Congenital heart disease pathology

Question 1

Define “congenital heart disease”

Answer 1

present at birth

Question 2

List 4 examples of left-to-right CHD shunt. Which is most common

Answer 2

Patent ductus arteriosus, ventricular septal defect, atrial septal defect,atrioventricular septal defect. VSD is most common. Baby is pink

Question 3

Define the underlying defect and process that results in Eisenmenger’s Syndrome

Answer 3

Reversal of a L to R shunt. RV hypertrophy occurs and pulmonary HTN, which reverses the flow and results in cyanosis

Question 4

List at least 3 examples of right-to-left CHD

shunt. Most frequent?

Answer 4

tetralogy of fallot, transposition of great vessels, truncus arteriosus, tricuspid atresia, truncus arteriosus, Eisenmenger syndrome. Tetralogy is most common

Question 5

Name 2 examples of obstructive Congenital heart disease

Answer 5

Aortic coarctation, stenotic aortic valve, stenotic pulmonary valve

Question 6

most common congenital cardiac anomaly

Answer 6

VSD

Question 7

most common congenital cardiac anomaly that can remain asymptomatic until adulthood

Answer 7

ASD

Question 8

most common cyanotic congenital heart disease

Answer 8

Tetralogy of fallot

Question 9

Congenital heart defects in down syndrome

Answer 9

ASD, VSD, AVSD, AV valve malformations

Question 10

What causes congenital heart disease

Answer 10

sporadic processes include maternal diabetes (toxic-metabolic) and Rubella (infectious) which causes PDA. Familial processes include Trisomy 13, 18, 15, Down syndrome, Turner syndrome (causes coarctation of aorta, bicuspid aortic valve, hypoplastic left heart syndrome), DiGeorge (tetralogy of fallot, truncus arteriosus, aortic arch defect), Noonan syndrome (pulmonic stenosis) and williams syndrome (supravalvar aortic stenosis)

Question 11

What is cyanosis

Answer 11

Blue peripheral extremities and perioral region due to poorly oxygenated blood

Question 12

Clubbing?

Answer 12

Distal fingers and toes- increased nail convexity due to hypoxia seen in cyanotic diseases

Question 13

Types of aortic coarctation

Answer 13

infantile hypoplasia of arch, or adult infolding of area of ligamentum arteriosum. Also Bicuspid valve in 50%

Question 14

Fors of aortic valve stenosis

Answer 14

subvalvular(ring or collar), valvular(small and/or dysplastic cusps or abnormal number of cusps), supravalvular (stenosis is above valve in aorta)

Question 15

Forms of pulmonary stenosis

Answer 15

isolated or with tetralogy of fallot