[7] Cyanotic Congenital Heart Disease

Question 1

Most common congenital heart disease?

Answer 1

Ventricular Septal Defect

Question 2

Main Etiology for CHD

Answer 2

Idiopathic (Probably multifactorial)

Question 3

Noted infection that causes Patent Ductus Arteriosus and Peripheral Pulmonary Stenosis

Answer 3

Rubella

Question 4

Normal O2 Saturation on the Right Side of the Heart

Answer 4

75

Question 5

Clinically Apparent O2 Saturation of Cyanosis

Answer 5

< 85%

Question 6

How much reduced Hg to detect Cyanosis?

Answer 6

> 5 gm/dL

Question 7

Clinical Features of Tetralogy of Fallot

Answer 7

Progressive Cyanosis

Retarded Growth NOT Mental Development

Question 8

Why do children with ToF often squat?

Answer 8

To improve pulmonary blood flow

Question 9

What are the Malformations of the Tetralogy of Fallot

Answer 9

1. Ventricular Septal Defect
2. Pulmonary Stenosis
3. Dextroposition of Aorta
4. Right Ventricular Hypertrophy

Question 10

Describe the murmur present in ToF

Answer 10

Systolic Ejection Murmur

Question 11

Most severe spectrum of ToF

Answer 11

Presents with Pulmonary Atresia

Question 12

Least severe spectrum of ToF

Answer 12

Pink ToF

Question 13

Proper Squat Form to Relieve ToF

Answer 13

Knee Chest Position

Question 14

What does the body do to try to compensate for the Cyanosis?

Answer 14

Polycythemia

Because it thinks you need more Hb in the body, but that doesn’t do anything

Question 15

Why are patients with ToF prone to brain abscess?

Answer 15

Since you have virtually a single ventricle, when you have bacteria (usually coming from the dental flora) it can go from RV -> LV and instantly enter systemic circulation

Since the carotids are straight, it can go straight to the brain

Question 16

Medical Management of ToF

Answer 16

Prevent Fe deficiency
Phlebotomy
Propanolol for Hypoxic Spells

Question 17

Surgical Management of ToF

Answer 17

Palliative: Shunts (Blalock-Taussig Shunt, Central)
Definitive: VSD Closure
Infundibulectomy
Transannular Patching

Question 18

Describe Murmur of Transposition of Great Arteries (TGA)

Answer 18

Single loud S2

Soft Systolic Murmur

Question 19

Describe the shape of the heart in TGA

Answer 19

Egg-Shaped

Question 20

Natural History of TGA w/o Intervention

Answer 20

Death

Question 21

How do patients survive TGA?

Answer 21

If they have a VSD or PS

Question 22

Palliative Care of TGA

Answer 22

Prostaglandin Infusion

Create interatrial communication

Question 23

Definitive Care of TGA

Answer 23

Atrial Switch: Senning or Mustard Procedure

Arterial Switch: Jantene Procedure

Question 24

Wall to Wall Heart is Characteristic of?

Answer 24

Ebstein’s Anomaly

Question 25

What do you need when you have Pulmonary Valve Atresia to survive?

Answer 25

Tricuspid Regurgitation
Patent Ductus Arteriosus
Atrial Septal Defect

Question 26

What kind of blood is in the Right Atrium in Total Anomalous Pulmonary Venous Return?

Answer 26

All of it, oxygenated and non-oxygenated

Question 27

Requirement of TAPVR for Survival

Answer 27

Atrial Septal Defect

Patent Foramen Ovale

Question 28

What type of TAPVR is considered an emergency for neonates

Answer 28

Infracardiac TAPVR

Question 29

Requirements of Hypoplastic Left Heart Syndrome

Answer 29

Atrial Communication

PDA big enough to support descending and ascending aorta

Question 30

Describe Murmur of Truncus Arteriosus

Answer 30

Loud S2

Diastolic murmur at the Left Upper Sternal Border