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

This study aims to provide a comprehensive review of the literature regarding the postoperative outcome of TOF in the Philippines GENERAL OBJECTIVE

SPECIFIC 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Health Research Development Information (Herdin) of the Philippine Council for Health Research & Development (PCHRD)  unpublished research initiatives by fellows of Adult & Pediatric Cardiology at PHC METHODOLOGY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

Table 1. Recommendation strength and quality of evidence.

Table 2.Summary of the studies reviewed. Table 2. Summary of the studies reviewed. PUBLISHED STUDIES

Table 2.Summary of the studies reviewed. Table 2. Summary of the studies reviewed. PUBLISHED STUDIES

Table 2. Summary of the studies reviewed. UNPUBLISHED STUDIES

Table 2.Summary of the studies reviewed. Table 2. Summary of the studies reviewed. UNPUBLISHED STUDIES

Table 2.Summary of the studies reviewed. Table 2. Summary of the studies reviewed. UNPUBLISHED STUDIES

Table 2. Summary of the studies reviewed. UNPUBLISHED STUDIES

Figure I. Mortality rate of various studies on TOF done at PHC. Mortality Rate

ONE-STAGE ONE-STAGE APPROACH APPROACHTWO-STAGE

The Common Palliative Procedures in TOF Modified Blalock- Taussig Taussig Waterston Shunt Classic Blalock Taussig Potts Shunt

SURGICAL INTERVENTION A. PALLIATIVE 1.Progressive cyanosis may still be noted due to: a.worsening RVOT obstruction b.gradual stenosis of palliative aorto pulmonary shunts. c.development of pulmonary hypertension d.progressive aortic dilatation and aortic regurgitation Bote-Nuñez, J.R., PHC.R , Unpublished paper Villanueva, N.J., PHC.R , Unpublished paper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found in terms of morbidity and mortalities with a two-stage compared with one-stage approach Villanueva, N.J., PHC Unpublished paper

1.Close the VSD 2.Relieve the RV outflow obstruction 3.Repair any stenosis in the pulmonary arteries Claudio, M.T.E, (2004), crf , Unpublished paper Nichols, David G. et al: Critical Heart Diseases in Infants & Children, Missouri, 1995, Mosby Year-Book, Inc. GOALS OF OPERATION WITH ONE-STAGE APPROACH WITH ONE-STAGE APPROACH

SURGICAL INTERVENTION A. DEFINITIVE (one stage) 1.Majority of the patients are asymptomatic although there are long-term complications: a.atrial tachyarrhythmias b.ventricular tachyarrhythmias c.progressive RV dilatation d.progressive aortic root dilatation Perfecto, S.M., PHC.R , Unpublished paper Bote-Nuñez, J.R., PHC.R , Unpublished paper

Figure II. Surgical Approach in TOF Correction Number of cases

Figure IIIMean age and range at which TOF patients are operated Figure III. Mean age and range at which TOF patients are operated Mean Age

TAP: transannular patching; RVOT: right ventricular outflow tract. Table 3. Various techniques used in relieving right outflow tract obstruction.

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

RECOMMENDATION In patients who show clinical symptoms, several examinations may be performed:  Chest X-Ray  ECG  2-D Ech0 Doppler  Magnetic Resonance Imaging

RECOMMENDATION 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

POIRIER’S MODIFIED CLASSIFICATION GROUP 1 patient has NO symptoms NO restriction in activity NO known residual VSD NO significant residual pulmonary outflow obstruction NO reoperation NO related medications administered at the time of follow-up NO related medications administered at the time of follow-up Poirier R. etal, J. Thorac Cardiovascular Surgery, 1977

POIRIER’S MODIFIED CLASSIFICATION GROUP 2 mild symptoms with activity mild restriction with activity minimal residual VSD residual outflow gradient of mmHg. a need for Digoxin or diuretics Poirier R. etal, J. Thorac Cardiovascular Surgery, 1977

POIRIER’S MODIFIED CLASSIFICATION GROUP 3 moderate-to-severe symptoms and restrictions residual VSD with shunt greater than 1.5:1 residual VSD with shunt greater than 1.5:1 outflow gradient greater than 50 mmHg. outflow gradient greater than 50 mmHg. reoperation secondary to any cause other than bleeding in the early postoperative period reoperation secondary to any cause other than bleeding in the early postoperative period Poirier 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 1996 Delfin, D.P., Phil Journal of Cardiology, 1991; 2:

COMPLICATIONS 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 Unpublished paper

COMPLICATIONS 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): Delfin, D.P., Phil. Journal of Cardiology, 1991; 2: Bonow, R.O., A Textbook of Cardiovascular Med, 9 th ed. Philadelphia: Elsevier, Gatzoulis MA, et al., Lancet 2000; 356: 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 , 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%).

CONCLUSION 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).

CONCLUSION RE-INTERVENTION has been recommended in patients with: residual VSD with a shunt > 1.5:1  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  severe pulmonary regurgitation with RV dilatation or dysfunction exercise intolerance  exercise intolerance Sommer, R.J., Circulation 2008; 117: Bonow, R.O, A Textbook of Cardiovascular Medicine, 9 th ed. Philadelphia: Elsevier, 2012

CONCLUSION 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.

CONCLUSION 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!!)

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 , Unpublished paper

COMMON PALLIATIVE PROCEDURES IN TETRALOGY OF FALLOT COMMON PALLIATIVE PROCEDURES IN TETRALOGY OF FALLOT

COMPLICATIONS 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  patient may present with a low-grade fever and chest discomfort Mild leukocytosis and pleural effusion on chest radiograph  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 , Unpublished paper 2 Bonow, R.O., 2012

Perfecto, S.M., PHC.R , Unpublished paper COMPLICATIONS The QRS prolongation reflects right ventricular dilatation rather than an increase in mass as seen in hypertrophy.

COMPLICATIONS To alleviate these episodes the following should be done:  Positioning Calming the patient  Calming the patient Pharmacologic treatment  Pharmacologic treatment Del Campo, J.F.M., (2000), PHC.R , Unpublished paper

COMPLICATIONS Pharmacologic Treatment includes:  Sodium Bicarbonate Morphine sulfate  Morphine sulfate Oxygen inhalation (Grade B 1)  Oxygen inhalation (Grade B 1) Del Campo, J.F.M., (2000), PHC.R , Unpublished paper

CONCLUSION RE-INTERVENTION has been recommended in patients with: sustained arrhythmias  sustained arrhythmias substantial LV dysfunction or QRS > 180 ms.  substantial LV dysfunction or QRS > 180 ms. significant AR with symptoms or progressive LV dilatation  significant AR with symptoms or progressive LV dilatation aortic root enlargement > 55 mm  aortic root enlargement > 55 mm rapidly enlarging RVOT aneurysm  rapidly enlarging RVOT aneurysm Sommer, R.J., Circulation 2008; 117: Bonow, R.O, A Textbook of Cardiovascular Medicine, 9 th ed. Philadelphia: Elsevier, 2012

CAUSES OF EARLY DEATH 1. Myocardial Failure 2. Massive bleeding 3.Multiple embolism 4. Intra cranial Bleed 5.Intractable ventricular tachycardia 1 Villanueva N.J. Results of Repair of TOF at the PHC, PHC.R Unpublished Paper 2 Claudio, M.T.E. Preoperative Echocardiographic Predictors of Outcome of Pediatric Patients Undergoing Total Correction of TOF CRF Griffin, B.P., 2009 (14%) ( 7%) (3.5%)

CAUSES OF LATE DEATH 1. Infective Endocarditis Bote-Nuñez, J.R. Long Term Results after Total Repair of TOF PHC.R: Unpublished 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, unpublished PHC.R:053.01, unpublished 2 Villanueva, N.J. (1988) PHC.R:039.88, unpublished PHC.R:039.88, unpublished

Primary total correction in infants and young children is now being advocated (Grade B1) 1 Gamponia, R.T., January Claudio, M.T.E., PHC.R: (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 2008 Bonow, R.D., 2012

RECOMMENDATION 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):

Only one parameter had a good predictive value with a p value of 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 , 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 , Unpublished paper

Del Campo, J.F.M., PHC.R , 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.

COMPLICATIONS 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)

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

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

CONCLUSION 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.

SURGICAL INTERVENTION After definitive surgery several murmurs may be auscultated: a.low-pitched diastolic murmur of PR b.Systolic ejection murmur from residual RVOT obstruction c.high-pitched diastolic murmur of AR d.pansystolic murmur from a VSD leak Delfin et al Phil Journal of Cardiology, 1991: 2:

RESULTS 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. lack of internal validity.

RECOMMENDATION Perfecto, S.M., 2008, PHC.R , 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/m 2  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 , Unpublished paper 2 Villanueva, N.J., PHC.R , Unpublished paper 3 Del Campo, J.F.M., PHC.R , Unpublished paper COMPLICATIONS

Postoperative TOF patient Perfecto, S.M., PHC.R , Unpublished paper COMPLICATIONS development of arrhythmias  development of arrhythmias sudden cardiac death (SCD)  sudden cardiac death (SCD)