Long-Term Follow-Up in Patients with Modified Fontan: Assessing Outcomes, Complications, and Management Strategies for Single-Ventricle Survivors
Main Article Content
Abstract
The modified Fontan procedure, a pivotal surgical intervention for patients with single-ventricle congenital heart defects, has drastically improved survival rates and quality of life for individuals who would otherwise face limited options. Despite these advancements, patients with modified Fontan physiology experience a unique set of hemodynamic challenges that require vigilant long-term follow-up. The Fontan circulation, characterized by passive venous return to the pulmonary arteries without a ventricular pump, predisposes patients to a variety of late complications, including Fontan-associated liver disease (FALD), arrhythmias, protein-losing enteropathy (PLE), thromboembolic events, and systemic venous hypertension. This review aims to evaluate the outcomes and long-term complications associated with modified Fontan patients, emphasizing the importance of early detection, multidisciplinary care, and individualized management strategies. The article explores current evidence on surveillance protocols, pharmacological treatments, and surgical interventions, as well as emerging therapeutic options like Fontan conversion, catheter-based therapies, and heart transplantation. This comprehensive analysis seeks to provide healthcare providers with a roadmap for optimizing care and improving the long-term prognosis of these patients.
Article Details
This work is licensed under a Creative Commons Attribution 4.0 International License.
References
I. Geva T. Tetralogy of Fallot repair: ready for a new paradigm. J Thorac Cardiovasc Surg.
;143:1305-6.
II. .Luijten LW, van den Bosch E, Duppen N, Tanke R, Roos-Hesselink J, Nijveld A, van Dijk A, Bogers AJ, van Domburg R and Helbing WA. Long-term outcomes of transatrial-transpulmonary repair of tetralogy of Fallot. Eur J Cardiothorac Surg. 2015;47:527-34.
III. Hickey EJ, Veldtman G, Bradley TJ, Gengsakul A, Manlhiot C, Williams WG, Webb GD and McCrindle BW. Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades. Eur J Cardiothorac Surg. 2009;35:156-64; discussion 164.
IV. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD and Redington AN. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356:975-81.
V. Frigiola A, Tsang V, Bull C, Coats L, Khambadkone S, Derrick G, Mist B, Walker F, van Doorn C, Bonhoeffer P and Taylor AM. Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation. 2008;118:S182-90.
VI. Abd El Rahman MY, Abdul-Khaliq H, Vogel M, Alexi-Meskishvili V, Gutberlet M and Lange PE. Relation between right ventricular enlargement, QRS duration, and right ventricular function in patients with tetralogy of Fallot and pulmonary regurgitation after surgical repair. Heart. 2000;84:416-20.
VII. Eindhoven JA, Menting ME, van den Bosch AE, Cuypers JA, Ruys TP, Witsenburg M, McGhie JS, Boersma E and Roos-Hesselink JW. Associations between N-terminal pro-B-type natriuretic peptide and cardiac function in adults with corrected tetralogy of Fallot. Int J Cardiol. 2014;174:550-6.
VIII. Westhoff-Bleck M, Kornau F, Haghikia A, Horke A, Bertram H, Treptau J, Widder J, Bauersachs J and Brehm MU. NT-proBNP Indicates Left Ventricular Impairment and Adverse Clinical Outcome in Patients With Tetralogy of Fallot and Pulmonary Regurgitation. Can J Cardiol. 2016;32:1247 e29-1247 e36.
IX. Muller J, Hager A, Diller GP, Derrick G, Buys R, Dubowy KO, Takken T, Orwat S, Inuzuka R, Vanhees L, Gatzoulis M and Giardini A. Peak oxygen uptake, ventilatory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol. 2015;196:158-64.
X. Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, Johansson B, Bouzas B, Mullen MJ, Poole-Wilson PA, Francis DP and Gatzoulis MA. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828-35.
XI. Orwat S, Diller GP, Kempny A, Radke R, Peters B, Kuhne T, Boethig D, Gutberlet M, Dubowy KO, Beerbaum P, Sarikouch S, Baumgartner H and German Competence Network for Congenital Heart Defects I. Myocardial deformation parameters predict outcome in patients with repaired tetralogy of Fallot. Heart. 2016;102:209-15.
XII. Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE, del Nido PJ and Geva T. Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart. 2008;94:211-6.
XIII. Diller GP, Kempny A, Liodakis E, Alonso-Gonzalez R, Inuzuka R, Uebing A, Orwat S, Dimopoulos K, Swan L, Li W, Gatzoulis MA and Baumgartner H. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of fallot. Circulation. 2012;125:2440-6.
XIV. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM and Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139:e698-e800.
XV. De Wolf D, Suys B, Verhaaren H, Matthys D and Taeymans Y. Low-dose dobutamine stress echocardiography in children and young adults. Am J Cardiol. 1998;81:895-901.