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.
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)
5.
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.
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
19.
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.
21.
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
25.
26. I Choi, S & V Simone, J & W Jackson, C. (1974). Megakaryocytopoiesis in
experimental iron deficiency anemia. Blood. 43. 1