08-23-07 Zaman - Heart 4 - Congenital Heart Diseases

Front 1

The most common cause of heart disease in children in the Western world

Back 1

congenital heart disease

Front 2

3 major groups of congenital heart disease

Back 2

L to R shunt
R --> L shunt
Obtructions

Front 3

cyanotic congenital heart disease is part of which group?

Back 3

right to left shunt

Front 4

infective endocarditis is common to which major group of congenital HD

Back 4

all 3

Front 5

the most common type of congenital malformation

Back 5

left to right shunt

Front 6

what specific malformations cause L to R shunts?

Back 6

VSD, ASD, PDA

Front 7

What is "tardive cyanosis"?

Back 7

when a left to right shunt flow reverses

Front 8

most common type of ASD (75%)

Back 8

ostium secundum ASD
(septum secundum fails to cover the ostium scundum)

Front 9

second mos common type of ASD (15%)

Back 9

ostium primum ASD
(septum primum does not fuse with endocardial cushion)

Front 10

least common type of ASD (10%)

Back 10

sinus venosus ASD

Front 11

where are sinus venosus ASDs?

Back 11

high in the atrial septum

Front 12

Inferior
(rare picture of both ostia in one heart)

Back 12

which is the ostium primum ASD?

Front 13

most common congenital heart malformations diagnosed in adults

Back 13

ASDs
(b/c many VSDs close spontaneously)

Front 14

Most common congenital heart malformations in general

Back 14

VSDs

Front 15

Ostium Primum ASDs are more likely assoc with CHF b/c of high frequency of _________ insufficiency
(partially)

Back 15

mitral

Front 16

Clinical signs of ASD

Back 16

- wide fixed split P2 with pulmonary flow murmur
- pulmonary vasculature normal to increased
- Eisenmenger's syndrome

Front 17

Type of ASD that is well-tolerated, especially if it is less than 1cm diameter.

Back 17

ostium secundum
(pulmonary HTN develops slowly and shunt reverses --> cyanosis)

Front 18

most common congenital heart lesion at birth

Back 18

VSD

Front 19

where are the basal (membranous) and muscular parts of the ventricular septum

Back 19

Membranous part grows from endocardial cushions
Muscular part grows up from apex

Front 20

Site where 70% of VSDs are located

Back 20

in the thinner membranous part of the septum

Front 21

murmur type heard with VSD

Back 21

pansystolic

Front 22

genetic diseases associated with VSD

Back 22

Trisomy 21, 13, 18

Front 23

____% of VSD cases occur alone w/o other malfomations

Back 23

30

Front 24

___% of VSD pts develop Eisenmenger's snyndrome

Back 24

10

Front 25

small or medium sized VSDs that produce jet lesions have the risk of __________

Back 25

superimposed infective endocarditis

Front 26

The 6th most common congenital Heart disease :)

Back 26

PDA
(10% of all cardiac malformations)

Front 27

What makes the ductus constrict after birth?

Back 27

1-increased levels of arterial oxygen,
2-decreasing pulmonary vascular resistance, and
3-declining levels of prostaglandin E2

Front 28

Membranous (basal)

Back 28

What type of VSD

Front 29

PDA is more common in females, T or F?

Back 29

true

Front 30

murmur heard with PDA

Back 30

machine-like, continuous, waxing and waning

Front 31

2 distinct presentations of PDA

Back 31

1- premature infants in postnatal period
2- term infants at 3 months

Front 32

What would delay closure of the ductus after birth

Back 32

hypoxia, resp. distress, heart disease

Front 33

There is higher incidence of PDA in the presence of maternal _________ infection.

Back 33

rubella

Front 34

polycythemia is associated with what congenital heart disease?

Back 34

PDA

Front 35

Treatment of PDA

Back 35

- surgical correction (cure)
- coil occlusion
- indomethacin in premies (80% success)

Front 36

right to left shunts (4)

Back 36

1- tetralogy of Fallot
2- transposition of the great vessels
3- tricuspid atresia
4- total anomalous pulonary venous connection (TAPVC)

Front 37

cyanotic heart defects at birth

Back 37

the Ts and Truncus Arteriosus

Front 38

Cyanotic heart defects after birth, when right-sided pressure increases

Back 38

ASD, VSD, PDA

Front 39

the most common cause of cyanotic heart disease

Back 39

tetralogy of Fallot

Front 40

The four components of the tetralogy are ________

Back 40

(1) a VSD,
(2) a "dextraposed" aortic root that overrides the VSD, (3) right ventricular outflow obstruction, and
(4) right ventricular hypertrophy

Front 41

What genetic disease is assoc. with Tetralogy of Fallot?

Back 41

Down syndrome

Front 42

What is the most likely lesion in a pt w/ right-sided arch and cyanosis

Back 42

tetralogy of Fallot

Front 43

Tetralogy of Fallot accounts for ____% of all congenital cardiac malformations

Back 43

6

Front 44

Shape of heart in tetralogy of Fallot

Back 44

Boot-shaped

Front 45

describe the proximal aortic root in tetralogy of Fallot

Back 45

enlarged

Front 46

describe pulmonary trunk in tetralogy of Fallot

Back 46

small with narrowing or stenosis

Front 47

When does pulmonary HTN start in Tetralogy of Fallot?

Back 47

never

Front 48

Effects of chronic cyanosis

Back 48

-hyperviscosity secondary to erythrocytosis
-digital clubbing
-systemic embolism

Front 49

the 2nd leading cause of cyanotic heart disease

Back 49

transposition

Front 50

patients with tetralogy of Fallot are also at increased risk for infective endocarditis, systemic emboli, and _________.

Back 50

brain abscesses

Front 51

the 2nd leading cause of cyanotic heart disease

Back 51

transposition

Front 52

patients with tetralogy of Fallot are also at increased risk for infective endocarditis, systemic emboli, and _________.

Back 52

brain abscesses

Front 53

treatment of transposition

Back 53

prostaglandin E2, balloon septostomy and the creation of shunts, followed by definitive surgical correction

Front 54

Truncus arteriosus is ___% of congenital heart malformations

Back 54

2

Front 55

Truncus arteriosus is a R to L shunt with heart failure and _________ infections

Back 55

respiratory tract

Front 56

Is truncus arteriosus correctable with surgery?

Back 56

yes

Front 57

complete occlusion of the tricuspid valve

Back 57

tricuspid atresia
(not discussed in Robbins 7th ed)

Front 58

For tricuspid atresia to allow life, what malforamtions are present?

Back 58

ASD, VSD (for the great artery communication in RV)
(not in Robbins 7th ed)

Front 59

Condition where no pulmonary veins directly join the left atrium

Back 59

total anomalous pulmonary vascular connections
(TAPVC)

Front 60

Instead of from the pulmonary veins, the left atrium gets blood how?

Back 60

Blood goes from coronary sinus --> to right atrium
--> ASD or patent foramen ovale
--> Left atrium

Front 61

congeniatl obstructive lesions (2)

Back 61

coarctation of aorta
valvular stenosis

Front 62

coarctation of the aorta is more common in the ____ gender and also _____ syndrome

Back 62

male

Turner syndrome

Front 63

coarctaion of the aorta is associated with ______ aneurysms of the CNS

Back 63

saccular

Front 64

most common type of coarctation of aorta

Back 64

post-ductal

Front 65

narrowing of the aortic isthmus

Back 65

preductal coarctation ("infantile")

Front 66

type of aortic coarctation wherer ductus is patent

Back 66

preductal

Front 67

type of aortic coarctation with LVH

Back 67

postductal

Front 68

"Anomaly":
RV is enlarged and there is abnormal septal and posterior tricuspid leaflets

Back 68

Ebstein's anomaly

Front 69

Ebstein's anomaly may be familial or associated with maternal _______ ingestion

Back 69

lithium

Front 70

condition that occurs with redundant and fenestrated leaflet

Back 70

Ebstein's anomaly

Front 71

condition that occurs with conduction abnormalities (Wolf-Parkinson-white syndrome)

Back 71

Ebstein's anomaly

Front 72

condition that causes atrialization of apical right ventricle

Back 72

Ebstein's anomaly

Front 73

treatment of Ebstein's anomaly

Back 73

-valvuloplasty and RV reduction
-tricuspid valve replacement
-ablation of conduction bypass pathways
-antiarrythmia agents

Front 74

clinical presentation of Ebstein's anomaly

Back 74

cyanosis
syncope
CHF
palpitation
clubbing
SCD

Front 75

13th most common CHD

Back 75

hypoplastic left heart syndromes

Front 76

a group of disorders characterized by underdeveloped left cardiac chambers

Back 76

hypoplastic left heart syndromes

Front 77

syndromes with marked cardiomegaly and increased pulmonary vascularity
Also, life depends on ductus areteriosus

Back 77

hypoplastic left heart syndromes

Front 78

Clinical presentation of hypoplastic left heart syndromes

Back 78

poor CO,
poor appetite and hypoglycemia,
eventual IHD,
acidosis,
maybe shock

Front 79

treatment of hypoplastic left heart syndromes?

Back 79

treat the acidosis and shock.

need surgery or transplant

Front 80

In severe cases the ostium primum defect is accompanied by a VSD and severe mitral and tricuspid valve deformities, with a resultant common ________

Back 80

atrioventricular canal

Front 81

69% of folks with atrioventricular canal have _______ syndrome

Back 81

Down

Front 82

heart is a mirror image of normal; sometimes assoc. with situs inversus

Back 82

dextrocardia

Front 83

3 types of congenital HD

Back 83

L-R, R-L, obstruction

Front 84

most common type (of 3) of Congenital HD

Back 84

left to right shunts

Front 85

what are right to left shunts due to?

Back 85

the T's;
tetralogy, transposition, TA, TAPVC

Front 86

COndition with abnormal tricuspid valve leaflets

Back 86

Ebstein's anomaly

Front 87

an endocardial cushion defect with failure to form a variety of structures

Back 87

AV canal