Complex Cardiac Abnormalities

Question 1

What is it called when the left heart is reduced in size?

Answer 1

hypoplastic left heart syndrome

Question 2

What is needed to diagnosis of HLHS?

Answer 2

LV < 10 mm
Ao trunk reduced in size
MV distorted or absent

Question 3

With HLHS, what is visible on U/S?

Answer 3

only the right side of the heart

Question 4

What is the key of treatment for HLHS?

Answer 4

early detection at 18-20 week scan

Question 5

What is the treatment for HLHS?

Answer 5

prostaglandin given at birth to maintain patency of PDA and PFO then surgery

Question 6

What is the downward displacement of TV into the RV?

Answer 6

ebstein’s anomaly

Question 7

What is associated with lithium in the diet?

Answer 7

ebstein’s anomaly

Question 8

What is the celemajor index?

Answer 8

distance of anterior leaflet of MV to insertion point of septal TV

> 1.5 indicated poor prognosis

Question 9

What is the most common malformation in children born with cyanotic heart disease (blue baby)?

Answer 9

tetralogy of fallot

Question 10

What are the 4 defects of tetralogy of fallot?

Answer 10

1. VSD
2. pulmonary stenosis
3. overriding aorta
4. RV hypertrophy

Question 11

What is a condition where the aorta and pulmonary artery arise from the RV?

Answer 11

double outlet RV

Question 12

What always includes a VSD?

Answer 12

DORV

Question 13

What are the symptoms of DORV?

Answer 13

pale/blue baby, trouble breathing, failure to thrive, baby tires while feeding, CHF

Question 14

What is the difference between DORV and PTA?

Answer 14

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

Question 15

What is the most common primary tumor of the heart found in children?

Answer 15

rhabdomyoma

Question 16

What is associated with tuberous sclerosis?

Answer 16

rhabdomyoma

Question 17

What is aka ectocardia or exocardia?

Answer 17

ectopia cordis

Question 18

What is the condition of a beating heart outside of thorax with no protection by skin or bone?

Answer 18

ectopia cordis

Question 19

What is a subvalvular membrane in the LA that creates 2 LA’s?

Answer 19

cor triatriatum

Question 20

What is the rare, congenital abnormality of RV where the RV myocardium is absent or hypoplastic?

Answer 20

uhl’s anomaly

Question 21

What is aka RV dysplasia or parchment heart?

Answer 21

uhl’s anomaly

Question 22

What is it called when the pulmonary veins partially or totally drain into the RA instead of the LA?

Answer 22

anomalous pulmonary veins

Question 23

What is the most common location for TAPVR?

Answer 23

supracardiac

Question 24

Where do the PV’s drain in supracardiac TAPVR?

Answer 24

into SVC via venous connection

Question 25

Where do the PV's drain in cardiac TAPVR?

Answer 25

coronary sinus then RA

Question 26

Where do the PV's drain in infracardiac TAPVR?

Answer 26

IVC or portal system

Question 27

What is aka Aarskog syndrome?

Answer 27

persistent left SVC

Question 28

What is it called when the left sinus horn remains?

Answer 28

persistent left SVC

Question 29

What is the cause of a missing crux?

Answer 29

complete endocardial cushion defect

Question 30

What is the cause of a straight or offset crux?

Answer 30

VSD, partial endocardial cushion defect

Question 31

What is the cause of an exagerrated offset crux?

Answer 31

ebstein's anomaly