastic left heart syndrome; left; underdeveloped; half a heart; 960; 3; single functioning ventricle; great vessels; Fontan

3 stages of Fontan circulation:

1. _______ within first ____ of life

2. ________ _____ within ___ months

3. _________ _________ _________ around ___-___ years old

Norwood; weeks; bidirectional Glenn; 6; extracardiac fenestrated Fontan; 2-3

Norwood:

1. ________ the ______ to make it bigger, ____ the PDA, surgically ______ ASD, and create ______-______ ______ from ___ to ___ to increase BF to lungs

Bidirectional Glenn:

1. attach ____ to ___ and divide the ______-______ _____

extracardiac fenestrated Fontan:

1. add a ________ to the ____ to attach it to the ___; add _______ between it and the ___

reconstruct; aorta; patch; enlarge; Blalock-Taussig shunt; Ao to PA; SVC; PA; Blalock-Taussig shunt; conduit; IVC; PA; fenestration; RA

Fontan Circulation:

1. 5-year survival rate is about ___%

2. the procedure is ________

3. failing Fontan is unfortunately ________

4. _____ _______ is always required in the long run

5. engineering solutions - creating ______ _______ in Purdue, India for the Fontan ______ created at PA

70; palliative; common; heart transplant; assist devices; cross

overview of issues in pediatric cardiac defects:

1. extreme _______ in defects and the _______ of defects

2. children are not _____ ______

3. risk of _______ exposure with frequent check-ups

4. _______ are big

5. ______ and _____ come into play with treatment

6. _________ anomalies are common

variability; combination; small adults; radiation; emotions; growth and size; concomitant