Module 4 : Cyanotic CHD's Flashcards
what is cyanosis
- blue, gray, dark purple discolouration of the mucous membranes caused by low blood oxygen
what causes cyanosis
- oxygenated blood mixing with unoxygenated blood
- either from blood not getting to the lungs or blood not getting to the body
what are the signs and symptoms of a cyanotic CHD
- cyanosis of lips, toes, fingers, tip of nose
- blueish skin
- hyperventilation
- rounded clubbed fingers
- delayed growth
- tachycardia
- sweating
- SOB
- chronic respiratory infections
how many CHDs are considered cyanotic
- 8
what are the 5 T’s + These (three)
- 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
what is CTGA (D transposition)
- aorta arises from the morphologic RV
- pulmonary artery arises from the morphologic LV
what is the incidence in male to female in CTGA
- 3:1 male to female
what are the 3 anomalies with CTGA and is it considered fatal
- PFO
- transposed PA and AO
- PDA
- fatal if not corrected
why is the otters name for complete TGA D-TGA and why is it dangerous
- DEXTRO TGA
- atrioventricular concordance with ventriculoarterial discordance
- left and right heart are parallel
- blood unable to travel from right to left heart
what is the embryology with D-TGA
- instead of truncus arteriosus spiralling during development they run parallel inside truncus arteriosus
what vessels are connected to what ventricles and
- anterior aortic valve connects to the right ventricle
- posterior pulmonic valve connects to the left ventricle
what is the blood flow direction in DTGA
- shunt dependant lesion
- lesion pumps deoxygenated blood to he aorta and the body and oxygenated blood to the lungs
why is DTGA considered a shunt dependant lesion
- without a shunt of any kind there is no mixing of blood as the blood goes around and in series
what is hypoplastic right ventricle
- secondary to pulmonary atresia with no VSD
- small RV and small or absent pulmonary artery
why’s is hypoplastic right ventricle a shunt dependant lesion
- with small or absent PV/PA and very small RV flow to the lungs is greatly affected
what are the common sonographic features of HLHS
- small LV
- aortic stenosis or atresia
- mitral valve atresia
- associated with hypoplstic aortic arch
- associated with coarctation of the aorta
- ASD
what is the what causes HLHS
- underdevelopment of the left aorta complex
-
what is the difference between HLHS and coarctation of the AO
- larger portion of the aorta is blocked and this condition is more serious
where is retrograde/biphasic flow seen with HLHS
- due to low aorta pressure relative to PA pressure
- Aortic Arch, ascending aorta, coronary arteries
what are the symptoms of HLHS
- born with cyanosis
- less severe clinical presentation if PDA is present
- If PDA closes sudden shock appears
what is tetralogy of Fallot
- complex set of CHDs
- involves 4 anomalies fo heart
what is the most common cyanotic CHD
- tetralogy of fallot
what are the 2 types of tetralogy of fallot
- cyanotic and acyanotic
what are the sonographic features of tetralogy of fallot
- large VSD
- overriding AO
- pulmonary stenosis or atresia anywhere along the FVOT
- hypertrophy of right ventricle
what are the hemodynamics of tetralogy of fallot
- 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
what is truncus arteriosus
- single large vessel arising form the base of the heart
- supplies coronary, pulmonary and systemic circulation
- large VSD large common AO
how many cusps with the common valve have with truncus arteriosus
- 1-6 cusps
what three vessels arise from the single trucks arteriosus trunk
- coronary arteries
- main pulmonary artery
- aortic arch
what is total anomalous pulmonary venous return TAPVR
- pulmonary veins have no connection to the left atrium rather they drain directly of indirectly into the right atrium
what associated anomaly needs to be present for TAPVR be survivable
- ASD or PFO
-
what are the sonographic features of TAPVR
- dilated RA/RV
- small LA
- may have supra cardiac or infracardiac PV connection
what are the treatments of TAPVR
- atrial septostomy
+ short term (puncture the IAS to allow blood mixing) - anastomosis of common pulmonary vein to the LA
- then ASD closure
what is the definition of valvular atresia
- congenital abscess or closure of a normal body opening or tubular structure
what valves are commonly affected by valvular atresia
- pulmonary or tricuspid
what is the circulation of blood with valvular atresia
- 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
what is tricuspid atresia
- 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
what are the 4 associated condition with tricuspid atresia
- VSD
- RV small or hypoplastic
- +/- transposition of great arteries
- ASD/PFO/VSD/PDA necessary for survival
what are the 2 types of pulmonary atresia
- pulmonary atresia with intact inter ventricular septum
- pulmonary atresia with ventricular septal defect
what is pulmonary atresia with intact IVS
- complete obstruction to RV outflow
- intact inter ventricular septum only survivable if ASD/PDA present
- hypoplasia of the tricuspid calc and right ventricle
what are the structures are secondarily affected by pulmonary atresia with intact IVS
- TV
- RV
- RVOT
- branch PA
- coronary arteries
