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Helens Tuesday test
Congenital Heart Disease
109
Anatomy
Professional
05/09/2012

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Cards

Term
Define Cardioplegia
Definition
The intentional and temporary cessation of the heart to produce asystole.  A specialized solution that is introduced into the coronary circulation.  Its done to slow the metabolism of the heart to prevent damage to the LV and RV.
Term
What are 4 types of ASD's( Atrial Septal Defects)
Definition

-Ostium Secundum

-Sinus Venosus

-Ostium Primum

-Common Atrium

Term
Out of the 4 ASD'a Which is the largest and most common?
Definition

Ostium Secundum

Term
What are some surgical corrections for the ostium secundum ASD?
Definition
septal occluders and closures or pericardial patches or gortex graphs.
Term
Where is the Sinus Venosus ASD located?
Definition
High within the IAS near or at the level of the SVC/PV's
Term
How is the ostium primum ASD caused.
Definition
When the septal primum and endocardial cushions do not meet.  Its also classified as endocardial cushion defect.
Term
Describe common atrium ASD?
Definition
Very large looks like one large atrium, a lot of atrial mixing
Term
What are some surgical corrections for common atrium ASD
Definition
large pericardial graphs and they have to rebuild the IAS
Term
With an ASD assuming its small what is the direction of flow?
Definition
Usually from left to right because the pressures in the LA are higher
Term
With an ASD and if we heard a murmur describe it?
Definition
Itwill be systolic, from left sternal 3rd or 4th intercostal space by the tricuspid valve from left to right
Term
How would an ASD look in a chest x-ray?
Definition
may look normal.  you might see an increased cardiac silhouette and see some pulmonary congestion
Term
How would an ASD effect the heart and which chambers would be impacted?
Definition
It depends on the severity of the lession.  If small nothing.  Eventually all chambers would be impacted but it would start with the RA and RV.
Term
What are some symptoms that couls accompany and ASD?
Definition

feeding difficulties

shortness of breath

arrhythmias

fatigue

*Varies with age*

*Normally right sided

Term
What is Eisenmengers
Definition
Right heart failure, seen in apical VSD's
Term
Listening with a stethescope, what is the sound a VSD would most likely mimic.
Definition
Sounds like an Aortic Stenosis
Term
What are size classifications for VSD's
Definition

1) insignificant

2) small

3) moderate

4) large

Term
What is a common ventrical VSD
Definition

absence of the IVS

may have remnants of tissue distally, sometimes ventricals are inverted and again mixing of blood.

Term
What is a supracristal ASD
Definition

Goes from left ventrical to right outflow track, under the PA

Term
Where would the Gerbode VSD be located
Definition
lies under the Ao, also left to right
Term
what type of murmur would be heard with a VSD
Definition
systolic murmur, very harsh sounding, between the 2nd and 3rd intercostal space
Term
How would a chest x-ray look like if the patient has a VSD
Definition
if significant, a bigger cardiac silhouett with right side bigger
Term
what are some VSD small defects
Definition

goes undetected

no change in growth

no symptoms

no feeding issues

EKG may be normal with mild murmur

Term
what are some large defects with a VSD
Definition

growth failure

large left to right shunt

decreased immune system

shortness of breath

Term

Which VSD is rare in adolescents but more often seen in adults

 

Definition
Post Infarct VSD
Term
What does atresia mean?
Definition
small, narrow, or not present
Term
what are some clinical findings with tricuspid atresia?
Definition

cyanosis after birth and increases with crying

causes cerebral hypoxia

rapid breathing, increased HR

may squat a lot(increases venous flow to heart)

Clubbing

Term
Define clubbing
Definition
swelling of the digits
Term
What are some treatments of TV atresia
Definition

make the ASD or VSD bigger

blood flow to lungs needs to be established or increased

keep the PDA open with ProstagladinE

Term
What is PDA
Definition
patten ductus arteriosis
Term
there are three surgeries over a two year period dealing with TV atresia, what are they?
Definition

Blalock-Tausigg

Glen Shunt

Fontane

Term
What is the reason behind Blalock Tausigg procedure
Definition
left subclavien artery is divided and connected to the left pulmonary artery, allowing blood flow to the lungs to pick up oxygen
Term
what is the glen shunt used for
Definition
seperates the Right Atrium from the SVC, attatch the SVC to the RPA and drains right into lungs
Term
What is the end result with a glen shunt procedure
Definition
completely seperate the right side
Term
What is the fontane procedure
Definition

3rd and final surgury hopefully

complete seperation of systemic from pulmonary circ.

IVC is connected to the RPA

ASD and VSD are closed

PA banding may be necessary

Term
What is ebstiens malformation
Definition

the TV is displaced inferiorly towards apex

leaflets are abnormal

RV becomes arterialized

is compatible with life unless really severe

RVOT is main pumping chamber

decreased bloodflow to lungs

associated with ASD and VSD and pulmonary HTN

Term
Define levocardia
Definition
normal cardiac position, anterior inferior and to the left
Term
Dextrocardia
Definition
Apex is still on the left owever the heart is on the right
Term
Mecocardia
Definition
Heart is in the middle
Term
What does it mean if the heart is in concordance
Definition
Normal alignment; LV w/LA and RV w/RA
Term
Define disconcordance
Definition
abnormal alignment with great vessels and chambers
Term
Cardiac Situs
Definition
Cardiac position
Term
Atrial situs
Definition
Normal atrial position
Term
Situs Inversus
Definition
Everything is flipped
Term
Where is the membranous VSD located
Definition
High in the IVS at the level of the Ao
Term
What is TET
Definition
tetrology of fallot
Term
What are characteristics of TET
Definition

RVOT Obstruction; due to Pulmonic Stenosis

RVH; response to volume overload

Large VSD; can be biderectional but normally L to R

Overriding the Ao

Term
Name Physical findings and symptoms of TET
Definition

Cyanosis

Clubbing

Squatting- changes pressure in the abdominal cavity vs. thoracic cavity

Term
What is the most severe form of TET
Definition

Pulmonary atresia- complete closure of RVOT

Term
Some congenital lessions in TET
Definition

Bicuspid pulmonic valve

right sided arch in 25% of cases

coronary anomalies

R to L shunt

Term
TET surgery:
Definition

there was pallative surgery first and a total repair over a 2 to 4 year period.

 

Now with advancement total repair is done within first 6 months

Term
Surgical complications with TET
Definition

Infection

Arrhythmias

CHF

SBE

Pacemaker

Death

Term
Define mortality
Definition
number of deaths from a specific disease
Term
Define morbidity
Definition
the rate or incidence of that specific disease
Term
Steps of TET surgical repair
Definition
  • Blalock Tausigg sunt is removed
  • VAD and ASD are closed
  • Close PDA
  • In PS the RVOT can be surgically resected and reconstructed
Term
How is the Eisenmengers complex characterized
Definition

Large VSD

Over riding Ao

No RVOT obstruction

PA can be dialated

Abnormal TV ( thickened or Stenotic)

increase in pulmonary vascular resistance

shunts reversed

Term
Findings with a bicuspid Ao valve
Definition

2 cusps rather than 3

can be smooth, thinned or abnormally thick

found more in males

Ao root can be normal or dialated

can be an incidental finding

Term
What is coarctation of the desending Ao
Definition
A narrowing, almost a pinch from malformation
Term
What are the symptoms/signs of a bicuspid Ao
Definition

SOB

Murmur (AS or AI)

evidence of failure

Pulmonary HTN

Vent. arrhythmias

Term
Surgical repair for Ao or Pa valves are possible due to technology advancements?
Definition

No, There is no repair only full replacements for semi-lunar valves.

Aortic valvuplasty

Term
Is it possible for congenital AS to be tolerated
Definition
Yes it can be well tolerated if there are no symptoms
Term
What is congenital AS
Definition
The cusps are fused and not thickened, may become calcific later in life
Term
What are some key symptoms for congenital AS
Definition

Fatigue

CHF, chest pain

Arrhythmias

dyspnea

LVH

murmur

pulmonary congestion

increased cardiac silhouette on the left side and wide media steinum b/c Ao dilated

Term
What is the Ross Procedure
Definition
Taking the PA valve and put it in the place of the Ao valve, then fully replace the PA valve
Term

Name some surgical options for Congenital AS

 

Definition
  • valvuplasty
  • Ao replacement
  • ross procedure
Term
Define Hypoplastic left heart syndrome.
Definition

A single ventrical.

The LV is small or not there at all, Ao can be narrowed, Ao valve may be stenotic and can have MS or coarctation.

Term
Additional Characteristics for hypoplastic syndrome
Definition
  • hypoplastic LA
  • RVE, RVH
  • hypoplastic LV might be filled with a clot
  • Coronaries perfused from PDA down ascending Ao
  • systemic perfusion
  • need a very large ASD for one common atrium
Term
Medical care for Hypoplastic syndrome
Definition
  • Keep PDA open
  • Correct metabolic acidosis
  • Manipulate PVR
  • Intubation
  • Cardiac medication managment
  • O2 supplement
Term
What are thenumber of stages in the Norwood Procedure
Definition
3
Term
Stage II of the Norwood Procedure is what?
Definition
also known as the Glenn procedure. preformed 4 to 6 months as a bridge to stage III.Blalock Tausigg shunts will be disconnected and removed from the RPA.  The SVC is excised and anastomised to the RPA creating systemic venous to pulmonary connection.  Intracardiac mixing still occurs through IVC
Term
What is stage I of the Norwood Procedure
Definition

Pallative and usually performed within first few days

Involves constructing a neo-aorta( dividing the MPA below its confluence with R and L pulmonary branches and attatching the pulmonary trunk together with the pulmonic valve to the hypoplastic base of the Ao.  The PA below R and L branches is closed.

Term
Which stage is also known as the Fontane completion?
Definition
Stage III
Term
What does SAM stand for
Definition
Systolic Anterior Motion
Term
Facts about idiopathic sub-aortic stenosis
Definition
  • small LV cavity demensions, EF@75-80%
  • a degree of LVOT obstruction
  • SAM of the MV
  • change in contour, shape of LV
  • the venture effect
  • preserves systolic function but will lead to diastolic failure
  • intracardiac pressures increase. high pressure in LV reflected in LA and back to Right heart 
Term
What are someclassifications of Heprthropic cardiomyopathies
Definition
  • Obstructive or non-obstructive( is there an outflow track obstruction)
  • ASH
  • LVH
  • abnormal LV chamber
  • Decreased LV volume and area
Term
What is transposition of the great vessels.
Definition
Vessels arise from the wrong ventrical
Term
Another name for transposition of great vessels
Definition
D-transposition
Term
How does D-transposition occur
Definition
abnormal septation of truncus, failure of septation
Term
what is the meaning of dextro
Definition
Right
Term
what is the blood path for a d-transposition case?
Definition

SVC/IVC-RA-RV-Ao

 

LA-LV-PA

Term
What is the most common form of d-transposition?
Definition
Ao is anterior and to the right of the PA
Term

True or False

 

D-transposition is fatal if not treated.

Definition
True
Term
Name 5 clinical features of TGA?
Definition
  • cyanotic at birth
  • heart failure
  • respiratory distress; pulmonary edema
  • cardio megley (R heart dilates)
  • intracardiac pressures abnormal
Term
Surgery steps for TGA are?
Definition
  • pallative ASD, VSD
  • jantene procedure
  • coronary arteries reattached
  • ASD, VSD closed
Term
What is L-transposition
Definition

L=Left

 

is when the heart tube convolutes to the left instead of the right resulting in abnormal reversal of the ventricals

Term
Waht are facts about L-Transposition
Definition

MV, TV remain in there normal position

RV has moderator band, myocardium smaller

atria are usually normal

can go undetected

ventricular inversion (RV-Ao;LV-PA)

ASD,VSD

Term
What is Persistant Truncus Arterious
Definition
Venus and atrial mixing;VSD
Term
Number of PTA's and description
Definition

3 types

 

Type I=single PA trunk, arise from common atrial trunk, w/R and L P. arteries originate from that trunk

 

Type II= L & R PA arise seperately from posterior portion. No PA just 1 large arterial vessel. Right Ao arch is common

 

Type III= L & R PA arise as seperate vessels very widly seperated.  Ao should be normal

Term
Surgical treatments for PTA
Definition

close VSD

Seperate PA from the atrial trunk which will be used fr Ao

make sure RVOT aligned w/ new PA

close ASD

Term
How many types of TAPVR are there
Definition
3
Term
What does TAPVR stand for
Definition
Total Anomalous Pulmonary Venous Return
Term
What does PPVR stand for
Definition
Partial Pulmonary Venous Return
Term
What is supra cardiac TAPVR
Definition
Pulmonary vein flow to an innominate to a vein above the heart
Term
Intra cardiac TAPVR
Definition
Pulmonary flow to coronary sinus and empties into RA then to LA ( due to ASD)
Term
Infra cardiac TAPVR
Definition
Pulmonary vein to IVC (RA-LA because of ASD)
Term
What are surgical treatments for TAPVR
Definition

can be performed in 1-2 months

depends of number of pulmonary veins

ASD closed

pulmonary veins re-anastomosed to the LA

may put baffle/conduit in to help redirect flow in LA. you dont want turbulent flow

Term
Do they usually perform surgery with an anomaly where RCA arises from LMC artery?
Definition
normally no
Term
What is the most serious of coronary artery anomalies
Definition
Left main from the PA
Term
name three anomolies of the coronary arteries
Definition
  • RCA arising from LMC artery
  • Left main from the PA
  • Left main from thr RCA
Term
Facts about  anomaly where the Left Main arises from PA
Definition
  • most common defect; requires surgery
  • LMC lies between PA and Ao, gets pinched with contraction, causes coronary spasm leading to ischemia
  • have to reattach LMC to Ao
Term
Facts about anomoly where Left main arises from RCA
Definition
  • Less serious defect
  • coarse between Ao and PA
  • Puts child or infant at risk for sudden death
  • can go undetected
Term
Define parachute MV
Definition
one pap. muscle, two leaflets; all chorde go to one pap. muscle
Term
Define congenital MS
Definition
Thick leaflets and stenotic; decrease MV area.
Term
MV Prolapse
Definition
one or both leaflets prolapse into LA, causes volume overload to LA; seen more in females; can go undetected
Term
Mixoma
Definition
Benign cardiac tumors, slow growing. predominatly in the LA for children typically they will grow in LV; can go undetected and mimic stenosis
Term
Facts about endocardial defects
Definition
  • ASD,VSD, cleft MV
  • cushions partially fuse resulting in division of AV canal
  • Is a R/L ostium; has R/L flow
  • Primum ASD (partial ECD)
  • cleft MV or TV
  • volume overload to right heart
Term
What are surgical repair and survival rate for ECD
Definition
  • if not repaired mortality rate is high @ 80% at 2 years due to increasing CHF and pulmonary disease
  • restore normal circ. by closing ASD, VSD with patches
  • Pulmonary band
  • reconstruction of MV and TV
  • repair additional defects like Pulmonary stenosis
Term
Clinical indings for ECD
Definition
  • fixed or split S2
  • failure to thrive
  • fatigue
  • dyspnea
  • PHTN
  • large cardiac silhouette
  • EKG
  • sign of CHF
  • RV enlargment, hypertrophy (RV)
Term
Levels of VSD's
Definition
  • High membranous
  • Mid Septal
  • mid muscular VSD, apical VSD, supra cristal VSD (shunted in RVOT)
Term
Where are membranous VSD's located
Definition
high in the IVS at level of the Ao
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