TOF

Question 1

Anatomical defect

Answer 1

1- PA STENOSIS ( infundibular or vulvular)

2- overriding aorta
3- RVH
4- large Vsd

Question 2

Hymodynamics

Answer 2

1.Blood in the RV pass through 2 pathways:
-Small part: Pulmonary artery (PS) → – PBF
-Large part: Aorta (Overriding) → Cyanosis (right to left shunt)
2.Mild RVH (Pressure overload )
3. No shunt across VSD as pressure is equal in both ventricles ( بيساعدوا بعض ف ضخ الدم للورتا بالتالي مفيش hf )

Question 3

History

Answer 3

1- Cyanosis:
- Onset: Usually delayed (3-4 months) due to gradual narrowing of the infundibulum and closure of the PDA( Delay due to reduced Saturtion)

• May appear in the neonatal period (Severe cases)
• May be absent( wide infundibulm) (Pink Fallot): symptoms appear with exercise only ( — sat o2 )

2. Dyspnea
3. Hypercyanotic spell
4. Squatting

Question 4

Why squatting

Answer 4

Bending of popliteal, femoral a ~> + systemic ( aorta ) resistance ~> ++ pbf~> — cyanosis

Question 5

Examination

Answer 5

General
1- ftt
2- central cyanosis اللي ف قلبي علي لساني
3- clubbing (1-2 years)

Cardiac
Ins and palp
1- MILD RVH ( difficult to palpate)
2- systolic thrills on pulmonary area

Ausc
1- single A2 بكبر الي صوته عالي واصغر اللي صوته واطي
2- harsh ejection systolic murmur on pulmonary area due to pulmonary stenosis not vsd 😒

Question 6

Why clubbing

Answer 6

Hypoxia ~> kidney secretes erythropoietin ~> polycythaemia ~> platelets destruction ~> pdgf~> abnormal growth

Question 7

Comp

Answer 7

1- polycythaemia ~> thrombosis
2- brain abscess , or infarction ~> hemiplagia ( loss of ling filter )
3- iron deficiency ( overuse in bm)
4- infective endocarditis

❗️no recurrent chest inf ( lung oligemia)
❗️no hf

Question 8

Inv

Answer 8

1- cbc : + hb , + hematocrit , microcytosis
2- cardiac catheterization
3- xray : coeur en sabot ( boot shape)
~> 1- RVH
~> 2- uplifted apex
~> 3- exaggerated waist ( - pa size)
And lung oligemia

4- ecg : RVH
5- echo : tetralogy (4)

Question 9

Ttt

Answer 9

Medical ( cp + comp)
• Hypercyanotic spells
• Prostaglandin (PG1): patency of da
• Iron
• Infective endocarditis (Prophylaxis and Rx)
• Partial exchange transfusion (using FFP or albuminor saline )If hematocrit is > 65-70%

Surgical

1- Palliative: Blalock-Taussig (anastomosis between Subclavian a, pa ) (artificial PDA)
2- Total correction (at 6-9 months): Closure of VSD, infundibular resection and pulmonary valvotomy

Question 10

Hypercyanotic spell
1- def
2- etiology
3- precipitating f
4- cp
5- murmur
6- comp
7- ttt

Answer 10

1-Transient attacks of deep cyanosis and dyspnea
2-Due to infundibular spasm <> –PBF
3- inf , crying , hunger , dehydration, hypoxia, acidosis , iron deficiency ~> adrenaline release ~> spasm , tachy

4-Dyspnea, deepening of cyanosis ,coma and convulsions
5- murmur is disappeared or diminished due to– pbf
6- mi , cerbrovascular accidents ~> death if untreated ( القلب والعقل )

7- *squatting
*IV alpha agonist (increase systemic resistance)(contraversial as it + spasm)

•IV B-Adrenergic blockers (Propranolol): to relax the infundibulum
•Sedation (SC Morphine)

~O2 therapy
~NaCO: to correct acidosis

Iv fluids

Question 11

Why hypoxia , acidosis involved in hypercyanotic spell

Answer 11

Shunt to aorta ~> hypoxia ~> anaerobic glycolysis ( lactic acidosis) ~> + vd ~> hypotension ~> more shunt ( as you facilitate aorta path by being hypotensive)

Question 12

What is the most common congenital cyanotic hd

Answer 12

Tof