Surgical Alternatives Beyond Fontan: Revisiting the Role of Carotid - Jugular Shunt in Single Ventricle Patients
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Abstract
Single ventricle physiology refers to a group of congenital heart diseases where only one functional ventricle supports both systemic and pulmonary circulation. These patients often experience chronic cyanosis and are at risk for long-term complications due to abnormal circulation. The current standard of treatment is staged surgical palliation, which includes multiple steps leading to the Fontan procedure, a surgery that allows systemic venous blood to flow directly to the lungs without passing through the heart. However, some patients are not eligible for Fontan due to unfavorable hemodynamics. In these cases, standard treatment pathways may not be applicable, and alternative approaches must be considered. One such alternative is the carotid-to-jugular vein shunt (CCA–IJV shunt), which aims to lower venous pressure, improve pulmonary blood flow, and reduce the heart’s workload. While evidence is still limited, this technique has been used in selected patients with promising outcomes. This article reviews the physiological rationale, surgical technique, and clinical results of CCA–IJV shunt, based on both international studies and experiences in Thailand, to explore its role as a palliative option for single ventricle patients who cannot undergo Fontan completion.
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References
Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890-900.
Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.
Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102(14):1081-6.
d'Udekem Y, Iyengar AJ, Galati JC, 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.
Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117(1):85-92.
Fenton KN, Siewers RD, Rebovich B, Pigula FA. Interim mortality in infants with systemic-to-pulmonary artery shunts. Ann Thorac Surg. 2003;76(1):152-6.
Talwar S, Marathe SP, Choudhary SK, Airan B. Common carotid artery to internal jugular vein shunt for managing hypoxemia after a cavopulmonary shunt. Ann Thorac Surg. 2012;94(3):e67-9.
Nakata T, Fujimoto Y, Hirose K, et al. Hemodynamic impact of fenestration in total cavopulmonary connection: computer simulation and clinical correlation. Interact Cardiovasc Thorac Surg. 2015;20(6):775-80.
Kaulitz R, Ziemer G, Luhmer I, Kallfelz HC. Modified Fontan operation in functionally univentricular hearts: preoperative risk factors and intermediate results. J Thorac Cardiovasc Surg. 1996;112(3):658-64.
Anderson RH, Baker EJ, Penny D, Redington AN, Rigby ML, Wernovsky G. Paediatric Cardiology. 3rd ed. Philadelphia: Churchill Livingstone Elsevier; 2010.
Mertens L, Friedberg MK. The gold standard for Fontan failure: death or transplantation? Heart. 2013;99(23):1706-7.
Jacobs ML, Mayer JE Jr. Congenital Heart Surgery Nomenclature and Database Project: single ventricle. Ann ThoracSurg. 2000;69(4 Suppl):S197-204.
Bureau of Registration Administration, Department of Provincial Administration, Ministry of Interior, Thailand. Vital Statistics 2023. Bangkok; 2024.
Ohye RG, Sleeper LA, Mahony L, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010;362(21):1980-92.
Rogers LS, Glatz AC, Ravishankar C, et al. 18 years of the Fontan operation at a single institution: results from 771 consecutive patients. J Am Coll Cardiol. 2012;60(11):1018-25.
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26(3):240-8.
Kreutzer G, Galindez E, Bono H, De Palma C, Laura JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1973;66(4):613-21.
Sthanakiya S, et al. Mid-term Outcomes of Carotid-Jugular Shunt in Single Ventricle Physiology: A Cohort Analysis. Asian Cardiovascular and Thoracic Annals. 2022;30(4):412-419.
Talwar S, et al. Common Carotid Artery to Internal Jugular Vein Shunt: A Palliative Option for Fontan-Ineligible Patients. Journal of Cardiac Surgery. 2021;36(8):2845-2852.
Kothari SS. Palliative Procedures for Single Ventricle: Beyond the Fontan Circuit. Annals of Pediatric Cardiology. 2020;13(3):214-221.
