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TETRALOGY OF FALLOTA PHILIPPINE HEART CENTER REVIEW
RAUL D. JARA, M.D. MIKAELA NIKKOLA A. JARA, M.D.
Philippine Heart Center
TETRALOGY OF FALLOT:A Philippine Heart Center Review
This study aims to provide a comprehensive review of the literature regarding the postoperative outcome of TOF in the Philippines
GENERAL OBJECTIVE
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewSPECIFIC OBJECTIVES
a. To be able to determine the surgical outcome of patients with TOF in the Philippines.
b. To be able to determine the appropriate diagnostic examinations for postoperative TOF patients.
c. To be able to determine the common complications of TOF in postoperative patients.
TETRALOGY OF FALLOT:A Philippine Heart Center Review
study design – A Review electronic search using Health Research Development Information (Herdin) of the Philippine Council for HealthResearch & Development (PCHRD) unpublished research initiatives by fellows of Adult & Pediatric
Cardiology at PHC
METHODOLOGY
TETRALOGY OF FALLOT:A Philippine Heart Center Review
All retrieved researches were reviewed by two investigators independently and articles that contained 20 patients with TOF were included in the review.
METHODOLOGY
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Quality of
Evidence
Definition Example
A
Recommendation based on consistent and good-quality evidence.
Good quality randomized controlled trial (RCT) or meta-analysis.
B
Recommendation based on inconsistent or limited-quality evidence.
Lower quality RCTs, clinical cohort studies, and case-controlled studies.
CRecommendation based on consensus, expert opinion, or case series.
Strength of Recommendation
1 Strong recommendation.
2 Weak recommendation.
Table 1. Recommendation strength and quality of evidence.
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Principal Author
Time Interval of the Study
Patientsn
Interventions
Comparisons
Outcomes StudyDesign
1 Delfin, D.P.1975-1989(9)
Adult patients with total correction
25
None MortalityMorbidityTechnique used for VSD and RVOT obstructionClinical statusFunctional classOxygen saturation2D Echo findings
Cross-sectional
2 Bensurto,E.S.1990-2000(17)
Pediatric patients with
total correction42
PreoperativeLeft ventricularindices
MortalityMorbidityTechnique used for RVOT obstruction
Cross-sectional
Table 2. Summary of the studies reviewed.PUBLISHED STUDIES
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Principal Author
Time Interval of the Study
Patientsn
Interventions
Comparisons
Outcomes StudyDesign
3 Daen, C.P.1994-1996 (15)
Patients with TOF correction with
pericardial monocusp valve
21
Pericardial monocusp
valve usage in TOF
correction
Late result of right ventricular outflow tract reconstruction: ECG, Chest radiograph, 2D Echo with Doppler
Cross-sectional
4 Lazaro,M.E.R.2000-2001(13)
Pediatric patients with total correction
30
RVOT repair technique: Goretexmonocuspversus non-valve transannular repair
Two-dimensional echocardiography parameters Functional class
Cohort
Table 2. Summary of the studies reviewed.PUBLISHED STUDIES
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Principal Author
Time Interval of the Study
Patientsn
Interventions
Comparisons
OutcomesStudyDesign
5 Bote-Nuñez,J.R.1980-1995
Pediatric patients with
total correction able to follow-up
49
None MortalityPresence of palliativeProcedureSurgical approachTechnique used for VSDand RVOT obstructionPost-operative functional class
Cross-sectional
6 Villanueva,N.J.1984-1987(10)
Pediatric patients with total correction
57
None MortalityPresence of palliative procedureTechnique used for VSDand RVOT obstruction
Cross-sectional
Table 2. Summary of the studies reviewed.UNPUBLISHED STUDIES
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Principal Author
Time Interval of the Study
Patientsn
Interventions
Comparisons
OutcomesStudyDesign
7 Del Campo,J.F.M.1992-1997(16)
Pediatric patients with total correction
165
Surgical Approach:
Ventriculotomy or
Atriotomy
Mortality (Arrhythmias)Presence of palliative procedureTechnique used for RVOT obstructionBypass time and ischemic time
Cross-sectional
8 Perfecto,S.M.1996-2005(6)
Total Correction of TOF157
None MorbidityECG2D Echo with DopplerSymptomatology
Cross-sectional
Table 2. Summary of the studies reviewed.UNPUBLISHED STUDIES
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Principal Author
Time Interval of the Study
Patientsn
Interventions
Comparisons
OutcomesStudyDesign
9 Claudio,M.T.E.1995-2004(5)
Pediatric patients with total correction
150
Good and bad surgical
outcome group
Preoperative Echocardiographicpredictors
Cohort
10 Claudio,M.T.E.1997-2002(11)
Pediatric patients with elective total correction of TOF
and uncomplicated intraoperative
course63
Presence or absence of
complication
Immediate postoperative physiologic parameters (heart rate, blood pressure, temperature, etc.)Bypass time and Ischemic time
Cohort
Table 2. Summary of the studies reviewed.UNPUBLISHED STUDIES
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Principal Author
Time Interval of the Study
Patientsn
Interventions
Comparisons
OutcomesStudyDesign
11 Del Campo,J.F.M..1999-2000(16)
Infants or children with TOF who had
hypoxic spells20
Treatment of tet spell with
or without esmolol
Therapeutic response in terms of heart rate, detection of murmur, and saturation
RCT
12 Yap, M.C.,2010(18)
Patients > 15 years old who underwent TOF total correction
29
TOF correction before or
after 5 years old
Immediate postoperative physiologic parameters (heart rate, blood pressure, temperature, etc.)Bypass time and Ischemic time
Cohort
Table 2. Summary of the studies reviewed.UNPUBLISHED STUDIES
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Figure I. Mortality rate of various studies on TOF done at PHC.
1975
-198
9 Del
fin
1984
-198
7 Vi
llanu
eva
1980
-199
5 Bo
te-N
uñez
1990
-199
7 Be
nsur
to
1997
-200
2 Cla
udio
2000
-201
1 PH
C Cen
sus
0%
10%
20%
30%
4%
28%
6% 5% 3% 6%
Mort
ality
Rate
TETRALOGY OF FALLOT:A Philippine Heart Center Review
ONE-STAGE
APPROACH
TWO-STAGE
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewThe Common Palliative Procedures in TOF
Modified Blalock- Taussig
Waterston Shunt
Classic Blalock
Taussig
Potts Shunt
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewSURGICAL INTERVENTIONA. PALLIATIVE
1. Progressive cyanosis may still be noted due to:a. worsening RVOT obstructionb. gradual stenosis of palliative aorto pulmonary shunts.c. development of pulmonary hypertensiond. progressive aortic dilatation and aortic regurgitation
Bote-Nuñez, J.R., PHC.R.053.01, Unpublished paperVillanueva, N.J., PHC.R.039.88, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
With a range of 3 to 6 years between palliative shunt to INTRACARDIAC REPAIR, no significant difference has been found in terms of morbidity and mortalities with a two-stage compared with one-stage approach
Villanueva, N.J., PHC.039.88. Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
1. Close the VSD2. Relieve the RV outflow obstruction3. Repair any stenosis in the pulmonary arteries
Claudio, M.T.E, (2004), crf.006.04, Unpublished paperNichols, David G. et al: Critical Heart Diseases in Infants & Children, Missouri, 1995, Mosby Year-Book, Inc.
GOALS OF OPERATION WITH ONE-STAGE APPROACH
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewSURGICAL INTERVENTIONA. DEFINITIVE (one stage)
1. Majority of the patients are asymptomatic although there are long-term complications:a. atrial tachyarrhythmiasb. ventricular tachyarrhythmiasc. progressive RV dilatationd. progressive aortic root dilatation
Perfecto, S.M., PHC.R.060.08, Unpublished paperBote-Nuñez, J.R., PHC.R.053.01, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Figure II. Surgical Approach in TOF Correction
1975-1989 Delfin
1980-1995 Bote-Nunez
1990-1997 Bensurto
1992-1997 Del Campo
1994-1996 Daen
0
20
40
60
80
100
120
140
160
180
256
6943
21
96
12
Column1atriotomy
Num
ber
of
case
s
TETRALOGY OF FALLOT:A Philippine Heart Center Review
0
5
10
15
20
25
30
25
7.4 7.725.37 6.12 5.6
Figure III. Mean age and range at which TOF patients are operated
Mean
Ag
e
TETRALOGY OF FALLOT:A Philippine Heart Center Review
InterventionsDelfin1975-1989
Bote-Nuñez1980-1995
Villanueva
1990-1997
Bensurto1990-1997
Del Campo1992-1997
Perfecto1996-2005
TOTAL
Infundibulectomy 17 52 38 107
Valvotomy 7 7
Monocusp 2 26 28
TAP 4 19 27 52 102
RVOT Patch 7 22 24 52 105
RVOT Reconstruction 67 67
Rastelli external conduit with porcine valve
1 1TAP: transannular patching; RVOT: right ventricular outflow tract.
Table 3. Various techniques used in relieving right outflow tract obstruction.
TETRALOGY OF FALLOT:A Philippine Heart Center Review
ECG FindingsBote-Nuñez1980-1995
Villanueva1984-1987
Del Campo1992-1997
Daen1994-1996
Perfecto1996-2005
TOTAL
CRBBB 20 32 67 12 53 184
Normal 22 14 36
1st AV Block 24 24
LAD + CRBBB 22 22
IRBBB 7 4 11
RAD 9 9
CHB 3 3
Atrial Tachycardia 1 1
Table 4. Post-operative electrocardiographic findings in Filipino TOF patients .
CRBBB: Complete right bundle branch block; AV Block: Atrioventricular block; IRBBB: Incomplete right bundle branch block; RAD; Right-axis deviation; CHB: Complete Heart Block
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewRECOMMENDATION
In patients who show clinical symptoms, several examinations may be performed:
Chest X-Ray ECG 2-D Ech0 Doppler Magnetic Resonance Imaging
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewRECOMMENDATION
After the first year of intervention, if the patient is clinically asymptomatic, Doppler studies may be performed every three years (Grade C1)
Daen, C.P., (1997), Phil. Heart Center Journal 1996
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewPOIRIER’S MODIFIED CLASSIFICATIONGROUP 1
patient has NO symptoms
NO restriction in activityNO known residual VSDNO significant residual
pulmonary outflow obstructionNO reoperation NO related medications
administered at the time of follow-up
Poirier R. etal , J. Thorac Cardiovascular Surgery , 1977
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewPOIRIER’S MODIFIED CLASSIFICATION
GROUP 2
mild symptoms with activitymild restriction with activityminimal residual VSDresidual outflow gradient of 30-50
mmHg.a need for Digoxin or diuretics
Poirier R. etal , J. Thorac Cardiovascular Surgery, 1977
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewPOIRIER’S MODIFIED CLASSIFICATIONGROUP 3
moderate-to-severe symptoms and restrictions residual VSD with shunt greater than 1.5:1 outflow gradient greater than 50 mmHg. reoperation secondary to any cause other than bleeding in the early postoperative periodPoirier R. etal , J. Thorac Cardiovascular
Surgery, 1977
TETRALOGY OF FALLOT:A Philippine Heart Center Review
GROUP I - 25 patients
RECORDED PHILIPPINE STUDIES
GROUP II - 21 patients
GROUP III - 3 patients
Daen, C.P., Phil Heart Center Journal 1996Delfin, D.P., Phil Journal of Cardiology, 1991; 2:675-680
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
In unoperated TOF patients, a life-threatening complication is the TET SPELL.
These severe hypoxic episodes can be brought about by STRESS, ANXIETY, and EXERCISE and is a particular problem during the first two years of life.
Del Campo, J.F..M., PHC.R.022.99 Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
Emphasis on the education of these children and possible speech therapy should be brought up with the parents early in life (Grade C 1)
Yap, M.C., (2010), Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
1 Fox, D., Cleveland Clinic Journal of Medicine, 2010; 77 (1):821-8282 Delfin, D.P., Phil. Journal of Cardiology, 1991; 2:675-6803 Bonow, R.O., A Textbook of Cardiovascular Med, 9th ed. Philadelphia: Elsevier, 20124 Gatzoulis MA, et al., Lancet 2000; 356:975-981
COMPLICATIONS
A resting ECG with a QRS duration of 180 milliseconds or more has been considered as a sensitive predictor of life-threatening ventricular arrhythmias.
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Del Campo, J.F.M., PHC.R. 151..98, Unpublished paper
COMPLICATIONS
ECG and 2-D echo monitoring of right ventricular function in all post-operative TOF patients is recommended (Grade C 1). It has been found that the overall prevalence rate of ventricular arrhythmias in post-operative Filipino TOF patients was higher (58%) than those reported in literature (18%).
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCONCLUSION
Because of the growing number of TOF patients who have undergone repair, it is important to have a consistent follow-up.
It is recommended to have an annual check-up with an EXPERT CARDIOLOGIST whose interest is in congenital heart disease (Grade C 1).
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCONCLUSION
RE-INTERVENTION has been recommended in patients with:
residual VSD with a shunt > 1.5:1 residual pulmonary stenosis with RV systolic pressure > 2/3 of systemic pressure severe pulmonary regurgitation with RV dilatation or dysfunction exercise intolerance
Sommer, R.J., Circulation 2008; 117:1340-1350Bonow, R.O, A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia: Elsevier, 2012
TETRALOGY OF FALLOT:A Philippine Heart Center Review CONCLUSION
As this review has aimed to discuss TOF and its context in the Philippines, it is limited by the fact that some of the data have overlapping time frames and this might have affected the results. The data regarding the topic has mostly been descriptive thus, the recommendations offered by the authors are also limited by the lower level of evidence of the data gathered.
TETRALOGY OF FALLOT:A Philippine Heart Center Review CONCLUSION
This endeavor pioneers in guiding the Filipino clinician regarding the management of cases of patients with TOF and highlights possible future directions of research studies regarding TOF.
Merci beaucoup !
(Thank you very much!!)
TETRALOGY OF FALLOT:A Philippine Heart Center Review
If a palliative shunt is present, once nutritional buildup or the original reason for deferral of extensive surgery is met, definitive corrective surgery is recommended (Grade C1)
Bote-Nuñez, J.R., 2001, PHC.R.053.01, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
PROCEDURE DESCRIPTION
Classic Blalock-Taussig Shunt
Subclavian artery-to-pulmonary artery anastomosis (end-to-side)
Modified Blalock-Taussig Shunt
Interposition graft between subclavian arteryand ipsilateral pulmonary artery
Waterston Shunt Ascending aorta-to-main or right pulmonary artery (side-by-side)No artificial material used; shunt grows with the patient
Pott’s Shunt Descending aorta-to-left pulmonary artery (side-by-side)Necessitates reconstructive surgery during repair
COMMON PALLIATIVE PROCEDURES IN TETRALOGY OF FALLOT
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
POST-PERICARDIOTOMY SYNDROME was found to be a problem in the early post-operative period. patient may present with a low-
grade fever and chest discomfort Mild leukocytosis and pleural effusion on chest radiograph
First line of treatment - use of NSAIDs for 2-3 weeks with response within 48 hrs. (Grade B 1).
1 Bote-Nuñez, J.R., PHC.R.053.01, Unpublished paper2 Bonow, R.O., 2012
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Perfecto, S.M., PHC.R.060.08, Unpublished paper
COMPLICATIONS
The QRS prolongation reflects right ventricular dilatation rather than an increase in mass as seen in hypertrophy.
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
To alleviate these episodes the following should be done:
Positioning Calming the patient
Pharmacologic treatment
Del Campo, J.F.M., (2000), PHC.R.022.99, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
Pharmacologic Treatment includes:
Sodium Bicarbonate Morphine sulfate
Oxygen inhalation (Grade B 1)
Del Campo, J.F.M., (2000), PHC.R.022.99, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCONCLUSION
RE-INTERVENTION has been recommended in patients with:
sustained arrhythmias substantial LV dysfunction or QRS > 180 ms. significant AR with symptoms or progressive LV dilatation aortic root enlargement > 55 mm rapidly enlarging RVOT aneurysm
Sommer, R.J., Circulation 2008; 117:1340-1350Bonow, R.O, A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia: Elsevier, 2012
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCAUSES OF EARLY DEATH1. Myocardial Failure
2. Massive bleeding3. Multiple embolism4. Intra cranial Bleed5. Intractable ventricular tachycardia
1 Villanueva N.J. Results of Repair of TOF at the PHC, PHC.R.039.88 Unpublished Paper2 Claudio, M.T.E. Preoperative Echocardiographic Predictors of Outcome of Pediatric Patients Undergoing Total Correction of TOF CRF.006-04 3 Griffin, B.P., 2009
(14%)( 7%)(3.5%)
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCAUSES OF LATE DEATH1. Infective Endocarditis
Bote-Nuñez, J.R.Long Term Results after Total Repair of TOFPHC.R:053.01Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Previous shunt has not been noted to have a significant independent influence on operative mortality or morbidity rate.
1 Bote-Nuñez 2001 PHC.R:053.01, unpublished2 Villanueva, N.J. (1988) PHC.R:039.88, unpublished
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Primary total correction in infants and young children is now being advocated (Grade B1)
1 Gamponia, R.T., January 20122 Claudio, M.T.E., PHC.R:049.02 (2002)
TETRALOGY OF FALLOT:A Philippine Heart Center Review
The fundamental abnormality with TOF is the anterior and cephalad deviation of the outlet septum and its misalignment with the trabecular septum which involves the terminal spiral portion narrowing the pathway from the RV to the PA and enlarges the aortic root.
Sommer, R.J., Circ 2008Bonow, R.D., 2012
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewRECOMMENDATION
Those who have lower LV end diastolic diameter, LV end diastolic volume, and LV mass have been shown to have significantly more postoperative events. Patients thus who have lower left ventricular indices should be followed up more frequently.
Bensurto, E.S., Philip Journal of Pediatriics, 2000 Apr-Jun 49(2):117-121
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Only one parameter had a good predictive value with a p value of 0.010. Mcgoon’s index is the sum of the diameters of the left and right pulmonary arteries divided by the diameter of the descending aorta. A Mcgoon’s ratio of 1.7 is defined to have the best predictive value for good outcome and a value of 0.7 or greater is recommended at the PHC (Grade B 1)
Claudio, M.T.E.,(2002), PHC.R.049.02, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
In Filipino TOF patients who have the financial capability to undergo cardiac MRI, it is recommended to have at least one post-operatively to assess RV systolic function (Grade B 2)
Claudio, M.T.E.,(2002), PHC.R.049.02, Unpublished paper
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Del Campo, J.F.M., PHC.R. 151..98, Unpublished paper
COMPLICATIONS
Patients who had their operations delayed after the eighth year of age were found to be more susceptible to these arrhythmias when ventriculotomy was performed but there was no difference found with the transatrial approach.
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
In older patients, it is thus important to emphasize the need to use atriotomy to lessen the risk of development of ventricular arrhythmias and SCD (Grade B 1)
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewCOMPLICATIONS
Finding of prolonged QRS duration > 180 msrequires re-intervention in the form of an implanted defibrillator (Grade B 2).
TETRALOGY OF FALLOT:A Philippine Heart Center Review CONCLUSION
Annual examinations with a Cardiologist trained in congenital heart disease should be emphasized to prevent the development of irreversible complications.
TETRALOGY OF FALLOT:A Philippine Heart Center Review CONCLUSION
This endeavor pioneers in guiding the Filipino clinician regarding the management of cases of patients with TOF and highlights possible future directions of research studies regarding TOF.
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewSURGICAL INTERVENTIONAfter definitive surgery several murmurs may be
auscultated:a. low-pitched diastolic murmur of PRb. Systolic ejection murmur from residual
RVOT obstructionc. high-pitched diastolic murmur of AR d. pansystolic murmur from a VSD leak
Delfin et alPhil Journal of Cardiology, 1991: 2:675-680
TETRALOGY OF FALLOT:A Philippine Heart Center Review RESULTS
27 Researches
5 Published
22 Unpublished
13/27 is the subject of this report, one unpublished study was excluded because of lack of internal validity.
TETRALOGY OF FALLOT:A Philippine Heart Center ReviewRECOMMENDATION
Perfecto , S.M., 2008, PHC.R.060.08, Unpublished paper
PULMONARY VALVE REPLACEMENT (Grade B 1) serial prolongation of the QRS
duration severe PR with a PR index < 0.77 pulmonary regurgitant fraction of > 40% significant RV dilatation as suggested by index right ventricular end diastolic volume > 150 ml/m2 right ventricular systolic dysfunction with ejection fraction less than 40%
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Of the studies that recorded complications, the percentage of TOF patients who experienced complications was 65% of 296 patients.
1 Bote-Nuñez, J.R., PHC.R.053.01, Unpublished paper2 Villanueva, N.J., PHC.R.039.88, Unpublished paper
3 Del Campo, J.F.M., PHC.R.151.98, Unpublished paper
COMPLICATIONS
TETRALOGY OF FALLOT:A Philippine Heart Center Review
Postoperative TOF patient
Perfecto, S.M., PHC.R.060.08, Unpublished paper
COMPLICATIONS
development of arrhythmias sudden cardiac death (SCD)