Module 4 : Cyanotic CHD's

Question 1

what is cyanosis

Answer 1

• blue, gray, dark purple discolouration of the mucous membranes caused by low blood oxygen

Question 2

what causes cyanosis

Answer 2

• oxygenated blood mixing with unoxygenated blood

- either from blood not getting to the lungs or blood not getting to the body

Question 3

what are the signs and symptoms of a cyanotic CHD

Answer 3

• cyanosis of lips, toes, fingers, tip of nose
• blueish skin
• hyperventilation
• rounded clubbed fingers
• delayed growth
• tachycardia
• sweating
• SOB
• chronic respiratory infections

Question 4

how many CHDs are considered cyanotic

Answer 4

• 8

Question 5

what are the 5 T’s + These (three)

Answer 5

• complete TRANSPOSITION of great arteries
• TETROLOGY of fallot
• TRUNCUS arteriosus
• TOTAL anomalous pulmonary venous return
• TRICUSPID atresia
• pulmonary atresia
• hypoeplastic left heart syndrome
• double outlet right ventricle

Question 6

what is CTGA (D transposition)

Answer 6

• aorta arises from the morphologic RV

- pulmonary artery arises from the morphologic LV

Question 7

what is the incidence in male to female in CTGA

Answer 7

• 3:1 male to female

Question 8

what are the 3 anomalies with CTGA and is it considered fatal

Answer 8

• PFO
• transposed PA and AO
• PDA
• fatal if not corrected

Question 9

why is the otters name for complete TGA D-TGA and why is it dangerous

Answer 9

• DEXTRO TGA
• atrioventricular concordance with ventriculoarterial discordance
• left and right heart are parallel
• blood unable to travel from right to left heart

Question 10

what is the embryology with D-TGA

Answer 10

• instead of truncus arteriosus spiralling during development they run parallel inside truncus arteriosus

Question 11

what vessels are connected to what ventricles and

Answer 11

• anterior aortic valve connects to the right ventricle

- posterior pulmonic valve connects to the left ventricle

Question 12

what is the blood flow direction in DTGA

Answer 12

• shunt dependant lesion

- lesion pumps deoxygenated blood to he aorta and the body and oxygenated blood to the lungs

Question 13

why is DTGA considered a shunt dependant lesion

Answer 13

• without a shunt of any kind there is no mixing of blood as the blood goes around and in series

Question 14

what is hypoplastic right ventricle

Answer 14

• secondary to pulmonary atresia with no VSD

- small RV and small or absent pulmonary artery

Question 15

why’s is hypoplastic right ventricle a shunt dependant lesion

Answer 15

• with small or absent PV/PA and very small RV flow to the lungs is greatly affected

Question 16

what are the common sonographic features of HLHS

Answer 16

• small LV
• aortic stenosis or atresia
• mitral valve atresia
• associated with hypoplstic aortic arch
• associated with coarctation of the aorta
• ASD

Question 17

what is the what causes HLHS

Answer 17

• underdevelopment of the left aorta complex

-

Question 18

what is the difference between HLHS and coarctation of the AO

Answer 18

• larger portion of the aorta is blocked and this condition is more serious

Question 19

where is retrograde/biphasic flow seen with HLHS

Answer 19

• due to low aorta pressure relative to PA pressure

- Aortic Arch, ascending aorta, coronary arteries

Question 20

what are the symptoms of HLHS

Answer 20

• born with cyanosis
• less severe clinical presentation if PDA is present
• If PDA closes sudden shock appears

Question 21

what is tetralogy of Fallot

Answer 21

• complex set of CHDs

- involves 4 anomalies fo heart

Question 22

what is the most common cyanotic CHD

Answer 22

• tetralogy of fallot

Question 23

what are the 2 types of tetralogy of fallot

Answer 23

• cyanotic and acyanotic

Question 24

what are the sonographic features of tetralogy of fallot

Answer 24

• large VSD
• overriding AO
• pulmonary stenosis or atresia anywhere along the FVOT
• hypertrophy of right ventricle

Question 25

what are the hemodynamics of tetralogy of fallot

Answer 25

- tighter subpulmonary and PV stenosis the more blood shunts across the IVS into the aorta as it takes the path of lest resistance - overloads the systemic circulation with low oxygenated blood - if TOF occurs with pulmonary atresia very severe

Question 26

what is truncus arteriosus

Answer 26

- single large vessel arising form the base of the heart - supplies coronary, pulmonary and systemic circulation - large VSD large common AO

Question 27

how many cusps with the common valve have with truncus arteriosus

Answer 27

- 1-6 cusps

Question 28

what three vessels arise from the single trucks arteriosus trunk

Answer 28

- coronary arteries - main pulmonary artery - aortic arch

Question 29

what is total anomalous pulmonary venous return TAPVR

Answer 29

- pulmonary veins have no connection to the left atrium rather they drain directly of indirectly into the right atrium

Question 30

what associated anomaly needs to be present for TAPVR be survivable

Answer 30

- ASD or PFO | -

Question 31

what are the sonographic features of TAPVR

Answer 31

- dilated RA/RV - small LA - may have supra cardiac or infracardiac PV connection

Question 32

what are the treatments of TAPVR

Answer 32

- atrial septostomy + short term (puncture the IAS to allow blood mixing) - anastomosis of common pulmonary vein to the LA - then ASD closure

Question 33

what is the definition of valvular atresia

Answer 33

- congenital abscess or closure of a normal body opening or tubular structure

Question 34

what valves are commonly affected by valvular atresia

Answer 34

- pulmonary or tricuspid

Question 35

what is the circulation of blood with valvular atresia

Answer 35

- blood either makes its wy to the LV can either go out the aorta or cross the VSD to the RV and out the narrowed PV and PA

Question 36

what is tricuspid atresia

Answer 36

- absence of direct communication between the right atrium and right ventricle - floor of the RA is entirely muscular with complete separation from the hypoplastic RV - flow crosses the ASD and back across the VSD to the RV then out the PA

Question 37

what are the 4 associated condition with tricuspid atresia

Answer 37

- VSD - RV small or hypoplastic - +/- transposition of great arteries - ASD/PFO/VSD/PDA necessary for survival

Question 38

what are the 2 types of pulmonary atresia

Answer 38

- pulmonary atresia with intact inter ventricular septum | - pulmonary atresia with ventricular septal defect

Question 39

what is pulmonary atresia with intact IVS

Answer 39

- complete obstruction to RV outflow - intact inter ventricular septum only survivable if ASD/PDA present - hypoplasia of the tricuspid calc and right ventricle

Question 40

what are the structures are secondarily affected by pulmonary atresia with intact IVS

Answer 40

- TV - RV - RVOT - branch PA - coronary arteries