Case report discussion of management of pink tetralogy of Fallot in VACTERL association

Main Article Content

Bayushi Eka Putra
Toto Wisnu Hendrarto


Tetralogy of Fallot (TOF) and VACTERL are considered rare entities requiring medical attention with regards to diagnosis and management. When TOF is associated with VACTERL association, case management might differ from a simple case of TOF.

Tetralogy of Fallot (TOF) is defined as a complex congenital cardiac abnormality characterized by ventricular septal defect, right outflow tract obstruction, right ventricular hypertrophy, and overriding aorta.1 The term was coined in 1888 by Fallot of Marseilles, even though the defect had been described in 1673 by Steno of Denmark. The entity was then studied extensively to deliver better care management through medical and surgical approaches.2

Article Details

How to Cite
Putra B, Hendrarto T. Case report discussion of management of pink tetralogy of Fallot in VACTERL association. PI [Internet]. 25Feb.2019 [cited 24Jan.2020];59(3):164-. Available from:
Case Report
Author Biographies

Bayushi Eka Putra, Department of Cardiology and Vascular Medicine, Universitas Indonesia Medical School/National Cardiovascular Centre Harapan Kita, Jakarta

Bayushi Eka Putra

Department of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Indonesia, National Heart Centre Harapan Kita, Jakarta, Indonesia


Toto Wisnu Hendrarto, Subdivision of Perinatology, Department of Pediatric, Women and Children Hospital Harapan Kita, Jakarta

Toto Wisnu Hendarto

Subdivision of Perinatology, Department of Pediatrics, Women and Children Hospital Harapan Kita, Jakarta, Indonesia

Received 2018-08-07
Accepted 2019-02-25
Published 2019-02-25


1. van Doorn C. The unnatural history of tetralogy of Fallot: surgical repair is not as definitive as previously thought. Heart. 2002;88:447–8.
2. Neill CA, Clark EB. Tetralogy of Fallot. The first 300 years. Tex Heart Inst J. 1994;21:272–9.
3. Bhimji S, Mancini MC. Tetralogy of Fallot. [cited 2018 Aug 1]. Available from:
4. Siriapisith T, Wasinrat J, Tresukosol D. Uncorrected pink tetralogy of Fallot in an adult patient: incidental CT findings. J Cardiovasc Comput Tomogr. 2010;4:58–61.
5. Solomon BD. VACTERL/VATER Association. Orphanet J Rare Dis. 2011;6:56.
6. Özer E. VACTERL association [Internet]. 2016; [cited 2018 Aug 3]. Available from:
7. Park MK. Park’s pediatric cardiology for practitioners. Missouri: Mosby; 2014. p. 676.
8. Bailliard F, Anderson RH. Tetralogy of Fallot. Orphanet J Rare Dis. 2009;4:2.
9. Gupta R, Pandey C, Tripathi S. VACTERL rare association with rare survival pattern. J Indian Coll Cardiol. 2015;5:80–5.
10. Chen Y, Liu Z, Chen J, Zuo Y, Liu S, Chen W, et al. The genetic landscape and clinical implications of vertebral anomalies in VACTERL association. J Med Genet. 2016;53:431–7.
11. Simpson JM. Impact of fetal echocardiography. Ann Pediatr Cardiol. 2009;2:41–50.
12. Yoo SJ, Lee YH, Kim ES, Ryu HM, Kim MY, Yang JH, et al. Tetralogy of Fallot in the fetus: findings at targeted sonography. Ultrasound Obstet Gynecol. 1999;14:29–37.
13. Oster M. Newborn screening for critical congenital heart disease using pulse oximetry [Internet]. UpToDate. 2018; [cited 2018 Aug 3]. Available from:
14. Lee C-H, Kwak JG, Lee C. Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia. Korean J Pediatr. 2014;57:19–25.
15. Pozzi M, Trivedi DB, Kitchiner D, Arnold RA. Tetralogy of Fallot: what operation, at which age. Eur J Cardiothorac Surg. 2000;17:631–6.
16. Pettersen MD. Prostin VR Pediatric (alprostadil IV) dosing, indications, interactions, adverse effects, and more [Internet]. 2016; [cited 2018 Aug 4]. Available from:
17. Kanter KR, Kogon BE, Kirshbom PM, Carlock PR. Symptomatic neonatal tetralogy of Fallot: repair or shunt? Ann Thorac Surg. 2010;89:858–63.
18. Kirklin JW, Blackstone EH, Jonas RA, Shimazaki Y, Kirklin JK, Mayer JE Jr, et al. Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. A two-institution study. J Thorac Cardiovasc Surg. 1992;103:706–23.
19. Jonas RA. Early primary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;39–47.
20. Seliem MA, Wu YT, Glenwright K. Relation between age at surgery and regression of right ventricular hypertrophy in tetralogy of Fallot. Pediatr Cardiol. 1995;16:53–5.
21. Sharma M, Nair MNG, Jatana SK, Shahi BN. Congestive heart failure in infants and children. Med J Armed Forces India. 2003;59:228–33.
22. Farha S, Lundgrin EL, Erzurum SC. Novel therapeutic approaches to preserve the right ventricle. Curr Heart Fail Rep. 2013;10:12–7.
23. Simeoni M, Armeni A, Summaria C, Cerantonio A, Fuiano G. Current evidence on the use of anti-RAAS agents in congenital or acquired solitary kidney. Ren Fail. 2017;39:660–70.