Complex Cardiac Abnormalities Flashcards
What is it called when the left heart is reduced in size?
hypoplastic left heart syndrome
What is needed to diagnosis of HLHS?
LV < 10 mm
Ao trunk reduced in size
MV distorted or absent
With HLHS, what is visible on U/S?
only the right side of the heart
What is the key of treatment for HLHS?
early detection at 18-20 week scan
What is the treatment for HLHS?
prostaglandin given at birth to maintain patency of PDA and PFO then surgery
What is the downward displacement of TV into the RV?
ebstein’s anomaly
What is associated with lithium in the diet?
ebstein’s anomaly
What is the celemajor index?
distance of anterior leaflet of MV to insertion point of septal TV
> 1.5 indicated poor prognosis
What is the most common malformation in children born with cyanotic heart disease (blue baby)?
tetralogy of fallot
What are the 4 defects of tetralogy of fallot?
- VSD
- pulmonary stenosis
- overriding aorta
- RV hypertrophy
What is a condition where the aorta and pulmonary artery arise from the RV?
double outlet RV
What always includes a VSD?
DORV
What are the symptoms of DORV?
pale/blue baby, trouble breathing, failure to thrive, baby tires while feeding, CHF
What is the difference between DORV and PTA?
DORV the Ao and PA are connected to the RV while in PTA the Ao and PA are connected to the center of the heart over a VSD
What is the most common primary tumor of the heart found in children?
rhabdomyoma
What is associated with tuberous sclerosis?
rhabdomyoma
What is aka ectocardia or exocardia?
ectopia cordis
What is the condition of a beating heart outside of thorax with no protection by skin or bone?
ectopia cordis
What is a subvalvular membrane in the LA that creates 2 LA’s?
cor triatriatum
What is the rare, congenital abnormality of RV where the RV myocardium is absent or hypoplastic?
uhl’s anomaly
What is aka RV dysplasia or parchment heart?
uhl’s anomaly
What is it called when the pulmonary veins partially or totally drain into the RA instead of the LA?
anomalous pulmonary veins
What is the most common location for TAPVR?
supracardiac
Where do the PV’s drain in supracardiac TAPVR?
into SVC via venous connection
Where do the PV’s drain in cardiac TAPVR?
coronary sinus then RA
Where do the PV’s drain in infracardiac TAPVR?
IVC or portal system
What is aka Aarskog syndrome?
persistent left SVC
What is it called when the left sinus horn remains?
persistent left SVC
What is the cause of a missing crux?
complete endocardial cushion defect
What is the cause of a straight or offset crux?
VSD, partial endocardial cushion defect
What is the cause of an exagerrated offset crux?
ebstein’s anomaly
