Pulmonary vasodilator therapy and early postoperative outcome after modified Fontan operation.

Although mortality is low after the modified Fontan procedure, there is a significant percentage of patients with prolonged postoperative recovery. The objective of this study is to evaluate the usefulness of postoperative administration of oral sildenafil and inhaled nitric oxide on early postoperative outcome. A prospective interventional and comparison study with a historical cohort was conducted. Between January, 2010 and March, 2013, 16 patients received oral sildenafil during immediate modified Fontan postoperative period. Inhaled nitric oxide was also administered if the patient was kept intubated 12 hours after surgery. Early postoperative outcome was compared with a historical cohort of 32 patients on whom the modified Fontan procedure was performed between March, 2000 and December, 2009. Postoperative administration of sildenafil and nitric oxide had no influence on early postoperative outcome after the modified Fontan procedure in terms of duration of pleural effusions, mechanical ventilation time, length of stay in the ICU, and length of hospital stay.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study.

BACKGROUND: To evaluate indications and results of surgery for primary cardiac tumors in children. METHODS AND RESULTS: Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). CONCLUSIONS: Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

The role of airway stenting in pediatric tracheobronchial obstruction.

OBJECTIVE: Tracheobronchial obstruction is infrequent in the pediatric age group but it is associated with significant morbidity and mortality. The purpose of this study is to review the results of a single institution experience with endoscopic stent placement in children with benign tracheobronchial obstruction, and with special concern on safety and clinical effectiveness. MATERIALS AND METHODS: Twenty-one patients with severe airway stenosing disease in which stent placement was performed between 1993 and 2006. Inclusion criteria according to the clinical status were: failure to wean from ventilation, episode of apnea, frequent respiratory infections (>3 pneumonia/year), and severe respiratory distress. Additional criteria for stent placement were: failure of surgical treatment, bronchomalacia, and tracheomalacia refractory to previous tracheostomy. Selection of the type of stent depended on the site of the lesion, the patient's age, and the stent availability when time of presentation. The following variables were retrospectively evaluated: age, type of obstruction, associated malformations, stent properties, technical and clinical success, complications and related reinterventions, outcome and follow-up period. RESULTS: Thirty-three stents were placed in the trachea (n=18) and/or bronchi (n=15) of 21 patients with a median age of 6 months (range, 9 days-19 years). Etiology of the airway obstruction included severe tracheomalacia and/or bronchomalacia in 19 cases (90%), and postoperative tracheal stenosis in two. Twelve children had a total of 20 balloon-expandable metallic stents placed, and 10 had 13 silicone-type stents (one patient had both). In nine patients (42%) more than one device was placed. Stent positioning was technically successful in all but one patient. Clinical improvement was observed in 18 patients (85%) but complications occurred in five of them (27%). Eight patients died during follow-up but only in one case it was related to airway stenting. Thirteen patients (62%) are alive and in good condition with a mean follow-up of 39 months (1-13.8 years). CONCLUSIONS: Although the results were based on a small series, placement of stents in the pediatric airway to treat tracheobronchial obstruction seems to be safe and effective. Stenting is a satisfactory therapeutic option when other procedures have failed or are not indicated.

Mitochondrial membrane potential and reactive oxygen species content of endothelial and smooth muscle cells cultured on poly(epsilon-caprolactone) films.

A transitory but significant stimulation of mitochondrial activity, increase of reactive oxygen species (ROS) and oxidative stress were previously observed in L929 fibroblasts cultured on poly(epsilon-caprolactone) (PCL) films. ROS, mainly formed in mitochondria, play a physiological role but an excessive production can promote endothelial dysfunction, cause oxidative injury to vascular cells, oxidize lipoproteins and accelerate atherothrombogenesis. On the other hand, mitochondria have a crucial position in programmed cell death control and are responsible for ATP synthesis through the coupling of oxidative phosphorylation to respiration. This coupling requires the existence of a mitochondrial membrane potential (Deltapsi(m)). The aim of the present study was to evaluate by flow cytometry the ROS content and Deltapsi(m) of both endothelial (EC) and smooth muscle cells (SMC) cultured on PCL films as a potential substrate for vascular graft development. Cell size, internal complexity and cell cycle were also analyzed to detect the possible appearance of the subG(1) cell fraction, characteristic of apoptotic cells. The effect of treating PCL films with NaOH before culture was also studied. PCL decreases the ROS content of EC during the culture but produces an increase of these levels in SMC after 7 days. PCL also induces variations of Deltapsi(m) which show a significant parallelism with the changes observed in ROS levels proving the importance and sensitivity of these measurements as indicators of the mitochondrial function. The treatment of PCL with NaOH decreases these effects demonstrating the benefits of increasing the surface hydrophilicity before cell culture which improves cell adhesion and proliferation and reduces oxidative stress. Since no important changes have been detected in subG(1) fraction of EC and SMC cultured on either PCL or PCL-NaOH, the changes of Deltapsi(m) observed in the present study cannot be related to apoptosis. These results confirm the potential utility of PCL as a suitable scaffold in Vascular Tissue Engineering.

Treatment strategies in the management of severe complications following slide tracheoplasty in children.

OBJECTIVES: This study focuses on the different surgical and endoscopical treatment alternatives when dealing with severe complications after slide tracheoplasty (STP). METHODS: Retrospective study of patients with symptomatic congenital tracheal stenosis (CTS) admitted to a single institution, between January 1997 and January 2013, surgically treated by means of STP. The following variables were evaluated: demographics, preoperative tracheal stenosis characteristics, associated anomalies and outcome measures. RESULTS: Cohort included 14 patients (8 males and 6 females) with a mean age of 8.7 months when treated (range, 1-43 m). Eleven patients (78%) showed a long segment CTS (>30% of total tracheal length) and 9 (64%) had associated cardiac or great vessel anomalies (left pulmonary artery sling). Three patients (21%) showed severe postoperative complications that required significant airway reintervention: tracheal resection of a restenotic segment, laser division with balloon dilatation of a residual stenosis and placement of a biodegradable endotracheal stent in an extensive tracheal narrowing. All patients are in good clinical condition with a mean follow-up of 6.3 years (range, 2 months to 16 years). CONCLUSIONS: STP has become the procedure of choice when dealing with CTS. Although it shows clear advantages compared with other surgical techniques, severe and difficult to manage complications may occur. Surgeons involved in their treatment should be familiar with diverse surgical and endoscopical procedures. Biodegradable airway stenting is a new and promising technique when long and severe post-surgical tracheal stenosis is present.

Results of reparative surgery for tetralogy of Fallot: data from the European Association for Cardio-Thoracic Surgery Congenital Database.

OBJECTIVES: Right ventricular (RV) dysfunction and pulmonary insufficiency (PI) after tetralogy of Fallot (TOF) repair may contribute to early and late morbidity and mortality. RV dysfunction may be related to the ventriculotomy employed in the transventricular repair technique, particularly when it is combined with a transannular patch (TAP). Transatrial/transpulmonary (TA/TP) repair without ventriculotomy has been advocated as a method potentially diminishing such adverse events. However, the prevalence and early as well as the late results of these different surgical approaches in Europe have not been studied. To ascertain 'the current prevalence and associated early mortality' of various surgical approaches for repair of TOF, relevant data in the European Association for Cardio-Thoracic Surgery Congenital Database were analysed. METHODS: The study population was all types of reparative operations (n = 6654) for patients with primary diagnosis of TOF reported between 1999 and 2011. RESULTS: Overall hospital mortality (HM) was 2.58% (172 of 6654). Repair via ventriculotomy with TAP was the most prevalent technique (n = 3827, 57.5%), with HM 3.11%. Repair via ventriculotomy with non-TAP was performed in 1309 patients (19.7%, HM = 1.53%). Repair without ventriculotomy was performed in 1214 patients (18.2%, HM = 1.48%). Logistic regression analysis showed statistically significant association between HM risk and the type of surgery. In particular, ventriculotomy with TAP is associated with increased mortality risk significantly compared with ventriculotomy with non-TAP (crude odds ratio [OR] 2.13 (95% confidence interval [CI]: 1.33-3.63). Similar results were obtained by analysing for operative mortality risk (30-day mortality, OM). Operations that have been performed before 2005 have resulted in increased surgical risk compared with those performed after 2005 (ORs for OM 1.45, 95% CI: 1.03-2.013). CONCLUSIONS: Overall HM for TOF repair is low. TOF repair by means of ventriculotomy with TAP is the most prevalent approach and is associated with higher mortality. Repair with ventriculotomy but no TAP and repair without ventriculotomy are both less prevalent and with lower mortality. Surgical risk appears to be decreasing over time.

Fontan operation. Hemodynamic factors associated with postoperative outcomes.

INTRODUCTION AND OBJECTIVES: The Fontan operation is usually the final palliative procedure in patients with univentricular heart. The objectives of this study were, firstly, to describe the clinical and haemodynamic characteristics of a group of patients with univentricular physiology who had previously been palliated with a bidirectional Glenn procedure and, secondly, to identify risk factors that can influence postoperative outcomes after the Fontan operation. METHODS: Retrospective study with 32 patients who underwent a Fontan operation between March 2000 and December 2009. Clinical characteristics, catheterization data, type and duration of surgery were revised and analyzed as predictors of postoperative outcome. RESULTS: Hospital mortality was 3%. After a median follow-up of 44 months (interquartile range, 32-79), survival was 90%. Preoperative mean pulmonary artery pressure (measured during catheterization) was correlated with late mortality. Of the remaining variables analyzed, the Nakata and McGoon indices, and duration of cardiopulmonary bypass showed the highest correlations with postoperative outcomes. Interventional catheterization before the Fontan operation was performed in 42% of patients. CONCLUSIONS: Hospital mortality after the Fontan operation was very low. The performance of a haemodynamic study before the Fontan operation made it possible to select high-risk patients for surgery as well as permitting the performance of interventional procedures that could improve postoperative outcome in these patients.

Left arm underdevelopment secondary to an isolated left subclavian artery in tetralogy of Fallot.

The anomalous origin of the left subclavian artery is known to be associated with right aortic arch and tetralogy of Fallot. In our case, the left subclavian artery arose from the left pulmonary artery. Therefore, the left arm was perfused by poorly oxygenated blood from pulmonary arteries and some retrograde vertebral artery flow. Thus, the left arm was cyanotic and less developed than the right one. The patient underwent surgical repair with complete correction of tetralogy of Fallot and reimplantation of the left subclavian artery to the left carotid artery.

Surgery for complications of trans-catheter closure of atrial septal defects: a multi-institutional study from the European Congenital Heart Surgeons Association.

OBJECTIVE: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). METHODS: The records of all (n=56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. RESULTS: A wide range of ASD sizes (5-34mm) and devices of various types and sizes (range 12-60mm) were involved, including 13 devices less than 20mm. Complications leading to surgery included embolisation (n=29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n=12), significant residual shunt (n=12), aortic or atrial perforation or erosion (n=9), haemopericardium with tamponade (n=5), aortic or mitral valve injury (n=2) and endocarditis (n=1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% (p=0.001). CONCLUSIONS: Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory.

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.

Modification of the Norwood procedure in isomeric complete atrio-ventricular canal and hypoplastic right aortic arch.

We report a modification of the Norwood stage I procedure in a neonate with right aortic arch and complete atrio-ventricular canal in a variant of hypoplastic heart syndrome. Because of the unusual anatomy, the neo-aorta was reconstructed with a specially trimmed homograft patch and the pulmonary flow was maintained with implantation of a right ventricle to pulmonary artery shunt on the left side of the aorta. The patient had a favourable outcome and is now awaiting the stage II procedure.

The arterial switch operation in Europe for transposition of the great arteries: a multi-institutional study from the European Congenital Heart Surgeons Association.

OBJECTIVES: This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. METHODS: The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. RESULTS: A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. CONCLUSIONS: Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis.

Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association.

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.