Conversion of modified fontan procedure to lateral atrial tunnel cavopulmonary anastomosis

the bidirectional cavopulmonary anastomosis is commonly used in the palliation of patients with a functionally univentricular physiology. the management of alternative sources of flow of blood to the lungs, as well as the magnitude of acceptable accessory blood flow at the time of surgery, nonetheless, remains controversial. these issues are particularly significant when a cavopulmonary anastomosis is performed in infants who may become candidates for a fontan procedure. indeed, a long-standing volume overload, which is invariably associated with the maintenance of accessory sources of pulmonary blood flow, may result in systemic ventricular dysfunction. these observations prompted us to review the influence of antegrade pulmonary blood flow in the management of infants undergoing a cavopulmonary anastomosis.

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The role of angiogenesis in the development of pulmonary arteriovenous malformations in children after cavopulmonary anastomosis

Cardiology in the Young ◽

10.1017/s1047951100004352 ◽

1997 ◽

Vol 7 (4) ◽

pp. 370-374 ◽

Cited By ~ 18

Author(s):

Blair Marshall ◽

Brian W. Duncan ◽

Richard A. Jonas

Keyword(s):

Arteriovenous Malformations ◽

Fontan Procedure ◽

Pulmonary Arteriovenous Malformations ◽

Arterial Circulation ◽

Clinical Observations ◽

Bidirectional Glenn Shunt ◽

Cavopulmonary Anastomosis ◽

Pulmonary Arterial ◽

End Stage

AbstractPulmonary arteriovenous malformations are a frequent cause of progressive cyanosis after construction of a cavopulmonary anastomosis. Their formation complicates the management of children with single ventricle physiology after a bidirectional Glenn shunt or the Kawashima procedure. The key role of the liver in this phenomenon is suggested by the observation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circulation limit further development of the malformations. In addition, it is known that patients with end-stage hepatic failure develop pulmonary arteriovenous malformations that diminish after liver transplantation. We have begun purification of a factor derived from hepatocyte-conditioned media that is inhibitory for the proliferation of cultured endothelial cells. This substance is heat sensitive, and binds avidly to a copper-containing chromatography column. These clinical observations, and this preliminary experimental work, support the concept that hepatic-derived angiogenic factors may play a role in the development of pulmonary arteriovenous malformations after construction of cavopulmonary anastomoses.

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Total cavopulmonary anastomosis versus conventional modified Fontan procedure

The Annals of Thoracic Surgery ◽

10.1016/0003-4975(91)91335-s ◽

1991 ◽

Vol 52 (2) ◽

pp. 189-196 ◽

Cited By ~ 66

Author(s):

Jeffrey M. Pearl ◽

Hillel Laks ◽

Darryl G. Stein ◽

Davis C. Drinkwater ◽

Barbara L. George ◽

...

Keyword(s):

Fontan Procedure ◽

Cavopulmonary Anastomosis ◽

Total Cavopulmonary Anastomosis

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Risk Factors For Unfavorable Outcomes After Bidirectional Cavopulmonary Anastomosis

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135117728505 ◽

2017 ◽

Vol 8 (5) ◽

pp. 575-583 ◽

Cited By ~ 2

Author(s):

Nataliya R. Nichay ◽

Yuriy N. Gorbatykh ◽

Igor A. Kornilov ◽

Ilya A. Soynov ◽

Yuriy Y. Kulyabin ◽

...

Keyword(s):

Risk Factors ◽

Proportional Hazards ◽

Cox Regression ◽

Fontan Procedure ◽

Unfavorable Outcome ◽

Atrioventricular Valve Regurgitation ◽

Cavopulmonary Anastomosis ◽

Mean Pressure ◽

Bidirectional Cavopulmonary Anastomosis ◽

Anomalous Pulmonary Venous Connection

Background: Bidirectional cavopulmonary anastomosis (BCPA) is an important preliminary step toward the Fontan procedure; thus, understanding of risk factors for morbidity and mortality after BCPA may ultimately promote improved rates of success with Fontan completion and general survival. This study evaluated survival and predictors of unfavorable outcomes in patients after BCPA. Methods: Clinical data of 157 patients who underwent BCPA from 2003 to 2015 at a single center were retrospectively analyzed. Results: Three-year and nine-year survival after BCPA were 87.1% ± 2.8% and 85.8% ± 2.9%, respectively. Freedom from unfavorable outcomes (mortality, BCPA takedown, nonsuitability for Fontan procedure) was 83.8% ± 3.1% at three years and 73.5% ± 4.8% at nine years. Multivariate proportional hazards regression analysis revealed that total anomalous pulmonary venous connection (TAPVC; hazard ratio [HR]: 3.74, 95% confidence interval [CI]: 1.35-10.36; P = .01) and increased mean pressure in BCPA circuit (HR: 1.17, 95% CI: 1.02-1.34; P = .03) were independent risk factors for unfavorable outcomes. Postoperative mean pressure in BCPA circuit in patients with poor outcomes was median 16 mm Hg (interquartile range [IQR]: 14-18 mm Hg) versus median 14 mm Hg (IQR: 12-15.5 mm Hg) in patients with favorable outcomes ( P < .01). Preoperative (HR: 1.87, 95% CI: 1.20-2.91; P < .01) and postoperative atrioventricular valve regurgitation (AVVR; HR: 2.22, 95% CI: 1.24-3.94; P < .01) were also associated with unfavorable outcome in univariate Cox regression. Conclusions: Elevated mean pressure in the BCPA circuit is the main predictor of unfavorable outcome; therefore, thorough preoperative examination and careful patient selection are critical points for successful intermediate-stage and later Fontan completion. Total anomalous pulmonary venous connection and insufficient correction of AVVR worsen the prognosis in this patient group.

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Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection

Cardiology in the Young ◽

10.1017/s1047951112001175 ◽

2012 ◽

Vol 23 (3) ◽

pp. 335-343 ◽

Cited By ~ 9

Author(s):

Andrew M. Atz ◽

Thomas G. Travison ◽

Brian W. McCrindle ◽

Lynn Mahony ◽

Andrew C. Glatz ◽

...

Keyword(s):

Fontan Procedure ◽

Cross Sectional Study ◽

Univentricular Heart ◽

P Value ◽

Cross Sectional ◽

Ventricular Performance ◽

Cavopulmonary Anastomosis ◽

Bidirectional Cavopulmonary Anastomosis ◽

Fontan Surgery ◽

Cavopulmonary Connection

AbstractBackgroundA superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.MethodsWe compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).ResultsA prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.ConclusionsAfter controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.

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the effect of the position of an additional systemic-to-pulmonary shunt on the fluid dynamics of the bidirectional cavo-pulmonary anastomosis

Cardiology in the Young ◽

10.1017/s1047951104006547 ◽

2004 ◽

Vol 14 (S3) ◽

pp. 38-43 ◽

Cited By ~ 4

Author(s):

francesca gervaso ◽

silvia kull ◽

giancarlo pennati ◽

francesco migliavacca ◽

gabriele dubini ◽

...

Keyword(s):

Pulmonary Stenosis ◽

Fontan Procedure ◽

Pulmonary Arteries ◽

Volume Overload ◽

Additional Source ◽

Arteriovenous Fistulas ◽

Palliative Procedure ◽

Cavopulmonary Anastomosis ◽

One Step ◽

Bidirectional Cavopulmonary Anastomosis

the bidirectional cavo-pulmonary anastomosis is a well-established palliative procedure for patients with a functionally univentricular circulation. it is usually considered one step in preparation for fontan procedure, but it may be performed as a long-term palliation for patients deemed to be at high-risk. in this subset of patients, a valuable surgical option could be to add, or maintain, an additional source of flow of blood to the lungs, either derived from a patent but banded trunk or one protected by native pulmonary stenosis, or a systemic-to-pulmonary arterial shunt. the risk and benefits of providing an additional source of pulmonary flow after construction of a bidirectional cavopulmonary anastomosis are strongly debated. in terms of benefit, the arterial saturation of oxygen is increased due to the greater ratio of pulmonary-to-systemic flow, arteriovenous fistulas are prevented and, as a consequence of the arterial pulsatile flow, the pulmonary arteries are stimulated to grow. the most significant drawbacks are volume overload of the functionally single ventricle, and higher pressures compared to an isolated bidirectional cavopulmonary anastomosis.

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Functional state following the Fontan procedure

Cardiology in the Young ◽

10.1017/s1047951109990382 ◽

2009 ◽

Vol 19 (4) ◽

pp. 320-330 ◽

Cited By ~ 22

Author(s):

Ismee A. Williams ◽

Lynn A. Sleeper ◽

Steven D. Colan ◽

Minmin Lu ◽

Elizabeth A. Stephenson ◽

...

Keyword(s):

Right Ventricular ◽

Functional State ◽

Diastolic Pressure ◽

Fontan Procedure ◽

Fontan Circulation ◽

Functional Score ◽

Lower Income ◽

Cross Sectional ◽

Ventricular Ejection Fraction ◽

Cavopulmonary Anastomosis

AbstractBackgroundDespite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function.Methods and ResultsWe analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset.ConclusionsRight ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes.

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The clinical impact of percutaneous balloon expandable endovascular stents in the management of early postoperative vascular obstruction

Cardiology in the Young ◽

10.1017/s1047951100003243 ◽

1996 ◽

Vol 6 (1) ◽

pp. 48-53 ◽

Cited By ~ 7

Author(s):

Yoshiho Hatai ◽

David G. Nykanen ◽

William G. Williams ◽

Robert M. Freedom ◽

Lee N. Benson

Keyword(s):

Postoperative Period ◽

Fontan Procedure ◽

Symptomatic Improvement ◽

Clinical Impact ◽

Arterial Stenosis ◽

Low Cardiac Output Syndrome ◽

Endovascular Stents ◽

Percutaneous Balloon ◽

Cavopulmonary Anastomosis ◽

Bidirectional Cavopulmonary Anastomosis

AbstractTo assess the clinical impact of percutaneously implanted balloon expandable endovascular stents on patient management, we reviewed 20 children aged 14 days to 4.8 years (median 1.3 years) with residual vascular obstructions in the immediate postoperative period. Patients included 11 with pulmonary arterial stenosis, five with stenosis of venous pathways after a modified Fontan procedure or bidirectional cavopulmonary anastomosis, and four with a restrictive modified Blalock-Taussig shunt. Placement of the stent was optimal in 18 of 20 patients (24 of 26 Palmaz or Palmaz-Schatz implants). In two patients, rupture of the balloon resulted in malposition of the stent. Reoperation was avoided and symptomatic improvement was noted in 11 of 20 patients (55%), while procedural complications occurred in eight patients. Seven of 10 patients presenting with a low cardiac output syndrome died despite relief of the obstructions. This early experience supports consideration of the application of these devices in the management of significant vascular obstructive lesions in the immediate postoperative period, thus avoiding early reoperation in this profoundly compromised population.

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Lung Perfusion Patterns After Bidirectional Cavopulmonary Anastomosis (Hemi-Fontan Procedure)

Pediatric Cardiology ◽

10.1007/s002469900146 ◽

1997 ◽

Vol 18 (3) ◽

pp. 191-196 ◽

Cited By ~ 14

Author(s):

M.A. Seliem ◽

J. Murphy ◽

J. Vetter ◽

S. Heyman ◽

W. Norwood

Keyword(s):

Fontan Procedure ◽

Lung Perfusion ◽

Cavopulmonary Anastomosis ◽

Bidirectional Cavopulmonary Anastomosis

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Similarities and differences in the exercise performance of patients after a modified Fontan procedure compared to patients with complete transposition following a Senning operation

Cardiology in the Young ◽

10.1017/s1047951100009112 ◽

2000 ◽

Vol 10 (3) ◽

pp. 201-207 ◽

Cited By ~ 4

Author(s):

Gernot Buheitel ◽

Michael Hofbeck ◽

Stephan Gerling ◽

Andreas Koch ◽

Helmut Singer

Keyword(s):

Carbon Dioxide ◽

Minute Ventilation ◽

Fontan Procedure ◽

Fontan Operation ◽

Work Rate ◽

Control Group ◽

Control Subjects ◽

Complete Transposition ◽

Cavopulmonary Anastomosis ◽

Senning Operation

AbstractObjectiveTo investigate cardiopulmonary performance in patients after a Fontan procedure, comparing it to patients following a Senning operation.MethodsWe studied 21 children, with a mean age of 11.1 years, after a total cavopulmonary anastomosis, comparing them to 13 with complete transposition after a Senning procedure, having a mean age of 11.8 years, and 21 control subjects with a mean age of 11.2 years. All were tested on a bicycle ergospirometer.ResultsPeak consumption of oxygen, maximal work rate, peak oxygen pulse and endexpiratory pressure of carbon dioxide at a work rate of 1.5 Watt/kg were lowest in patients with a modified Fontan procedure, and highest in the control group (p≤ 0.0278). Production of carbon dioxide, and minute ventilation at a work rate of 1.5 Watt/kg, was highest in the patients after Fontan procedure, and lowest in the healthy subjects (p≤ 0.0163). Production of carbon dioxide per single breath was lower in those having a Fontan procedure (28.9 ml) than in the two other groups (35.1 ml; p = 0.0243). The tidal volume showed no significant differences between the three groups.ConclusionsThe reaction to exercise was identical qualitatively in both groups of patients, and comparable to the behaviour of patients with chronic heart failure. Quantitatively, the results of the patients following a Senning procedure lay between those of control subjects and those who had undergone a Fontan operation. The only exception was dead space ventilation, where the patients after a Fontan procedure differed from the two other groups because of their increased ventilation-perfusion mismatch.

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