Research Article

Clinical profile and surgical outcomes of children presenting with teratology of Fallot

Josephat M Chinawa*, Agarwal Vijay, Sarang Gaikwad, Bhadra Trivedi4 and Bartholomew Chukwu

Published: 14 September, 2020 | Volume 5 - Issue 3 | Pages: 157-162

Background: Tetralogy of Fallot (TOF) is a very common cyanotic congenital heart disease presenting early at birth with various degrees of cyanosis. If left uncorrected surgically, can lead to death.

Objectives: This study is aimed at determining pattern and surgical outcome of children with teratology of Fallot in a budding health facility in India over a year period.

Result: A total of 51 children were diagnosed of TOF over the period, of which 66.7% were males with mean age of 48.14 ± 45.36 months.

The surgical outcome showed only 3.9% mortality. The death was among children >1 to 5 years. The mean number of days in intensive care unit (ICU) was 5.8 ± 11.2 days. 82.4% of the patients were off-pump post-operatively, compared to 17.6% with re-pump. Among those who had re-pump, 77.8% were males and among those without re-pump, 64.3% were likewise males (χ2 = 0.6, p = 0.41). About 92.2% (47/51) of patients had pulmonary regurgitation post-op, ranging from mild to moderate regurgitation. 51.1% of the regurgitations were mild while 25.5% and 23.4% were moderate and severe regurgitations respectively.

Post-operative VSD was detected in 51% (26/51) of the patients. The post-op right ventricular pressure (RVOT) was significantly lower than that of pre-op pressure, 10.8 ± 1.5 mmHg vs. 31.7 ± 4.5 mmHg (pair t test = 8.7, p < 0.001).

Conclusion: Timely surgical repair is crucial in alleviating several morbidity and mortality associated with teratology of fallot. Pulmonary regurgitation is a very common sequel after surgery and can result in death.

Read Full Article HTML DOI: 10.29328/journal.jccm.1001103 Cite this Article Read Full Article PDF


Teratology of fallot; Children; Surgical repair; Death; Sequel


  1. van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis J Am Coll Cardiol. 2011; 58: 2241-2247. PubMed: https://pubmed.ncbi.nlm.nih.gov/22078432/
  2. Chinawa JM, Adiele KD, Ujunwa FA, Onukwuli VO, Arodiwe I, et al. Timing of cardiac surgery and other intervention among children with congenital heart disease: A review article. J Cardiol Cardiovasc Med. 2019; 4: 94-99. https://www.heighpubs.org/jccm/jccm-aid1047.php
  3. Valente AM, Gauvreau K, Assenza GE, Babu-Narayan SV, Schreier J, et al. Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Heart. 2014; 100: 247–253. PubMed: https://pubmed.ncbi.nlm.nih.gov/24179163/
  4. Kato A, Drolet C, Yoo SJ, Redington AN, Grosse-Wortmann L. Vicious circle between progressive right ventricular dilatation and pulmonary regurgitation in patients after tetralogy of Fallot repair? Right heart enlargement promotes flow reversal in the left pulmonary artery. J Cardiovasc Magn Reson. 2016; 18: 34. PubMed: https://pubmed.ncbi.nlm.nih.gov/27268132/
  5. Sen DG, Najjar M, Yimaz B, Levasseur SM, Kalessan B, et al. Aiming to preserve pulmonary valve function in tetralogy of Fallot repair: comparing a new approach to traditional management. Pediatr Cardiol. 2016; 37: 818–825. PubMed: https://pubmed.ncbi.nlm.nih.gov/26921062/
  6. Koyak Z, Harris L, de Groot JR, Silversides CK, Oechslin EN. Sudden cardiac death in adult congenital heart disease. Circulation 2012; 126: 1944-1954. PubMed: https://pubmed.ncbi.nlm.nih.gov/22991410/
  7. Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010; 122: 868-875. PubMed: https://pubmed.ncbi.nlm.nih.gov/20713900/
  8. Barron DJ, Ramchandani B, Murala J, Stumper O, de Giovanni JV, et al. Surgery following primary right ventricular outflow tract stenting for Fallot's Tetralogy and variants: rehabilitation of small pulmonary arteries. Eur J Cardiothorac Surg. 2013; 44: 656-662. PubMed: https://pubmed.ncbi.nlm.nih.gov/23650024/
  9. Mahmoud US, Mariya MY, Karaye KM. Spectrum of congenital heart disease in a tropical environment: An echocardiography study. J National Med Association. 2007; 99: 665-659. PubMed: https://pubmed.ncbi.nlm.nih.gov/17595936/
  10. Chinawa JM, Obu HA, Eke CB, Eze JC. Pattern and clinical profile of children with complex cardiac anomaly at University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu State, Nigeria. Niger J Clin Pract. 2013; 16: 462-467.
  11. Imran K, Zafar T, Saeed A, Madiha I, Tipu K, et al. Surgery for Tetralogy of Fallot in Adults: Early Outcomes. Braz. J. Cardiovasc. Surg. 2016; 300-303. PubMed: https://pubmed.ncbi.nlm.nih.gov/27849302/
  12. Conotruncal Abnormalities | Pediatric Practice. https://accesspediatrics.mhmedical.com/content.aspx?bookid=525§ionid=
  13. Edward JL, Jacqueline SC, David MI, Kathleen S, Kazutoyo OS, et al. Chromosomal abnormalities among children born with conotruncal cardiac defects. Birth Defects Res A Clin Mol Teratol. 2009; 85: 30–35. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2856481/
  14. Glen SV, Gyaandeo SM, Julie T, Vivek KR, John GC, et al. What is the Optimal Age for Repair of Tetralogy of Fallot? Circulation. 2000; 102: 123–129. PubMed: https://pubmed.ncbi.nlm.nih.gov/11082374/
  15. Asani M, Aliyu I, Kabir H. Profile of congenital heart defects among children at Aminu Kano Teaching Hospital Kano, Nigeria. Journal of Medicine in the Tropics. 2013; 15: 131-134.
  16. Khadim J, Issa S. Spectrum of congenital heart disease in Basra: an echocardiography study. The Medical Journal of Basra University. 2009; 27: 15-18.
  17. Kapoor R, Gupta S. Prevalence of congenital heart disease, Kanpur, India. Indian Pediatr. 2008; 45: 309-311.
  18. Kennedy N, Miller P. The spectrum of paediatric cardiac disease presenting to an outpatient clinic in Malawi. BMC Res Notes. 2013; 6: 53. PubMed: https://pubmed.ncbi.nlm.nih.gov/23388520/
  19. Arch Mal Coeur Vaiss. Tetralogy of Fallot. 20 Years' Experience in 144 Cases.1989; 82: 683-688.
  20. Meriem M, Lucile H, Damien B. Anatomy of the ventricular septal defect in congenital heart defects: a random association? Orphanet J Rare Dis. 2018; 13: 118. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6052685/
  21. Anderson RH, Weinberg PM. "The clinical anatomy of tetralogy of fallot". Cardiol Young. 2005; 15: 38–47. PubMed: https://pubmed.ncbi.nlm.nih.gov/15934690/
  22. Seddio L, Migliazza A, Borghi G, Crupi. Previous palliation in patients with tetralogy of Fallot does not influence the outcome of later repair. J Cardiovasc Med. 2007; 8: 119-122. PubMed: https://pubmed.ncbi.nlm.nih.gov/17299294/
  23. Sarris GE, Comas JV, Tobota Z, Maruszewski B. Results of reparative surgery for tetralogy of Fallot: data from the European Association for Cardio-Thoracic Surgery Congenital Database. Eur J Cardiothorac Surg. 2012; 42: 766-774. PubMed: https://pubmed.ncbi.nlm.nih.gov/23087090/
  24. Tirilomis T, Friedrich M, Zenker D, Seipelt RG, Schoendube FA, et al. Indications for reoperation late after correction of tetralogy of Fallot .Cardiol Young. 2010; 20: 396-401. PubMed: https://pubmed.ncbi.nlm.nih.gov/20456817/
  25. Pekka Y, Nieminen H, Olli MP, Eero J, Heikki S. Need of transannular patch in tetralogy of Fallot surgery carries a higher risk of reoperation but has no impact on late survival. European J Cardio-Thoracic Sur. 2015; 48: 91–97. PubMed: https://pubmed.ncbi.nlm.nih.gov/25326015/
  26. Ammash NM, Dearani JA, Burkhart HM, Connolly HM. Pulmonary Regurgitation After Tetralogy of Fallot Repair: Clinical Features, Sequelae, and Timing of Pulmonary Valve Replacement. Congenit Heart Dis. 2007; 2: 386-403. PubMed: https://pubmed.ncbi.nlm.nih.gov/18377431/
  27. Fraser CD, McKenzie ED, Cooley DA. Tetralogy of Fallot: surgical management individualized to the patient. Ann Thorac Surg. 2001; 17: 1556-1563. PubMed: https://pubmed.ncbi.nlm.nih.gov/11383800/
  28. Udekem YD, Ovaert C, Grandjean F, Gerin V, Cailteux M, et al. Tetralogy of Fallot: transannular and right ventricular patching equally affect late functional status. Circulation 2000; 102 :116-122.
  29. Andrew LD, Amber CD, Christopher JS, Jeffrey MG, Rahul D, et al. Characteristics and Hemodynamic Effects of Extubation Failure in Children Undergoing Complete Repair for Tetralogy of Fallot. Comparative Study Pediatr Cardiol. 2013; 34: 1455-1462. PubMed: https://pubmed.ncbi.nlm.nih.gov/23463132/
  30. Roos-Hesselink J, Perlroth MG, McGhie J, Spitaels S. Atrial arrhythmias in adults after repair of tetralogy of Fallot. Correlations with clinical, exercise, and echocardiographic findings. Circulation. 1995; 91: 2214-2219. PubMed: https://pubmed.ncbi.nlm.nih.gov/7697851/
  31. Harrison DA, Siu SC, Hussain F, MacLoghlin CJ, Webb GD, et al. Sustained atrial arrhythmias in adults late after repair of tetralogy of Fallot. Am J Cardiol. 2001; 87: 584–588. PubMed: https://pubmed.ncbi.nlm.nih.gov/11230843/
  32. Frank AP, Philipe NK, John EM, Pedro JN, Richard AJ. Repair of Tetralogy of Fallot in Neonates and Young Infants.Circulation. 1999; 100: 157–161. PubMed: https://pubmed.ncbi.nlm.nih.gov/10567296/
  33. Newburger JW, Jonas RA, Wernovsky G, Wypij D, Hickey PR, et al. Comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart surgery. N Engl J Med. 1993; 329: 1057–1034. PubMed: https://pubmed.ncbi.nlm.nih.gov/8371727/
  34. Bellinger DC, Jonas RA, Rappaport LA, Wypij D, Wernovsky G, et al. Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. N Engl J Med. 1995; 332: 549–545. PubMed: https://pubmed.ncbi.nlm.nih.gov/7838188/
  35. Robert A , Nancy RA, Joshua W, Serafin D. Bidirectional Glenn Shunt as an Adjunct to Surgical Repair of Congenital Heart Disease Associated with Pulmonary Outflow Obstruction: Relevance of the Fluid Pressure Drop-Flow Relationship. Pediatr Cardiol. 2008; 29: 910-917. PubMed: https://pubmed.ncbi.nlm.nih.gov/18551335/
  36. Kamal S, Iftikhar A, Mehboob S, Intisar Ul , Umair Y, et al. Bidirectional Glenn for Residual Outflow Obstruction in Tetralogy of Fallot. Cardiol Young. 2019; 29: 684-688. PubMed: https://pubmed.ncbi.nlm.nih.gov/31118113/
  37. Marco P, Dipesh BT, Denise K, Robert AA. Tetralogy of Fallot: what operation, at which age. European J Cardio-Thoracic Surg. 2000; 17: 631–636. PubMed: https://pubmed.ncbi.nlm.nih.gov/10856851/
  38. Brizard CP, Mas C, Sohn YS, Cochrane AD, Karl TR. Transatrial-transpulmonary tetralogy of Fallot repair is effective in the presence of anomalous coronary arteries. J Thorac Cardiovasc Surg. 1998; 116: 770-779. PubMed: https://pubmed.ncbi.nlm.nih.gov/9806384/
  39. Kalra S, Sharma R, Choudhary SK, Airan B, Bhan A, et al. Right ventricular outflow tract after non-conduit repair of tetralogy of Fallot with coronary anomaly. Ann Thorac Surg. 2000; 70: 723-726. PubMed: https://pubmed.ncbi.nlm.nih.gov/11016300/
  40. van Son JA. Repair of tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. J Thorac Cardiovasc Surg. 1995; 110: 561-562. PubMed: https://pubmed.ncbi.nlm.nih.gov/7637382/
  41. Kiran U, Aggarwal S, Choudhary A, Uma B, Kapoor PM. The Blalock and Taussig Shunt Revisited. Ann Card Anaesth. 2017; 20: 323–330. PubMed: https://pubmed.ncbi.nlm.nih.gov/28701598/
  42. Fraser CD Jr, McKenzie ED, Cooley DA. Tetralogy of Fallot: Surgical management individualized to the patient. Ann Thorac Surg. 2001; 71:1556-1561. PubMed: https://pubmed.ncbi.nlm.nih.gov/11383800/
  43. Azari A, Nezafati M, Bigdelu L. Early post operative mortality of Total Correction of Tetralogy of Fallot. J Cardio-Thoracic Med. 2017; 5: 222-225.
  44. Cuypers JA, Menting ME, Konings EE, et al. Unnatural history of tetralogy of Fallot: prospective follow-up of 40 years after surgical correction. Circulation. 2014; 130: 1944-1953. PubMed: https://pubmed.ncbi.nlm.nih.gov/25341442/
  45. Chang YH, Lee HD, Park JA, Lee YS. Early and late outcomes of total repair of tetralogy of Fallot: risk factors for late right ventricular dilatation. Interact Cardiovasc Thorac Surg. 2013; 17: 956-962. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3829492/

Similar Articles

Recently Viewed

Read More

Most Viewed

Read More