20.Congenital heart disease

Question 1

types causes of congenital heart defects

Answer 1

cyanotic

non cyanotic

Question 2

cyanotic causes of congenital heart defects

Answer 2

tetralogy of fallot

transposition of great arteries

persistent truncus arteriousus

ebsteins anomaly

tricuspid atresia

Question 3

non cyanotic causes of congenital heart defects

Answer 3

VSD
ASD
patent ductus arteriousus 
aortic stenosis
pulmonary stenosis
coarctation of the aorta

Question 4

epidemiology of congenital heart defects

Answer 4

affects 4-9/1000 fulterm live born babies

most common dg congenital condition

Question 5

risk factors of congenital heart defects

Answer 5

parent w/ congenital heart defect (higher risk in mothers)

intrauterine infections

intake of lithium/ alcohol during preg

genetic condtns

maternal phenylketonuria

lack of folic acid in first trimester assoc w/ neural tube defects

Question 6

which genetic conditions show risk of congenital heart defects

Answer 6

down’s (21 trisomy

digeorge (22 del)

turner (loss of X)

Question 7

what is digeorge syndrome

Answer 7

deletion of a small segment of chromosome 22 congenital heart problems, specific facial features, frequent infections, developmental delay, learning problems and cleft palate.

Question 8

examples of duct dependant congenital heart disease

Answer 8

hypoplastic left heart syndrome

pulmonary atresia

transposition of great arteries

critical aortic stenosis

interupted aortic arch

Question 9

sx children not dg at birth

Answer 9

murmurs
tachycardia
HF
failure to thrive 
SOB
cyanotic episodes during feeding

Question 10

when are murmurs d/2 CHD heard

Answer 10

first week-month of life

Question 11

why dont some pt w/ CHD present w/ murmurs

Answer 11

pulmonary vascular resistenc changes w/ cLOSURE OF DUCTUS ARTERIOUSUS

Question 12

why do older children w/ cyanotic CHD squat

Answer 12

to improve venous return

Question 13

investigations in CHD w/ severe R and CV distress

Answer 13

CXR,

blood gases,

assessment of renal function

assesment of electrolytes

assessment of other possible causes of
cardiovascular collapse, e.g full infection screen

cardiac catheterisation

echocardiography

Question 14

management of CHD in abscence of sx`

Answer 14

prophylaxis of infectious endocarditis

Question 15

management of CHD in Acute severe presentations

Answer 15

resuscitation

prostaglandin infusion for ductus dependant lesions

Question 16

when is prostaglandin indicated

Answer 16

utilized to maintain the patency of the ductus arteriosus until surgical ligation is performe

Question 17

when is balloon valvotomy indicated in CHD

Answer 17

if CHD causes imparied circulation and the oxygenation of the blood, or to put a strain
on the heart or lungs,

Question 18

when is a heart transplant indicated

Answer 18

severe cases e.g. hypoplastic heart

Question 19

what is Hypoplastic left heart syndrome (HLHS)

Answer 19

a rare congenital heart defect in which the left side of the heart is severely underdeveloped.

Question 20

which CHD has infectious endocarditis as a complication

Answer 20

all except ASD

Question 21

COMPLICATIONS OF CHD

Answer 21

infective endicarditis

failure to thrive

stroke d/2 embolism (in R-L shunts

cyanosis d.2 EISENMENGER COMPLEX

polycytemia d/2/ cyanosis in R-L shunts

Question 22

what is eisenmenger complex

Answer 22

L-R shunts dont cause cyanosis but high volume pumped by the right side may result
inpulmonary hypertensionand, if this builds up and exceeds systemic pressure, the shunt may reverse from right
to left.

Question 23

what is tetralogy of Fallot (TOF)

Answer 23

a cardiac anomaly that refers to a combination of four related heart defects that commonly occur together.

1) large ventricular septal defect (VSD), which is a hole between the two ventricles or pumping chambers in the heart; 2) pulmonary stenosis, which is narrowing beneath or in the blood vessel leading to the lungs; 3) overriding of the aorta, in which the aorta lies directly above the ventricular septal defect; and 4) as a result of these events, the right ventricle becomes thickened or hypertrophied.

Question 24

ebstein’s anomaly

Answer 24

he leaflets are malformed and are positioned too low in the right ventricle. Sometimes, one of the leaflets is displaced downward into the ventricle, while another leaflet is larger than usual and may be abnormally attached to the wall of the ventricle. This causes enlargement of the atrium, and it can lead to congestive heart failure — a back-up of blood flow that results in a fluid buildup in the body.