Acyanotic congenital heart defects: Pathology review

Question 1

What are acynotic congenital heart diseases ?

Answer 1

These are conditions that presents with left to right shunts.

Question 2

What are the symptoms of acynotic heart diseases ?

Answer 2

Exercise intolerance and SoB
Poor feeding and failure to thrive.

Question 3

What is the most common acynotic heart disease ?

Answer 3

VSD

Question 4

Where does VSD commonly occur ?

Answer 4

At the membranous part which is closer to the AV septum is the locus of VSD.

Question 5

What is the evolution of VSD and its symptoms ?

Answer 5

Most VSDs often close on its own.

Question 6

An auscultation a ____________can be heard along the left sternal border in VSD.

Answer 6

Systolic murmur (The smaller the defect the more audible the murmur).

Question 7

What is the developmental history of atrial septum ?

Answer 7

The first structure in the atrial septum to develop is the septum primum which grows out to close the ostium primum by fusing with the endocardial cushion. While the ostium secondum opens up at the proximal 1/3 of the septum primum. Concomitantly, the septum secondum grows downward and leave open the foramen ovale. At birth the septum secundum and primum close. including the foramen ovale and ostium secondum.

Question 8

The most common ASD is ?

Answer 8

Ostium secundum defect.

Question 9

Osteum primum defect is often associated with ?

Answer 9

Down syndrome (Trisomy 21)

Question 10

The patagnomnic auscultatory finding in ASD is ?

Answer 10

Fixed split S2.

Question 11

What is the clinical complication of PFO ?

Answer 11

Pardoxical emboli journey from the DVT to the Rt atrium to left atrium through the PFO and into the systemic circulation.

Question 12

What is the name of the stroke caused by pardoxical emboli of the PFO ?

Answer 12

Cryptogenic stroke.

Question 13

What is the factor that maintains the ductus areriosus inutero ?

Answer 13

Prostaglandine E2.

Question 14

What is the cause of PDA ?

Answer 14

In premature birth or congenital rubella due to low O2 tension the DA remains open which is called PDA.

Question 15

In PDA the blood moves from _____to ______?

Answer 15

aorta to the pulmonary artery

Question 16

What are the conditions in which PDA is necessary for survival ?

Answer 16

In Tetralogy of Fallot where there is RVOTO and in transposition of great vessels where there is transposition of aorta and pulmonary artery.

Question 17

cynotic lower limbs and normal upper limbs are seen in babies with what condition ( differential cyanosis ?

Answer 17

PDA

Question 18

What is the murmur of PDA ?

Answer 18

Continuous machine like murmur

Question 19

What is the Tx of PDA ?

Answer 19

Indomethacin which blocks the synthesis of PGE2.

Question 20

What is Eiesenmenger syndrome ?

Answer 20

It is the shifting of left to right shunt to right to left shunt commonly seen in adults in whom cogential acynotic diseases are not corrected. It is also associated with polycythemia and clubing.

Question 21

What are the two types of coarctation of the aorta ?

Answer 21

Pre-ductal ( infantile type) occurs above the ductus arteriosus and post ductal adult type occurs below the DA.

Question 22

What is the BP pattern in coarctation of the aorta?

Answer 22

high BP in UE as compared to LE. There is also radio and brachio -femoral delay.

Question 23

What are the conditions associated to the coarctation of the aorta ?

Answer 23

Turner syndrome and Bicuspid aortic valave