Current management of late failure after classic Fontan modifications: Fontan conversion

BACKGROUND: Re-establishment of Fontan circulation by eliminating the drawbacks of classic Fontan modifications has been proposed recently to improve the functional class and quality of life of patients with failed Fontan circulation. METHODS: Five patients underwent extracardiac and lateral tunnel Fontan conversion due to failure of the Fontan circulation, after classic Fontan modifications. All of the patients were female and mean age was 10.2 +/- 2.2 years (range: 8-14 years). Previous Fontan modifications were atrio-pulmonary anastomosis in 3 and Kawashima operation in 2 patients. Time to re-operation for Fontan conversion was 6 +/- 1.5 years (range: 4-8 years). Indications for re-operation were right heart failure, right atrial dilatation, and intractable atrial arrhythmias in patients with previous atrio-pulmonary anastomosis, progressive hypoxia and exercise intolerance in patients with previous Kawashima operation due to pulmonary arteriovenous fistulas. Previous atrio-pulmonary anastomoses were converted to intra-atrial or lateral tunnel Fontan operation with modified right atrial Maze procedure. Extracardiac Fontan completion was carried out after previous Kawashima operations by redirection of hepatic veins to the lungs. RESULTS: There was no mortality and no major morbidity. All patients were discharged from the hospital in good condition and in sinus rhythm. No prolonged or recurrent effusions were observed. On follow-up, all patients were in sinus rhythm and had NYHA class I functional capacity. In two Kawashima patients, SpO(2) gradually increased from 60 % to 90 % six months after the operation. DISCUSSION: We suggest that Fontan conversion should be considered in patients with previous atrio-pulmonary anastomosis, when right atrial dilatation or intractable atrial arrhythmias with deteriorating functional status develops. Redirection of hepatic venous flow to lungs induces regression of pulmonary arteriovenous fistulas and improves arterial saturation in patients with previous Kawashima operation.     

Failed transcatheter creation of fenestration in a Fontan patient with original fenestration closed by AngelWings septal occluder: a case report

The fenestrated Fontan procedure has helped to reduce the mortality and morbidity of pediatric patients who underwent the Fontan operation. We treated a patient (7-year-old, female) who had developed heart failure 1 year after fenestration closure using an AngelWings device, although the patient met the proposed criteria for test occlusion. Hemodynamic evaluation using the ventricular pressure-area relationship revealed that the patient had both systolic and diastolic dysfunction, which were difficult to recognize by conventional hemodynamic indices. We then tried to percutaneously create a fenestration using a Brockenbrough needle, but this procedure failed. The AngelWings device, which may have been covered by pseudointima (composed of fibroelastic tissue), was hard enough to break the Brockenbrough needle. Re-creating a fenestration after device closure in patients after the fenestrated Fontan operation appears to be difficult, so more accurate methods to predict Fontan physiology after fenestration closure are needed.     

改良房坦手术后右心房内球囊搏动辅助循环的实验研究

目的：为了提高改良房坦（Ｆｏｎｔａｎ）手术的早期生存率，我们研制了一种新型右心球囊辅助装置，通过机械辅助的方法帮助有房坦手术危险因素的患者。方法：该装置由标准的主动脉内球囊反搏机和一套并连的单球囊管和双球囊管组成，通过调节主动脉内球囊反搏机的充气时相，来驱动经外周静脉送入右心房和腔静脉内的球囊使之产生类似右心室收缩的效果，从而增加肺血流量。本实验比较了应用该装置在搏动频率１：１、１：２和停搏３种状态下对６条犬急性改良房坦模型的血液动力学的影响。各项指标由八导生理记录仪做同步记录，所有数据用配对ｔ检验做统计学分析。结果：研究发现，肺动脉血流量增加十分显著，当搏动频率为１：１和１：２时，分别比停搏时增加了８２％和６４％（Ｐ＜０．０５和Ｐ＜０．０１）。结论：可以认为新型辅助装置能有效地改善改良房坦手术后早期右心功能，这将为帮助有房坦手术危险因素的患者安全渡过围术期提供一种新的途径。     

Modified Fontan operation for complex cardiac anomalies--postoperative hemodynamics, cardiac function and clinical status

The purpose of this paper is to compare and define the postoperative hemodynamics, cardiac function and clinical status after the modified Fontan operation in patients with complex cardiac anomalies. Thirteen consecutive patients (6 with double-outlet right ventricle [DORV] [SLL : 4, SDL : 2], 5 with single ventricle [SV] [A-III : 3, B-III : 1, C-III : 1] and 2 with tricuspid atresia [TA] [Ib : 1, IIb : 1]) underwent the modified Fontan operation. These 13 patients ranging in age from 7 to 42 years of age (mean 18) were catheterized at 7 to 46 months (mean 8 months) postoperatively. Pressure tracings at rest demonstrated a dominant "a" wave in both the right atrium and the pulmonary artery suggesting a marked right atrial contraction after this operation. Left ventricular filling pressure was significantly decreased after the modified Fontan operation in DORV and SV. The ejection fraction and left ventricular end-diastolic volume index were unchanged or slightly increased after the modified Fontan operation. Cardiac index in 6 patients with DORV was significantly increased from rest to exercise by +48% (p less than 0.05) with a significant increase in the stroke volume (p less than 0.05) and with a slight increase in heart rate, but this index in 5 patients with SV and in 2 patients with TA was not significantly increased from rest to exercise. In the postoperative clinical status, 11 of 13 patients were in NYHA class I and 2 in class II at follow-up periods ranging from 4 to 75 months (mean 48 months). These results suggest that the modified Fontan operation can be of value and can provide excellent exercise tolerance for patients with complex cardiac lesions.     

Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation

OBJECTIVES: The purpose of this study was to compare the clinical and echocardiographic features of adults who developed atrial tachyarrhythmias (ATs) late after a Fontan procedure with those who have remained free of arrhythmias. BACKGROUND: Atrial tachyarrhythmias are a frequent complication of the Fontan operation. However, the outcomes in adult patients with AT who have had the Fontan operation have not been well defined. METHODS: We reviewed the outcomes of 94 consecutive patients who underwent the Fontan operation between 1977 and 1994 and were followed as adults at the University of Toronto Congenital Cardiac Centre for Adults. Sixty patients had an atriopulmonary connection, 21 patients had an atrioventricular connection, and 13 patients received a lateral tunnel connection. RESULTS: Thirty-nine patients (41%) had sustained AT (atrial fibrillation, atrial flutter or supraventricular tachycardia) after their Fontan procedure. Compared with patients who did not develop AT, those who did were more likely to develop heart failure (46% vs. 13%, p = 0.003) and right atrial thrombus (31% vs. 4%, p = 0.006), exhibit left atrial enlargement (mean [+/-SD] diameter: 44 +/- 10 vs. 37 +/- 9 mm, p = 0.002), exhibit right atrial enlargement (mean [+/-SD] volume: 139 +/- 149 vs. 76 +/- 54 ml, p = 0.040) and have moderate-to-severe systemic valve regurgitation (31% vs. 7%, p = 0.010). The mean survival time was not significantly different between the arrhythmia group and the arrhythmia-free group (21.2 +/- 1.3 and 18.0 +/- 0.7 years, respectively; p = 0.900). CONCLUSIONS: Systemic atrioventricular valvular regurgitation and biatrial enlargement are commonly observed in patients who develop AT after the Fontan procedure. These patients are more likely to develop right atrial thrombus and heart failure.     

Long-term transvenous AV-sequential pacing in a failing atriopulmonary Fontan patient

We report on a 32-year-old man with tricuspid atresia, pulmonary stenosis and hypoplastic right ventricle. He had received a Fontan-Kreutzer procedure (anastomosis between the right atrial appendage and the pulmonary artery) at the age of 14 years. At the age of 2 years, an abdominal VVI-pacemaker with an epicardial ventricular lead had been implanted because of symptomatic third degree AV-block. The patient was now hospitalized with symptoms of severe congestive heart failure. A least invasive approach restoration of AV-synchrony by a dual chamber pacer was performed. Therefore a complete transvenous approach to avoid thoracotomy was attempted. A specially designed CS lead was advanced via the CS to a left lateral ventricular vein for ventricular stimulation. After institution of dual chamber pacing the patient recovered of his heart failure. During a follow-up time of 20 months the patient was clinically stable in the AV-sequential pacing. Conclusion: Dual chamber pacing using a transvenously placed coronary sinus lead for ventricular stimulation may improve congestive heart failure in patients after the classic Fontan operation. The minimally invasive transvenous approach might be the best solution for patients who need a pacemaker and are not candidates for surgery or heart transplantation.     

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

OBJECTIVE: To investigate cardiopulmonary performance in patients after a Fontan procedure, comparing it to patients following a Senning operation. METHODS: We 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. RESULTS: Peak 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 < or = 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 < or = 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. CONCLUSIONS: The 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.     

Transesophageal echocardiography detects thrombus formation not identified by transthoracic echocardiography after the Fontan operation

Transesophageal echocardiography demonstrated six instances of venous thrombus formation in the inferior vena cava, right atrium and caval-pulmonary anastomosis region in four children after a modified Fontan operation. Transthoracic surface echocardiography failed to identify these thrombi in five of the six cases because of the posterior location of the thrombus or imaging interference from surgical hardware. These thrombotic episodes occurred 2 days to 5 years after the Fontan operation in children 25 to 168 months of age. Clinical features of compromised cardiac performance with cyanosis or inadequate perfusion were present during four of the six episodes. In two patients, thrombi occurred around transvenous permanent atrial pacing leads. Therapy to eliminate thrombus included surgery (two cases), anticoagulation with warfarin (three cases) and streptokinase thrombolysis (one case). Disappearance of the thrombus was confirmed by transesophageal study in three of the four cases with follow-up echocardiography. Transesophageal echocardiographic demonstration of atrial and pulmonary thrombi that could not be seen by transthoracic imaging suggests that these thrombi occur with greater frequency in patients who have undergone the Fontan operation than was previously suspected.     

Transvenous right atrial and left ventricular pacing after the Fontan operation: long-term hemodynamic and electrophysiologic benefit of early atrioventricular resynchronization

We report a case of long-term, successful, endocardial atrioventricular pacing in a 32-year-old man who had severe heart failure and ascites after having undergone a Fontan procedure for tricuspid atresia 9 years earlier. The patient was referred to our hospital for Fontan revision. However, electroanatomic mapping of the right atrium revealed viable tissue at the interatrial septum above the os of the coronary sinus, and it appeared that the left ventricle could be paced from a coronary sinus branch. Therefore, instead of Fontan revision, an endocardial atrioventricular pacemaker was implanted transvenously. On 5-year follow-up, the patient remained in New York Heart Association functional class I and had not been readmitted to the hospital for congestive heart failure or arrhythmias. His atrial and ventricular leads continued to show excellent pacing and sensing results.     

Langzeitergebnisse nach modifizierter Fontan-Operation

Since the introduction of palliative surgical therapy for single ventricle defect more and more patients have reached adulthood. Thus, nowadays the long-term results of large cohorts can be analyzed. The results of the modified Fontan operation performed with modern surgical technique are characterized by low morbidity and mortality rates. Therefore in the past 40 years a new patient population with very specific anatomical and physiological principles has arisen and continues to increase in size. The long-term follow-up reveals stable hemodynamics under non-cyanotic conditions. The preconditions for optimal long-term outcome are strict preoperative selection and subject-specific postoperative supervision. The long-term results can be significantly improved if therapy is started early enough. Nevertheless the Fontan circulation is limited through the non-physiological flow principle and decreased function of the single ventricle. Essential for the long-term stability of the hemodynamics are the reduction of pulmonary vascular resistance and improvement of ventricular filling. The conventional medication for heart failure is not applicable in treatment of the failing Fontan circulation. Severe Fontan complications, such as protein-losing enteropathy with chronic ascites and chronic Fontan failure are rare. Nevertheless the possibility for conventional treatment in such cases is limited and heart transplantation is the ultima ratio therapy.     

Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

BACKGROUND. We reviewed the outcome of 76 consecutive patients (age range, 5 months to 6 years; median age, 19 months) who underwent a modified Fontan procedure after initial palliative surgery for hypoplastic left heart syndrome (HLHS) between January 1984 and December 1989. METHODS AND RESULTS. Modifications of the Fontan procedure included transatrial baffle of pulmonary venous return to the tricuspid valve (n = 10) or inferior vena cava baffle within the right atrium to the superior vena caval-pulmonary artery anastomosis, with pulmonary artery augmentation (n = 66). Actuarial survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52% (4 years). Of the 43 survivors, 25 patients have returned for postoperative cardiac catheterization at a medium of 13 months after the Fontan procedure. Mean +/- SD hemodynamic values were cardiac index, 2.8 +/- 0.6 l/min/m2; right arterial pressure, 11 +/- 2 mm Hg; pulmonary artery wedge pressure, 6 +/- 3 mm Hg; and arterial oxygen saturation, 94 +/- 3%. No patient had significant tricuspid or native pulmonary valve insufficiency. CONCLUSIONS. Survival after the Fontan procedure in patients with HLHS is comparable to survival after a Fontan procedure in patients with other complex congenital heart lesions. In the subgroup of patients with HLHS who survived both reconstructive surgery and a Fontan procedure and have been evaluated by cardiac catheterization after a Fontan procedure, the use of the right ventricle as the systemic ventricle yielded excellent intermediate results for Fontan physiology.     

Influence of ventricular morphology on outcome after the Fontan procedure

The modified Fontan procedure has gained wide acceptance in the treatment of various congenital heart defects. Determination of risk factors for mortality remains an important issue for optimizing patient selection for the Fontan procedure. Conflicting results have been reported about whether ventricular morphology is a risk factor in these patients. Survival free of Fontan takedown or cardiac transplantation was assessed in the first 500 patients undergoing the Fontan procedure at our institution. This survival was correlated with ventricular morphology as evaluated by angiography. Both multivariate and univariate analyses indicated ventricular morphology was predictive of early survival free of Fontan takedown or cardiac transplantation following the procedure. However, there was no statistical evidence for ventricular morphology being a risk factor for mortality in patients alive 6 months after the procedure. Ventricular morphology is a risk factor for early survival in patients undergoing a Fontan procedure, with left ventricular morphology associated with a better early survival than right ventricular morphology.     

Orthotopic heart transplantation in patients with univentricular physiology

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) [1]. 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 [5]. Improved early and late survival has not yet abolished late mortality secondary to myocardial failure, therefore increasing the referrals for cardiac transplantation [6]. Interstage attrition [7] is moreover expected in staged palliation towards completion of a Fontan-type circulation, while Fontan failure represents a growing indication for heart transplantation [8]. Heart transplantation has therefore become the potential "fourth stage" [9] or a possible alternative to a high-risk Fontan operation [10] in a strategy of staged palliation for single ventricle physiology. Heart transplant barely accounts for 16% of pediatric solid organ transplants [11]. The thirteenth official pediatric heart transplantation report- 2010 [11] 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 [9], while others suggest that rescue-OHT after failing Fontan seems unwarranted [10]. Moreover, OHT outcome appears to be different according to the surgical staging towards the Fontan operation and surgical technique of Fontan completion [12].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.     

Usefulness of cardiac magnetic resonance imaging combined with low-dose dobutamine stress to detect an abnormal ventricular stress response in children and young adults after fontan operation at young age

After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 microg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with beta-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate.     

Somatic growth failure after the Fontan operation

Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (-0.91 +/- 0.99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were -0.67 +/- 1.1 and -0.89 +/- 1.2 respectively. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a mean time from the Fontan operation of 4.4 +/- 1.4 years, the mean Z score for height was -1.15 +/- 1.2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.     

Management and outcome of infants and children with right atrial isomerism.

OBJECTIVES: To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. SETTING: Tertiary referral centre. METHODS: 20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. RESULTS: Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine). CONCLUSIONS: Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.     

A case of PAGOD syndrome with hypoplastic left heart syndrome

PAGOD syndrome is an extremely rare congenital malformation complex involving multiple organs. These include pulmonary artery and lung hypoplasia, diaphragm defects, omphalocele, sex reversal or ambiguous genitalia, and complex cardiac defects. Eight cases have been reported to date. We report a case of PAGOD syndrome that is manifested by right diaphragm eventration, horseshoe lung with right lung hypoplasia, hypoplastic left heart syndrome (mitral atresia, aortic atresia), scimitar syndrome, agonadism with ambiguous genitalia. A karyotype revealed normal 46-XY. This patient received a modified Norwood procedure for hypoplastic left heart syndrome as an initial palliation and bidirectional cavopulmonary anastomosis as a second stage of operation. The postoperative courses were uneventful. This patient is waiting for Fontan operation.     

Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation

Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients' characteristics, Fontan modifications employed, early mortality, long‐term survival and frequency of relevant complications was extracted. Ultimately, thirty‐one studies published by the largest surgical centers with an overall number of 9390 patients were included in this review. The extracardiac total cavopulmonary anastomosis is the most frequently used Fontan modification. Hemodynamic data demonstrate a rigorous overall adherence to suggested Fontan selection criteria. The analysis showed a clear trend toward improved early and long‐term survival over the time period covered. Although inconsistently reported, severe complications such as arrhythmias, thromboembolic events and protein‐losing enteropathy as well as reoperations and reinterventions were frequent. In conclusion, patients palliated for complex univentricular heart malformations nowadays benefit from the experience and technical developments of the past decades and have a significantly improved long‐term prognosis. However, important issues concerning postoperative long‐term morbidity and mortality are still unsolved and clear intrinsic limitations of the Fontan circulation are becoming evident as the population of Fontan patients ages.     

Fontan's operation: results, immediate and long-term postoperative complications

The Fontan operation and its modifications can be relatively safely performed in the treatment of many complex congenital heart diseases, such as univentricular heart and tricuspid atresia. The main postoperative complications following the Fontan procedure and the incremental risk factors involved in their development are investigated. Between January 1984 and January 1988 eleven patients (6 females and 5 males), ranging in age from 2 to 15 years (mean age: 7.3 +/- 3.7) and in weight from 10.8 to 50 Kg (mean weight 22.3 +/- 12.7) underwent the Fontan operation in our Department. No hospital death occurred. The mean postoperative stay in the Intensive Care Unit was 6.3 +/- 3.9 days. There were two surgical re-entries for the same patient: cardiac tamponade (the day of operation) and residual atrial septal defect (2 days following the operation). Eight patients had significant signs of venous stasis, with severe hepatomegaly and pleural effusion. The 11 patients discharged were followed-up for a period of between 3 and 48 months (mean follow-up: 26.4 +/- 18). There were 2 cases of recurrent pleural effusion, 10 to 15 days after discharge. One late death occurred 2 years after the Fontan procedure (massive pulmonary embolism after re-operation). Short and medium-term rate of complications is related to increased post-operative values of mean right atrial pressure (greater than 15 mmHg). Mean right atrial pressures greater than 15 mmHg appear to be correlated with Nakata index values less than 250 mm2/m2. The experience reviewed confirms the excellent results of the Fontan operation, as regards survival and functional recovery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pre-operative assessment of ventricular function in patients considered for Fontan procedure.

In 1978 Choussat and Fontan established ten criteria, which should be fulfilled to achieve a successful outcome in Fontan operation. Recent data suggest that while some of these ten criteria need not be necessarrily be fulfilled, new criteria should be added. These include 1. good diastolic function, 2. normal or only slightly increased ventricular mass, and 3. absence of systemic outflow obstruction. In addition the morphology of the single ventricle may be important as long-term results in patients with single ventricle of right ventricular morphology may be worse than results in patients with single ventricle of left ventricular morphology. Ventricular size and pump function can be assessed by cardiac catherization, echocardiography or magnetic resonance imaging. Estimation of ejection fraction under stress by nuclear angiography may be indicated. Diastolic function can be examined using Doppler echocardiography or nuclear angiography. Myocardial mass may be assessed by echocardiography or magnetic resonance imaging. Normal reference values for different parameters of systolic and diastolic function are listed in the enclosed tables. Patients scheduled for a Fontan operation should have an ejection fraction less than 50%. Patients with borderline ejection fraction should be examined by echocardiography to determine the end-systolic wall stress, a parameter of ventricular contraction, which is independent of pre- and afterload. As afterload may decrease after a Fontan operation some patients with reduced ejection fraction but normal end-systolic wall stress may still be suitable candidates for Fontan operation.(ABSTRACT TRUNCATED AT 250 WORDS)