Results of 100 consecutive extracardiac conduit Fontan operations.

Throughout the years, the experience with Fontan's operation has increased and has opened the way to a much wider application of this principle. A number of major risk factors have been identified and managed by several modifications of the original Fontan procedure. In the past 15 years, operative and postoperative risks have been controlled to a major extent by the application of intermediate surgical procedures. Modifications to the original technique have been designed to simplify surgery and better protect the myocardium by avoiding, as much as possible, prolonged ischemic time. In 1988, we developed a new form of total extracardiac right heart bypass by means of associating an extracardiac conduit placed between the inferior vena cava (IVC) and the pulmonary artery with a bidirectional cavopulmonary anastomosis (BCPA)--the so-called total extracardiac cavopulmonary connection (TECC). Between November 1997 and October 2003, 100 patients with complex cardiac anomalies underwent a modified Fontan operation by TECC. In 88 patients, the repair was staged by preliminary BCPA that was bilateral in 15 patients and associated with a modified Damus-Kaye-Stansel procedure to bypass a subaortic obstruction in 24 patients. Early (in hospital) deaths occurred in 6% of patients and the extracardiac conduit was taken down in 2 additional patients for a total early failure rate of 8%. The cause of death was myocardial failure in 5 patients. Pulmonary artery distortion or hypoplasia appeared to be the cause of death in 1 patient and the cause of failure in 1 patient. Atrioventricular valve regurgitation was the cause of failure in 1 patient. Follow-up to December 2003 was available in all surviving patients. There were no late deaths. At follow-up, 87 patients (89%) were in New York Heart Association (NYHA) functional class I, 4 in class II, and 3 in class III due to moderate to severe atrioventricular valve regurgitation. Use of the following guidelines can result in the achievement of orthoterminal repair with complete separation of pulmonary and systemic circulation, with negligible early mortality and a long-term NYHA class I: (1) Not all patients with univentricular heart (UVH) should undergo the extracardiac Fontan procedure; (2) in UVH with favorable streaming, a Q (p)/Q (s) ratio of approximately 1 to 1, and mild cyanosis, natural history might be preferable to surgical history; (3) any form of fenestration is contradictory to orthodox application of Fontan's principle. Children in whom a planned fenestration seems necessary because of suboptimal conditions should instead undergo a combination of BCPA and associated forward pulmonary blood flow; and (4) negligible mortality should be considered mandatory in UVH, normal pulmonary arteries, and absent cardiomegaly after appropriated and correct staging.

Shelhigh No-React porcine pulmonic valve conduit: a new alternative to the homograft.

BACKGROUND: The Shelhigh No-React pulmonic valve conduit is a new porcine conduit that is glutaraldehyde-treated and detoxified using a proprietary heparin process. In our institution it has been implanted in 25 patients. The aim of this present contribution is to evaluate the short-term follow-up after its implantation. METHODS: From November 1997 to August 1999, 25 patients (mean age, 20.2 years; range, 0.6 to 28.3 years) were operated on using this conduit. Seventeen patients underwent a Ross procedure for aortic valve disease, with the conduits implanted in anatomic position; 6 patients underwent right ventricular outflow tract reconstruction; 2 patients underwent the Rastelli operation. The follow-up was complete. Preoperative and postoperative two-dimensional echocardiography data were collected. RESULTS: There were two non-conduit-related deaths. Two conduits needed to be exchanged because of an increase in the gradient. Overall, all patients were improved in terms of New York Heart Association class. Comparison of preoperative and postoperative two-dimensional echocardiography gradient showed significant improvement. At the 30-month follow-up, no calcification was seen on the explanted conduits or on the two-dimensional echocardiography, although many of the patients are children. CONCLUSIONS: The Shelhigh conduits seem to be an alternative to homograft especially in infants. These experiences are preliminary, and longer follow-up is required.

Analysis by mathematical model of haemodynamic data in the failing Fontan circulation.

Several late complications jeopardize the clinical performance of recipients of the Fontan operation. The underlying causes have been referred to disturbed flow dynamics in the cavopulmonary connections. Presumably, the large pressure drops occurring in the inferior and superior connections play a pivotal role in the pressure level of the entire circulation, especially in the venous. To address this issue, we retrospectively reviewed catheterization data of six patients with failing Fontan circulation and compared them with those of six patients with functioning Fontan circulation. The impact on the systemic and pulmonary pressure of the increase in the cavopulmonary connection resistances was studied through a steady-state mathematical model of the univentricular closed-loop circulation. In the patients with failing Fontan, pressure in the venae cavae was found to be significantly higher, especially at the inferior cava (19.3 +/- 2.2 versus 12.5 +/- 2.3 mmHg) with the pressure drop at the inferior cavopulmonary connection significantly increased (4.7 +/- 3.1 versus 0.33 +/- 0.82 mmHg). The proposed mathematical model permits us to clearly relate the pressure increase in the venae cavae to an increased resistance in the cavopulmonary connections. Therefore, the present analysis confirms that, to avoid possible congestion of venous circulation, the definitive palliation of univentricular heart should not cause pressure drops at the cavopulmonary connections.

Fast-track congenital heart operations: a less invasive technique and early extubation.

BACKGROUND: Many novel techniques have been described for "minimally invasive" congenital cardiac operations to achieve an improved cosmetic result. There is little information on incorporation of such techniques into fast-track congenital heart operations. METHODS: We have developed an approach to fast-track congenital heart operations, which includes a cosmetic approach for repair of congenital heart defects without sacrificing adequate exposure or requiring specialized equipment, along with a simple approach to intraoperative anesthetic management that allows extubation in the operating room. The heart is exposed through a short midline skin incision and a full median sternotomy. The conventional technique of cannulation is performed. Between October 1997 and January 1999, 88 patients were operated on with this method. Cardiac anomalies included simple and complex ostium secundum atrial septal defect, sinus venous atrial septal defect, partial atrioventricular septal defect, simple and complex ventricular septal defect, and bicuspid aortic valve stenosis. RESULTS: There were no operative or late deaths. The majority of patients were extubated in the operating room or within 2 hours of operation. No patient underwent reoperation and the mean length of hospital stay was 3.9 days. Sternal instability or wound infection were not observed. CONCLUSIONS: We believe that our approach to fast-track congenital heart operation is safe and effective. The surgical technique provides good exposure and has excellent cosmetic results. Moreover, it is easy to learn and, if necessary, the surgeon can quickly gain direct access to the heart. The anesthetic management facilitates early tracheal extubation and a shorter duration of postoperative stay.

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.

Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt.

A 21-year-old white woman, born with a univentricular heart, had undergone staged procedures before Fontan correction. She then began to develop edema, protein-losing enteropathy, and ascites refractory to diuretic therapy. Cardiac angiography showed a patent right Blalock-Taussig shunt, with turbulent cavopulmonary circulation. After undergoing an unsuccessful attempt at coil embolization she then underwent shunt ligation, with resolution of symptoms and normalization of protein levels. This report draws attention to the importance of cavopulmonary laminar flow to prevent the development of protein-losing enteropathy.

Intrapericardial teratoma in a newborn: a case report.

Intrapericardial teratomas are unusual tumors that often cause respiratory distress and might be lethal in the newborn. The purpose of this article is to present the clinical and pathological findings in a 12-day-old male successfully treated for a big intrapericardial teratoma. Given that the diameter of this tumor is generally related to the age at the time of diagnosis, the rarity of our case is the presence of a huge intrapericardial teratoma in a newborn. The surgical resection was lifesaving.

[The conversion of a modified Fontan procedure to a total extracardiac cavo-pulmonary conduit. The Medico-Surgical Cardiology Group]. / Conversione della procedura di Fontan modificata a connessione cavopolmonare totale extracardiaca. Raggrupamento di Cardiologia Medico Chirurgica.

BACKGROUND: After a modified Fontan procedure with atriopulmonary or atrioventricular conduit, some patients present stress intolerance, supraventricular arrhythmia, recurrent pleuropericardial or ascitic effusions, and protein-losing enteropathy, all of which are signs that the previous procedure has failed. The aim of this study was to evaluate the midterm outcome after surgical therapy for this condition. MATERIAL AND METHODS: Between August 1994 and December 1997, nine patients (6 males and 3 females), age 10 to 39 (mean 21.5) years, underwent conversion of previous modified Fontan procedure to total extracardiac cavo-pulmonary connection. Time from the previous procedure was 6 to 18 years (mean 10). Diagnosis was tricuspid atresia with pulmonary stenosis (n = 2), double-inlet left ventricle and concordant ventriculoarterial connection (n = 3), double-inlet left ventricle and discordant ventriculoarterial connection (n = 3), Holmes heart (n = 1). Nine patients presented decreased stress tolerance, seven had arrhythmia, five had pleuropericardial effusions and two had protein-losing enteropathy. In all but one patient, right atrial pressure was higher than 15 mmHg, while in six patients the cardiac index was less than 2 l/min/m2. A polytetrafluoroethylene non-valved conduit was interposed between the inferior vena cava and the right pulmonary artery for conversion in all patients. A bidirectional cavo-pulmonary anastomosis (modified Glenn) was associated in all patients. Evaluation was done by NYHA Class and by an arbitrary score assigned to patients based on 7 parameters. RESULTS: There was no perioperative mortality. All patients were clinically improved at a mean follow-up of 24 months (range: 3 to 46). No patient had effusions, and the arrhythmias disappeared in 4 patients and were controlled by medical therapy in one. The two patients with protein-losing enteropathy improved markedly within 30 days and the score dropped below 10 points. CONCLUSIONS: The conversion of the modified Fontan procedure to total extracardiac cavo-pulmonary connection improves clinical condition by decreasing the right atrium-pulmonary gradient and right atrial preload, and by providing a laminar cavo-pulmonary flow without any need for intracardiac anastomoses. This procedure should be undertaken early in this subset of patients, before ventricular failure ensues.

[The role of a bidirectional cavopulmonary anastomosis in the correction and palliation of complex congenital cardiopathies]. / Ruolo dell'anastomosi cavo-polmonare bidirezionale nella correzione e palliazione delle cardiopatie congenite complesse.

Between May 1990 and January 1998, 68 patients underwent bidirectional cavo-pulmonary anastomosis. We evaluated all patients in whom the bidirectional cavo-pulmonary anastomosis was associated with additional pulmonary flow (group A) and those in whom it was associated with biventricular repair (group B). Group A included 23 patients (33.8%), 14 males and 9 females, mean age 25 years and 6 months (range 4 months-16 years). Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). Group B included 13 patients (19.1%), 6 males and 7 females, mean age 13 years and 7 months (range 1 year-37 years). Diagnoses were pulmonary atresia with intact ventricular septum (4), Ebstein's anomaly (3), tetralogy of Fallot (3), atrioventricular canal (1), hypoplastic right ventricle (1), and pulmonary and tricuspid insufficiency (1). Four patients (17.3%) in group A died in the postoperative period, whereas there was no postoperative mortality in group B. Follow-up data were available in 31 patients (19 from group A, 13 from group B). Mean follow-up was 1 year and 6 months (range 30 days to 6 years). Evaluation was done by NYHA class functional status. In group A, 14 patients are doing well (NYHA I or II), while five patients (26.3%) underwent Fontan operation with one death. All group B patients are currently doing well (NYHA class I or II). In group A, complications were pericardial effusion (7), transient superior vena cava syndrome (5), pleural effusion (4), chylothorax (1) and rhythm disturbance (1). Complications in group B involved neurological events (2), pleural effusion (1) and rhythm disturbance (1). Bidirectional cavo-pulmonary anastomosis can be associated with additional pulmonary flow with good short- and intermediate-term outcome. Concern remains for the ability to properly regulate the amount of effective pulmonary blood flow. Bidirectional cavo-pulmonary anastomosis can be associated with biventricular repair in patients with diminutive right ventricles, amenable to anatomic complete correction, with good clinical outcome.

Aortic pulmonary autograft implant: medium-term follow-up with a note on a new right ventricular pulmonary artery conduit.

BACKGROUND: The Ross operation has been applied to various aortic valve pathologies, particularly when somatic growth is an issue. However, associated cardiac disease and technical problems may limit its use with regard to associated procedures and issues of right ventricular outflow reconstruction. MATERIALS AND METHODS: From December 1992 to March 1998, 24 patients underwent aortic pulmonary autograft implantation. There were 14 males and 10 females, 15+/-10 years of age (mean +/- SD) (range 1 to 50 years), weighing 42.8+/-20 kg (mean +/- SD) (range 8 to 78 kg). Aortic insufficiency was present in 15 (62.5%) patients, stenosis in 8 (33.3%) patients, and valvar stenosis associated with left ventricular outflow tract obstruction in 1 (4.1%) patient. Etiology was rheumatic in 17 patients and congenital in 7. The Ross procedure was accompanied by a partial-Konno left ventricular outflow enlargement in one patient, and mitral valve annuloplasty, mitral commissurotomy, and tricuspid valve replacement in three other patients, respectively. The right ventricular outflow was reconstructed with a valved pulmonary homograft in 14 patients and with a Shelhigh No-React porcine pulmonary conduit in 10 patients. Evaluation was done by New York Heart Association (NYHA) Class and by echocardiography at a follow-up of 22.8+/-24 months (mean +/- SD) (range 3 to 63 months). RESULTS: There were no operative mortalities and no postoperative arrhythmias. One (4.1%) patient required intra-aortic balloon pump (IABP) support for 3 days, one (4.1%) patient died 2 years later of probable arrhythmia, and one (4.1%) patient required mechanical aortic valve replacement 2 years later for severe autograft insufficiency. Left ventricular ejection fraction was unchanged (preoperative 62.4%+/-30%, postoperative 64.2%+/-30% [mean +/- SD], [p = NS]) and no significant gradient was documented by echocardiographic Doppler in the right and left ventricular outflow tracts. The aortic insufficiency scale decreased from a mean of 3.9+/-0.2 to a mean of 1+/-0 (p < 0.01). NYHA Class decreased to I in all patients, from III (10) and II (14). CONCLUSIONS: The pulmonary autograft in the aortic position is suitable for aortic valve replacement in pediatric and adult patients with good medium-term results and in patients with rheumatic etiology, and it provides a desirable solution in the presence of associated pathologies, such as left ventricular tract obstruction or associated multivalvular disease. The development of new means of right ventricular outflow reconstruction must parallel the progress achieved for the left side.

Primary repair of isolated ventricular septal defect in infancy guided by echocardiography.

Between 1989 and 1995, 96 consecutive infants affected by isolated ventricular septal defect (VSD) were submitted for primary correction at a median age of 4 months. Of the 96, 60 (group I) underwent surgery on the base of a 2-dimensional Doppler echocardiographic (DD echo) test alone. The preoperative DD echo anatomic definition of the type of VSD was confirmed at surgery in all 60 patients without false-positive results in terms of additional cardiac anomalies. There were 2 false-negatives: in 2 patients an associated cardiac anomaly was not detected by DD echo and required a second surgical procedure after postoperative cardiac catheterization. During the same period 36 infants (group II) underwent surgical closure of isolated VSD on the basis of cardiac catheterization and angiocardiography in addition to DD echo. The retrospective comparison between the 2 groups revealed no significant difference in terms of sensitivity and specificity of the diagnostic tools, early and late mortality after surgical correction, postoperative hospital stay, and need for late cardiac catheterization and surgery. We conclude that after an accurate selection, most of the infants with uncomplicated VSD can safely undergo primary repair on the basis of DD echo alone.

Extracardiac Fontan operation for complex cardiac anomalies: seven years' experience.

METHODS: Between 1988 and 1995, 60 patients with complex cardiac anomalies underwent a total extracardiac cavopulmonary connection, a combination of a bidirectional cavopulmonary anastomosis with an extracardiac conduit interposition between the inferior vena cava and pulmonary arteries, except in one patient in whom direct anastomosis was possible. In 40 patients the total extracardiac cavopulmonary connection followed preliminary bidirectional cavopulmonary anastomosis, associated with a modified Damus-Kaye-Stansel anastomosis in 16. The conduits were constructed of Dacron fabric (n = 34), homografts (n = 3), and polytetrafluoroethylene (n = 22). RESULTS: Total early failure rate was 15% (n = 9). Six patients died, and three more had conduit takedown owing to pulmonary artery stenosis and hypoplasia (n = 2) and severe atrioventricular valve regurgitation (n = 1). Two other patients required anastomosis revision owing to stricture. In a mean follow-up of 48 months (6 to 86 months) there were no late deaths (actuarial 5-year survival 88% +/- 4%); 52 of 54 patients are in New York Heart Association class I or II. Two patients required pulmonary artery balloon dilation or stent implantation, or both, after total extracardiac cavopulmonary connection. Late tachyarrhythmias were detected in four of 54 patients: two had sick sinus syndrome with flutter necessitating a pacemaker implantation and two had recurrent flutter (actuarial 5-year arrhythmia-free rate 92% +/- 4%). Conduit patency was evaluated by serial magnetic resonance imaging studies. Preliminary data showed a 17.8% +/- 7.6% mean reduction in conduit internal diameter during the first 6 months after total extracardiac cavopulmonary connection, with no progression over the next 5 years. CONCLUSION: These results demonstrate that the total extracardiac cavopulmonary connection provides good early and midterm results and may reduce the prevalence of late arrhythmias in patients undergoing the Fontan operation.

[Combined heart-kidney transplantation in pediatric age]. / Trapianto combinato cuore-rene in età pediatrica.

A case of simultaneous heart and kidney transplantation is presented in a 12 years old girl. The patient suffered from dilated cardiomyopathy and end-stage kidney disease. Bilateral vesico-renal refluxes were corrected at the same time of the duplex transplantation. A multiorgan harvesting from a pediatric 8 years old donor was utilized. Follow-up is 40 months. Cytomegalovirus (CMV) infection and acute tubular necrosis from haemoglobinuria were transient complications. Hemolysis from bypass and extracorporeal circulation was involved as pathogenetic factor of the acute tubular necrosis. Cardiac and renal function remain excellent and the child is fully recovered and resumed a normal and active life. Combined heart and kidney transplantation may be of benefit in selected patients, also in pediatric age.

Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options.

BACKGROUND: An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure. METHODS: From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter. RESULTS: There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy. CONCLUSIONS: Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.

[Functional evaluation by treadmill in children and adolescent following correction of Tetralogy of Fallot]. / Valutazione funzionale al treadmill in bambini ed adolescenti dopo correzione di Tetralogia di Fallot.

AIM OF THE STUDY: The Authors have examined 22 children (16m and 6f), mean age 9.64 +/- 2.63 years, range 5-15, after total correction for Tetralogy of Fallot, to evaluate the response of their cardiovascular apparatus during an exercise testing on treadmill (Bruce protocol). METHODS: Parameters examined: exercise duration, maximal heart rate (HR), maximal systolic blood pressure (max BP), non invasive cardiac output at rest and at the peak of exercise (CO), arterial oxygen saturation; lung function test at rest, echocardiogram and 24 hours Holter monitoring. CONTROL GROUP: 22 healthy peers, same gender, height and weight, not practising competitive sports. RESULTS: The exercise duration was significantly lower in the first group (77.8 +/- 11.8%; 86.5 +/- 8.2%; P = 0.006). Also max HR and max BP were significantly lower (max HR: 162 +/- 12 b/m'; 187 +/- 8 b/m'; P = 0.000), (max BP: 119 +/- 9 mm Hg; 126 +/- 12 mm Hg; P = 0.042). There were no differences for CO at rest (3.70 +/- 1.09 l/m'; 3.95 +/- 1.07 l/m'). In the first group, CO at peak of exercise was lower but not significantly (6.51 +/- 2.56 l/m'; 7.95 +/- 2.77 l/m'; NS). CONCLUSIONS: These results make more complete the not invasive functional evaluation for a better follow-up of these patients and a better choice for their physical activity.

Myocardial rhabdomyoma with rhabdomyoblastic moiety.

The clinical, histologic, histochemical, immunohistochemical, and ultrastructure studies of a primary neoplasia of the myocardium with onset in a 14-year-old boy led to the conclusion that this was a benign neoplasia of myocardial origin, apparently not previously described. The authors propose to call this entity "myocardial rhabdomyoma with rhabdomyoblastic moiety".