Non-surgical intervention for palliative treatment in Late-presentation Tetralogy of Fallot (TOF): Is there any hope?
Abstract
Background: Late congenital heart disease (CHD) in patients with tetralogy of Fallot (TOF). Due to the variable severity of defects in patients with TOF, late presentation of CHD may only be discovered beyond the neonatal period. Chronic polycythemia from TOF may increase the risk of hemorrhaging during surgery and patients with untreated TOF risk developing CHD-related pulmonary hypertension. Non-surgical transcatheter palliation in patients with TOF may be applied; however, the efficacy and safety of the method remained very scarce. Therefore, we report two cases of late-presenting TOF treated with non-surgical transcatheter palliation due to high perioperative risks for surgical repair of the defects.
Case Illustration: A 41-year-old (Case 1) and 19-year-old man (Case 2) were admitted to the emergency room due to chief complaint of dyspnea and severe headache with previous history of hypoxic spell, respectively. Both patients had presented with signs of right ventricular hypertrophy and cardiomegaly from physical examination. Echocardiography had confirmed TOF in both cases. Non-surgical palliation for both cases were performed with right ventricular outflow tract (RVOT) stenting and balloon pulmonary valvuloplasty (BPV), respectively. Both patients had shown clinical and systolic function improvement after both interventions.
Conclusion: Late-presentation TOF may present with major comorbidities that contraindicates definitive repair of the defects due to high perioperative risk. Palliative interventions in late-presentation TOF may be considered as a bridging procedure prior to the definitive repair to minimize the risk of complications from untreated TOF beyond neonatal period.
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References
2. Mouws EMJP, de Groot NMS, van de Woestijne PC, de Jong PL, Helbing WA, van Beynum IM, et al. Tetralogy of Fallot in the Current Era. Semin Thorac Cardiovasc Surg. 2019;31(3):496–504.
3. Iyer PU, Moreno GE, Fernando Caneo L, Faiz T, Shekerdemian LS, Iyer KS. Management of late presentation congenital heart disease. Cardiol Young. 2017 Dec;27(S6):S31–9.
4. Starr JP. Tetralogy of fallot: yesterday and today. World J Surg. 2010 Apr;34(4):658–68.
5. Therrien J, Siu SC, McLaughlin PR, Liu PP, Williams WG, Webb GD. Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol. 2000 Nov;36(5):1670–5.
6. Koppel CJ, Jongbloed MRM, Kiès P, Hazekamp MG, Mertens BJA, Schalij MJ, et al. Coronary anomalies in tetralogy of Fallot – A meta-analysis. Int J Cardiol. 2020 May;306:78–85.
7. Bodhey NK, Beerbaum P, Sarikouch S, Kropf S, Lange P, Berger F, et al. Functional analysis of the components of the right ventricle in the setting of tetralogy of Fallot. Circ Cardiovasc Imaging. 2008 Sep;1(2):141–7.
8. Karl TR, Stocker C. Tetralogy of Fallot and Its Variants. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc. 2016 Aug;17(8 Suppl 1):S330-336.
9. Sen S, Dalvi B. Palliative Balloon Pulmonary Valvotomy in Tetralogy of Fallot: Is There a Role in 2021? Hearts. 2021 Jun;2(2):224–33.
10. Khan SM, Drury NE, Stickley J, Barron DJ, Brawn WJ, Jones TJ, et al. Tetralogy of Fallot: morphological variations and implications for surgical repair. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2019 Jul 1;56(1):101–9.
11. Dohlen G, Chaturvedi RR, Benson LN, Ozawa A, Van Arsdell GS, Fruitman DS, et al. Stenting of the right ventricular outflow tract in the symptomatic infant with tetralogy of Fallot. Heart Br Card Soc. 2009 Feb;95(2):142–7.
12. van der Ven JPG, van den Bosch E, Bogers AJCC, Helbing WA. Current outcomes and treatment of tetralogy of Fallot. F1000Research. 2019;8:F1000 Faculty Rev-1530.
13. Linnane N, Walsh KP, McGuinness J, Linnane N, Damien K. Evolution of Approach to Right Ventricular Outflow Tract Stenting in Infants ≤ 2Kgs. J Struct Heart Dis. 2018 Apr 30;4(2):50–5.
14. Pizzuto A, Cuman M, Assanta N, Franchi E, Marrone C, Pak V, et al. Right Ventricular Outflow Tract Stenting as Palliation of Critical Tetralogy of Fallot: Techniques and Results. Hearts. 2021 Jun;2(2):278–87.
15. Godart F, Rey C, Prat A, Muilwijk C, Francart C, Vaksmann G, et al. Early and late results and the effects on pulmonary arteries of balloon dilatation of the right ventricular outflow tract in tetralogy of Fallot. Eur Heart J. 1998 Apr;19(4):595–600.
16. Castleberry CD, Gudausky TM, Berger S, Tweddell JS, Pelech AN. Stenting of the right ventricular outflow tract in the high-risk infant with cyanotic teratology of Fallot. Pediatr Cardiol. 2014 Mar;35(3):423–30.
17. Karlsen S, Dahlslett T, Grenne B, Sjøli B, Smiseth O, Edvardsen T, et al. Global longitudinal strain is a more reproducible measure of left ventricular function than ejection fraction regardless of echocardiographic training. Cardiovasc Ultrasound. 2019 Sep 2;17(1):18.
18. Ingle L, Cleland JG, Clark AL. The long-term prognostic significance of 6-minute walk test distance in patients with chronic heart failure. BioMed Res Int. 2014;2014:505969.
19. Tiksnadi BB, Ambari AM, Adriana M. Uji Jalan 6 Menit (UJ6M) pada Pasien Pasca Sindrom Koroner Akut. Indones J Cardiol [Internet]. 2019 Sep 11 [cited 2022 Dec 8];40(1). Available from: http://ijconline.id/index.php/ijc/article/view/913
20. Porcari JP, Foster C, Cress ML, Larson R, Lewis H, Cortis C, et al. Prediction of Exercise Capacity and Training Prescription from the 6-Minute Walk Test and Rating of Perceived Exertion. J Funct Morphol Kinesiol. 2021 Jun 14;6(2):52.
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