6000 CHD - Dr. Blue

normal CV embryological development:

1. CV development begins in the ___ week of gestation

2. embryo dependent on _________ for O2 and nutrition

3. paired ________ heart _____ develop

4. primitive heart begins to beat at ___-___ days

3rd; placenta; endothelial; tubes; 22-23

fusing and separating:

1. the two tubes fuse to make a ______ ________ ______

2. heart tube develops series of ________ and _________

3. heart tube then forms a ___-_______ ______ (this places the future ______ on top and future _________ on bottom

single endothelial tube; constrictions and dilations; U-shaped loop; atria; ventricles

septation:

1. ridge forms in _____ of the common atrium and grows _______ (called septum ________) but leaves a large opening called ________ ________

2. septum ________ develops inferiorly and grows _______; fuses with _______ but leaves patent _______ ______

3. superior edge of septum _______ forms flaplike valve that only allows ____-___-_____ flow through _______ ______

roof; downwards; primum; ostium primum; secundum; upwards; primum; foramen ovale; primum; right-to-left; foramen ovale

more septation:

1. muscular ridge arises in the ________ _________ ventricle; this develops into _________ __________ __________

2. later portions of the ________ ________ fuse to form the ________ portion of the IVS

common primitive; muscular interventricular septum; endocardial cushion; membranous

spiraling:

1. ______ _______ and ______ _______ fuse and spiral 180 degrees to form aorticopulmonary septum

2. this septum creates two _______ ________ (future Ao and PA)

bulbus cordis and truncus arteriosus; arterial channels

valves:

1. tissue develops at origin of _______ and ________ orifices and these develop into _______ and ________ valves

2. tissue develops in the __________ region and differentiates into ______ and ________ valves

aorta and pulmonary; aortic and pulmonary; endocardial; mitral and tricuspid

fetal circulation:

1. occurs in ________ and transitions

2. purpose is to supply ________ _______ to the growing fetus while bypassing the _______ where no ________ is occurring

3. _________ blood comes from the _________ through the ______ _______ into the ___; passes into RA and most passes through _____ into LA (only a small amount goes to ______ since no oxygenation is occurring)

4. _________ blood in RV is pumped out through the ___ but the ________ ________ detours blood away from the ______ to the body before birth

phases; oxygenated blood; lungs; oxygenation; oxygenated; placenta; ductus venosus; IVC; PFO; lungs; oxygenated; PA; ductus arteriosus; lungs

ductus arteriosus:

1. _________ in the blood keep the DA open in fetal life

2. at birth, __________ levels _____ and DA closes

3. sometimes if DA fails to close, they give ______ which _______ the effects of ___________ which can help induce closure; indomethacin commonly used

4. in certain CHDs, (like _____) it may be beneficial to keep DA _______ (or _______) to allow for continued _________ until surgery can be performed

prostaglandins; prostaglandin; drop; NSADIs; inhibit; prostaglandins; TGA; patent; open; circulation

two broad categories of CHD…

acyanotic and cyanotic

acyanotic:

1. includes intracardiac or vascular ______, vascular ________, and _______ that result in ____-___-_____ shunting of blood; over time, because of increased _________ ________ ________, the shunt may reverse to _____-___-______ leading to ________

cyanotic:

1. occurs when _______ ________ blood from the _____ side of the heart is shunted to the ______ side, bypassing the ______

cyanosis:

1. a _____-______ color of the skin and mucous membranes caused by elevated concentration of __________ __________

stenosis; regurgitation; defects; left-to-right; pulmonary vascular pressure; right-to-left; cyanosis; poorly oxygenated; right; left; lungs; blue-purple; deoxygenated hemoglobin

5 categories of CHD based on physiology…

1. ________ lesions

2. _______ _______ ________ lesions

3. _______ _______ ________ lesions

4. _________ lesions

5. ________ _________

shunting; right sided obstructive; left sided obstructive; regurgitant; parallel circulation

shunting lesions:

1. these lesions result from _______ defects at _______ or ________ or a ____ at great vessel level; cause abnormal _____ of ______ from the _________, _____ side of the heart (or ________ circulation) into the ______ side of the heart (or _________ circulation)

2. examples = ____, ____, and ____

septal; atrial or ventricular; PDA; flow of blood; oxygenated, left; systemic; right; pulmonic; ASD, VSD, PDA

right sided obstructive lesions:

1. variable degrees of _______ or _______ at valvular or great vessel level leading to _______ ________ of RV; often with _____ to _____ shunting allowing __________ blood to bypass lungs and producing _________

2. examples = ________ ________ and ____

stenosis or atresia; volume overload; right to left; deoxygenated; cyanosis; pulmonary stenosis and TOF

left sided obstructive lesions:

1. lesions of the left heart with ________ or _______

2. examples = ________ ______ _________, _________ of the ______, and __________ _______ ________ syndrome

stenosis or atresia; congenital aortic stenosis; coarctation of the aorta; hypoplastic left heart syndrome

regurgitant lesions:

1. regurgitation of the ______ or ______ valves which can cause ________ ________ of the respective V

2. example = _______ ________

aortic or pulmonary; volume overload; epstein anomaly

parallel circulation:

1. physiologic class reserved for _________ of the ______ _______ whereby the ________ and ________ circulation exist in ________, resulting in ________

2. example = _____

transposition of the great arteries; systemic and pulmonary; parallel; cyanosis; TGA

acyanotic lesions:

1. ____

2. ____

3. ____

4. ________ _______ _________

5. ________ _______ ________ _________

6. _________ of the ________

ASD; VSD; PDA; congenital aortic stenosis; congenital pulmonary valve stenosis; coarctation; aorta

cyanotic CHDs:

1. ________ of ______

2. _________ of the ________ _______

3. __________ _________

tetralogy of Fallot; transposition of the great arteries; Eisenmenger’s syndrome

who was the African American janitor that ended up being a really good heart surgeon?

Vivien Thomas

who did Thomas work under at Vanderbilt?

Alfred Blalock

who discovered leaving PDA open helped TGA?

Dr. Helen Taussig

ASD:

1. persistent opening in the ________ ________ that results in direct communication between ____ and ____ atria

2. one of the more ________ types of CHD

3. most common ASD is ________ _______ that occurs in the region of the _______ _____

4. less common is _______ _______ in the _______ portion of the septum

5. hemodynamically similar but embryologically different is ______ ______ defect

6. ____ normal in utero but flaps fail to fuse and persistent defect remains

interatrial septum; left and right; common; ostium secundum; foramen ovale; ostium primum; inferior; sinus venosus; PFO

ASD continued:

1. typically there is BF from _____ atria to _____ atria; slowly over time RV is _______; rarely progresses to _________; they may be _________ for decades

2. usually ________ and ________ are asymptomatic

3. ______ murmur

4. _____ is diagnostic study of choice

5. if hemodynamically significant, then ______ is recommended (________ or _________)

left; right; overloaded; Eisenmenger’s; asymptomatic; newborns and infants; systolic; echo; repair; surgical or percutaneous

VSD:

1. abnormal opening in the _________ _______; usually ____-___-_____ shunt but over time may reverse; _____ matters

   1. ________ or _________ portions can have VSDs

2. if ____, either early or late, then repair

3. in about ___% of small to moderate VSDs in ________ septum there will be spontaneous ______ as child grows

4. repair may be _______ or _________

interventricular septum; left-to-right; size; muscular or membranous; CHF; 50; muscular; closure; surgical or percutaneous

PDA:

1. DA is a ______ vessel that connects ___ to ________ ______; if can fail to ______ after birth; rare, but more likely if mom had ______ in first trimester, baby was _______ born, or a birth at ______ ______

2. remember that ________ keep it open; trial with _________ to get closure, otherwise, _______ closure required

fetal; PA to descending aorta; close; rubella; prematurely; high altitude; prostaglandins; indomethicin; surgical

congenital aortic stenosis:

1. usually from _______ valve

2. not uncommonly associated with _______ of the ______

3. if severe obstruction at birth and CHF, then _______ or _______ and ______ when child is larger

4. most frequently the obstruction _______/______ over time and is not detects, found, or become asymptomatic until patient is ___-___ yo

5. frequent association with pathology of the _______ _______

bicuspid; coarctation of the aorta; repair or valvuloplasty and repair; develops/worsens; 30-40; ascending aorta

pulmonic valve stenosis:

1. usually the valve ______ is stenosed, but can also be in the _______ ______ of RV or in the ___ beyond valve

2. mild PS does not require ________; if increasing ___ ______ and ___ _____, then repair

3. most commonly fixed with _________ _________ __________

itself; outflow tract; PA; treatment; RV size; RV CHF; transcatheter balloon valvuloplasty

coarctation of the aorta:

1. discrete ______ ______ narrowing of the lumen of aorta

2. frequently associated with _______ ________ valve; also an occur in ________ sydrome

3. later in life may be detected due to ______ ___ in the arms and _____ ___ in the legs

4. if severe in ______ then give _________ infusion to keep DA open until surgery; if older and larger, can sometimes be done with ________ ________

napkin ring; bicuspid aortic; Turner’s; high BP; low BP; neonate; prostaglandins; balloon dilation

cyanotic lesions of tetralogy of fallot:

1. ____ caused by _________ of IVS

2. __________ PS

3. _____-______ aorta that receives blood from _____ ________

4. RVH secondary to _____ ______ caused by ___

VSD; malalignment; subvalvular; over-riding; both ventricles; high pressure; PS

TOF repair:

1. initially treatment is creating anatomic communications between ______ (or one of its main branches) to ___, establishing a ____-___-____ shunt to increase ________ BF

2. now may do initial ________ procedure to allow _______ of the child and then do repair

3. history of TOF surgery:

   1. _______ to ___ shunt by Blalock, Taussig, and Thomas in 1944

   2. first complete repair 10 years late by Lillehei using _____-________ and by Kirlin with early/primitive ____

aorta; PA; left-to-right’ pulmonary; palliative; growth; systemic to PA; cross-circulation; CPB

transposition of great arteries:

1. in transposition, each artery arises from the _______ ________; RV to ______ and LV to ___

2. possible due to failure to ______

3. ______ if not recognized and treated

4. treatment

   1. maintain ______ ________ patency with ________ then do the _______ procedure which is _______ _______ at level of intra-atrial septum

   2. definitive corrective surgery presently is an ______ _______ or ______ procedure

opposite ventricle; aorta; PA; spiral; lethal; ductus arteriosus; prostaglandins; Rashkind; balloon dilation; arterial switch or Jantene

Eisenmenger’s syndrome:

1. when there is severe ________ ________ and ________ ________ ________ secondary to chronic ____-___-______ shunting and over time, pressures get high on the ____ and reverses the shunt to be ____-___-____ making patient _______

2. maybe ____, ____, or combination and a few other rare ____-___-____ shunts that go undetected

3. no _________ treatment

4. shunt closure usually causes _____

5. becoming more rare because most CHD is being pick up ______

6. only effective treatment is ______-______ __________

pulmonary hypertension and pulmonary vascular resistance; left-to-right; right; right-to-left; cyanotic; ASD, VSD; left-to-right; successful; death; earlier; heart-lung transplant

GUCH - grown up CH

1. many/most CHD kids make it to _________ and do _____

2. however, they need to be _________ ________ by GUCH specialist

3. depending on underlying lesion, may be at risk for late ____

4. particularly prone to __________ and ____, once again depending on original lesion; ____ is particularly a problem in these patients

adulthood; well; monitored regularly; CHF; arrhythmias and SCD; AF