Bi-directional cavopulmonary shunt: is accessory pulsatile flow, good or bad?

OBJECTIVE: Evaluation of the effect and long-term outcome of accessory pulsatile blood flow versus classical bi-directional cavopulmonary connection (BCPC). METHODS: Retrospective review of the medical and surgical records. RESULTS: Two-hundred and five patients (119 boys, 86 girls) underwent BCPC from 1990 to 1996. Accessory pulsatile flow was present in 68%, flow being maintained through the pulmonary trunc in 46%, systemic-to-pulmonary artery shunt in 13% and mixed in 7%, or patent ductus arteriosus in 2%. Patients with accessory pulsatile flow had lower hospital mortality (3% versus 5%), while mean pulmonary artery pressure (14.1 versus 12.6 mmHg P = 0.050) and increase of oxygen saturation (12.4 versus 8.7, P = 0.034) were significantly higher. The period of artificial ventilation (1.9 day) and ICU stay (6 days) did not differ for both groups. Late mortality was higher following accessory pulsatile flow (6% versus 1%). At late follow-up patients with accessory pulsatile flow had significantly higher oxygen saturation (mean 85 +/- 4%, versus 79 +/- 4%; P < or = 0.005). If subsequent completion of Fontan is considered the optimal palliation and subsequent systemic to pulmonary artery shunt, arteriovenous fistula and transplantation is considered a failure, patients with accessory pulsatile flow had significantly more and earlier completion of the Fontan procedure (mean 1.7 +/- 2.4 years, versus 2.7 +/- 4.4 years; P = 0.008). Survival is not influenced by age at bi-directional cavopulmonary shunt surgery, left or right functional ventricular anatomy or previous palliative surgery. One patient with accessory pulsatile flow developed systemic-to-pulmonary collateral's eventually requiring lobectomy. CONCLUSION: Despite two different initial palliative techniques the outcome was not significantly different. Accessory pulsatile blood flow appeared not to be a contra-indication for a completion Fontan procedure. Moreover, the data suggest that after accessory pulsatile flow can safely be performed, at late follow-up oxygen saturation is higher, while, significantly more and earlier completion of Fontan occurred. Age at bi-directional cavopulmonary shunt, basic left or right ventricular anatomy or previous palliative surgery did not influence survival.     

Preoperative small pulmonary artery did not affect the midterm results of Fontan operation.

OBJECTIVE: It was well discussed, in the primary Fontan era, that small pulmonary arterial size could affect the results immediately after the Fontan procedure. The objective of the present study is to determine whether this feature remains as a risk factor in the era of the staged Fontan completion and poses functional impediments in the longer terms. METHODS: Between June 1991 and November 2004, the staged Fontan completion was carried out subsequent to the bidirectional Glenn procedure in consecutive 57 patients with a preoperative pulmonary artery index less than 250 mm2/m2 (Group-S; minimum index 104 mm2/m2). Clinical data were reviewed retrospectively. As background and reference information, similar data were collected in another consecutive 64 patients with larger pulmonary arteries who underwent the staged Fontan completion during the same period (Group-L; maximum index 697 mm2/m2). RESULTS: No patients died after the Fontan procedure in Group-S, while six early deaths in Group-L. No takedown of the Fontan circulation was carried out in either group. The latest catheterizations, at 2.8+/-2.7 years postoperatively, showed a pulmonary artery index significantly lower than the preoperative index (Group-S: 198+/-37-176+/-49 mm2/m2; P=0.0082, Group-L: 360+/-94-266+/-89 mm2/m2; P<0.0001). Hemodynamics in Group-S during the intermediate term were identical with those in Group-L in mean pulmonary arterial pressure (10+/-2 in Group-S and 10+/-3 mmHg in Group-L), mean atrial pressure for the systemic chambers (5+/-2 and 6+/-3 mmHg, respectively), mean transpulmonary gradient (5+/-2 and 4+/-2 mmHg, respectively), cardiac index (3.0+/-0.7 and 3.0+/-0.6l/min/m2, respectively), and arterial oxygen saturation (93+/-3% and 94+/-2%, respectively). Similarly, brain natriuretic peptides concentration in the serum (19.4+/-15.6 in Group-S and 28.3+/-37.2 pg/ml in Group-L) and peak oxygen consumption on exercise testing (24.8+/-4.5 and 24.0+/-6.3 ml/kg/min, respectively) were not inferior in Group-S to those in Group-L. Conclusions: The outcome after the Fontan completion, including functional ones in the intermediate term, was acceptable in patients having a preoperative PA index smaller than 250 mm2/m2. Pulmonary artery index decreased still further postoperatively, but did not obviously militate against functional efficacy of the Fontan circulation.     

Fontan procedure for hypoplastic left heart syndrome.

Since 1985, 354 neonates have undergone palliative reconstruction for hypoplastic left heart syndrome with 109 early deaths and 12 late deaths. Of the survivors, before 1989, 77 patients underwent a subsequent modified Fontan operation, consisting of baffling the atrial septal defect to the tricuspid valve (initial 25 patients) or intraatrial baffling of the inferior vena cava to the pulmonary arteries and superior vena cava (52 patients). There were 17 early deaths and three late deaths. Major serous effusions developed in 42 patients (54%) after Fontan operation. Since 1989, a staged approach to Fontan's operation was undertaken in an effort to reduce the volume load of the right ventricle as early as possible, to minimize the impact of rapid changes in ventricular geometry and diastolic function that can accompany a primary Fontan operation, and to reduce effusive complications. Thus, at a mean age of 6 months, 121 patients have undergone closure of aortopulmonary shunt, augmentation of central pulmonary arteries, and association of the superior vena cava with the branch pulmonary arteries (hemi-Fontan procedure). Of these, 61 patients have already undergone completion of the Fontan procedure with six early deaths and three late deaths. Major serous effusions developed in 28 patients (46%) with the staged Fontan. For perspective, the contemporary experience since January 1991 consists of 58 neonates who have undergone initial palliation with 11 deaths (19%), 17 patients who have undergone the hemi-Fontan procedure with one death (6%), and 21 patients who have undergone completion of the Fontan operation with one death (5%).(ABSTRACT TRUNCATED AT 250 WORDS)     

Bidirectional cavopulmonary connection without additional pulmonary blood flow in patients below the age of 6 months.

Objective: The bidirectional cavopulmonary connection (BCPC) has shown excellent results as an intermediate step towards permanent palliation in functional single ventricle morphology. The optimal timing of the BCPC, though, remains unclear. This investigation focuses on results in a subgroup of patients under 6 months of age. Methods: Between 2001 and 2006, 124 patients received a BCPC followed by an extracardiac total cavopulmonary connection (TCPC). Review of 84 angiograms before BCPC and before TCPC allowed for analysis of haemodynamic findings and measurement of the diameters of the pulmonary arteries. Twenty-eight patients were below 6 months of age at the time of BCPC (group 1), whereas 56 were above 6 months of age (group 2). Results: Age at time of BCPC was 4.6+/-1 months (range 2-6 months) for group 1, compared to 16.6+/-17 months (range 6-98 months) for group 2 (p<0.001). The most common diagnosis was a hypoplastic left heart syndrome (HLHS) in 16 patients (19%). The rate of patients with HLHS was higher in group 1 than in group 2 (p=0.006). There was a significant increase in oxygen saturation from 73+/-11% prior BCPC to 83+/-6% prior to TCPC in group 1 (p=0.001) and from 75+/-8% to 83+/-6% in group 2 (p=0.003) respectively. The mean pulmonary artery pressure decreased from 18+/-10mmHg before BCPC to 9+/-2mmHg before TCPC in group 1 (p<0.001) and from 14+/-7mmHg to 8+/-3mmHg in group 2 (p=0.001). The pulmonary artery diameters increased in both groups between BCPC and TCPC, except for the left pulmonary artery in group 2. Nevertheless, the pulmonary arteries remained too small for the given body surface area. Conclusion: Early unloading of the functional univentricular heart by means of BCPC without additional pulmonary blood flow is feasible even in very young patients. Favourable haemodynamics and reasonable increase in pulmonary artery size allow for good condition prior to completion to TCPC.     

Extracardiac Fontan operation without cardiopulmonary bypass

AIM: The avoidance of cardiopulmonary bypass and aortic cross-clamping in patients possessing single ventricular physiology has potential advantages including preservation of ventricular and pulmonary functions; early extubation, decreased incidence of pleural effusions and decreased requirement of inotropic agents and blood products. In this study, we assessed the postoperative outcome of patients who have undergone extracardiac Fontan operation performed without cardiopulmonary bypass. METHODS: Between March 1999 and August 2002, 10 consecutive patients (6 males and 4 females) underwent extracardiac Fontan operation without cardiopulmonary bypass. The age of patients ranged between 1.5 to 12 (5.2+/-3.1) years. All the patients requiring any intracardiac intervention were excluded from the study. Previous operations of the patients were modified Blalock-Taussig shunt procedure in 3 patients, bidirectional cavopulmonary shunt operation in 2 patients and pulmonary arterial banding in 1 patient. All operations were performed without cardiopulmonary bypass. Bidirectional cavopulmonary anastomosis was performed by using a transient external shunt constructed between the superior vena cava and right atrium. An appropriate sized tube graft was anastomosed to the inferior surface of right pulmonary artery. Finally, inferior vena cava to tube grafts anastomosis was performed with the aid of another external shunt constructed between inferior vena cava and right atrium. During the procedure central venous pressure, blood pressure and arterial oxygen saturation levels were continuously monitored and recorded. RESULTS: The mean intraoperative Fontan pressure was 16.1+/-2.75 mmHg. Intraoperative fenestration was required in 4 patients with a Fontan pressure above 18 mmHg. There were no intra and postoperative deaths. Three patients required mild doses of inotropic support during the postoperative period. All patients were weaned off mechanical ventilation within 24 h. The mean arterial oxygen saturation raised from 74.5+/-4.2% to 93.5+/-2%. Arterial oxygen saturation was 95+/-0.6% in 6 patients without fenestration and 91.2+/-0.5% in 4 patients with fenestration (P=0.001). All patients were in sinus rhythm postoperatively. Only 2 patients required blood transfusion. Two patients suffered from prolonged pleural effusion (more than 7 days). The mean intensive care unit and hospital stay periods were 3.3+/-1.5 and 15.4+/-5.3 days, respectively. CONCLUSIONS: The extracardiac Fontan operation performed without cardiopulmonary bypass provides good results in short and midterm follow-up periods with improved postoperative hemodynamics.     

The modified Fontan operation for double inlet left ventricle : Surgical results, early haemodynamic and functional assessment

Twenty-one patients with a double inlet left ventricle underwent a modified Fontan between May 1979 and August 1987. All but the first patient in this series were operated upon after August 1984. Their age at operation ranged from 4 to 23 years with a median age of 8 years. Initial palliation was required in 12 patients and the mean interval to repair was 77.1 months, ranging from 38 to 112. Only one of the criteria of Choussat was exceeded in 12 patients. The pulmonary vascular resistance was always less than 4 Um². A direct atriopulmonary anastomosis was performed in 19 patients and an aortic homograft interposition was used in 2. There was one hospital death due to acute cardiac failure and no late deaths. The average of the mean right and left atrial pressures measured early postoperatively was 12.9 and 8 mmHg respectively. A prolonged hospitalization with a mean of 23 days, ranging from 9 to 69, was required because of recurrent pleural and pericardial effusions which usually resolved after the first 6 postoperative months. The need for early anticoagulation is recommended to prevent the risk of pulmonary thromboembolism observed in 2 of our patients. Cardiac catheterization performed in 19 patients within 1 year after surgery showed that the average of the mean right atrial pressure had decreased to 11.3 mmHg without a gradient across the anastomosis in any case. Two patients required reoperation: one for enlargement of a restrictive ventricular septal defect and the other for closure of a recurrent dehiscence of the patch used for closure of the right a-v valve. Three patients developed a significant subaortic obstruction and have been scheduled for reoperation. Ventricular function was assessed at a mean interval of 11.9 months (17 patients) and 25.5 months (13 patients) from surgery. Gated equilibrium radionuclide ventriculography showed that the ejection fraction was normal (≥50%) and it had increased with time in 10. Yet, the response to exercise was normal in only 5 of the 13 patients re-evaluated. Treadmill exercise testing showed an increase in exercise tolerance from a mean of 7.4 min to a mean of 8.7 min. At the last follow-up, as of August 1987, 17 out of 20 survivors were in NYHA functional class I and only 2 of them remained on diuretic therapy. Two patients with protein-losing enteropathy were in class II, while the last patient who suffered cerebral haemorrhage was in class IV. We conclude that the Fontan operation can be performed in patients with double inlet left ventricle with overall results similar to those reported in the literature for repair of tricuspid atresia.     

Incidence and implications of systemic to pulmonary collaterals after bidirectional cavopulmonary anastomosis

BACKGROUND: Systemic to pulmonary arterial collaterals often develop after bidirectional cavopulmonary anastomosis (BCPA). It has been proposed that such collaterals may be related to perioperative outcome and duration of effusions after the modified Fontan procedure. However, the incidence and significance of collaterals after BCPA remain uncertain. METHODS: To evaluate risk factors for, and significance of, such collaterals, we reviewed angiographic and clinical data for all 76 patients who underwent BCPA between January 1990 and June 1996 and had follow-up catheterization during or before 1997. RESULTS: The median age at BCPA was 10 months, and the median duration from BCPA to follow-up catheterization was 18 months. Arterial collaterals were detected on follow-up catheterization in 45 patients (59%). Factors associated with collateral development included a prior right-sided systemic-to-pulmonary arterial shunt, lower pre-BCPA end-diastolic ventricular pressure and pulmonary vascular resistance, and use and duration of cardiopulmonary bypass during the BCPA operation. Fourteen of the 45 patients (30%) underwent coil embolization of the collaterals. Forty-three patients have undergone extracardiac conduit Fontan, with 1 early and 1 late death. Collaterals were present in 22 of these patients, 7 of whom underwent pre-Fontan embolization. The duration from BCPA to Fontan was longer in patients with collaterals, but these patients were not more likely to have prolonged effusions than those without, and the duration of tube thoracostomy was significantly shorter in patients with collaterals. Embolization of collaterals did not affect the duration of effusions. CONCLUSIONS: Systemic-to-pulmonary arterial collaterals are common after BCPA. In contrast to prior reports, collaterals were not associated with a higher incidence of prolonged effusions after the Fontan procedure in our experience, and did not correlate with poor outcome.     

Long-term clinical outcome of atrial isomerism after univentricular repair

Abstract   Objectives: We retrospectively reviewed the long-term outcome of atrial isomerism patients after Fontan completion. Methods: Since 1972, 58 patients underwent a palliative procedure prior to the Fontan-type operation. Twenty-eight out of 58 patients could not reach Fontan-type operation. Twenty-five patients underwent Fontan-type operation, and 12 of them expired less than five years after the Fontan completion. Eleven patients survived more than five years after the Fontan completion and were identified as long-term survivors. The mean follow-up period was 13 ± 5 years. Results: During follow-up period, four of the 11 patients expired. The actuarial survival rates at 10, 15, and 20 years after univentricular repair (UVR) were 100%, 71.4%, and 53.6%, respectively. The significant predictors of long-term survival by univariate analysis were the staged strategy (p = 0.019), total cavo-pulmonary connection with extracardiac conduit (p = 0.019), and the absence of postoperative common atrioventricular valve regurgitation (p = 0.040). Six out of the seven present survivors showed New York Heart Association class I activity. All present survivors' mean percutaneous oxygen saturation, mean pulmonary arterial pressure, pulmonary capillary wedge pressure, single ventricular end diastolic volume index, and single ventricular ejection fraction were 88.8 ± 6.8%, 11.0 ± 2.6 mmHg, 5.8 ± 2.0 mmHg, 104 ± 37 mL/m², and 52.0 ± 6.5%, respectively. Conclusions: There are still life-threatening problems 10 years after the UVR. However, the excellent performance status of the present long-term survivors suggests that these problems can all be overcome by the present strategies established for the Fontan-type operation.     

Total Extracardiac Right Heart Bypass Using a Polytetrafluoroethylene Graft

A bstract Background : With regard to hemodynamics and late arrhythmias, total cavopulmonary connection has been accepted as a superior technique as compared to Fontan type procedures. However, intra-atrial baffles for lateral tunnel or conduit remain construction retain some similar disadvantages. Patients and Methods : As an alternative to total cavopulmonary connection, total extracardiac right heart bypass using a polytetrafluoroethylene tube for the inferior vena cava to pulmonary artery connection may obviate some problems. Five patients with complex heart disease necessitating one ventricle repair underwent this procedure successfully. Results : Aortic cross-clamp time ranged from 0 to 24 minutes (mean = 15.8 min). No case required takedown or an additional step. Although the follow-up periods have been relatively short (mean = 19 months), all patients are well and no arrhythmic event or thromboembolic episode has occurred. Conclusions : As a simple, safe, and reproducible procedure, total extracardiac right heart bypass is an alternative to Fontan or total cavopulmonary connection procedure.     

Extracardiac conduit Fontan procedure: early and intermediate results

Objective: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. Methods: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7–38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. Results: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6±3.2 and 8.5±3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26–105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. Conclusions: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.     

Fenestrated Fontan operation with delayed transcatheter closure of atrial septal defect. Improved results in high-risk patients.

Ten patients, each with two or more risk factors for morbidity and death, underwent a fenestrated Fontan procedure in which a 4 to 6 mm circular fenestration was left between the systemic and pulmonary venous chambers. None died; a similar group of high-risk patients without fenestration had a mortality rate of 2 of 8. Patients with fenestration had significantly less drainage from the chest tube, less need for inotropic support, and shorter intensive care and hospital stays than did patients without fenestration. Comparison with a group of low-risk patients undergoing the Fontan operation showed no statistical difference in these postoperative parameters. Fenestrations were closed in all 10 patients at from 9 days to 6 months after operation by means of the transcatheter clamshell occluder device. Two patients had left pulmonary artery balloon angioplasty and three patients had other atrial communications closed with additional clamshell devices. During short-term follow-up periods averaging 18 months, all patients were clinically well; however, one patient with mitral atresia required reoperation for obstruction between the left atrium and the tricuspid valve, not related to the clamshell device. These data indicate that fenestration may be one method of achieving lower morbidity and mortality rates among high-risk patients undergoing the Fontan procedure.     

The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection.

OBJECTIVE: This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology. METHODS: Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n=44, 42%) or right ventricular morphology (n=59, 58%). The TCPC was performed using an extracardiac conduit (n=84, 82%) or a lateral atrial tunnel (n=19, 18%), and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation; early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days-5.2 years). RESULTS: Early mortality was 1.9% (n=2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (+/-1 SEM) was 95.6+/-2.5%. Forty-two patients (41%) had prolonged pleural drainage (> or =14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (+/-1 SEM) were 92.2+/-5.0 and 73.4+/-6.0%, respectively. The Fontan procedure was associated with an improved functional class (P<0.005) and all current survivors (n=99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P<0.05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P<0.05), increased postoperative pulmonary artery pressures (P<0.005) and an unfenestrated Fontan procedure (P<0.01). CONCLUSIONS: In this contemporary series, the modified Fontan procedure was characterised by low early mortality, excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival.     

Anesthetic care during modified fontan procedure without cardiopulmonary bypass

OBJECTIVE: The purpose of this study was to report the anesthetic care of patients during performance of a Fontan procedure without cardiopulmonary bypass (CPB). DESIGN: Retrospective chart review. SETTING: Operating room of a university hospital. PARTICIPANTS: Seven pediatric-patients undergoing inferior vena cava (IVC)-to-pulmonary artery (PA) anastomosis for completion of the Fontan procedure. INTERVENTIONS: Charts were reviewed for anesthetic technique, hemodynamic and ventilatory changes occurring during the procedure, and anesthetic interventions that were provided. MEASUREMENTS AND MAIN RESULTS: The off-bypass Fontan procedure was attempted in 7 patients (age: 26 months-7 years, weight: 13 to 28 kg). Exposure of the PA was not feasible in 1 patient because of a markedly enlarged right atrium. In the remaining 6 patients, before cross-clamping of the PA to allow for the proximal anastomosis between the PA and the conduit, alkalosis (pH > or =7.5) was maintained by the administration of sodium bicarbonate. After PA cross-clamping, fluid administration was necessary in 5 patients and dopamine (3-7 microg/kg/min) was necessary in 4 patients. The minute ventilation was increased by 18 +/- 7% to maintain baseline PaCO2 values. Before the placement of the PA cross-clamp, the end-tidal PaCO2 difference was 7 +/- 4 mmHg and the transcutaneous (TC)-PaCO2 difference was 3 +/- 2 mmHg. The end-tidal PaCO2 difference increased to 14 +/- 6 mmHg during cross-clamping of the PA, whereas no change was noted in the TC-PaCO2 difference. Once the proximal anastomosis was completed, a bridge was placed to redirect blood from the IVC to the right atrium while the IVC was clamped and attached to the distal end of the conduit. After placement of the distal end of the bridge into the IVC, fluid administration to maintain the blood pressure was necessary in 3 patients. In 1 patient, 20 minutes after placement of the bridge, the authors noted a progressive increase in the central venous pressure reading measured from the left femoral vein and the need for the administration of volume to maintain the mean arterial pressure. Examination of the bridge revealed occlusion with thrombus despite an activated coagulation time value of 250 to 300 seconds. The tracheas of 3 of the 6 patients were extubated in the operating room, whereas the other 3 were extubated in the pediatric intensive care unit within 4 hours of completion of the procedure. The 1 patient who required the use of CPB required reintubation and had a protracted intensive care unit course. The other 6 patients were discharged home on postoperative days 7 to 12. CONCLUSIONS: With alteration of the anesthetic technique, the Fontan procedure can be performed in selected patients without the need for CPB.     

Endotracheal balloon dilatation and stent implantation in benign stenoses

BACKGROUND: Tracheal reconstruction is the treatment of choice in nontumorous tracheal stenoses, but recurrences and concomitant medical conditions limit this approach. We investigated the outcome after balloon dilatation and silicone stent implantation. METHODS: Forty-two patients with inoperable tracheal stenoses underwent balloon dilatation and afterward silicone stent implantation. Patients were divided into two groups, in group A 24 patients received tracheal stents as a temporary treatment. In group B, definitive stenting was done in 18 patients with severe concomitant medical conditions that did not allow for stent removal. RESULTS: Immediate results were satisfactory in all patients. In group A, stents could be removed in 12 patients after a mean interval of 20 months. Restenting was not required during the following 18.9 months. Twelve patients are still waiting for stent removal after a mean follow-up of 20 months. In group B, mean follow-up is now 48.4 months. Complications included retained secretions, dislocation, and granuloma formation. CONCLUSIONS: Stenting after balloon dilatation is safe and effective in benign tracheal stenoses. After temporary use, stents can be removed when local and general conditions permit. In all other patients, stenting proved beneficial for 5 years as more definitive treatment.     

Fontan type operation for complex lesions. Surgical considerations to improve survival

Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used. Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previous pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 +/- 6.5 torr), and hospital stay longer (31 days). In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 +/- 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlusion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13%). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing well. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valve closure, avoidance of valved conduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.     

Right partial anomalous pulmonary venous connection to the superior vena cava following the Warden procedure

Background and Aims

Surgical repair of right partial pulmonary anomalous connection to the superior vena cava (SVC) with the Warden procedure can be complicated by SVC obstruction, pulmonary veins obstruction, and sinus node dysfunction. We review our 20 years of experience with Warden procedures for the repair of right partial pulmonary venous connection to the SVC.

Methods

This was a single‐center retrospective study of all patients (pediatric and adult) with right partial pulmonary abnormal venous connections who underwent a complete repair with the Warden procedure between 1997 and 2016. A total of 59 patients were included. The median age was 14 years (5 months to 61 years) and the median weight was 44 kg (4.9‐92 kg).

Results

The mean term follow‐up was 7 (±5) years. No deaths were reported. One (1.7%) patient required a pacemaker implantation; two (3.4%) suffered from a SVC stenosis successfully treated with balloon dilatation and stent implantation; and eight (13.6%) patients had transient rhythm disturbances, one had junctional ectopic tachycardia, three had sick sinus syndrome, three had an atrial flutter, and one had an atrioventricular block. None suffered from pulmonary vein obstruction.

Conclusion

The Warden procedure is safe and can be performed with very low morbidity, mortality, rhythm disturbances, and decreased pulmonary and SVC obstructions.     

Definitive repair in patients with pulmonary atresia and intact ventricular septum

Between 1977 and 1988, 22 patients underwent definitive repair for pulmonary atresia with intact ventricular septum. Fifteen underwent biventricular repair (mean age 24 months). All had mild to moderate right ventricular hypoplasia at the time of definitive repair. Repair consisted of closure of the atrial septal defect with enlargement of the right ventricular cavity and outflow tract with a patch in eight, insertion of a valved homograft in three, and superior vena cava-pulmonary artery connection in four. There was one operative death (7%). Seven patients had severe right ventricular hypoplasia at the time of definitive repair and underwent a Fontan procedure (mean age 46 months). In two a valved connection was made to the right ventricle and in five a right atrial-pulmonary artery nonvalved connection. There were two operative deaths (29%). Three patients had right ventricular-coronary sinusoids: Two of them underwent a Fontan procedure and one a biventricular repair. We conclude that with adequate early palliation a biventricular repair may be successfully performed for patients with mild or moderate right ventricular hypoplasia, and the Fontan procedure may be used for those with severe right ventricular hypoplasia.     

Is laparoscopic refundoplication feasible in patients with failed primary open antireflux surgery?

Background: Due to the widespread availability and acceptance of minimal-access surgery, laparoscopic antireflux surgery has become the standard procedure for the treatment of severe gastroesophageal reflux disease (GERD). However, open and laparoscopic antireflux procedures sometimes result in failure, so that redosurgery is required in some cases. The aim of this prospective study was to evaluate the surgical outcome and quality of life of patients who underwent refundoplication after the failure of primary open antireflux surgery. Methods: Twenty patients with a mean age of 52 years (range, 33–69) underwent laparoscopic refundoplication after primary open antireflux surgery. Four of them had undergone surgery twice previously. Preoperative and postoperative data, including esophageal manometry, 24-h pH monitoring, and assessment of quality of life, were reviewed prospectively. Quality of life was evaluated using the Gastrointestinal Quality of Life Index (GIQLI). Results: In 18 patients (90%), the reoperation was completed successfully laparoscopically. Two others (10%) required conversion to an open procedure. One of them had an injury of the gastric wall; in the other case, severe bleeding of the spleen necessitated the conversion. The average operating time was 245 min. Preoperatively, the main symptoms were recurrent reflux in 14 cases and a combination of re-reflux and dysphagia in six cases. The anatomic findings were telescope phenomenon (n = 6), hiatal disruption (n = 10), and wrap breakdown (n = 4). Postoperatively, two patients suffered from dysphagia and required pneumatic dilatation. The lower esophageal sphincter (LES) pressure increased significantly from a preoperative value of 6.08 mmHg to 12.2 mmHg at 3 months and 11.9 mmHg at 1 year after surgery. The DeMeester score decreased from a preoperative value of 69.8 to 17.1 at 3 months and 14.6 at 1 year postoperatively. The GIQLI score increased from a preoperative value of 84.9 points to 119.6 points at 3 months and 120.1 points at 1 year. Conclusion: Laparoscopic refundoplication after the failure of a primary open intervention is an effective procedure that can be performed safely by experienced laparoscopic surgeon. The procedure yields excellent functional results and leads to significant improvement in the patient's quality of life.     

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

BACKGROUND: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS: Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.     

Results of right atrial to right ventricular and right atrial to pulmonary artery conduits for complex congenital heart disease.

The Fontan procedure was initially proposed for tricuspid atresia (TA). We describe our experience with modifications of this operation and its application to previously uncorrectable congenital lesions. From 1975 to 1979, 26 patients have undergone a Fontan-like procedure, at a mean age of 14 years (range: 3 months to 23 years). Nineteen patients had TA, one patient had mitral atresia, four patients had a univentricular heart, one patient had a "criss-cross" heart, and one patient had pulmonary atresia. Previous procedures included eight Glenn shunts, 18 systemic to pulmonary artery (PA) shunts, two PA bands, and two atrial septectomies. Thirteen patients underwent right atrial (RA) to PA connections, with a valved conduit in nine patients and without in four. Two patients had simultaneous Glenn shunts. Thirteen had RA to right ventricular (RV) conduits, with a valve in 12 patients and without in one. There were five early deaths (<30 days, 19%) and no significant difference between the RA to PA connection (four deaths of 13) versus the RA to RV connection (one death of 13). The mortality rate was higher in patients undergoing RA to PA connection without valved conduit (one death of nine with a valve, three deaths of four without, p < 0.05), while the Glenn shunt did not affect mortality (one death of seven with, three of six without, p < 0.1). There were no late deaths (mean follow-up: 24 months). Seven patients underwent recatheterization with a mean RA pressure of 14 mmHg (range: 9--25 mmHg). Thus, the "Fontan" procedure can be done with an acceptably low mortality with good functional results, both for TA and other complex lesions.