The Fontan Procedure: What Have We Learned and Accomplished?

Published on May 1, 1998in Acc Current Journal Review
· DOI :10.1016/S1062-1458(98)00030-0
Douglas D. Mair53
Estimated H-index: 53
(Mayo Clinic)
#1Mary M. Canobbio (UCLA: University of California, Los Angeles)H-Index: 13
#2Douglas D. Mair (Mayo Clinic)H-Index: 53
Last. Brian J. Koos (UCLA: University of California, Los Angeles)H-Index: 19
view all 4 authors...
Abstract Objectives . This study sought to determine risks and outcome of pregnancy and delivery after the modified Fontan operation. Background . Increasingly, female Fontan patients reaching child-bearing years are interested in having children. To date, the number of reported pregnancies is small, and pregnancy has therefore been discouraged. Methods . One hundred ten of 126 female patients from the Fontan registries of the Mayo Clinic and University of California Los Angeles Medical Center r...
188 CitationsSource
#1Robert H. Feldt (Mayo Clinic)H-Index: 31
#2David J. Driscoll (Mayo Clinic)H-Index: 71
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 10
view all 8 authors...
Abstract Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hem...
273 CitationsSource
#1Frank Cetta (Mayo Clinic)H-Index: 46
#2Robert H. Feldt (Mayo Clinic)H-Index: 31
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 104
view all 12 authors...
Objectives. This study sought to evaluate changes in early morbidity and mortality as well as predictors of outcome in our most recent 339 patients undergoing modified Fontan operations. Background. The Fontan operation is the preferred definitive palliation for patients with functional single ventricles. Previously reported early mortality rates after Fontan operation have been substantial. Methods. Records of 339 consecutive patients who had a Fontan operation at the Mayo Clinic between 1987 a...
171 CitationsSource
#1Luc Mertens (Katholieke Universiteit Leuven)H-Index: 68
#2Monique DumoulinH-Index: 20
Last. Marc GewilligH-Index: 76
view all 3 authors...
59 CitationsSource
: The modified Fontan procedure for univentricular heart disease results in the full spectrum of significant cardiac arrhythmias: tachycardias and bradycardias. The tachycardias are primarily supraventricular in origin: 1. atrial flutter, 2. primary atrial tachycardia, 3. atrial fibrillation, and 4. accelerated junctional rhythm or junctional tachycardia; however, ventricular tachyarrhythmias occur also, but less frequently. Bradycardia usually is a result of 1. sinoatrial node dysfunction resul...
31 Citations
#1Nancy D. Bridges (Boston Children's Hospital)H-Index: 43
#2James E. Lock (Boston Children's Hospital)H-Index: 126
Last. Aldo R. Castaneda (Boston Children's Hospital)H-Index: 84
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Ventricular dysfunction, elevated pulmonary vascular resistance, and residual distal pulmonary artery distortion contribute to early mortality after a Fontan operation; they may be transient or reversible. A baffle fenestration, allowing right-to-left shunting, maintains cardiac output and limits right atrial pressure. A baffle fenestration was surgically created at the time of a modified Fontan repair in 20 consecutive patients. Risk factors included pulmonary artery pressure of 18 mm Hg or mor...
355 CitationsSource
#1Guy L. ReedH-Index: 15
#2Gary R. Matsueda (Harvard University)H-Index: 32
Last. Edgar Haber (Princeton University)H-Index: 100
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Recent experiments in vitro have shown that inhibition of human alpha 2-antiplasmin by a monoclonal antibody (MAb RWR) markedly enhances clot lysis by plasminogen activators. To extend these studies in vivo, we tested whether inhibition of clot or fibrin-bound alpha 2-antiplasmin by MAb RWR could enhance the lysis of a human clot by tissue-type plasminogen activator (t-PA) in a rabbit jugular vein thrombosis model. Compared with a saline placebo or a control antibody, MAb RWR significantly incre...
61 CitationsSource
#1Douglas D. Mair (Mayo Clinic)H-Index: 53
#2Donald J. Hagler (Mayo Clinic)H-Index: 88
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 104
view all 6 authors...
Between May 1974 and March 1989, 155 patients with double-inlet left ventricle had the Fontan procedure performed at the Mayo Clinic. Age at operation ranged from nearly 2 to 41 years (median 10). The operative mortality rate from 1974 through 1980 (39 patients) was 21%, but from 1981 through 1989 (116 patients) it was reduced to 9%. The 17 late deaths were secondary to reoperation (n = 8), progressive myocardial failure (n = 5), sudden arrhythmia (n = 3) and bleeding varices (n = 1). Neither op...
57 CitationsSource
#1M. de LevalH-Index: 16
#1M R de LevalH-Index: 53
Last. C Bull (UR: University of Rochester)H-Index: 1
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To understand better the contribution of a right atrium in a valveless atriopulmonary connection, we performed some basic hydrodynamic studies. Pulsation of a valveless chamber in a simple continuous flow circuit was found to generate turbulence and thereby to increase resistance to net forward flow. Visualization of flow through cavities and around corners and measurements of energy losses across nonpulsatile cavities, corners, and stenoses indicated the importance of streamlining. These studie...
846 Citations
#1Puga FjH-Index: 1
#1Francisco J. PugaH-Index: 73
Last. Donald J. HaglerH-Index: 88
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Two modifications of the Fontan operation are proposed for prevention of pulmonary venous obstruction in patients with left atrioventricular valve atresia or single atrioventricular valve. In the first technique, a bipedicled flap of right atrial wall is used to separate systemic and pulmonary venous drainages; an atriopulmonary connection is constructed by use of the right atrial appendage, and the defect in the right atrial wall is repaired with a pericardial patch. In the second technique, th...
129 Citations
Cited By5
#1Andrew L. Cheng (SC: University of Southern California)H-Index: 5
#2Niema M. Pahlevan (SC: University of Southern California)H-Index: 11
Last. Morteza Gharib (California Institute of Technology)H-Index: 74
view all 5 authors...
The Fontan procedure for univentricular heart defects creates a unique circulation where all pulmonary blood flow is passively supplied directly from systemic veins. Computational simulations, aimed at optimizing the surgery, have assumed blood to be a Newtonian fluid without evaluating the potential error introduced by this assumption. We compared flow behavior between a non-Newtonian blood analog (0.04% xanthan gum) and a control Newtonian fluid (45% glycerol) in a simplified model of the Font...
5 CitationsSource
#1Coleen A. Vernick (Thomas Jefferson University Hospital)H-Index: 1
#2Jiri Horak (HUP: Hospital of the University of Pennsylvania)H-Index: 7
Last. William J. Vernick (HUP: Hospital of the University of Pennsylvania)H-Index: 13
view all 4 authors...
Abstract The management of a patient who had previously undergone surgical palliation of tricuspid atresia at the age of two, and who required right parietal craniotomy for resection of seizure focus, is presented. The anesthetic considerations in patients with Fontan physiology are also presented, as well as the impact on patients undergoing neurosurgical procedures, specifically, craniotomy for seizure focus resection. The physiologic demands of modern surgical practice requires that there is ...
1 CitationsSource
#1Tain-Yen Hsia (Johns Hopkins University)H-Index: 29
#2Francesco Migliavacca (Polytechnic University of Milan)H-Index: 60
Last. Marc R. de Leval (GOSH: Great Ormond Street Hospital)H-Index: 52
view all 7 authors...
Objectives and background In the Fontan circulation, pulmonary and systemic vascular resistances are in series. The influence of various inferior vena cava to pulmonary artery connections in this unique circulatory arrangement was evaluated using computation fluid dynamics methods. Methods Realistic three-dimensional models of total cavopulmonary connections were created from angiographic measurements to include the hepatic vein, superior vena cava, and branches of the pulmonary arteries. Steady...
89 CitationsSource
#1Matthew S. Lemler (UTSW: University of Texas Southwestern Medical Center)H-Index: 14
#2William A. Scott (UTSW: University of Texas Southwestern Medical Center)H-Index: 23
Last. Claudio Ramaciotti (UTSW: University of Texas Southwestern Medical Center)H-Index: 22
view all 5 authors...
Background— The Fontan procedure is the definitive operation for palliation of complex congenital heart disease with single-ventricle physiology. Fenestration of the Fontan circuit allows for shunting of deoxygenated blood to the systemic circulation. This procedure improved the clinical outcomes of patients who are at high risk for poor Fontan results. However, it is controversial whether fenestration is beneficial for standard-risk patients. Methods and Results— This prospective, randomized tr...
297 CitationsSource
#1Tain-Yen Hsia (GOSH: Great Ormond Street Hospital)H-Index: 29
#2Sachin KhambadkoneH-Index: 33
Last. Marc R. de LevalH-Index: 52
view all 6 authors...
Background —In the Fontan circulation, pulmonary and systemic vascular resistances are in series. The implications of this unique arrangement on infradiaphragmatic venous physiology are poorly understood. Methods and Results —We studied the effects of respiration and gravity on infradiaphragmatic venous flows in 20 normal healthy volunteers (control) and 48 Fontan patients (atriopulmonary connection [APC] n=15, total cavopulmonary connection [TCPC] n=30). Hepatic venous (HV), subhepatic inferior...
147 CitationsSource