Stefano M. Marianeschi

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.

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.

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.

Partial biventricular repair for complex congenital heart defects: an intermediate option for complicated anatomy or functionally borderline right complex heart

BACKGROUND Partial biventricular repair consists of bidirectional cavopulmonary anastomosis in the presence of separated systemic and pulmonary circulations, with antegrade flow of inferior caval return through an intact or reconstructed pulmonary outflow tract. This versatile procedure may be used as a definitive repair for patients with a functional right heart complex incapable of supporting an entire cardiac output or in patients with complicated anatomy. METHODS From July 1992 to April 1997, 23 patients (median age 5.2 years) underwent partial biventricular repair. In 15 of these cases the entire repair, including bidirectional cavopulmonary anastomosis, intracardiac repair, and right ventricular outflow reconstruction, was performed as a planned procedure at our institution. The other eight patients had previously been placed on a Fontan track and had undergone bidirectional cavopulmonary anastomosis; their circulations were converted to a partial biventricular circulation. RESULTS There were no early deaths. Complete atrioventricular block developed in two patients with straddling tricuspid valve. At a median follow-up of 17 months, there were no late deaths and three patients had undergone reintervention. Partitioning of the pulmonary arteries to create a classic Glenn anastomosis with antegrade flow to the left lung was performed in one case; another patient underwent an atrial septectomy, and the third patient required revision of tricuspid valve repair. All patients are in New York Heart Association functional class I. CONCLUSIONS Partial biventricular repair is a versatile strategy that can be used to manage a variety of forms of complex congenital heart disease. Cases for which the repair is useful include those in which complete biventricular repair is unlikely to be achieved because of limited size or function of the right side of the heart and those in which a patient with a ventricle capable of supporting inferior vena caval return was previously placed on a Fontan track.

Current strategies in tetralogy of Fallot repair: pulmonary valve sparing and evolution of right ventricle/left ventricle pressures ratio

OBJECTIVE Chronic volume overload in repair of tetralogy of Fallot (TOF) with transannular patch leads to significant late morbidity and mortality. Preserving pulmonary valve integrity offers a better long-term prognosis, despite a risk of residual stenosis. In our study we analyzed the evolution of pressure gradients in patients operated with conservative approaches, with particular regard to those babies with an immediate postoperative Prv/Plv ratio >or=0.70. METHODS Between January 2000 and June 2008, 24 patients with TOF underwent reparative surgery with a valve sparing procedure (median age 8.1 months, range 1.1-86.6). The intraoperative post-repair echocardiography showed a Prv/Plv ratio >or=0.70 in eight patients (33%, group A) and <0.70 in 16 patients (67%, group B). We realized a retrospective study of pre-, intra-, and postoperative data and of clinical and echocardiographic follow-up data. RESULTS There was no early or late mortality, nor functional or rhythmic disturbances. One patient required re-operation for residual stenosis at annular level at one year. After a median follow-up of 32.8 months (range 0.6-73.1), the Prv/Plv ratio decreased by 16% (p=0.001) in all patients. In group A the reduction was 28% (p=0.018) and in group B it was 12% (p=0.14). CONCLUSIONS After a valve sparing procedure there is a reduction of Prv/Plv ratio at medium-term follow-up; in our study this reduction was statistically significant in all patients and in the subgroup with higher postoperative ratios. A valve sparing strategy reduces pulmonary regurgitation, preserves RV function and decreases the incidence of late arrhythmias, which are the determinants of long-term outcome.

Balloon-expandable metallic stents in the management of tracheomalacia in neonates

Tracheomalacia is a challenging problem after operations for certain congenital heart malformations in neonates and infants. 1 It may often prevent the patients from being weaned from mechanical ventilation. Management of this condition remains difficult. Many surgical approaches have been suggested, although none of them is universally accepted. 2 Conversely, a major role could be played by stent implantation at the level of the malacic segment. However, both silicone stents and self-expanding metallic stents present major technical or physiologic problems in pediatric patients. 35 This article reports on our preliminary experience with the use of endovascular balloon-expandable metallic stents in the treatment of neonatal tracheomalacia after surgical repair of congenital heart malformations. Patient population. During 1993, three patients (Table I) were subjected to implantation of a balloon-expandable metallic stent at the airway level. The stent was used to treat tracheobronchial malacia that caused failure to wean from assisted ventilation after operations for congenital cardiovascular malformations. In each case, informed parental consent was obtained before stent implantation. Technique. The morphologic features of the malacic segment were evaluated by tracheography during spontaneous respiration. Peak respiratory pressure and pulmonary compliance were measured before and after stent implantation by a Capnomac Ultima monitor (Datex Medical Instrumentation, Tewksbury, Mass.). Endovascular balloon-expandable Palmaz-Schatz stents (Johnson & Johnson, New Brunswick, N.J.), 128 mm long, were used to support the tracheomalacic segment. The stent was mounted on the Lo-Bow (Mallinckrodt Medical, Inc., St. Louis, Mo.) 6 mm balloon guidewire (0.035 inch) and positioned, under fluoroscopic guidance, at the level of the malacic segment through the endotracheal tube used for mechanical ventilation. After the endotracheal tube had been withdrawn as far as possible, mechanical ventilation was briefly suspended and the balloon rapidly inflated to expand the stent. Statistical analysis. Morphologic and functional data were expressed as mean values _-2-1 standard deviation. Statistical analysis was performed by the paired Students t test. Results. Balloon-expandable stent implantation was simply, quickly, and successfully performed in all cases.

Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross Registry

Objective Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken. Methods 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross–Konno in 73. Results There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies. Conclusions Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft.

Pulmonary Valve Implantation With the New Shelhigh Injectable Stented Pulmonic Valve

BACKGROUND Pulmonary regurgitation (PR) occurs frequently after tetralogy of Fallot (TOF) repair, impairing long-term prognosis and necessitating reinterventions. Myocardial damage, invasiveness, and the risks of pulmonary valve replacement (PVR) therefore need to be minimized. The new Shelhigh Injectable Stented Pulmonic Valve (Shelhigh Inc, Union, NJ) allows implantation without cardiopulmonary bypass (CPB) under direct control. METHODS Twelve symptomatic patients (age, 21.3 +/- 12.5; range, 5.8 to 53.5 years) with severe PR and progressive right ventricular (RV) dilatation with dysfunction received the Shelhigh valve in sizes 21 (n = 1), 25 (n = 4), 27 (n = 3), 29 (n = 2), and 31 mm (n = 2). RESULTS Valve insertion was successful and hemodynamic performance excellent in all: peak systolic gradient, 14.5 +/- 4.6 (range, 10 to 20) mm Hg; mean gradient, 6.3 +/- 1.6 (range, 4 to 8) mm Hg. Four patients underwent concomitant procedures on CPB: one reduction plasty of a dilated main pulmonary artery, two tricuspid valve repairs, and one VSD closure. Early recovery was uneventful. There were no reoperations. During a mean follow-up of 5.4 +/- 4.3 months (range, 0.3 to 10.6 months) echocardiography showed good results, with low gradients and recovered RV function in all. All presented in New York Heart Association functional class 1 at the latest follow-up. CONCLUSIONS The Shelhigh valve allows easy PVR without CPB up to large valve sizes, with less invasiveness compared with a conventional approach. Further follow-up is needed to assess its durability and long-term performance.

Total cavopulmonary direct anastomosis: a logical approach in selected patients.

A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.

Pulmonary valve implantation using self-expanding tissue valve without cardiopulmonary bypass reduces operation time and blood product use

OBJECTIVE The study objective was to review our initial experience with newly developed off-pump pulmonary valve implantation techniques and compare outcomes with the conventional approach. METHODS Thirteen symptomatic patients with severe pulmonary regurgitation underwent pulmonary valve implantation, 6 without cardiopulmonary bypass (group 1: age, 28 ± 21 years; range, 12-62; body surface area range, 1.38-2.39 m(2)) and 7 with cardiopulmonary bypass (group 2: age, 23 ± 13 years; range, 10-46; body surface area range, 1.31-1.89 m(2)). Ten patients had previous repair of tetralogy of Fallot, and 3 patients had pulmonary valvotomy/valvuloplasty. RESULTS Mean operation times were 166 minutes (range, 110-240) in group 1 and 299 minutes (range, 221-375) in group 2 (P < .001). Hemoglobin level after chest closure was 13.4 and 9.8 g/dL in groups 1 and 2, respectively (P < .001). Postoperative chest drainage (median) was 78 and 300 mL in groups 1 and 2, respectively (P = .003). Blood product requirement was zero and 3 units (median) in groups 1 and 2, respectively (P < .014). There was no significant difference in postoperative ventilation time or lengths of intensive care unit and hospital stays between the 2 groups. Mean follow-up was 15 months; all patients are in New York Heart Association I/II. Echocardiography showed that peak velocity across the pulmonary valve was 2.2 and 2.0 in groups 1 and 2, respectively (P = .46). No patient had a paravalvular leak or more than mild pulmonary regurgitation. CONCLUSIONS Off-pump pulmonary valve implantation is a good alternative for pulmonary valve replacement. The procedure reduces operating time, blood loss, and blood product requirement.