RAUL D. JARA, M.D. MIKAELA NIKKOLA A. JARA, M.D. Philippine Heart Center.

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.


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Health Research Development Information (Herdin) of the Philippine Council for HealthResearch & Development (PCHRD) unpublished research initiatives by fellows of Adult & Pediatric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


All retrieved researches were reviewed by two investigators independently and articles that contained 20 patients with TOF were included in the review.

METHODOLOGY


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.


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


Principal Author


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


Principal Author


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)


57

None MortalityPresence of palliative procedureTechnique used for VSDand RVOT obstruction

Cross-sectional

Table 2. Summary of the studies reviewed.UNPUBLISHED STUDIES


Principal Author


Patientsn

Interventions

Comparisons

OutcomesStudyDesign

7 Del Campo,J.F.M.1992-1997(16)


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



Principal Author


Patientsn

Interventions

Comparisons

OutcomesStudyDesign

9 Claudio,M.T.E.1995-2004(5)


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



Principal Author


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



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


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


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


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


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


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


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.


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


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


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


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


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.


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).


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.


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!!)


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


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


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


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.


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


Pharmacologic Treatment includes:

Sodium Bicarbonate Morphine sulfate

Oxygen inhalation (Grade B 1)

Del Campo, J.F.M., (2000), PHC.R.022.99, Unpublished paper


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


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


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)


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


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


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


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


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.


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)


Finding of prolonged QRS duration > 180 msrequires re-intervention in the form of an implanted defibrillator (Grade B 2).


Annual examinations with a Cardiologist trained in congenital heart disease should be emphasized to prevent the development of irreversible complications.


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.


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%


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


Postoperative TOF patient

Perfecto, S.M., PHC.R.060.08, Unpublished paper

COMPLICATIONS

development of arrhythmias sudden cardiac death (SCD)

RAUL D. JARA, M.D. MIKAELA NIKKOLA A. JARA, M.D. Philippine Heart Center.

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