Tetralogy of Fallot: total correction in the first year of life

1
Tetralogy of Fallot total correction in the
firstyear of life
Vasyl V.Lazorychynets,M.D.,Ph.D., Vitaliy
Demianchuk, M.D., Miroslav Glagola,
M.D.,Ph.D., Igor Mokryk, M.D. Kyiv Institute of
Cardiovascular surgery AMS of Ukraine
2
Schematic representation of the rotation of the
truncoconal septum
1/3
1/2
2/3
1/2
Diplaced toward the pulmonary side In tetralogy
of Fallot
Normal 180
Angelini P Texas Heart Institute Journal
1995221-12
3
Epidemiology

• Tetralogy of Fallot is a rather common complex
  cardiac malformation with an incidence of
• 2,2/10 000
• live births
• Francannet C et al. European Journal of
  Epidemiology 19939607-16.

4
Natural history without treatment

• 1 year survival rate of 66
• 2 years survival rate of 49
• 20 years and more only 10-15
• Bertranou EG et al. Am J Cardiol 197842458-66

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History of surgical management of TOF
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Types of surgical repair for RVOTO relief
C
B
A
Transanular patch
Transatrial transpulmonary
RV outflow patch
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Objective
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Methods

• The myocardium specimens
• of 69 patients (age from 3 months to 23 years)
  with TOF were studied by light and electron
  microscopy. The biopsy was taken from RV outflow
  tract during intracardiac repair, immediately
  after cardioplegia.

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RV myocardium structure - fetal type Group 1
(n24) - 3 months to 3 years
1-year-old patient x 250
10
Myocardium cells hypertrophy with interstitial
fibrosis Group 2 and 3
17-years-old patient x250
11
RV myocardium fetal type with the preservation
of cells ultrastructure Group 1 (n24) - 3
months to 3 years
mc
cap
mc
1-year-old patient x14 000
12
Early stage of myocardium cells sclerosis Group 2
(n29) - 4 to 10 years
cap
cw
mc
10-years-old patient x 14 000
13
Myocardium cells dystrophy with atrophy Group 3
(n16) - 11 to 23 years
Mega corpuscles
Fat granulaes
X 14 000
X 34 850
18-year-old patient
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Outcome after repair of tetralogy of Fallot in
the first year of life (literature review)
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41-years experience of the surgical treatment of
TOF

• 3216 cases of complete repairfrom 1960 to 2001
  
• in the Institute of Cardio-vascular Surgery of
  the Acamedy of Medical Sciences of Ukraine

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Proportion of patients undergoing repair of TOF
during the 1 year of life
17
Age distribution of patients undergoing repair of
TOF in the 1 year of life
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Objective

• The purpose of this study was to evaluate the
  early and mid-term results after repair of
  tetralogy of Fallot (TOF) in the first year of
  life

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Methods

• From 01.01.1996 to 01.01.2001,
• 74 consecutive infants with a mean age of 9,9?2,2
  months
• (range, 4 to 12 months) underwent repair of TOF.
• A median weight of patients was
• 8,7?1,6 kg (from 4,7 to 12 kg).
• There were 45 (60,8) male and 29 (39,2) female.

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Quantity of patients after primary vs staged
repair of TOF
78,4
21,6
n68
n16
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Incidence of TAP vs PA preservation
n33
n41
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Surgical approach for TAP(n33)
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Surgical approach for PAP(n41)
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Operative mortality

• Subdural hematoma - 2
• Air embolism - 1
• Fungal sepsis -1
• Pneumonia - 1

25
Early postoperative complications in 13 patients
(17,5 )
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Late reoperations and mortality

• Follow-up was received from 93 of patient,
  averaging 28,7?13,5
• (range, 1,5 to 54 months)
• 5 patients required reoperation with 1 operative
  mortality
• 4/0 - RVOT- aneurysmectomy
• 1/1 - infective endocarditis

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Conclusions

• RV hypertrophy in TOF is a dynamic process.
• In infancy the growth of RV myocardium is
  provided mainly by the fetal hyperplasia
  prolongation with the preservation of cells
  ultrastructure.
• Later irreversible degenerative processes are
  prevalent.
• In order to improve the results of surgery
  complete repair of TOF has to be performed in the
  early stages of the disease before the
  development of an irreversible changes in
  myocardium occurred.

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Conclusions

• Early repair of tetralogy of Fallot is
  associated with an acceptable operative risk

• Yonger age for time of operation does not
  increase of incidence of transanular patching

• Alternative surgical techniques can be
  accomplished for patients of the first year of
  life

• Follow-up data suggest that biventricular
  function is good