One-and-a-half ventricle repair in adult patients: a word of caution.

OBJECTIVES: The "one-and-a-half ventricle repair" (1.5 VR) is applied to several congenital heart diseases with hypoplastic right ventricle (RV), with good functional and clinical results in infants and children, but feasibility in adult population is being explored. MATERIALS AND METHODS: This is a retrospective analysis of medical records of all adult patients undergoing 1 + 1/5 VR between 2000 and 2012. RESULTS: Five patients (M/F = 4/1, mean age of 42.3 years, range 29-66.8) underwent 1 + 1/5 VR. Underlying diagnoses were Ebstein's anomaly (EA) in three, hypoplastic RV and tricuspid valve (TV) in two. Mean TV annulus Z-score was -3.0; mean pulmonary vascular resistance was 1.6 WU/m(2) . Eleven associated procedures were performed simultaneously to 1.5 VR. There were no operative deaths. All patients were discharged home alive and in good clinical condition on anticoagulation regimen. At a mean follow-up of 8.6 ± 5.2 years, there was one cerebral stroke-related death; two patients experienced major complications. Two patients are in NYHA Class I, one is in NYHA Class II, and the last in NYHA Class III on a transplant list. CONCLUSIONS: The one-and-a-half ventricle repair in adult patients is feasible with low mortality. However, advanced age-associated co-morbidities and prolonged chronic RV preoperative dysfunction may jeopardize long-term results when compared to children.

One-and-a-half ventricle repair as a surgical alternative to Fontan revision in an adult.

We report a case of a young man with hypoplastic right ventricle, who presented with recurrent untreatable arrhythmias after a Bjork Fontan procedure in infancy. He underwent one-and-a-half ventricle repair as an alternative surgery to Fontan revision.

European Contegra multicentre study: 7-year results after 165 valved bovine jugular vein graft implantations.

OBJECTIVE: The valved bovine conduit "Contegra" for RVOT reconstruction became available for clinical use within a 100 % source data monitored and echo core lab controlled prospective European Multicentre Study, carried out from 1999 to 2006. We present the results of this study. METHODS: A total of 165 Contegras were implanted in 8 centres. The mean patient age was 3.9 years (2 days - 18 years, median 2.0). Total follow-up was 687 patient years. Diagnoses included: tetralogy of Fallot (64 patients, 39 %), truncus arteriosus (50, 30 %), double outlet right ventricle (16, 10 %), aortic valve disease/Ross procedure (11, 7 %), pulmonary valve atresia (10, 6 %), transposition of the great arteries (10, 6 %), 4 other malformations (2 %). Previous procedures were: 82 patients (50 %) - none; 37 (22 %) - valved conduit implantation; 14 (8 %) aortopulmonary shunt; 6 (4 %) catheter intervention. Follow-up appointments which included standardised echocardiography investigations were scheduled at 1, 3, 6, and 12 months, then annually. We evaluated freedom from death, explantation, intervention, stenosis, insufficiency, and degeneration. Results were stratified by age, diagnosis group and conduit size. RESULTS: The 5-year freedom-from rates were: explantation - 90 % (for patients aged 1 to 10 years) and 68 % (for younger patients); endocarditis - over 92 %; catheter intervention - 74 % (patients with congenital malformations); stenosis - 75 % and more (any group); insufficiency - 50 % (12 and 14 mm diameter conduits); any event - 13 % (patients under 1 year), 58 % (1 to 10 years), 82 % (> 10 years). Trace or mild insufficiency was a frequent, but not progressive finding. Mild calcification was detected in only 8 examinations. CONCLUSIONS: The performance of the Contegra conduit compares well with that of homografts when used to reconstruct paediatric right ventricular outflow tracts.

The use of "2-octyl cyanoacrylate" as skin adhesive in pediatric and congenital cardiac surgery.

AIM: The aim of this paper was to evaluate the safety and cost-effectiveness of "2-octyl-cyanoacrylate" as skin adhesive in congenital heart surgery. METHODS: From April 2010 to December 2011, we collected data from 300 patients who underwent cardiac surgery for congenital heart disease. We divided our population into 3 groups: group-1 (N.=100):"2-octyl-cyanoacrylate" has been used to replace the intra-dermal suture line; group-2 (N.=100):"2-octyl-cyanoacrylate" has been utilized as a barrier ("add-on measure") in addition to the intra-dermal suture line, group-3 (N.=100) with a standard intra-dermal suture line. RESULTS: Median age of patients was 1.36 years. One-hundred and thirty-nine patients were younger than 12 months and 56 older than 16 years. There were 11 wound dehiscence (3.6%) (2 in group-1 and 9 in group-3, P=0.001) and 1 superficial wound infection (group-1). Six patients (2%) required surgical wound revision (2 in group-1 and 4 in group-3, P=NS). Wound complication was significantly associated to delayed sternal closure (3/12 patients, 25% versus 13/288 patients, 4.5%) (P=0.04). Median cost (intra-/postoperative) for wound treatment was lower in group-1 and 2 (19±5.5 and 23.9±7.4 € respectively) when compared to Group-3 (26.7±3.2) (P<0.0001). CONCLUSION: The use of "2-octyl-cyanoacrylate" proved to be safe and effective; the "add-on measure" strategy provided the best cost-effective solution.

Comparison between echocardiographic subtraction method and first-pass radionuclide ventriculography for measuring right ventricular volume after operative "repair" of patients with tetralogy of Fallot.

In the long-term follow-up of patients after repair of tetralogy of Fallot, monitoring right ventricular function is mandatory. The echocardiographic subtraction method proposed by Tomita seems to be easily applicable to a pediatric age population and accurate enough to be included in the longitudinal surveillance of such a group of patients.

Aortic atresia with interrupted aortic arch: reparative operation.

Aortic atresia is a form of congenital cardiac disease always complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. Rarely, there is an associated ventricular septal defect (VSD) and, consequently, a well-developed left ventricle. This complex malformation is universally lethal and survived after a reparative operation has not been previously reported. The following is an account of an infant with aortic atresia, VSD, and Type C aortic arch interruption who presented with a unique ductus-dependent physiology and is clinically well 11 months following a reparative operation.

The surgical anatomy of the Taussig-Bing malformation.

There is no consensus as to the most appropriate definition of the use of the term Taussig-Bing malformation. In this study, we endorse the suggestion that the lesion be considered a spectrum of anomalies, unified by a juxtapulmonary ventricular septal defect with malalignment of the infundibular septum. The ends of the spectrum are hearts with, on the one hand, double-outlet right ventricle and, on the other hand, a discordant ventriculoarterial connection. We studied 10 hearts within this spectrum, eight with a double outlet and two with discordant ventriculoarterial connection. We found that the same basic morphology could exist with a bilaterally complete infundibulum or in the presence of fibrous continuity between the mitral and the pulmonary valves. Features of most significance for surgical correction were the arrangement of the outlet septum (particularly the presence or absence of chordal attachments from the atrioventricular valves), the morphology of the coronary arteries, and the state of the subaortic infundibulum. The options for surgical correction are determined by the internal morphology of the lesions rather than by the precise ventriculoarterial connection.

The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and noncommitted ventricular septal defect.

In describing hearts with double-outlet right ventricle, we have had problems with how best to use the term noncommitted as applied to the ventricular septal defect. We reviewed, therefore, 63 hearts with double-outlet right ventricle in the setting of usual atrial arrangement and a concordant atrioventricular connection. From these, 18 hearts with potentially noncommitted defects were identified and studied in detail. The defect was unequivocally remote from the ventricular outflow tracts in 16 of these hearts, being perimembranous with excavation to open into the inlet of the right ventricle in 12, two of these also having straddling of an atrioventricular valve. One heart had a muscular defect situated in the inlet part of the muscular septum, whereas the defect was the ventricular component of an atrioventricular septal defect in the other three. In the remaining two hearts the defect was anatomically juxtaposed to a subarterial outlet. The pathway to the outflow tract, however, was obstructed by leaflets of a straddling valve. Our study shows, therefore, the need to distinguish between anatomic "commitment" of the defect from the problems in terms of commitment that may confront the surgeon in the operating room. Not only does the distance between the interventricular communication and one of the subarterial outflow tract need to be assessed (the anatomic commitment), but also the presence and nature of any intervening extraneous tissues (the surgical commitment) requires assessment.

Experience with operation for total anomalous pulmonary venous connection in infancy.

Twenty patients presenting with total anomalous pulmonary venous connection (TAPVC) in infancy underwent cardiac repair. Four had associated major intracardiac anomalies (complex TAPVC) and 16 had isolated TAPVC. All patients with complex lesions died during or shortly after the operation; they all had pulmonary venous obstruction (PVO). The associated malformations were critical pulmonary stenosis (one case), multiple ventricular septal defects (VSDs) (one case), mitral atresia (one case), and asplenia syndrome with common atrioventricular valve, double-outlet right ventricle, and pulmonary stenosis (one case). Among the patients with isolated lesions there were two surgical deaths, both in infants less than 1 month of age, with severe PVO and subdiaphragmatic drainage. Statistical analysis of these data shows a strongly incremental risk of surgical death due to the presence of associated malformations. Analysis of just isolated TAPVC shows a possible incremental effect due to neonatal age (less than 1 month) and PVO, these two factors being not clearly separated. There have been no late deaths and no late complications among the 14 survivors followed from 7 months to 10 years (mean 44 months). An aggressive surgical approach regardless of the age, degree of pulmonary hypertension, and type of anatomic connection is advised for isolated TAPVC. Some caution is recommended for complex TAPVC, in which a much higher risk is anticipated, particularly when a palliative pulmonary vein-to-left atrium anastomosis is performed.

Long-term performance of the Hancock porcine bioprosthesis in the tricuspid position. A review of forty-five patients with fourteen-year follow-up.

Because little information is available regarding the clinical performance of the Hancock porcine bioprosthesis (Johnson & Johnson Cardiovascular, King of Prussia, Pa.) implanted in the tricuspid position, we reviewed the long-term follow-up of patients who had tricuspid valve replacement with this device. From March 1970 to December 1983, 45 patients had tricuspid valve replacement, either isolated (seven patients) or combined with replacement of other valves (38 patients) by means of a standard, glutaraldehyde-preserved Hancock porcine bioprosthesis. Follow-up ranged from 0.2 to 14.7 years (mean, 7.6 +/- 3.6 years) and was complete. The late mortality rate was 6.6% +/- 1.6%/pt-yr and the actuarial survival rate at 14 years was 23% +/- 9%. Reoperation because of structural deterioration of the tricuspid, the mitral, or both bioprostheses was performed in nine patients (3.7% +/- 1.2%/pt-yr) from 40 to 177 months (mean, 112 +/- 43 months) and resulted in no deaths. Actuarial freedom from structural deterioration of a Hancock tricuspid porcine bioprosthesis at 14 years is 68% +/- 13%. Morphologic examination of explanted porcine bioprostheses showed that those implanted in the tricuspid position had lower degrees of calcification and less severe structural changes than those simultaneously explanted from the mitral position. We conclude that the Hancock porcine bioprosthesis has an acceptable long-term durability and satisfactory performance after tricuspid valve replacement, and we continue to favor its use in the tricuspid position even in association with mechanical prostheses in the left side of the heart.

Repair of congenitally malformed mitral valve in children.

We report our experience with 30 children under 15 years of age treated surgically for congenital mitral valve disease from March 1972 to July 1986. Valve reconstruction was possible in 26 patients (87%), whereas in four the valve was replaced with a mechanical prosthesis. Five patients died in the hospital (16.6%), four after conservative operations and one after mitral valve replacement. There was only one late death in a child in chronic congestive heart failure. Three patients, treated conservatively, required valve replacement 2, 22, and 24 months, respectively, after the operation. Follow-up data reveal that 22 of 24 patients are asymptomatic 5 months to 15 years after operation. Two-dimensional echocardiographic studies were performed in 19 patients treated conservatively, 17 of whom are asymptomatic. Eleven of them have no signs of mitral regurgitation or stenosis, six show only mild mitral incompetence, and two have moderate mitral regurgitation or stenosis. Peak pulmonary artery pressure is within normal limits in all. Our results indicate that mitral valve reconstructive procedures for congenital valve dysplasia may be effective and reliable in children despite the frequent severity of valve malformation. Although no major prosthesis-related complications were observed in the present series, we believe that mitral valve repair should always be attempted in the pediatric population to avoid the drawbacks of the currently available prostheses.

Surgical management of double-outlet right ventricle.

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.

Pulmonary atresia, intact ventricular septum, and Ebstein anomaly of the tricuspid valve. Anatomic and surgical considerations.

Pulmonary atresia with intact ventricular septum is a disorder that involves the whole right ventricle. An associated Ebstein deformity of the tricuspid valve is found in 10% of the cases, further complicating the anatomy and the function of the right ventricle. From January 1966 to December 1990, pulmonary atresia, intact ventricular septum, and Ebstein deformity of the tricuspid valve were observed in 11 cases in our institution; four of them were necropsy findings and the remaining seven were patients treated surgically. Of the latter, two were boys and five were girls (age range 1 day to 18 days). Two patients were managed by pulmonary valvotomy, three by systemic-pulmonary artery shunt, and two had a combination of the two. Operative mortality was 57%. Eight hearts were available for morphologic study. All had viscero-atrial situs solitus, D-loop ventricular structure, and normally related great arteries. A correlation between the degree of tricuspid valve dysplasia and right ventricular cavity size was observed in all. Furthermore, in five cases the anterior leaflet of the tricuspid valve obstructed the right ventricle at the ostium infundibuli level. In two of our surgical patients, a protruding anterior tricuspid valve leaflet was identified and excised and both patients survived. Ebstein anomaly of the tricuspid valve further complicates surgical management and outcome of pulmonary atresia and intact ventricular septum. Potential obstruction at the ostium infundibuli level should always be considered during repair. Various degrees of right ventricular inlet dysplasia, always present in this complex, may contraindicate an anatomic correction. In these cases a modified Fontan-type procedure should be considered as a valid surgical option.

Postoperative pathology of complete atrioventricular defects.

Necropsy data on 15 patients, who died after operations for complete atrioventricular defect (CAVD), were reviewed in order to analyze the possible determinants of death. Two infants had undergone a palliative procedure--pulmonary artery banding (Group I); three patients had had prosthetic replacement of one or both atrioventricular (AV) valves (Groups II); and the remaining 10 had had conservative repair with plastic reconstruction of the AV valves (Group III). The most notable pathological findings related to death were as follows: Group I, left ventricular hypoplasia in both infants; Group II, prosthetic dysfunction in all patients; Group III, extracardiac diseases in two-patients and cardiac lesions in six. In Group III severe pulmonary vascular obstructive disease (PVOD) was observed as an isolated finding in two patients and in combination with residual intracardiac anomalies in another two. Analysis of the incidence of PVOD demonstrated that irreversible changes may occur within the first year of life in babies with Down's syndrome, and that these changes well correlate with the calculated pulmonary vascular resistance (PVR) values. From the results of this study we have reached the following conclusions: (1) Prosthetic valve replacement has to be avoided because of a high incidence of prosthesis-related complications and the effectiveness of conservative repair; (2) residual untreated anomalies may affect the outcome of operation and should be accurately recognized and weighted preoperatively; (3) PVOD can occur in CAVD even before the first year of life and can be reliably assessed by the measurement of PVR. High degrees of PVOD frequently interfere with the surgical success, particularly when residual intracardiac anomalies are present. Therefore, we suggest that surgical correction be planned, when indicated, during the first 6 months of life.

Tricuspid atresia versus other complex lesions. Comparison of results with a modified Fontan procedure.

Several modifications of the Fontan principle are currently applied to the treatment of tricuspid atresia with low mortality. The use of these modifications in other malformations has most frequently been associated with less satisfactory results. At our institution, from June 1977 to October 1986, 35 consecutive patients, whose ages ranged from 8 months to 20 years (median age 3.4 years), underwent a modified Fontan procedure. Twenty patients with a median age of 3.2 years (group I) having tricuspid atresia (16 patients) or hypoplastic right heart syndrome (four patients) were treated by means of a right atrium-pulmonary artery anastomosis (12 patients) or right atrium-subpulmonary chamber connection (eight patients). Fifteen patients (group II) with a median age of 3.6 years, having a single left ventricle (10 patients), left atrioventricular valve hypoplasia or atresia (three patients), or double-outlet right ventricle (two patients), underwent right atrium-pulmonary artery anastomosis, together with a repositioning of the atrial septum to the right of the right atrioventricular valve, which thus left intact the inlet to the ventricle(s). The operative mortality rate was 25% in group I and 0% in group II. One patient in group I and one in group II died late postoperatively. All the 28 survivors are free of symptoms 3 months to 9 years after correction. According to our results, low risk can be associated with modified Fontan procedures in the treatment of complex heart malformations other than tricuspid or pulmonary atresia. Preserving the integrity of the entire inlet to the ventricle(s) by repositioning the interatrial septum, as done in group II malformations, might be helpful in improving the quality of the repair.

Description of a continent jejunal gastrostomy.

A new technique of permanent gastrostomy interposes a vascularized jejunal conduit with an intussuscepted valve between the stomach and abdominal wall, creating a "continent jejunal gastrostomy". In a series of dogs undergoing gastrostomy, the continent jejunal gastrostomy functioned well, with no leakage, and was superior to gastric tube gastrostomy in this regard. Furthermore, there was no evidence of marginal ulceration. A patient who underwent continent jejunal gastrostomy has an excellent one-month result.

Cavopulmonary anastomosis in staging toward Fontan operation: pathologic substrates.

BACKGROUND: A review of the history and practice of cavopulmonary connections in staging toward Fontan operation and the pathologic experience at Padua University is presented. METHODS: Gross and histologic assessment of the heart-lung blocks removed at autopsy in the cases in which the cause of death could be related to a dysfunction of the cavopulmonary anastomosis were performed. RESULTS: The main complications were distortion of the intraatrial tunnel and thrombosis of the pulmonary branches of the cavopulmonary anastomosis. CONCLUSIONS: The cavopulmonary anastomosis remains a widespread procedure, both as a preliminary step to a Fontan operation and as an integral part of a Fontan or modified Fontan procedure in all those cardiac malformations characterized by a hypoplastic right or left ventricle in which these ventricles are too small to support the whole circulation.

Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy.

BACKGROUND: We present a new understanding of the anatomic position of apical ventricular septal defects and its surgical relevance. These defects occur between the left ventricular apex and the infundibular apex, rather than between the left and right ventricular apices. Often a sizable apical recess, the infundibular apex lies anteriorly and inferiorly to the moderator band and is the most leftward part of the right ventricle. METHODS: Four patients (2 boys and 2 girls) with a mean age of 109 days (range, 48 to 217 days) underwent patch closure through an apical infundibulotomy, which allowed complete visualization of the muscular apical ventricular septal defect. RESULTS: There were no early or late deaths at operation. No significant residual shunt at ventricular level was detected by postoperative two-dimensional and Doppler echocardiography. Intraoperative comparison of right atrial and pulmonary arterial blood samples showed a difference of less than 5%. At a mean follow-up of 18 months, all the patients are asymptomatic and growing well. CONCLUSIONS: The successful outcome of these 4 patients indicates that surgical closure of apical ventricular septal defects can be achieved safely and completely in early infancy through a limited right ventricular apical infundibulotomy. Long-term follow-up of these and similar patients is needed to provide further evaluation of this approach.

Left atrioventricular valve incompetence after repair of common atrioventricular canal defects.

BACKGROUND: Reoperation rate for residual atrioventricular (AV) valve regurgitation after repair of common atrioventricular canal defect (AVCD) is currently between 5% and 10%. This retrospective analysis evaluates the impact of AV valve anatomy, age at repair, and surgical technique on postoperative AV valve incompetence. METHODS: Between January 1982 and July 1994, 205 patients with common AVCD underwent surgical repair at our institution. A complete form with common atrioventricular orifice (CCAVCD) was present in 81 patients. One hundred twenty-four had separate AV valves and orifices; 22 of these had a restrictive ventricular septal defect component. Preoperative moderate to severe AV valve regurgitation (AVVR) was present in 74 (36%), with a lower incidence in the CCAVCD group (20/81, 25%) versus the transitional (8/22, 36%) or the partial forms (46/102, 45%) (p = 0.02). The incidence was 12.5% (3/24) for CCAVCD patients less than 4 months of age (p = 0.02). Repair of the left AV valve was performed according to the trifoliate approach in most cases (142/205, 70%). Follow-up was 98% complete. RESULTS: By Kaplan-Meier analysis, survival at 12.3 years was 97.8% +/- 1% in partial AVCD, 95.4% +/- 4% in transitional AVCD, and 73.2% +/- 5% in CCAVCD (median follow-up, 60 months). Freedom from reoperation at 12.3 years was 93.5% +/- 2% for partial AVCD, 76.9 +/- 9% for the transitional form, and 68.3% +/- 5% for CCAVCD: Postoperative moderate to severe AVVR occurred in 42 patients (21%), with lower incidence for CCAVCD (10/81, 12.5%) versus transitional AVCD (8/22, 36%) and partial AVCD (24/102, 24%) (p = 0.02). Postoperative moderate AVVR was found in only 1 patient with CCAVCD less than 4 months of age (p < 0.01). Nine patients (5%) underwent reoperation for residual postoperative AVVR Valve repair was performed in all with no operative death. By Cox proportional risk multivariate analysis, preoperative AVVR and double orifice "mitral" valve were associated with increased risk of postoperative left AVVR (p < 0.01), whereas a bifoliate approach appeared to reduce the risk of this event in partial AVCD (p = 0.03). CONCLUSIONS: Postoperative AVVR is related to the type of anatomy of the AV valve, to the age at repair, and to the surgical technique employed. Residual AVVR can still be corrected with conservative techniques at low mortality rates. Early repair of common AVCD is associated with a lower incidence of preoperative and postoperative AVVR in CCAVCD and seems to prevent progression of annular dilation and preoperative AVVR in partial AVCD.

Repair of ventricular septal defect and aortic regurgitation associated with severe hemophilia B.

A child with severe factor IX deficiency who underwent an open-heart operation using extracorporeal circulation is described. The factor IX level was normalized immediately before operation and at the end of cardiopulmonary bypass by infusing prothrombin complex concentrate and fresh frozen plasma. Partial thromboplastin time and factor IX serum levels were monitored for 20 days postoperatively and showed factor IX activity higher than 50%.