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TETRALOGY OF FALLOT a Philippine Heart Center Review

TETRALOGY OF FALLOT a 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. GENERAL OBJECTIVE.

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TETRALOGY OF FALLOT a Philippine Heart Center Review

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  1. TETRALOGY OF FALLOTa Philippine Heart Center Review RAUL D. JARA, M.D. MIKAELA NIKKOLA A. JARA, M.D. Philippine Heart Center

  2. TETRALOGY OF FALLOT:A Philippine Heart Center Review GENERAL OBJECTIVE This study aims to provide a comprehensive review of the literature regarding the postoperative outcome of TOF in the Philippines

  3. TETRALOGY OF FALLOT:A Philippine Heart Center Review SPECIFIC OBJECTIVES To be able to determine the surgical outcome of patients with TOF in the Philippines. To be able to determine the appropriate diagnostic examinations for postoperative TOF patients. To be able to determine the common complications of TOF in postoperative patients.

  4. TETRALOGY OF FALLOT:A Philippine Heart Center Review METHODOLOGY • 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Health Research Development Information (Herdin) of the Philippine Council for Health Research & Development (PCHRD) • unpublished research initiatives by fellows of Adult & Pediatric Cardiology at PHC

  5. TETRALOGY OF FALLOT:A Philippine Heart Center Review METHODOLOGY All retrieved researches were reviewed by two investigators independently and articles that contained 20 patients with TOF were included in the review.

  6. TETRALOGY OF FALLOT:A Philippine Heart Center Review

  7. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 2.Summary of the studies reviewed. PUBLISHED STUDIES

  8. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 2.Summary of the studies reviewed. PUBLISHED STUDIES

  9. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 2. Summary of the studies reviewed. UNPUBLISHED STUDIES

  10. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 2.Summary of the studies reviewed. UNPUBLISHED STUDIES

  11. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 2.Summary of the studies reviewed. UNPUBLISHED STUDIES

  12. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 2. Summary of the studies reviewed. UNPUBLISHED STUDIES

  13. TETRALOGY OF FALLOT:A Philippine Heart Center Review Mortality Rate Figure I. Mortality rate of various studies on TOF done at PHC.

  14. TETRALOGY OF FALLOT:A Philippine Heart Center Review ONE-STAGE APPROACH TWO-STAGE

  15. TETRALOGY OF FALLOT:A Philippine Heart Center Review The Common Palliative Procedures in TOF Classic Blalock Taussig Modified Blalock- Taussig Potts Shunt Waterston Shunt

  16. TETRALOGY OF FALLOT:A Philippine Heart Center Review SURGICAL INTERVENTION • 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.053.01, Unpublished paper Villanueva, N.J., PHC.R.039.88, Unpublished paper

  17. 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

  18. TETRALOGY OF FALLOT:A Philippine Heart Center Review GOALS OF OPERATION WITH ONE-STAGE APPROACH 1. Close the VSD 2. Relieve the RV outflow obstruction 3. Repair any stenosis in the pulmonary arteries Claudio, M.T.E, (2004), crf.006.04, Unpublished paper Nichols, David G. et al: Critical Heart Diseases in Infants & Children, Missouri, 1995, Mosby Year-Book, Inc.

  19. TETRALOGY OF FALLOT:A Philippine Heart Center Review SURGICAL INTERVENTION • DEFINITIVE (one stage) 1. Majority of the patients are asymptomatic although there are long-term complications: a. atrialtachyarrhythmias b. ventricular tachyarrhythmias c. progressive RV dilatation d. progressive aortic root dilatation Perfecto, S.M., PHC.R.060.08, Unpublished paper Bote-Nuñez, J.R., PHC.R.053.01, Unpublished paper

  20. TETRALOGY OF FALLOT:A Philippine Heart Center Review Number of cases Figure II. Surgical Approach in TOF Correction

  21. TETRALOGY OF FALLOT:A Philippine Heart Center Review Mean Age Figure III. Mean age and range at which TOF patients are operated

  22. TETRALOGY OF FALLOT:A Philippine Heart Center Review Table 3. Various techniques used in relieving right outflow tract obstruction. TAP: transannular patching; RVOT: right ventricular outflow tract.

  23. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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

  24. TETRALOGY OF FALLOT:A Philippine Heart Center Review RECOMMENDATION • In patients who show clinical symptoms, several examinations may be performed: • Chest X-Ray • ECG • 2-D Ech0 Doppler • Magnetic Resonance Imaging

  25. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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

  26. TETRALOGY OF FALLOT:A Philippine Heart Center Review POIRIER’S MODIFIED CLASSIFICATION GROUP 1 patient has NO symptoms NOrestriction in activity NO known residual VSD NO significant residual pulmonary outflow obstruction NO reoperation NO related medications administered at the time of follow-up       Poirier R. etal , J. Thorac Cardiovascular Surgery , 1977

  27. TETRALOGY OF FALLOT:A Philippine Heart Center Review POIRIER’S MODIFIED CLASSIFICATION GROUP 2 mild symptoms with activity mild restriction with activity minimal residual VSD residual outflow gradient of 30-50 mmHg. a need for Digoxin or diuretics      Poirier R. etal , J. Thorac Cardiovascular Surgery, 1977

  28. TETRALOGY OF FALLOT:A Philippine Heart Center Review POIRIER’S MODIFIED CLASSIFICATION GROUP 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 period     Poirier R. etal , J. Thorac Cardiovascular Surgery, 1977

  29. TETRALOGY OF FALLOT:A Philippine Heart Center Review RECORDED PHILIPPINE STUDIES GROUP I - 25 patients GROUP II - 21 patients GROUP III - 3 patients Daen, C.P., Phil Heart Center Journal 1996 Delfin, D.P., Phil Journal of Cardiology, 1991; 2:675-680

  30. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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.022.99 Unpublished paper

  31. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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

  32. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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. 1 Fox, D., Cleveland Clinic Journal of Medicine, 2010; 77 (1):821-828 2 Delfin, D.P., Phil. Journal of Cardiology, 1991; 2:675-680 3 Bonow, R.O., A Textbook of Cardiovascular Med, 9th ed. Philadelphia: Elsevier, 2012 4 Gatzoulis MA, et al., Lancet 2000; 356:975-981

  33. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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%). Del Campo, J.F.M., PHC.R. 151..98, Unpublished paper

  34. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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 CARDIOLOGISTwhose interest is in congenital heart disease (Grade C 1).

  35. TETRALOGY OF FALLOT:A Philippine Heart Center Review CONCLUSION 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; 117:1340-1350 Bonow, R.O, A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia: Elsevier, 2012

  36. 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.

  37. 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.

  38. Merci beaucoup ! (Thank you very much!!)

  39. 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

  40. TETRALOGY OF FALLOT:A Philippine Heart Center Review COMMON PALLIATIVE PROCEDURES IN TETRALOGY OF FALLOT

  41. TETRALOGY OF FALLOT:A Philippine Heart Center Review 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 • 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 paper 2 Bonow, R.O., 2012

  42. TETRALOGY OF FALLOT:A Philippine Heart Center Review COMPLICATIONS The QRS prolongation reflects right ventricular dilatation rather than an increase in mass as seen in hypertrophy. Perfecto, S.M., PHC.R.060.08, Unpublished paper

  43. TETRALOGY OF FALLOT:A Philippine Heart Center Review COMPLICATIONS 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

  44. TETRALOGY OF FALLOT:A Philippine Heart Center Review COMPLICATIONS Pharmacologic Treatment includes: • Sodium Bicarbonate • Morphine sulfate • Oxygen inhalation (Grade B 1) Del Campo, J.F.M., (2000), PHC.R.022.99, Unpublished paper

  45. TETRALOGY OF FALLOT:A Philippine Heart Center Review CONCLUSION 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; 117:1340-1350 Bonow, R.O, A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia: Elsevier, 2012

  46. TETRALOGY OF FALLOT:A Philippine Heart Center Review CAUSES OF EARLY DEATH • Myocardial Failure • Massive bleeding 3. Multiple embolism • Intra cranial Bleed 5. Intractable ventricular tachycardia (14%) ( 7%) (3.5%) 1 Villanueva N.J. Results of Repair of TOF at the PHC, PHC.R.039.88 Unpublished Paper 2 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

  47. TETRALOGY OF FALLOT:A Philippine Heart Center Review • Infective Endocarditis CAUSES OF LATE DEATH Bote-Nuñez, J.R. Long Term Results after Total Repair of TOF PHC.R:053.01 Unpublished paper

  48. 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, unpublished 2 Villanueva, N.J. (1988) PHC.R:039.88, unpublished

  49. 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 2012 2 Claudio, M.T.E., PHC.R:049.02 (2002)

  50. 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 2008 Bonow, R.D., 2012

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