PATH: 10-19 Congenital Heart Dz

Question 1

A: There must be ______% Vascular Obstruction for Physiological adverse effect

B: Although small-moderate size [Ventricular septum defects] tend to close spontaneously during _______ ….these pts are at risk for developing _______​

Answer 1

A: There must be [Greater than 65%] Vascular Obstruction for Physiological adverse effect

B: Although small-moderate size [Ventricular septum defects] tend to close spontaneously during 1st few years of life…these pts are at risk for developing infective endocarditis

Question 2

Defect in the ATRIAL septum (4)

Answer 2

FOSC

Atrial septum Defect (ASD)

1. Fossa ovalis / secundum type - Common
2. Ostium primum type- [endocardium cusion defect] near distal atrium tht affects mitral valve–> mitral regurgitation
3. Sinus venosus or [proximal type of ASD] - UnCommon
4. Coronary sinus type - rare

Question 3

List the sx of a LARGE Septal Defect in the heart (2)

Answer 3

• Volume Hypertrophy of R Vt / L Atrium / L Vt
• Pressure of L Vt —> R Vt —> [R Vt Pressure Hypertrophy]

Question 4

The pulmonary vascular tree is able to accommodate with INC pressure in pulmonary circuit.

However, pulmonary HTN may still develop from what 3 reasons?

B: pulmonary HTN is more commonly associated with which septal defect?

Answer 4

1. Flow beyond distensibility of lung vasculature.
2. Vasoconstriction of pulmonary vascular bed.
3. Secondary pathologic changes in the Lung [intima and media] of [muscular arteries and arterioles] —> restrict pulmonary bed.

B: Large [Ventricular Septal Defects]

Question 5

Eisenmenger Complex

Answer 5

Occurs when [L to R shunt] produces [R Vt Pressure Hypertrophy] and as a result DEC blood volume in the [L Vt] –> [L Vt Volume Atrophy]

[L Vt Volume Atrophy] will REVERSE the direction of shunt so that it now is a [R to L shunt]!

Question 6

A: Describe Hypertrophic Cardiomyopathy

B: What 2 things may it cause?

Answer 6

Hypertrophic cardiomyopathy is an [autosomal dominant] inherited genetic disease–> asymmetrical septal hypertrophy of muscular septum.

B: This may produce

• SubAortic obstruction
• Sudden death

Question 7

A: AORTA COARCTATION

B: Sx (3)

Answer 7

narrowing of transverse arch in the region of the isthmus (segment between origin of [left subclavian artery] and [ligamentum arteriosus] )

B:

• [L Vt and atrial Pressure hypertrophy]
• HTN proximal and distal to narrowing
• Collateral anastomoses that bypass [aortic narrowing]

Question 8

Tetralogy of Fallot is a shunt with obstruction

• A: Name the 4 Sx*
• B: Define Infundibulum and how it plays a role in* Tetralogy of Fallot
• C: What are the 2 types of* Tetralogy of Fallot. Which is more common?
• D: Which type is associated with [L —> R Shunt] that results in biventricular hypertrophy and INC pulmonary flow?*

Answer 8

” VOIR is French like… Fallot “

1. Infundibular pulmonary stenosis.
2. Right ventricular hypertrophy.
3. Ventricular septal defect (U-shaped deformity confluent w/aortic valve –> aortic valve can override defect)
4. Overriding Aorta.

B: Outflow tract of R Ventricle that consist of septal and parietal [muscle band groups]. Can become hypertrophied —> [Infundibular pulmonary stenosis]

C: Cyanotic (MOST COMMON and caused by [infundibular pulmonary obstruction]) vs. Acyanotic

D: Acyanotic