Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve)

Protein-losing enteropathy occurred in a 7-year-old girl with tricuspid atresia, concordant ventriculo-arterial connexions and a relatively large hypoplastic right ventricle, one year after an atrio-ventricular type of Fontan operation by means of a valveless woven Dacron conduit. Severe conduit regurgitation and a marked enlargement of the hypoplastic right ventricle were demonstrated at recatheterization. Insertion of a bioprosthetic valve at the base of the right atrial appendage led to a dramatic clinical recovery of the patient. The use of a valved conduit is recommended when an atrioventricular type of Fontan repair is planned in patients with tricuspid atresia, concordant ventriculo-arterial connexions and relatively large hypoplastic right ventricle.     

The modified Fontan operation for children less than 4 years old

Of 500 patients who had a modified Fontan operation at this institution between 1973 and 1987, 54 (33 boys and 21 girls) were less than 4 years old. This retrospective study related preoperative clinical and hemodynamic data to subsequent survival. Twenty patients less than 4 years old had tricuspid atresia, 13 had double inlet ventricle and 21 had other complex heart defects. There were 14 early deaths (less than 30 days after operation) and 6 late deaths. Multivariate analysis of survival for the entire group of 500 patients revealed the following factors to be significantly associated with poorer survival: absence of tricuspid atresia (p = 0.011), asplenia (p less than 0.001), age less than 4 years at operation (p = 0.042), atrioventricular valve dysfunction (p = 0.017), early calendar year of operation (p less than 0.001) and the presence of either one or more of the following: left ventricular ejection fraction less than 60%, mean pulmonary artery pressure greater than 15 mm Hg and pulmonary arteriolar resistance greater than 4 U.m2 (p less than 0.001). On the basis of this study of 500 patients, age less than 4 years at operation appears to be an independent risk factor for poorer survival after the modified Fontan operation.     

Clinical and hemodynamic results of the Fontan operation for tricuspid atresia

Thirty patients aged 3 months to 26 years (median 9 years) with tricuspid atresia or severe tricuspid stenosis underwent a modified Fontan operation at Children's Hospital, Boston, between 1973 and 1980. Thirty-six palliative operations had been performed previously in 20 patients. Preoperative hemodynamic measurements included: mean pulmonary arterial pressure 6 to 18 mm Hg (median 9); pulmonary vascular resistance 0.3 to 2.2 Woods units/m² (median 1.5) and left ventricular end-diastolic pressure 3 to 14 mm Hg (median 7).There were five hospital deaths (17 percent), including two in the last 24 operations. The 25 survivors have been followed up for 4 to 85 months (median 23) with no late deaths. Twenty patients are in New York Heart Association class I and four in class II; one infant was not classified. Two patients have required reoperation, at, respectively, 71 and 26 months postoperatively; the former for late-developing conduit obstruction and residual bidirectional shunting; and the latter for a residual right to left shunt at atrial level. Early postoperative monitoring of hemodynamics in the intensive care unit revealed that a mean right atrial pressure of 17 mm Hg or greater, without obstruction of the anastomosis, was invariably associated with serious morbidity or mortality.Fifteen patients underwent elective cardiac catheterization 9 to 21 months (median 13) postoperatively. The mean right atrial pressure ranged between 5 and 12 mm Hg (median 8) and mean pulmonary arterial pressure between 4 and 11 mm Hg (median 7). The cardiac index (measured in 12 patients) was 1.9 to 4.0 liters/min per m² (median 3.2). The left ventricular ejection fraction measured in 11 patients did not change significantly postoperatively, remaining normal in 10. Three patients had a residual right to left shunt at atrial level while the arterial oxygen saturation in the remaining 12 patients was 93 to 97 percent. Thus the modified Fontan operation can be performed with excellent clinical and hemodynamic results in selected patients with tricuspid atresia.     

Surgical management of subaortic obstruction in single left ventricle and tricuspid atresia

Subaortic obstruction caused by either a restrictive bulboventricular foramen in single left ventricle with an outflow chamber or by a restrictive ventricular septal defect in tricuspid atresia with transposition of the great arteries can lead to a hypertrophied, noncompliant ventricle and excessive pulmonary blood flow. This combination is disadvantageous to potential Fontan procedure candidates because they are dependent on good ventricular function and low pulmonary vascular resistance for survival. The results of surgical procedures to directly or indirectly relieve significant subaortic obstruction (gradient greater than 30 mm Hg) in 24 patients, 16 with single left ventricle and 8 with tricuspid atresia, were reviewed. Four patients had a left ventricular apex to descending aorta valved conduit; none survived. Seven patients had resection of subaortic tissue; four survived and four developed heart block at surgery. Adequate gradient relief was evident in only one of the four survivors. Thirteen patients had a main pulmonary artery to ascending aorta anastomosis or conduit; six survived. All survivors had adequate gradient relief. The overall survival was 42% (10 of 24). None of seven patients with a subaortic gradient greater than 75 mm Hg survived. These data show that: Surgical relief of established subaortic obstruction in patients with single left ventricle and tricuspid atresia carries a high mortality rate, especially if the subaortic gradient is greater than 75 mm Hg. The best procedure appears to be the pulmonary artery to ascending aorta anastomosis. A clearer understanding of the factors leading to the development of significant subaortic obstruction is necessary to prevent it or to devise improved therapeutic strategies.     

Reconstruction of the pulmonary outflow tract with a valved conduit (author's transl)]

The authors describe 25 patients who underwent open heart surgery for reconstruction of the pulmonary outflow tract with a valved conduit. The following anomalies were observed: six cases of great vessels transposition, five cases of pulmonary artery atresia, five cases of truncus, three cases of tetralogy of Fallot and six cases of single ventricle. Two cases of single ventricle were treated in order to be transformed into a systemic ventricle. The pulmonary outflow was reconstructed with a valved conduit anastomosed between right atrium and pulmonary artery. This study includes pre- and postoperative hemodynamic values, surgical evaluation and a 5-months to 3-years follow-up. The authors emphasize that it is important that the surgical indications are closely related to the morphology and hemodynamics of pulmonary circulation for a better result.     

A Carpentier-Edwards porcine-valved dacron conduit: at twenty-five years

We report the longest duration of implantation of a porcine bioprosthesis in the English language literature--a Carpentier-Edwards valved conduit excised after twenty-five years. This 50-year-old man had undergone a modified Fontan operation for tricuspid atresia. He presented with progressive dyspnea, fatigue and edema. The bioprosthesis' dysfunction was due to the pathologic findings of pannus, significant thrombus (on the sinus surface of a cusp), mild structural valve deterioration and pseudo-intimal formation within the conduit. The bioprosthesis' longevity is likely related to low-pressure circulation in the right heart and to host tissue (pannus) overgrowth that covered the cusps and thereby reduced tissue degeneration.     

Fontan operation: modification of the valve position in conduit with brief review of the literature

A Fontan operation was performed on a 10-year-old child for correction of tricuspid atresia. A xenograft, valved conduit was used to establish continuity between the right atrium and the small right ventricle. Atrial and ventricular septal defects were repaired with Dacron patches. Two hours postoperatively, the patient suffered a cardiac arrest secondary to hypoxia and was successfully resuscitated. Partial recurrence of a right-to-left shunt at the atrial level necessitated the patient's return to surgery. The insufficient conduit was replaced and the leaking patches were resutured. The conduit was shortened to one-third its length, and the conduit valve was repositioned directly at the proximal end, over the right atriotomy. Ten months postoperatively, the patient remains in stable condition.     

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Persistence and effects of sinus rhythm after Fontan procedure for tricuspid atresia.

Four patients who had had a Fontan type of procedure for tricuspid atresia 23, 6, 6, and 11 months previously were investigated by ambulatory electrocardiographic recording and simultaneous recording of the jugular venous pressure and echocardiogram of the conduit or pulmonary valve. All had been considerably improved by the operation. In 1 patient episodes of supraventricular tachycardia were recorded but no rhythm disturbance was detected in the other 3. Pulmonary blood flow was shown to be pulsatile and atrial systole is an important factor in this. The conduit valve showed delayed opening and slow closure suggesting that its presence in the pulmonary circuit may be unnecessary.     

Persistence and effects of sinus rhythm after Fontan procedure for tricuspid atresia.

Four patients who had had a Fontan type of procedure for tricuspid atresia 23, 6, 6, and 11 months previously were investigated by ambulatory electrocardiographic recording and simultaneous recording of the jugular venous pressure and echocardiogram of the conduit or pulmonary valve. All had been considerably improved by the operation. In 1 patient episodes of supraventricular tachycardia were recorded but no rhythm disturbance was detected in the other 3. Pulmonary blood flow was shown to be pulsatile and atrial systole is an important factor in this. The conduit valve showed delayed opening and slow closure suggesting that its presence in the pulmonary circuit may be unnecessary.     

Total cavo-pulmonary bypass. Short-term results]

In order to reduce the complications of the Fontan procedure, 2 techniques of total cavopulmonary connection using a right intra-atrial baffle (Pugas' technique, 19 patients), and a modified de Kreutzer (19 patients) were assessed in 38 consecutive patients operated between November 1980 and November 1991. All patients had complex cyanotic congenital heart disease: tricuspid atresia (13), single ventricle (12), hypoplasia of one of the two ventricles (8), pulmonary atresia with an intact septum (5). The average age was 5.8 +/- 5.6 years (range 1 to 30 years), the weight was 18 +/- 11.7 kg (range 8 to 59.7 kg). Six patients (15.8%) died, 4 of whom (10.5%) in the operative period. Five of these deaths were related to extremely high right heart pressures without any technical fault, and the other to thrombosis of the conduit in a patient not taking anticoagulants. Two immediate and 10 secondary reoperations were necessary. All 3 patients who had disconnection of the anastomosis because of high right heart pressures died. The postoperative course was often difficult: right heart failure (63%), pleural effusion (57%), and pericardium effusion (13%). Of the risk factors analysed, age had no influence on postoperative outcome. The two poor prognostic factors were: presence of atresia or hypoplasia of the systemic atrioventricular valve (mortality 42.8% versus 6.5%, p = 0.05 and prolongation of the hospital period: 39.5 +/- 32 days versus 21 +/- 11 days, p less than 0.05) and previous banding (reoperation rate 62.5% versus 16.6%, p less than 0.05, and longer stay in the intensive care unit and hospital period).(ABSTRACT TRUNCATED AT 250 WORDS)     

Left ventricular function in tricuspid atresia: a radionuclide study

Left ventricular function was evaluated by radionuclide angiocardiography in patients with tricuspid atresia before and after the Fontan operation. Ejection fraction was 0.54 +/- 0.13 in 29 patients without the Fontan procedure and 0.56 +/- 0.14 in 14 postoperative patients. Group means were not statistically different, but both were less than normal values for the institution (p less than 0.001). Function did not correlate significantly with age or aortic oxygen saturation. Serial studies demonstrated little change in eight of nine patients examined over 1 to 3 years preoperatively. From the preoperative group of nine patients with abnormal ejection fraction, three underwent surgery, with two survivors. Although 5 of 14 patients had abnormal systolic function after the Fontan operation, only 1 was symptomatic. Depressed response to isometric exercise was found in two patients with a normal ejection fraction at rest. Group systolic function after Fontan surgery did not correlate significantly with age at surgery but all patients were operated on at more than 5 years of age. Of seven patients studied both preoperatively and postoperatively two had an increase and three a decrease in ejection fraction. The trend in these seven studied serially after surgery was toward early improvement in ejection fraction. This study shows variability in left ventricular performance in patients with tricuspid atresia before and after surgery. Individuals undergoing the Fontan procedure after 5 years of age do not necessarily have an increase in ejection fraction over preoperative values, and may even have a decrease. Serial improvement over early postoperative results may be expected, without significant deterioration, during the next 1 to 3 years.     

Relief of an early pulmonary pathway obstruction with stenting after modified Fontan operation in an adult

The prognosis of Tricuspid Atresia, a rare congenital heart disease, has been changed by surgery. The criteria for Fontan operation have been well established in the literature and adult patients rarely fulfil these criteria; however, in very selected cases Fontan operation can be performed also in adults. A 33 year old woman with tricuspid atresia and previous palliation with classical right Glenn and with left modified Blalock-Taussing, underwent modified Fontan operation because increasing cyanosis and moderate exercise intolerance. Three weeks after operation the patient was readmitted with severe heart failure because of a tight obstruction at the anastomosis between right atrium and main-left pulmonary artery. The stenosis was treated with balloon and stenting achieving large pathway. Our experience confirms that after a modified Fontan, if cardiac failure occurs, an immediate full investigation have to be done.     

心外管道全腔肺动脉连接术在复杂先心病中的应用

目的：总结心外管道全腔肺动脉连接（total cavopulmonary connection,TCPC）术治疗复杂先天性心脏病的临床经验。方法：2002年1月～2011年5月,采用外管道TCPC术矫治复杂先天性心脏病47例（病种包括三尖瓣闭锁、单心室、肺动脉闭锁、右心室发育不良、右室双出口）患者,手术年龄（8±4）岁。一期手术25例,分期手术22例。结果：术后早期（术后1月）死亡4例（8%）。死亡原因：多脏器功能衰竭、心脏骤停、感染、蛋白丢失性肠病和低心排。术后早期并发症发生率为17%,主要为肺水肿3例、胸腔积液2例、心律失常1例、心包积液1例、蛋白丢失性肠病和低心排1例。术后均随访,随访时间9～87月。远期再住院率为9%（4/43）,再次手术7%（3/43）。2例再次出现胸腔积液,2例因自行停服肠溶阿司匹林后出现外管道堵塞再次手术,1例于术后出现低心排死亡。其余患者恢复良好。结论：对于适应证明确的复杂先天性心脏病患者行心外管道TCPC手术治疗,近期临床效果满意。     

Doppler echocardiographic evaluation of pulmonary artery flow after modified Fontan operation: importance of atrial contraction.

Doppler echocardiography was used to evaluate blood flow in the pulmonary artery in 14 patients 2 to 42 months (mean (SD) 17 (12) months) after a modified Fontan operation incorporating a direct atriopulmonary anastomosis. Preoperatively six patients had tricuspid atresia, six had a double inlet left ventricle, and two had pulmonary atresia with an intact ventricular septum. The postoperative rhythm was sinus in 11 patients, junctional in one, ventricular pacing in one, and atrioventricular sequential pacing in one. In one patient the Doppler trace was unsatisfactory for analysis. In all patients forward flow in the pulmonary artery had biphasic peaks related to both atrial and ventricular contraction. The mean (SD) peak flow velocity that was synchronous with atrial contraction was 80 (30) cm/s and that synchronous with ventricular contraction was 74 (23) cm/s. The atrial contribution to total pulmonary artery flow, assessed by velocity-time integrals, varied between 22% and 73% (mean (SD) 45 (14)%). In patients with tricuspid atresia the mean (SD) peak flow velocity with atrial contraction was 90 (27) cm/s and that with ventricular contraction was mean (SD) 68 (24) cm/s. In patients with double inlet left ventricle the mean (SD) peak flow velocity was 67 (36) cm/s with atrial contraction and 80 (25) cm/s with ventricular contraction. The atrial contribution to total pulmonary blood flow in patients with tricuspid atresia was significantly higher (53 (11)%) than in those with double inlet left ventricle (37 (14)%). Pulmonary artery flow after modified Fontan operation was biphasic and was related to both atrial and ventricular contraction. The atrial contribution to pulmonary blood flow is greater in patients with tricuspid atresia than in those with a double inlet left ventricle. The mechanism of the second peak related to ventricular contraction is unknown.     

Doppler echocardiographic evaluation of pulmonary artery flow after modified Fontan operation: importance of atrial contraction

Doppler echocardiography was used to evaluate blood flow in the pulmonary artery in 14 patients 2 to 42 months (mean (SD) 17 (12) months) after a modified Fontan operation incorporating a direct atriopulmonary anastomosis. Preoperatively six patients had tricuspid atresia, six had a double inlet left ventricle, and two had pulmonary atresia with an intact ventricular septum. The postoperative rhythm was sinus in 11 patients, junctional in one, ventricular pacing in one, and atrioventricular sequential pacing in one. In one patient the Doppler trace was unsatisfactory for analysis. In all patients forward flow in the pulmonary artery had biphasic peaks related to both atrial and ventricular contraction. The mean (SD) peak flow velocity that was synchronous with atrial contraction was 80 (30) cm/s and that synchronous with ventricular contraction was 74 (23) cm/s. The atrial contribution to total pulmonary artery flow, assessed by velocity-time integrals, varied between 22% and 73% (mean (SD) 45 (14)%). In patients with tricuspid atresia the mean (SD) peak flow velocity with atrial contraction was 90 (27) cm/s and that with ventricular contraction was mean (SD) 68 (24) cm/s. In patients with double inlet left ventricle the mean (SD) peak flow velocity was 67 (36) cm/s with atrial contraction and 80 (25) cm/s with ventricular contraction. The atrial contribution to total pulmonary blood flow in patients with tricuspid atresia was significantly higher (53 (11)%) than in those with double inlet left ventricle (37 (14)%). Pulmonary artery flow after modified Fontan operation was biphasic and was related to both atrial and ventricular contraction. The atrial contribution to pulmonary blood flow is greater in patients with tricuspid atresia than in those with a double inlet left ventricle. The mechanism of the second peak related to ventricular contraction is unknown.     

The bovine jugular vein: a totally integrated valved conduit to repair the right ventricular outflow

BACKGROUND AND AIMS OF THE STUDY: Current techniques to correct valvular anomalies of the right ventricular outflow tract (RVOT) include repair and replacement of the pulmonary valve. However, the performance of currently used conduits has been less than ideal because of unfavorable hemodynamics and mid- to long-term complications. An early experience with a totally integrated Contegra valved conduit derived from a bovine jugular vein is reported; this conduit has the advantage that there is no discontinuity between its lumen and the valve it incorporates. METHODS: Between October 1999 and October 2001, a total of 22 Contegra valved conduits (12-22 mm) was implanted in 21 children aged <5 years, and in one patient aged 21 years. Diagnosis included tetralogy of Fallot (n = 13), pulmonary atresia (n = 3), double outlet right ventricle with pulmonary stenosis (PS) (n = 3), transposition of the great arteries, ventricular septal defect and PS (n = 2) and truncus arteriosus (n = 1). In 15 of these patients, distal and proximal anastomoses were performed on the beating heart. RESULTS: There was no mortality and no valved-conduit-related early morbidity. Intraoperative invasive assessment demonstrated excellent hemodynamic characteristics: mean peak pressure increase was 8.5+/-6.3 mmHg (varying between 4 mmHg in the 20-mm conduit and 18 mmHg in the 14-mm conduit). These values were confirmed by pre-discharge transthoracic pulsed-wave Doppler echocardiography. Because of endocarditis, one conduit was explanted after 11 months and replaced with a pulmonary homograft. Two patients required reintervention. CONCLUSION: The Contegra valved conduit is an excellent immediate substitute in the treatment of RVOT lesion when a pulmonary valve has to be inserted. Both systolic and diastolic valve functions are promising. Further data are required to confirm the favorable hemodynamics, as well as the durability and efficacy of this conduit in the long term.     

Bicuspidized pulmonary homograft for truncus arteriosus repair

Primary repair is preferable to palliation in infants with truncus arteriosus. At our institute, an appropriately small homograft valved conduit is not available for every patient; a bicuspidized pulmonary valve homograft is an alternative. Between December 1996 and August 2005, 24 patients aged 28 days to 21 months with truncus arteriosus underwent primary repair with a homograft valved conduit; bicuspidized homografts were used in 15 of them. In the 18 (75%) patients who survived to hospital discharge, 5-year survival was 94% (75% for tricuspid homografts and 100% for bicuspidized homografts, which was not significantly different). Freedom from reoperation or balloon angioplasty in all 18 survivors was 89% at 5 years. Freedom from reoperation in tricuspid and bicuspidized homograft groups at 5 years was 67% and 100%, respectively; the difference was not statistically significant. Bicuspidized homografts worked as well as tricuspid conduits in the intermediate term. The remodeled homografts showed excellent hemodynamic characteristics and appear to be a reasonable alternative when an appropriate size of valved homograft is unavailable.     

Tricuspid atresia with pulmonary atresia

The clinical presentation and long-term follow-up of 14 cases of tricuspid atresia associated with pulmonary atresia were reviewed. The electrocardiograms, hemodynamic findings and a definition of anatomic types were outlined in order to facilitate therapeutic decisions. In these types of tricuspid atresia the clinical presentation depends on the patent ductus. Despite the caliber of the ductus arteriosus, the poor tolerance to the malformation is frequent and the clinical presentation is similar to those malformations with decreased pulmonary blood flow (cyanosis since birth and hypoxic spells). In considering the age of these patients, the modified Blalock-Taussig anastomosis is the initial procedure. The Fontan procedure should be considered carefully as an alternative in older children.