Article Sidebar

PDF
Issue: Vol. 56
Section: Research Article
DOI: 10.47972/vjcts.v56i.1795
Date Published: 31/07/2026
Views 72
Downloads 32
How to Cite
Le, N. M., Nguyen, B. P., Luong, T. B., & Do, A. T. (2026). Tetralogy of Fallot Associated With a Restrictive Ventricular Septal Defect in an Infant: A Case Report. The Vietnam Journal of Cardiovascular and Thoracic Surgery, 56, 76 - 82. https://doi.org/10.47972/vjcts.v56i.1795

Tetralogy of Fallot Associated With a Restrictive Ventricular Septal Defect in an Infant: A Case Report

Ngoc Minh Le1,2, , Ba Phong Nguyen1, Tuan Bao Luong1, Anh Tien Do1,3
1 E Hospital
2 University of Medicine and Pharmacy, VNU
3 University of Medicine and Pharmacy

Main Article Content

Abstract

Background : Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart defects. In most patients the ventricular septal defect (VSD) is large and non-restrictive, allowing equalization of ventricular pressures. TOF associated with a restrictive VSD is a rare variant in which the interventricular pressure gradient elevates right ventricular pressure, altering the hemodynamic profile and complicating both diagnosis and surgical management.


Case presentation : A 3-month-old female infant (4.8 kg) presented with progressive cyanosis and oxygen saturation of 70–72% in room air. Echocardiography and cardiac computed tomography demonstrated TOF with a restrictive perimembranous VSD (approximately 3 mm; interventricular gradient 52 mmHg) and severe right ventricular outflow tract (RVOT) obstruction (peak gradient 77 mmHg). Complete repair was performed through a minimally invasive lower partial sternotomy. The early postoperative course was complicated by right ventricular dysfunction with myocardial edema and fluid overload, requiring delayed sternal closure, multiple inotropes, and peritoneal dialysis. The patient recovered gradually; at 3-month follow-up she was thriving, with good biventricular function, no residual shunt, and only mild residual RVOT obstruction.


Conclusion : TOF with a restrictive VSD is a rare variant whose distinct hemodynamics predispose to postoperative right ventricular dysfunction. Accurate preoperative diagnosis, an individualized surgical strategy, and anticipatory management of right heart failure are essential for a favorable outcome, even in young infants.

Keywords

Tetralogy of Fallot, Restrictive ventricular septal defect, Infant, Right ventricular dysfunction, Minimally invasive cardiac surgery.

Article Details

References

1. Flanagan MF, Foran RB, Van Praagh R, Jonas RA, Sanders SP. Tetralogy of Fallot with obstruction of the ventricular septal defect: spectrum of echocardiographic findings. J Am Coll Cardiol. 1988;11(2):386-395.
2. McBrien A, Kirk R, Crossland D. Tetralogy of Fallot with restrictive ventricular septal defect. Pediatr Cardiol. 2010;31(8):1267-1269. doi:10.1007/s00246-010-9773-0.
3. Soni MR, Sharma S, Tiwari A, Bhalodiya YN. Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid leaflet tissue. Indian Heart J. 2012;64(6):618-620. doi:10.1016/j.ihj.2012.08.020.
4. Amano K, Hirata Y, Shibata M, Ono M. Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid valve tissue: repair with valve preservation. Gen Thorac Cardiovasc Surg Cases. 2023;2:76-80.
5. Rajendra Kumar P, Rakshak D, Rajagopal P, Jaishanker S. Tetralogy of Fallot with flap-valve ventricular septal defect producing suprasystemic right ventricular pressure: echocardiographic observations. Indian Heart J. 1998;50(2):211-214.
6. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. 2009;374(9699):1462-1471. doi:10.1016/S0140-6736(09)60657-7.
7. Bailliard F, Anderson RH. Tetralogy of Fallot. Orphanet J Rare Dis. 2009;4:2. doi:10.1186/1750-1172-4-2.
8. Kannan BRJ. Tetralogy of Fallot. Ann Pediatr Cardiol. 2008;1(2):135-138. doi:10.4103/0974-2069.43882.
9. Kirklin JW, Barratt-Boyes BG. Cardiac Surgery. 4th ed. Philadelphia (PA): Churchill Livingstone Elsevier; 2013. Chapter 37, Tetralogy of Fallot; p. 1679-1743.
10. Anderson RH, Baker EJ, Penny DJ, Redington AN, Rigby ML, Wernovsky G, editors. Paediatric Cardiology. 3rd ed. Philadelphia (PA): Churchill Livingstone Elsevier; 2010. Chapter 29, Tetralogy of Fallot; p. 853-890.
11. Van Arsdell GS, Williams WG, Freedom RM, Rebeyka IM, Coles JG, Trusler GA. Tetralogy of Fallot: surgical management and outcomes. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:44-62.
12. Cullen S, Shore D, Redington A. Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot: restrictive physiology predicts slow postoperative recovery. Circulation. 1995;91(6):1782-1789.