Congenital Heart Defects Flashcards
Shunts that progress from R to L present _________ than shunts from L to R
earlier
How do we remember the R to L shunts?
5 T’s
are Terrible, i.e. present earlier
(Tetralogy of Fallot, Transposition, Truncus arteriosus, Tricuspid atresia, TAPVR)
How to we remember the L to R shunts?
VSD/ASD/PDA
L to R = Late
Tetralogy refers to what in Tetralogy of Fallot?
Pulmonary Stenosis
RVH
Overriding aorta
VSD
(PROVe it!)
A very young child presents because his mom notices his face turns blue every now and then. He squats down, and the symptoms get better. What is the most important determinant in the prognosis of the child?
Degree of PULMONARY STENOSIS
presents young, so must start with a T and be a R to L shunt
What is the mechanism of squatting in a Tetralogy of Fallot patient?
increases systemic pressure, which decreases the R to L shunt, which pushes blood into the pulmonary artery (decreasing symptoms)
What is often the shape of the heart on x ray in Tetralogy of Fallot?
boot shaped
seen in RVH
The cause of all the symptoms of Tetralogy of Fallot comes down to what anatomical defect?
anterosuperior displacement of the infundibular septum
VSD/ASD/PDA is to pulmonary HYPERtension as Tetralogy of Fallot is to __________
HYPOtension
due to stenosis; will show decreased pulmonary vascular markings on x ray
Describe the flow of events (Eisenmenger syndrome) that occurs in children with late cyanosis.
starts as L-R shunt (VSD/ASD/PDA)
slowly develop pulmonary HTN (due to hypertropy)
resistance becomes super hi, get reversal of shunt and appearance of cyanosis/clubbing/polycythemia
Contrast the location of the two types of coarctation of the aorta
Infantile: often btw branches of aorta and ductus
Adult: often AFTER the ligamentum arteriosum
(INfantile = IN closer; aDult=Distal to Ductus)
In adult form of coarctation of the aorta, what vessels will be grossly dilated?
IMA artery (internal mammary, aka internal thoracic)
sends blood via anterior intercostal arteries to bypass the stenotic aorta
leads to RIB NOTCHING
Describe the physical exam of a pt with coarctation of the aorta.
HTN/flushing/strong pulse in the UE
hypotension/cyanosis/weak pulse in the LE
What two key associations are linked to infantile coarctation of the aorta?
Turner syndrome (45 XO)
PDA
Adult coarctation of the aorta is associated with ________ and can lead to ______________
bicuspid aortic valve
aortic regurgitation
An infant pops out and has cyanosis. Her aorta is anterior to the pulmonary trunk. What should be given immediately?
PGE1 (alprostadil)
keeps ductus arteriosus open until sx
Alprostadil is also used for?
erectile dysfunction
An infant pops out and has cyanosis. Her aorta is anterior to the pulmonary trunk. What condition did her mother most likely have?
Maternal diabetes
high assoc with transposed great vessels and sacral agenesis
D-transposition of great vessels is to ‘failure to spiral’ as Persistent Truncus Arteriosus is to _____________
failure of aorticopulmonary septum to develop
A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. What treatment is given to correct the issue?
Indomethacin closes a PDA
A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. What are the characteristics of the most likely infectious organism that mom had?
Rubella (German measles)
Togavirus family; Enveloped RNA, SS + linear
A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. His mom had a congenital infection. What should we check this little dude for?
cataracts (assoc with maternal Rubella)
A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. What are the most likely manifestations mom had as a result of an infection?
Fine Rash (starts at head, moves down)
LAD
Arthraligia/arthritis
An infant presents with early cyanosis. Imaging reveals a hypoplastic RV and an ASD. What is the most likely etiology?
Tricuspid Atresia
