Outcomes of patients with single ventricle physiology undergoing cavopulmonary palliations depend on pulmonary vascular resistance (PVR) and have been suggested to be adversely affected by living at elevated altitude. We compared the pulmonary hemodynamic data in correlation with postoperative outcomes at the 3 centers of Denver, Edmonton, and Toronto at altitudes of 1604, 668, and 103 meters, respectively.
Hemodynamic data at pre-bidirectional cavopulmonary anastomosis (BCPA) and pre-Fontan catheterization between 1995 and 2007 were collected. Death from cardiac failure or heart transplantation in the same period was used to define palliation failure.
There was no significant correlation between altitude (ranged from 1 to 2572 meters) and PVR, pulmonary artery pressure (PAP) or transpulmonary gradient (TPG) at pre-BCPA and pre-Fontan catheterization. BCPA failure occurred in 11 (9.2%) patients in Denver, 3 (2.9%) in Edmonton, and 34 (11.9%) in Toronto. Fontan failure occurred in 3 (6.1%) patients in Denver, 5 (7.2%) in Edmonton, and 11 (7.0%) in Toronto. There was no significant difference in BCPA and Fontan failure among the 3 centers. BCPA failure positively correlated with PVR and the presence of a right ventricle as the systemic ventricle. Fontan failure positively correlated with PAP and TPG.
Moderate altitude is not associated with an increased PVR or adverse outcomes in patients with a functional single ventricle undergoing BCPA and the Fontan operation. The risk factors for palliation failure are higher PVR, PAP, and TPG and a systemic right ventricle, but not altitude. Our study reemphasizes the importance of cardiac catheterization assessments of pulmonary hemodynamics before BCPA and Fontan operations.
The bidirectional Glenn procedure (BDG) is a routine intermediary step in single-ventricle palliation. In this study, we examined risk factors for death or transplant and failure to reach Fontan completion after BDG in patients, who had previously undergone stage one palliation (S1P).
All patients at our institution, who underwent BDG following S1P between 2002 and 2009 (n = 194), were included in the analysis.
Transplant-free survival through 18 months post BDG was 91%. Univariable competing risk analyses showed atrioventricular valve regurgitation (AVVR) >mild, age ≤3 months at BDG, ventricular dysfunction >mild, and prolonged hospital stay after S1P to be associated with increased risk of death or orthotopic heart transplant. Multivariable competing risk analysis through 5 years of follow-up showed >mild AVVR (hazard ratio (HR) 7.5, 95% confidence interval (CI) 3.0–18.8), prolonged hospitalization after S1P (HR 4.5, 95% CI 1.8–11.5), and age ≤3 months at BDG (HR 6.8, 95% CI 2.3–20.0) to be independent risk factors for death or transplant. Concomitantly, >mild AVVR and age ≤3 months were independently associated with an overall decreased rate of Fontan completion.
Pre-BDG AVVR, age ≤3 months at time of BDG, and prolonged hospitalization after S1P are independently associated with decreased successful progression of staged palliation in midterm follow-up after BDG.
Congenital heart disease; CHD; Hypoplastic left heart syndrome; Outcomes; Pediatric
The success of a Fontan circulation depends on several factors including low pulmonary vascular resistance. Pulmonary vascular resistance rises in response to hypoxia. Hypoxia is associated with altitude. Therefore, we wondered whether altitude is a risk factor for early failure after the Fontan operation. The aim was to test this hypothesis.
Data were obtained from all published series of 'total cavopulmonary' Fontan operations since 1990. The early failure rate from each series and the altitude of the respective cities were recorded. Early failure was defined as death, takedown of Fontan, or transplantation during the same hospital admission. The association between altitude and failure rate was investigated by rank correlation and logistic regression.
24 series were identified from centres situated at altitudes ranging from sea level to 520 metres. The plot of failure rate versus altitude suggests that failure rate increases with altitude. Logistic regression did not fit the data adequately. This was possibly due to the influence of unmeasured and unknown factors affecting the results, as well as the fact that centres were not randomly chosen but were self-selected by virtue of publishing their results. However, Spearman's rank correlation was 0.74 (p = 0.001).
The early outcome of the Fontan circulation appears to be adversely affected by altitude.
We ranked the haemodynamics and pulmonary artery (PA) configurations of Norwood with bidirectional Glenn shunt (NW-G) patients among the other staged Fontan completion (conventional) patients in this study.
Between August 2001 and April 2010, 91 consecutive patients completed staged Fontan operations. Among them, NW-G was performed in 11.
There was no operative or late death except in one case which resulted from a reoperation after Fontan completion. Although the age at bidirectional Glenn (BDG) and Fontan completion was younger in NW-G (4.2 vs. 10.9 months, P < 0.01, and 2.3 vs. 3.3 years, P < 0.01), the interval between BDG and Fontan completion was not significantly different in these groups (23.6 vs. 28.0 months, P = 0.71). On Fontan completion, the extracardiac conduit size was significantly different (16.4 vs. 17.7 mm, P < 0.01). However, the pulmonary artery size (PA index) and the PA pressure were not significantly different (165.4 vs. 205.1 mm2/m2, P = 0.23, and 13.4 vs. 11.0 mmHg, P = 0.08). Particularly in hypoplastic left heart syndrome patients, the PA index was not significantly different between these groups (165.4 vs. 174.9 mm2/m2, P = 0.59).
The NW-G patients could have a subsequent Fontan completion with good results. There was no significant difference with respect to Fontan completion.
Norwood with bidirectional Glenn shunt; Staged Fontan operation; Haemodynamics; Pulmonary artery configuration
OBJECTIVE—To determine whether patients with cavopulmonary connection have higher levels of vasoactive/water-salt regulating hormones and if so, whether hormone levels are related to postoperative haemodynamics and postoperative follow up.
DESIGN—Cross sectional study.
PATIENTS—20 patients (New York Heart Association functional class I-II), mean age 11 years (range 4 to 22), were studied at a mean of 2 years (0.5 to 6) after a total cavopulmonary connection (TCPC, n = 12) or a bidirectional Glenn anastomosis (BDG, n = 8).
INTERVENTIONS—Cardiac catheterisation was performed and blood samples were drawn. Control blood samples were drawn from 33 healthy children, mean age 12 years (6 to 16).
MAIN OUTCOME MEASURES—Plasma levels of angiotensin II, renin, aldosterone, arginine, vasopressin, atrial natriuretic factor (ANF), brain natriuretic peptide (BNP).
RESULTS—All neurohormones were significantly increased in both TCPC and BDG patients (p < 0.05), with a fourfold increase in angiotensin II, renin, and aldosterone, and a twofold increase in vasopressin, ANF, and BNP (compared with healthy controls). There was no correlation between haemodynamic variables and hormone levels. Angiotensin II and renin were inversely correlated with time to follow up. All subjects over 15 years (n = 5) had normal neurohormonal levels.
CONCLUSIONS—Neurohormones were raised for years after successful cavopulmonary operations but lower levels were observed with time on follow up. This supports the hypothesis that neurohormonal activation is primarily related to altered postoperative physiology and that adaptation takes place over time.
Keywords: neurohormones; cavopulmonary connection; congenital heart disease; paediatric cardiology
A male infant with the rare lesion of unguarded tricuspid orifice in the setting of pulmonary valve atresia, intact ventricular septum, and a hypoplastic right ventricle is described. The patient presented with cyanosis at 1 day old; transcutaneous oxygen saturations were between 20% and 30% in room air, and 60% in 100% inspired oxygen. Pre-excitation was found incidentally on the ECG and the potential for rapid antegrade conduction of atrial tachyarrhythmias, after eventual extended palliation with the Fontan procedure, was demonstrated at electrophysiological study. By 11 months old the patient was becoming increasingly cyanosed and interim palliation with a bidirectional cavopulmonary shunt was proposed. Successful radiofrequency ablation of the accessory pathway was performed before bidirectional cavopulmonary shunt, which would have prevented access to the heart via the superior vena cava. Difficulty with femoral venous access because of previous occlusion of a femoral vein was overcome by the use of 2 F pacing electrodes and a 5 F ablation catheter.
Keywords: radiofrequency ablation; accessory pathway; unguarded tricuspid orifice
Ventricle‐pulmonary artery connections in patients after the Fontan procedure lead to ineffective volume loading and can cause long term problems. In patients with a cavopulmonary shunt anterograde pulmonary blood flow is frequently maintained, but can cause significant volume loading of the heart or complicate the subsequent Fontan procedure.
To evaluate the use of transcatheter closure of a ventricle‐pulmonary artery communication in the setting of a cavopulmonary shunt or after the Fontan procedure.
Patients and methods
Retrospective study at a tertiary referral centre. Eight patients (age 1.5–18 years, mean 7.8 years). Indications: cardiac failure or persistent pleural effusions after cavopulmonary shunt (n = 2) or after Fontan (n = 3) and abolishing the volume load of the single ventricle prior to Fontan completion (n = 3).
Devices used: Rashkind Umbrella (n = 1), Amplatzer PDA (n = 7) and Amplatzer ASD (n = 1). One patient required two devices. There were no procedural complications. All 3 patients with prolonged pleural effusions (1 post CP shunt and 2 post Fontan) showed complete resolution between 4 and 10 days after catheter closure. Two patients underwent transcatheter occlusion for progressive ventricular dilatation and cardiac failure. The first patient was post Fontan and showed gradual improvement in ventricular function. The second patient (post CP shunt) was in end stage cardiac failure due to severe AV valve regurgitation. The patient died 48 hours after an uncomplicated procedure due to ventricular failure and electromechanical dissociation (non‐procedure‐related cardiac death). Three patients underwent catheter closure to off‐load the systemic ventricle prior to the Fontan procedure. The device had to be removed prior to release in one patient, due to unsatisfactory position.
Transcatheter closure of ventricle‐pulmonary artery communication is a safe and effective technique in the treatment of selected patients after cavopulmonary shunt or Fontan procedure with early or late complications due to inappropriate pulmonary blood flow. This intervention should also be considered in the preparation for the Fontan procedure in selected patients with ventricular overload.
OBJECTIVE: In adults, race-based disparity in access to cardiovascular care has been documented. Racial differences in cardiac care for children have not been evaluated previously. METHODOLOGY: We analyzed timing of single-ventricle palliation as a function of race and geography at Duke University Medical Center (DUMC) from 1997-2000. RESULTS: African American children underwent bidirectional Glenn (BDG) at a median age of 11 months (13.8 +/- 10.8, n = 11); white children at five months (5.6 +/- 2.3, n = 29), p = 0.01. African American children underwent Fontan at 60 months (106.8 +/- 84.0, n = 9); white children at 36 months (45.6 +/- 36.0, n = 18), p = 0.005. CONCLUSIONS: African American children at DUMC underwent palliation at a later age and with more variability in age than did white children. Further investigation is needed to determine possible causes of these race-associated differences in health care delivery.
Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.
Keywords: persistent left superior vena cava; cavopulmonary connection; Fontan procedure; congenital heart disease
To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders.
Design and patients
Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18–45 years): atriopulmonary anastomosis (n = 23), atrioventricular connection (n = 5) and total cavopulmonary connection (n = 10).
Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live‐birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non‐specified secondary infertility (n = 2), uterus bicornis (n = 1) and related to endometriosis (n = 1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n = 15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population).
Women can successfully complete pregnancy after adequate Fontan palliation without important long‐term sequelae, although it is often complicated by clinically significant (non‐)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.
The clinical benefits of the Fontan operation in treating single-ventricle defects have been well documented. However, perioperative mortality or morbidity remains a critical problem. The purpose of the present study was to identify the cardiovascular factors that dominate the transient hemodynamic changes upon the change of a bidirectional cavopulmonary (Glenn) anastomosis (BCPA) into a total cavopulmonary connection (TCPC). For this purpose, two computational models were constructed to represent, respectively, a single-ventricle circulation with a BCPA and that with a TCPC. A series of model-based simulations were carried out to quantify the perioperative hemodynamic changes under various cardiovascular conditions. Obtained results indicated that the presence of a low pulmonary vascular resistance and/or a low lower-body vascular resistance is beneficial to the increase in transpulmonary flow upon the BCPA to TCPC change. Moreover, it was found that ventricular diastolic dysfunction and mitral valve regurgitation, despite being well-known risk factors for poor postoperative outcomes, do not cause a considerable perioperative reduction in transpulmonary flow. The findings may help physicians to assess the perioperative risk of the TCPC surgery based on preoperative measurement of cardiovascular function.
Pulmonary arteriovenous malformations (PAVMs) are a common source of morbidity after bidirectional superior cavopulmonary anastomosis (Glenn). The diversion of hepatic venous effluent away from the pulmonary circulation after Glenn appears to play a significant role in the pathogenesis of PAVMs. Although the liver is known to produce factors that regulate vascular development, specific hepatic inhibitors of angiogenesis have not been described in the post-Glenn population. Endostatin, produced from its precursor collagen XVIII, is a potent inhibitor of angiogenesis produced by the liver. This study aimed to investigate the hypothesis that endostatin levels decrease in patients after Glenn. Levels of endostatin and its precursor, long-type collagen XVIII, were determined by enzyme-linked immunoassay and immunoprecipitation, respectively, for serum samples from 38 patients undergoing Glenn, total cavopulmonary anastomosis (Fontan), or biventricular repair of cardiac defects. Samples were obtained before surgery and 24 h afterward. In patients undergoing a bidirectional Glenn procedure, endostatin levels decreased after surgery (n = 17; 4.42 vs 3.34 ng/ml; p < 0.001), and long type-collagen XVIII levels increased by 200 % (n = 10; p = 0.0001). However, endostatin levels did not change after surgery in patients undergoing Fontan (n = 13) or biventricular repair (n = 8). In patients undergoing Fontan, long-type collagen XVIII increased by 18 % (p < 0.01), whereas in control subjects, the levels were unchanged. These data suggest that the diversion of hepatic blood flow away from the pulmonary circulation in patients after the Glenn procedure inhibits endostatin production from collagen XVIII, resulting in decreased circulating serum endostatin levels. A decrease in endostatin may promote angiogenesis. The mechanism whereby the pulmonary circulation processes endostatin and its potential role in the pathogenesis of PAVMs warrant further study.
Angiogenesis inhibitor; Cardiac repair; Collagen XVIII; Congenital heart disease; Endostatin; Pulmonary arteriovenous malformations
An 8 year old child with tricuspid atresia had developed right sided pulmonary arteriovenous malformations following a previous classic Glenn procedure. These became clinically manifest immediately after Fontan conversion because of severe systemic desaturation. The pathophysiology and postoperative medical management of this case is described and related to current understanding of the aetiology of acquired pulmonary arteriovenous malformations following cavopulmonary shunt.
Keywords: classic Glenn shunt; bidirectional cavopulmonary anastomosis; pulmonary arteriovenous malformations; Fontan circulation
OBJECTIVE--To define the lowest age at which the bidirectional superior cavopulmonary anastomosis can safely be used in infants with complex congenital heart defects. DESIGN--A retrospective analysis of clinical, echocardiographic, haemodynamic, and angiographic data in four consecutive patients undergoing bidirectional superior cavopulmonary anastomosis below the age of 2 months. PATIENTS--Between November 1990 and September 1993, four infants less than 8 weeks of age (3, 4, 6, and 7 weeks) underwent bidirectional superior cavopulmonary anastomosis as a primary palliation for complex heart disease. The indication for early surgical intervention was progression of cyanosis (n = 3) and high pulmonary blood flow causing heart failure (n = 1). In two infants with tricuspid atresia, surgery was performed through a right thoracotomy without the use of cardiopulmonary bypass. In one infant with double inlet left ventricle and discordant ventriculoarterial connection, cavopulmonary anastomosis was combined with an arterial switch procedure. The final infant had double inlet left ventricle with pulmonary atresia; the central pulmonary arteries were virtually discontinuous and each branch was supplied by a separate arterial duct. The central pulmonary arteries were reconstructed using the subaortic innominate vein. Temporary prostacyclin infusion was used in three patients in the immediate postoperative period. RESULTS--Early postoperative extubation (5, 7, and 48 h) was successful in three patients. The youngest child required ligation of the ductus arteriosus four days later because of severe upper body oedema. The postoperative course was complicated by prolonged effusions in two patients. All were alive and well 14-48 months postoperatively, with satisfactory systemic saturations (80-87%) and haemodynamic indices. CONCLUSIONS--This limited experience challenges the widely held belief that the bidirectional superior cavopulmonary anastomosis cannot be used as a primary palliation for complex heart disease in early infancy. A wider experience is required to determine the safety and indications for this approach.
To determine flow distribution in the cavopulmonary connections of Fontan patients with and without bilateral superior vena cavae. No large series exists that establishes the flow distributions in Fontan patients, which would be an important resource for everyday clinical use and may impact future surgical reconstruction.
We studied 105 Fontan patients (ages 2 - 24 years) with through-plane phase contrast velocity mapping to determine flow rates in the inferior and superior vena cava, and left and right pulmonary arteries. Superior caval anastomosis type included 40 bidirectional Glenns (of which 15 were bilateral) and 53 hemi-Fontans, while Fontan type included 69 intra-atrial baffles, 28 extracardiac conduits, and 4 atriopulmonary connections.
Total caval flow was 2.9±1.0 l/min/m2, with an inferior vena cava contribution of 59%±15%. Total pulmonary flow was 2.5±0.8 l/min/m2, statistically less than caval flow and not explained by fenestration presence. The right pulmonary artery contribution (55%±13%) was, statistically greater than the left. In patients with bilateral superior cavae, the right cava accounted for 52%±14% of the flow, with no difference in pulmonary flow splits (50%±16% to the right). Age and body surface area correlated with percent inferior caval contribution (r = 0.60 and 0.74 respectively). Superior vena cava anastomosis and Fontan type did not significantly affect pulmonary flow splits.
Total Fontan cardiac index was 2.9 l/min/m2, with normal pulmonary flow splits (55% to right lung). Inferior vena cava contribution to total flow increases with body surface area and age, consistent with data from healthy children.
cardiac magnetic resonance imaging; Fontan procedure; total cavopulmonary connection; single ventricle physiology; blood flow; congenital heart disease; pediatric cardiology
Since the Fontan/Kreutzer procedure was introduced, evolutionary clinical advances via a staged surgical reconstructive approach have markedly improved outcomes for patients with functional single ventricle. However, significant challenges remain. Early stage mortality risk seems impenetrable. Serious morbidities - construed as immutable consequences of palliation - have hardly been addressed. Late functional status is increasingly linked to pathophysiologic consequences of prior staged procedures. As more single ventricle patients survive into adulthood, Fontan failure is emerging as an intractable problem for which there is no targeted therapy. Incremental solutions to address these ongoing problems have not had a measurable impact. Therefore, a fundamental reconsideration of the overall approach is reasonable and warranted. The ability to provide a modest pressure boost (2-6 mmHg) to existing blood flow at the total cavopulmonary connection can effectively restore more stable biventricular status. This would impact not only treatment of late Fontan failure, but also facilitate early surgical repair. A realistic means to provide such a pressure boost has never been apparent. Recent advances are beginning to unravel the unique challenges which must be addressed to realize this goal, with promise to open single ventricle palliation to new therapeutic vistas.
univentricular Fontan circulation; cavopulmonary assist; mechanical circulatory support
OBJECTIVE—To compare the relative merits of cavopulmonary or aortopulmonary shunts, or both, as definitive non-Fontan palliations for patients with single ventricle physiology.
DESIGN—Clinical data, ECG, echocardiographic data, surgical records, and available postmortem material were reviewed in all patients with single ventricle physiology identified from the University of Toronto Congenital Cardiac Centre for Adults (UTCCCA) database who had not undergone a Fontan operation. Current status of patients was assessed from clinic reviews and patient contact. Two groups of patients were identified: those with cavopulmonary shunt (group 1, n = 35); and those with aortopulmonary shunt(s) only (group 2, n = 15).
RESULTS—50 adults (21 male/29 female) who underwent the last palliation at a median age of 11 years (range 1 day to 53 years) were identified. During a mean (SD) follow up of 13.0 (6.2) years at the UTCCCA, 19 patients died. Survival is 89.4% and 51.9% at 10 and 20 years, respectively, from the time patients were first seen at UTCCCA, with no differences between the groups. Most recent New York Heart Association (NYHA) classification was I-II in 21 patients, III in 25, and IV in four patients; mean haemoglobin was 190 (28) g/l, and oxygen saturation was 82 (4)%, with no group differences. Arrhythmia developed in 25 patients (atrial flutter/fibrillation in 20 and/or sustained ventricular tachycardia in 11). Atrial flutter/fibrillation was more common in patients in group 2, who also showed a greater decline in ventricular function with time. Age at last palliation, cardiothoracic ratio, and inclusion in group 2 were predictive of atrial flutter/fibrillation, poor ventricular function predictive of ventricular tachycardia, NYHA class > III, and prior ventricular tachycardia predictive of death.
CONCLUSIONS—Cavopulmonary or aortopulmonary shunts, or both, provide sustained palliation for selected patients with single ventricle physiology. Survival for both compares favourably with published Fontan series. Compared to aortopulmonary shunts, cavopulmonary shunts convey a beneficial long term effect on ventricular function. Arrhythmia is a major cause of late morbidity in these patients, relating to both ventricular dysfunction and death. Onset of sustained ventricular tachycardia is an ominous sign.
Keywords: congenital heart defects; cavopulmonary shunt; cyanosis; Fontan procedure
Systemic to pulmonary collateral flow (SPCF) is common in single ventricle patients (pts) with superior cavopulmonary connections (BDG). Because no validated method to quantify SPCF exists, neither its hemodynamic burden nor clinical impact can be systematically evaluated. We hypothesize that (1) the difference in total ascending aortic (Ao) and caval flow (SVC+IVC) and (2) the difference between pulmonary vein and artery flow (PV − PA) provide two independent estimators of SPCF.
Methods and Results
We measured Ao, SVC, IVC, right (RPA) and left (LPA) PA, left (LPV), and right (RPV) PV flows in 17 BDG pts during routine cardiac magnetic resonance imaging studies using through-plane phase contrast velocity mapping. Two independent measures of SPCF were obtained: (1) Ao − (SVC + IVC). (2) (LPV−LPA) + (RPV−RPA). Values were normalized to body surface area (BSA), Ao, and PV and comparisons made using linear regression and Bland-Altman analysis. SPCF ranged from 0.2–1.4 L/min for (1) and 0.2–1.6 L/min for (2) for an average indexed SPCF of 0.5–2.8 L/min/m2 : 11–53% (mean 37%) of Ao and 19–77% (mean 54%) of PV. The mean difference between (1) and (2) was 0.01 L/min (p=0.40, 2 S.D. range −0.45–0.47 L/min).
We present a noninvasive method for the SPCF quantification in pts with BDG. It should provide an important clinical tool in managing these patients. Furthermore, we show that SPCF is a significant hemodynamic burden in many patients with BDG physiology. Future investigations will allow objective study of the impact of collateral flow on outcome.
single ventricle; collateral circulation; magnetic resonance imaging; blood flow; superior cavopulmonary connection
Extra-cardiac conduit (ECC) and Lateral atrial tunnel (LAT) total cavopulmonary connection are both widely used in management of functionally univentricular hearts. The impact of the type of connection on early outcomes after Fontan operation remains unclear. We evaluated the impact of Fontan type on early outcome in a large clinical database.
Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing the Fontan operation (2000–2009) were included. In multivariable analysis, we evaluated the impact of Fontan type (ECC vs. LAT) on in-hospital mortality, Fontan takedown/revision, Fontan failure (in-hospital mortality or Fontan takedown/revision), post-operative length of stay (LOS), and complications, adjusting for patient, procedural, and center factors.
A total of 2747 patients from 68 centers were included: 61% were male and 45% had a right dominant ventricle. ECC Fontan (vs. LAT) was performed in 63%; in all 65% were fenestrated. In multivariable analysis with adjustment for patient, procedural (including fenestration), and center factors (including Fontan volume), ECC Fontan was associated with significantly higher Fontan takedown/revision (OR 2.73, 95%CI 1.09–6.87) and Fontan failure (OR 2.28, 95%CI 1.13–4.59), and longer post-operative length of stay (adjusted estimated difference in LOS: +1.4 days).
These multicenter data suggest that of the two prevalent forms of Fontan connection in current use, the LAT Fontan may be associated with superior early outcomes.
Congenital Heart Disease; Outcomes; Fontan
Parallel advancements in surgical technique, preoperative and postoperative care, as well as a better understanding of physiology in patients with duct-dependent pulmonary or systemic circulation and a functional single ventricle, have led to superb results in staged palliation of most complex congenital heart disease (CHD) . The Fontan procedure and its technical modifications have resulted in markedly improved outcomes of patients with single ventricle anatomy [2,3,4]. The improved early survival has led to an exponential increase of the proportion of Fontan patients surviving long into adolescence and young adulthood . Improved early and late survival has not yet abolished late mortality secondary to myocardial failure, therefore increasing the referrals for cardiac transplantation . Interstage attrition  is moreover expected in staged palliation towards completion of a Fontan-type circulation, while Fontan failure represents a growing indication for heart transplantation . Heart transplantation has therefore become the potential “fourth stage”  or a possible alternative to a high-risk Fontan operation  in a strategy of staged palliation for single ventricle physiology. Heart transplant barely accounts for 16% of pediatric solid organ transplants . The thirteenth official pediatric heart transplantation report- 2010  indicates that pediatric recipients received only 12.5% of the total reported heart transplants worldwide. Congenital heart disease is not only the most common recipient diagnosis, but also the most powerful predictor of 1-year mortality after OHT. Results of orthotopic heart transplantations (OHT) for failing single ventricle physiology are mixed. Some authors advocate excellent early and mid-term survival after OHT for failing Fontan , while others suggest that rescue-OHT after failing Fontan seems unwarranted . Moreover, OHT outcome appears to be different according to the surgical staging towards the Fontan operation and surgical technique of Fontan completion .
The focus of this report is a complete review of the recent literature on OHT for failing single ventricles, outlining the clinical issues affecting Fontan failure, OHT listing and OHT outcome. These data are endorsed reporting our experience with OHT for failing single ventricle physiology in recent years.
Pediatric heart transplantation; fontan failure; univertricular physiology.
The impact of age and weight on outcomes following the Fontan operation is unclear. Previous analyses have suggested that lower weight-for-age z-score is an important predictor of poor outcome in patients undergoing bidirectional Glenn. We evaluated variation in age, weight, and weight-for-age z-score at Fontan across institutions, and the impact of these variables on post-operative morbidity and mortality.
Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing the Fontan operation (2000–2009) were included. Center variation in age, weight, and weight-for-age z-score were described. Multivariable analysis was performed to evaluate the impact of age, weight, and weight-for-age z-score on in-hospital mortality, Fontan failure (combined in-hospital mortality and Fontan takedown/revision), post-operative length of stay, and complications, adjusting for other patient and center factors.
A total of 2747 patients (68 centers) were included: 61% male; 45% right dominant lesions (38% left dominant, 17% undifferentiated). An extracardiac conduit Fontan (vs. lateral tunnel) was performed in 63%; 65% were fenestrated. Median age and weight at Fontan operation and proportion with weight-for-age z-score <−2 varied across centers ranging from 1.7–4.8 yrs, 10.5–16.1 kg, and 0%–30%, respectively. In multivariable analysis, age and weight were not significantly associated with outcome. Weight-for-age z-score <−2 was associated with increased in-hospital mortality (OR 2.73, 95%CI 1.09–6.86), Fontan failure (OR 2.59, 95%CI 1.24–5.40), and longer length of stay (+1.2 days, 95%CI 0.1–2.4).
Weight-for-age z-score <−2 is associated with significant morbidity and mortality following the Fontan operation independent of other patient and center characteristics.
Congenital Heart Disease; Outcomes
SETTING—Tertiary adult congenital cardiac referral centre.
DESIGN—Retrospective cross sectional analysis.
OBJECTIVES—To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation.
PATIENTS AND MAIN OUTCOME MEASURES—Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation.
RESULTS—61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up.
CONCLUSION—The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.
Keywords: Fontan operation; univentricular heart; cavopulmonary shunt; aortopulmonary shunt
A 34 months-old male patient with double inlet right ventricle with nonconfluent pulmonary arteries who underwent successful extracardiac fenestarated Fontan procedure using pre-designed T-shaped PTFE vascular graft after multi-step rehabilitation of the diminutive hilar pulmonary arteries. At first we performed 6 mm confluent pulmonary artery vascular graft implantation with 4 mm BT shunt at patient's 4 weeks old. At 9 months of patient, we upsized the confluent pulmonary arterial graft to 8 mm with bidirectional cavopulmonary connection, and, at 34 months, we performed extracardiac conduit Fontan procedure with pre-designed T-shape conduit including the confluent pulmonary arterial portion at last. Patient shows excellent functional status and development.
We quantify the geometric and hemodynamic characteristics of extracardiac and lateral tunnel Fontan surgical options and correlate certain anatomic characteristics with their hemodynamic efficiency and patient cardiac index.
Methods and Results
The study was conducted retrospectively on 22 patients undergoing Fontan operations (11 extracardiac and 11 lateral tunnel operations). Total cavopulmonary connection geometric parameters such as vessel areas, curvature, and offsets were quantified using a skeletonization method. Energy loss at the total cavopulmonary connection junction was available from previous in vitro experiments and computational fluid dynamic simulations for 5 and 9 patients, respectively. Cardiac index data were available for all patients. There was no significant difference in the mean and minimum cross-sectional vessel areas of the pulmonary artery between the extracardiac and lateral tunnel groups. The indexed energy dissipation within the total cavopulmonary connection was strongly correlated to minimum cross-sectional area of the pulmonary arteries (R2 value of 0.90 and P < .0002), whereas all other geometric features, including shape characteristics, had no significant correlation. Finally, cardiac index significantly correlated with the minimum pulmonary artery area (P = .006), suggesting that total cavopulmonary connection energy losses significantly affect resting cardiac output.
The minimum outlet size of the total cavopulmonary connection (ie, minimum cross section of pulmonary artery) governs the energy loss characteristics of the total cavopulmonary connection more strongly than variations in the shapes corresponding to extracardiac and lateral tunnel configurations. Differences in pulmonary artery sizes must be accounted for when comparing energy losses between extracardiac and lateral tunnel geometries.
Objectives: To assess the early results of conversion from atriopulmonary to total cavopulmonary connection in patients with failing Fontan operation.
Design: Early clinical and instrumental evaluation of patients undergoing conversion from atriopulmonary to total cavopulmonary connection from April 1999 to November 2000.
Setting: Tertiary referral centre for congenital heart disease.
Patients: 11 Fontan patients (mean (SD) age 20.9 (6.7) years) with refractory arrhythmias or ventricular dysfunction.
Interventions: Total cavopulmonary connection, intraoperative ablation, and AAIR pacemaker implantation.
Main outcome measures: Holter monitoring, transoesophageal atrial stimulation, ergometric test, and myocardial scintigraphy at a mean (SD) follow up of 16.8 (5.6) months.
Results: One early postoperative death occurred. During follow up three patients had relapse of atrial tachycardia, controlled by medical treatment, and two were pacemaker dependent. Transoesophageal stimulation did not induce atrial tachycardia in any patient. Ergometric test showed a diminished exercise tolerance in all but one patient. Mean minute ventilation and maximum oxygen consumption were 62% and 40% of their respective predicted values. Myocardial scintigraphy showed reversal of rest or exercise dysfunction in five patients and improved systemic ventricular function in seven. Mean basal ejection fraction increased from 39.4% (95% confidence interval (CI) 32% to 46%) to 46.5% (95% CI 41.7% to 51.2%) and ejection fraction on effort from 42.3% (95% CI 33.9% to 50.7%) to 50.2% (95% CI 44.5% to 55.9%).
Conclusions: Our data show that total cavopulmonary connection associated with intraoperative ablation and pacemaker implantation allows for better control of arrhythmias and improves ventricular function in the majority of patients with failing Fontan.
atriopulmonary connection; transoesophageal atrial stimulation; ergometric test; myocardial scintigraphy