Clinical Case of Treating a Child with Functionally Single Ventricle Complicated by the Blood Clot Formation in the Rudimentary Ventricle and Acute Cerebrovascular Accident
Single ventricle is a congenital heart defect in which there is only one ventricle that may be identified by all anatomical clues. It provides both systemic and pulmonary circulation. If there is another ventricular chamber, it is usually represented only by the outflow tract. Most often, this defect is diagnosed in the first days or months of life. Clinical presentation de-pends on the type of defect, in most cases is characterized by cyanosis after birth. In case of natural course of the disease most patients die in childhood, therefore, they need surgical treatment. Today Fontan procedure allows patients with a single ventricle to survive until adulthood.
Objective. To represent a clinical case of treating a patient with single ventricle and analyze the tactics of surgical inter-vention and anticoagulant therapy taking into account blood clot formation in the rudimentary ventricle and acute cerebro-vascular accident caused by it.
Materials and methods. The article describes a case of surgical treatment of single ventricle in a child, which was com-plicated by the formation of blood clot in the rudimentary right ventricle causing multiple acute cerebrovascular accidents.
Discussion. Anticoagulant therapy does not guarantee freedom from blood clots formation. That is why the use of surgical methods is more reasonable in order to reduce the risk of such complications.
Conclusion. Along with the prescription of anticoagulant therapy in patients with single ventricle, special attention should be paid to the surgical modifications of the Fontan procedure with the so-called «blind pouch», which may become a site for the blood clots formation due to the possible stagnation of blood and propensity of such patients to hypercoagulation for a number of reasons.
- van Doorn CA, de Leval MR. The lateral tunnel Fontan. Oper Tech in Thorac and Cardiovasc Surg. 2006;11(2):105– 22. https://doi.org/10.1053/j.optechstcvs.2006.05.001
- Nakano T, Kado H, Tatewaki H, Hinokiyama K, Oda S, Ushinohama H, et al. Results of extracardiac conduit total cavopulmonary connection in 500 patients. Eur J Cardiothorac Surg. 2015;48(6):825–32; discussion 832. https://doi.org/10.1093/ejcts/ezv072
- Egbe AC, Connolly HM, Niaz T, Yogeswaran V, Taggart NW, Qureshi MY, et al. Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation. Am Heart J. 2017;183:10–7. https://doi.org/10.1016/j.ahj.2016.09.014
- d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Redefining expectations of long-term survival after the Fontan procedure: Twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014;130(11 Suppl 1):S32–8.
- Iyengar AJ, Winlaw DS, Galati JC, Wheaton GR, Gentles TL, Grigg LE, et al. No difference between aspirin and warfarin after extracardiac Fontan in a propensity score analysis of 475 patients. Eur J Cardiothorac Surg. 2016 Nov;50(5):980–7.
- Ohuchi H, Yasuda K, Miyazaki A, Ono S, Hayama Y, Negishi J, et al. Prevalence and predictors of haemostatic complications in 412 Fontan patients: their relation to anticoagulation and haemodynamics. Eur J Cardiothorac Surg. 2015 Mar;47(3):511–9. https://doi.org/10.1093/ejcts/ezu145
- Attard C, Huang J, Monagle P, Ignjatovic V. Pathophysiology of thrombosis and anticoagulation post Fontan surgery. Thromb Res. 2018 Dec;172:204–13. https://doi.org/10.1016/j.thromres.2018.04.011
- Sinclair AJ, Fox CK, Ichord RN, Almond CS, Bernard TJ, Beslow LA, et al. Stroke in Children With Cardiac Disease: Report From the International Pediatric Stroke Study Group Symposium. Pediatr Neurol. 2015 Jan;52(1):5–15. https://doi.org/10.1016/j.pediatrneurol.2014.09.016
- Mandalenakis Z, Rosengren A, Lappas G, Eriksson P, Hansson PO, Dellborg M. Ischemic Stroke in Children and Young Adults With Congenital Heart Disease. J Am Heart Assoc. 2016 Feb 23;5(2). pii: e003071. https://doi.org/10.1161/JAHA.115.003071
- Alsaied T, Alsidawi S, Allen CC, Faircloth J, Palumbo JS, Veldtman GR. Strategies for thromboprophylaxis in Fontan circulation: A meta-analysis. Heart. 2015;101:1731–7. https://doi.org/10.1136/heartjnl-2015-307930
- Feldt RH, Driscoll DJ, Offord KP, Cha RH, Perrault J, Schaff HV, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996;112:672–80. https://doi.org/10.1016/S0022-5223(96)70051-X
- McCrindle BW, Manlhiot C, Cochrane A, Roberts R, Hughes M, Szechtman B, et al. Factors associated with thrombotic complications after the Fontan procedure: A secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure. J Am Coll Cardiol. 2013;61:346–53. https://doi.org/10.1016/j.jacc.2012.08.1023
- Rychik J. The Relentless Effects of the Fontan Paradox. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2016;19(1):37–43. https://doi.org/10.1053/jpcsu.2015.11.006
- Cheung YF, Chay GW, Chiu CS, Cheng LC. Long-term anticoagulation therapy and thromboembolic complications after the Fontan procedure. Int J Cardiol. 2005;102:509–13.
- Coon PD, Rychik J, Novello RT, Ro PS, Gaynor JW, Spray TL. Thrombus formation after the Fontan operation. Ann Thorac Surg. 2001 Jun;71(6):1990–4.
- Lee SY, Baek JS, Kim GB, Kwon BS, Bae EJ, Noh CI, et al. Clinical Significance of Thrombosis in an Intracardiac Blind Pouch After a Fontan Operation. Pediatr Cardiol. 2012 Jan;33(1):42–8. https://doi.org/10.1007/s00246-011-0074-x
- Streif W, Andrew M, Marzinotto V, Massicotte P, Chan AK, Julian JA, et al. Analysis of warfarin therapy in pediatric patients: A prospective cohort study of 319 patients. Blood. 1999;94:3007–14. PubMed PMID: 10556183.
- Tait RC, Ladusans EJ, El-Metaal M, Patel RG, Will AM. Oral anticoagulation in paediatric patients: Dose requirements and complications. Arch Dis Child. 1996;74:228–31. https://doi.org/10.1136/adc.74.3.228