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42 Cards in this Set

  • Front
  • Back

Out of 1000 babies, how many are born with congenital heart diseases?

8

What percentage of babies born with CHD are cyanotic?

20%

Which 5 disorders at birth lead to cyanosis?

The 5 T's
1) Tetralogy of Fallot


2) Transposition of the Great Arteries


3) Truncus Arteriosus


4) Tricuspid Atresia


5) Total Anamolous Pulmonary Venous Return

What 4 types of shunts are acyanotic?

1) ASD


2) VSD


3) PDA


4) AVSD

What 3 types of obstruction are acyanotic?

1) AS


2) PS


3) Coarctation of the aorta

What is another term for Eisenmenger's syndrome?

Tardive/Late onset cyanosis


(due to pulmonary scarring)

What chromosomal abnormalities can lead to CHD? (5)

- Trisomy 13, 15, 18, 21


- Turner Syndrome (Xo)

What single gene defects can lead to CHD? (3)

1) DiGeorge Syndrome


2) Noonan Syndrome


3) Williams Syndrome

What in utero disease states can lead to CHD? (2)

1) Congenital Rubella Syndrome


2) Diabetic Mother

What CHD is likely in a neonate with Down syndrome?



Turner Syndrome?

Down - AVSD



Turner - Coarctation of the aorta

What CHDs are likely in DiGeorge, Noonan, and William's syndromes?

DiGeorge - Truncus arteriosus



Noonan - Pulmonic Stenosis



Williams - Supravalvar Aortic Stenosis

What CHD would in utero rubella infection cause?

Patent Ductus Arteriosus

What CHD would in utero diabetic mother cause?

Transposition of the Great Arteries

Does blood oxygen saturation decrease with left to right shunt?

No

Why does the right ventricle experience hypertrophy over time in a left to right shunt CHD?

Pulmonary capillaries constrict leading to less surface area and thus greater pressure in the right ventricle due

What kind of murmur is heard in a ASD?

Flow murmur over right ventricular outflow tract

What is the most common ASD?

Secundum

What relation does VSD size have with murmur sound?

The smaller the VSD, the louder the murmur

What are 2 types of VSD?

Membranous and muscular

What can occur with untreated PDA?

CHF due to lost of pressure in aorta that should have gone to coronary arteries

What are the treatments for PDA? (3)

- Surgical ligation


- Transcatheter coil occlusion


- Indomethacin

What does cyanosis mean in terms of blood flow?

Blood is shunted right to left

What would an x-ray show missing in a cyanotic baby?

No vascular markings in pulmonary blood flow


(AKA black lungs)

If CHD is not repaired in a cyanotic baby, what 5 complications can occur?

1) Growth retardation


2) Polycythemia (upping RBCs and Hb)


3) Clubbing (swelling of fingers)


4) Cerebral hypoxia


5) Paradoxic embolism

How is a large VSD in ToF measured?

Whether it is larger than aortic valve

What is the area under the pulmonary valve called?

Infundibulum of the right ventricle

What is an overriding aorta?

In ToF, aorta is shifted right so part of it is over the right ventricle

What risk does stress or valsalvic pressure have for a baby with ToF?

Spasms in muscle beneath pulmonary valve, cutting pulmonary circulation (Tet spells)

How would a neonate survive with TGA?

Some kind of shunt (VSD, PDA, PFO)

How is TGA mitigated at birth?

Balloon atrial septostomy

What occurs if TGA with shunt is unrepaired?

RVH

What is another term for surgical switch of great arteries in TGA?

Mustard

What two problems occur with Congenitally Corrected TGA in later years? (2)

Right ventricular failure and tricuspid valve failure

What is truncus arteriosus?



With what other CHD is it associated?

Failure to divide the trunk into an aorta and pulmonary artery



VSD

What is tricuspid atresia?

No valve between RA and RV, just a solid muscular floor



(ASD and VSD must be present to survive in utero)

What is TAPVR?

Total Anamolous Pulmonary Venous Return: pulmonary veins fail to drain into LA and instead drains into the coronary sinus or portal venous system

How does coarctation of the aorta present later in life if it is not discovered in infancy?

Hypertension in a teenager

What is the characteristic appearance in children with Williams syndrome? (4)

- Elven faces


- Lateral upper eyelid fullness


- Small nose


- Full lips

What psychological signs do children with Williams syndrome have? (3)

- Irritability


- Mimicking (cocktail party personality)


- Mental retardation

What gene is defective in Williams syndrome?

Elastin gene on chromosome 7

What are the facial features of Noonan syndrome? (4)

- Short webbed neck


- Epicanthal folds


- Ptosis


- Ear malformation

What other physical signs besides the face are associated with Noonan Syndrome? (3)

- Hypoplastic nipples


- Pectus excavatus


- Cryptorchidism