Management of Tetralogy of Falot - case presentation of a School going child presenting with central and peripheral Cyanosis, finger nail clubbing Grade IV with a history easy fatiguability and occasional Tet spells since the age of 2.

1. TOF MX
Kasoka Lukama k
7th year
UNZA-School of Medicine
11hrs Tutorial

2. OBJECTIVES
 Brief Background
 Mx
 MOST COMMON CAUSE OF CHD – 4/10, 000

3. ANATOMY

4. 4 THINGS
 Subvalvar pulmonary stenosis with ? Hypoplasia
of PA- valve and arteries
 Non restrictive VSD
 RVH
 Overriding Aorta
 Neonates: Pulmonary blood flow maintained by
PDA and MAPCA ( from ascending and
descending arteries)

6. C/P
 Central cyanosis usually in the first year of life.
Cyanosis dependant on stenosis.
 Squatting - ↑SVR → ↑ aortic pressure and
hence ↑pulmonary blood flow.
 Grey Sclerae and Engorged Blood vessels
during tet spells.
 Clubbing
 Hypoxic “tet” spells: due to infudibular spams.
Occur in te morning, after crying, feeding and
exercabated by stress.

7. TET SPELLS
 Hyperapnea, restless
 Cyanosis
 Reduced mummer
 Followed by generalised weakness and sleep.
 Severe ones can lead to LOC, CONVULSIONS,
HEMIPARESIS
 NOTE: Infants without cyanosis and have TOF
cyanosis suffer the most spells.

8. COMPLICATIONS OF TOF
 Cerebral Thrombosis: Causing CVA
- long standing hypoxemia causing ↑EPO →
polycythemia →sluggish flow.
- Fe deficiency anaemia ( due to ↑EPO,
Phlebotomy) disinhibits megakaryocytes.
o Brain Abscess: Due to septic emboli and lack of
pulmonary filtration. Presents as low grade fever
with behavioural changes, Headaches,
seizures, focal neurologic deficits.

9.  Bacterial Endocarditis
 Pulmonary TB
 Reduced School perfomance
 FTT
 NO HEART FAILURE
 Associated with: DiGeorge Syndrome (22q11.2
deletion) and CATCH 22( Cardiac, Abnormal
facies, cleft palate, hypocalcemia and 22 deletion)

10. GENERAL PRINCIPLES OF CHD MX
 Parents Counselling
 Modify Physical activity appropriate for age
(don’t restrict totally but avoid high impact
sports)
 Routine immunization and prophylaxis against
bacterial infections(dental surgery) and
RSV/Influenza.
 Anticipate complications and look for them.
 Avoid dehydration and you avoid stroke-viscosity
 Phlebotomy indicated in very symptomatic pts or
HCT>65

11. INVESTIGATIONS
1. Hyperoxic Test, O2 sats
2. FBC – Hb 19.8 ( 23.2 in 2014) - HCT 69.1 ( 73.1 in
2014)
3. Coagulation Profile
4. ABG – Sr lactates, base excess, PaO2
5. Blood cultures in febrile patients.
6. Knee arthrocentesis for knee swelling
7. ESR, CRP and ASOT
8. ANA, RF

12. IMAGING
 CXR – boot shaped heart with oligeamic lungs.
 ECG – RBBB with RAD and RVH (2014)
 ECHO – Infundibular stenosis, Overriding Aorta,
Normal AV valves, Hypertrophic infundibular
septum (2014)
 Cardiac catheterization: assesses pressure in Rt
ventricles and pulmonary artery, and anatomy.
 MRI – GOLD STANDARD
 X RAY joint
 Others: Chromosomal studies at birth.

13. BOOT SHAPED HEART

14. TX OF HYPERCYANOTIC SPELLS
 Knee chest position
 Avoid Iatrogenic stressors
 Remove constrictive clothes
 Adminster O2 (of limited value until propranolol)
 Sodium bicarbonate to correct acidosis
 Propranolol 0.1mg/kg
 Morphine 0.1mg/kg SC or midazolam IV
0.1mg/kg or ketamne 1-2mg/kg IV
 If still not responsive, admit to PICU, intubate
and sedate. This usually does the job.
 Monitor O2 sats and other vitals

15. SUBSEQUENT TX AFTER SPELL
 Oral prophyaxis propranolol 2-6mg/kg/d
 Avoid digitalis as it may induce infundibular
spasms.
 Vasodilator drugs can be used too such as
sildenafil.
 Phlebotomy with replacement of normal saline is
a long time therapeutic modality

16. THERAPEUTIC PHLEBOTOMY ( OTHER
INDICATIONS)
 Polycythermia vera
 Hemochromatosis
 Porphyria cutanea tarda
 NAFLD with hyperferritinemia
ADVERSE EFFECTS
o Post phlebotomy thrombosis
o Hematoma at site of injection
o syncope

17. AVOID CEREBRAL THROMBOSIS
 Tx of Fe deficiency Anemia
 Avoid dehydration
 Partial exchange transfusion with albumen or
normal saline if HCT>65
 PGE2 infusion at birth if severe cyanosis
 Look out for and treat Infective endocarditis.

18. SURGERY – PALLIATIVE SHUNTS
 Idea: anastomosis btwn aorta and PA to
↑pulmonary blood flow
 Indication: cyanotic infants < 3mo
 TYPES
- Modified Blalock Taussig: anastomosis btwn
subclavian artery and ipsilateral PA.
- Waterstone operation: Ascending Aorta and Rt
PA
- Potts operation: descending Aorta and Lt PA

20. CORRECTIVE SURGERY
 Timing : 4 to 12 months
 Involves enlargement of RVOT and VSD closure.
 Done by using cardiopulmonary bypass with
cardioplegia.
 Replacement of valves
 Postop monitoring for complications.
 Patient education that repair is not curing.

22. PROGNOSIS
 Historically, untreated TOF average life
expectancy is 12 years.
 Survival rates after Repair
 – 1 month = 100 percent
 - 1 year = 89 percent
 - 5 years = 95 percent

23. RARELY REPORTED
COMPLICATIONS
 Pépé Mfutu Ekulu, Orly Kazadi-wa-Kazadi, Paul
Kabuyi Lumbala, and Michel Ntetani Aloni,
“Nephrotic Syndrome in a Child Suffering from
Tetralogy of Fallot: A Rare Association,” Case
Reports in Pediatrics, vol. 2015, Article ID
128409, 3 pages, 2015.

24.  Ischemic Retinopathy and Uveitis in a
Patient with Tetralogy of Fallot Wu, Wei-Chi
et al.Ophthalmology , Volume 112 , Issue 11 ,
1936 - 1940

26. I Choi, S & V Simone, J & W Jackson, C. (1974). Megakaryocytopoiesis in
experimental iron deficiency anemia. Blood. 43. 1