Cyanotic Congenital Heart diseases

Question 1

Cyanotic Lesions

Answer 1

Tetralogy of Fallot
Transposition of the Great Vessels
Hypoplastic Left Heart Syndrome
Truncus Arteriosus
Total Anomalous Pulmonary Venous Return
Tricuspid Valve Atresia

Question 2

Acyanotic Lesions

Answer 2

Ventricular Septal Defect
Atrial Septal Defect
Patent Ductus Arteriosus (PDA)
Coarctation of the Aorta

Question 3

Tetralogy of Fallot

is a condition characterized by:

Answer 3

1 Overriding aorta
2 Pulmonary stenosis
3 Right ventricular hypertrophy
4 Ventricular septal defect (VSD)

Question 4

Tetralogy of Fallot Patho

Answer 4

It is associated with chromosome 22 deletions.

Question 5

Tetralogy of Fallot Dx

Answer 5

Chest x-ray: boot-shaped heart

Decreased pulmonary vascular marking

Question 6

VSDs are more common in what genetic diseases?

Answer 6

Down
(trisomy 21), Edwards (trisomy 18),
and Patau (trisomy 13).

Question 7

the only 3 holosystolic murmurs are:

Answer 7

Mitral regurgitation
Tricuspid regurgitation
Ventricular septal defect

Question 8

The most common congenital heart

defect in Down syndrome is:

Answer 8

endocardial cushion defect of atrioventricular canal.

Question 9

Transposition of the Great Vessels Dx

Answer 9

Early and severe cyanosis is seen. A single S2 is heard. Chest x-ray will show an
“egg on a string.”

Question 10

Transposition of the Great Vessels. Tx

Answer 10

Avoid NSAIDs (indomethacin*)
2 Qx

Question 11

Hypoplastic Left Heart Syndrome Patho

Answer 11

left ventricular hypoplasia, mitral valve atresia,

and aortic valve lesions.

Question 12

Hypoplastic Left Heart Syndrome Cx Fx

Answer 12

Absent pulses with a single S2
Increased right ventricular impulse
Gray rather than bluish cyanosis
Inaudible murmurs
Hyperdynamic precordium

Question 13

Hypoplastic Left Heart Syndrome Dx

Answer 13

Chest x-ray will show a globular-shaped heart with pulmonary edema.
Echocardiogram is the most accurate diagnostic test.

Question 14

Hypoplastic Left Heart Syndrome Tx

Answer 14

3 staged surgeries or a heart transplant.

Question 15

Truncus Arteriosus Cx Fx

Answer 15

Symptoms occur within the first few days of life and are characterized by:

• Severe dyspnea
• Early and frequent respiratory infections

Question 16

Truncus Arteriosus Dx

Answer 16

Chest x-ray: cardiomegaly + increased pulmonary markings.

Question 17

Truncus Arteriosus Tx

Answer 17

Qx, before pulmonary HTN occurs

Question 18

Total Anomalous Pulmonary Venous Return Patho

Answer 18

no venous return between pulmonary veins and the left atrium, oxygenated blood instead returns to the superior vena cava.

Question 19

TAPVR
with
obstruction Onset

Answer 19

Early in life with
respiratory
distress and
severe cyanosis

Question 20

TAPVR
without
obstruction ONSET

Answer 20

Age 1–2 years
with right heart
failure and
tachypnea

Question 21

TAPVR
with
obstruction DX

Answer 21

CXR shows pulmonary
edema.
Echocardiography is
definitive.

Question 22

TAPVR
without
obstruction DX

Answer 22

CXR shows snowman or
figure 8 sign. Most accurate
diagnostic test is an
echocardiogram.
Diagnosed with
echocardiogram and not by
x-ray.

Question 23

Tricuspid Valve Atresia Patho

Answer 23

No communication between the right heart chambers >
hypoplastic right
ventricular and pulmonary outflow tract >
underdevelopment of
the pulmonary valve and/or artery.

Question 24

Tricuspid Valve Atresia Asoc. Patho

Answer 24

PFO, ASD, or VSD.

Otherwise death

Question 25

Tricuspid Valve Atresia Dx

Answer 25

CXR: decreased pulmonary flow
EKG: left axis
deviation

Question 26

Tricuspid Valve Atresia Tx

Answer 26

-Prostaglandin E1 to keep the PDA open until an aortopulmonary shunt can
be performed
-Possible atrial balloon septostomy to enlarge the ASD
-Staged surgical correction