Search

Acute myocarditis leading to severe heart failure in paediatric patients is an uncommon but potentially life-threatening condition. The prompt implant of mechanical circulatory devices such as veno-arterial extra-corporeal membrane oxygenation remains the best treatment option to restore an adequate perfusion and improve patient survival in case of refractory cardiogenic shock cases. While few reports describe the in-hospital course of this dramatic disease, with an in-hospital mortality under veno-arterial extra-corporeal membrane oxygenation support around 30%, our study aims to analyse both short- and long-term outcomes after extra-corporeal membrane oxygenation implant.

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database. Primary outcome was cohort's mortality, also considering different decades, while secondary outcomes were postoperative complications and reintervention. In the last 3 decades, there were 897 patients undergoing Fontan operation, M/F 512/384, median age: 4.5 years (IQR 3.3-6.4), median weight 16 kg (IQR 14-22). A first palliation was deemed necessary in 710 patients (80%), and most patients underwent a staged Fontan (93%); an extracardiac conduit was used in 790 patients (88%). Postoperative complications (mild to severe) occurred in 410 patients (46%), and early reinterventions were required in 66 patients (7.5%). Overall operative mortality was 1.7% (15 patients). Age at Fontan greater than 4 years was associated with an early need for transcatheter reintervention (adj p value = 0.037) and a higher incidence of postoperative complications (adj p value = 0.017). The Fontan operation has seen significant improvements in immediate outcomes, notably a remarkable reduction in overall mortality to just 1.35% in the last decade. While minor complications have remained steady, there has been a substantial decrease in major early complications, deaths, and the need for reinterventions. Notably, patients aged over 4 years seem to face a higher risk of postoperative morbidity, underscoring the critical role of age in preoperative assessment and management strategies for Fontan patients., (© 2024. The Author(s).)

Pulmonary valve replacement (PVR) consists of substituting a patient's original valve with a prosthetic one, primarily addressing pulmonary valve insufficiency, which is crucially relevant in Tetralogy of Fallot repairment. While extensive clinical and computational literature on aortic and mitral valve replacements is available, PVR's post-procedural haemodynamics in the pulmonary artery and the impact of prosthetic valve dynamics remain significantly understudied. Addressing this gap, we introduce a reduced Fluid-Structure Interaction (rFSI) model, applied for the first time to the pulmonary valve. This model couples a three-dimensional computational representation of pulmonary artery haemodynamics with a one-degree-of-freedom model to account for valve structural mechanics. Through this approach, we analyse patient-specific haemodynamics pre and post PVR. Patient-specific geometries, reconstructed from CT scans, are virtually equipped with a template valve geometry. Boundary conditions for the model are established using a lumped-parameter model, fine-tuned based on clinical patient data. Our model accurately reproduces patient-specific haemodynamic changes across different scenarios: pre-PVR, six months post-PVR, and a follow-up condition after a decade. It effectively demonstrates the impact of valve implantation on sustaining the diastolic pressure gradient across the valve. The numerical results indicate that our valve model is able to reproduce overall physiological and/or pathological conditions, as preliminary assessed on two different patients. This promising approach provides insights into post-PVR haemodynamics and prosthetic valve effects, shedding light on potential implications for patient-specific outcomes., (© 2024 The Author(s). International Journal for Numerical Methods in Biomedical Engineering published by John Wiley & Sons Ltd.)

Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Congenital heart disease is defined as abnormality in the cardiovascular structure or function that is present at birth and it is the most common cause of congenital anomalies. Approximately 90% of more than 1,000,000 children born per year with congenital heart disease worldwide receive suboptimal care or have no access to care at all. Furthermore, the mortality is likely underreported in Low-and Middle-Income Countries. Mission Bambini Foundation is an Italian NGO founded in 2000, aiming at "helping and supporting children who are poor, sick, without education or physically and morally abused" in Italy and worldwide. In 20 years, through 1.700 projects, 1.4 million children have been supported in 75 Countries. In 2005, Mission Bambini launched the "Children's Heart Program," based on long-term partnerships and on medical/surgical volunteering, in order to provide multidisciplinary education and training and technical support., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Marianeschi, Albano, Viola, Barenghi and Gusella.)

Background: Every year, around 15 million children, in developing countries, die or develop life-long disabilities because of congenital cardiac diseases. In this report we measure the effect of a pediatric cardiac surgery humanitarian project on the health of the individual and on the potential influence this has on the countries economy and its growing health services. Methods: We collected and analyzed data from the Italian NGO, Mission Bambini's database, including all congenital cardiac missions undertaken in Cambodia between 2012 and 2019. DALY's (Disability Adjusted Life Years) saved by the humanitarian mission were estimated and used to reflect on the impact this had on the populations economy. Progression in the local medical teams skills emulated the advancements made in the health sector of the region. Results: Between 2012 and 2019, 128 patients underwent a congenital cardiac operation at Angkor Hospital for Children at Siem Reap, Cambodia. The median age was 6 years. The majority of the pathologies included VSD, TOF, ASD. The mean Aristotle's Complexity Score was 6. Post-operative mortality was 0.8% (1/128). The cost-effectiveness analysis identified 5.360 DALY's saved by surgery. The competency of the local team was progressive with them being able to handle more complex cases on subsequent missions. Conclusion: In developing Countries, performing congenital cardiac surgery cases can be carried out successfully with improvement in both the economy and the health system of the country by increasing the years and the quality of life of the working population and developing the expertise of the regional team., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Marianeschi, Uricchio, Cerri, Ghiselli, Carro, Albano and Viola.)

More than 4 millions of children with congenital heart disease (CHD) are waiting for cardiac surgery around the world. Few of these patients are treated only thanks to the support of many non-governmental organizations (NGOs). Starting in December 2019, the so-called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic and has dramatically impacted on all the international humanitarian activities for congenital heart disease. We analyzed data from all the Italian congenital cardiac surgery centers with the aim to quantify the impact of the pandemic on their charities. Fifteen Italian centers participated in the study and contributed to data collection. We analyzed and compared data regarding humanitarian activities carried out abroad and on site from two periods: year 2019 (pre-COVID-19) and year 2020 (COVID-19 pandemic). In 2019, 53 international missions were carried out by Italian congenital cardiac surgeons, resulting in the treatment of 471 CHD patients. In the same period 11 Italian cardiac centers operated on 251 foreign patients in Italy. In 2020, the pandemic led to a reduction of this activity by 96% for the surgery performed overseas and 86% for the interventions carried out in Italy. In conclusion our study shows the important quantitative impact of the pandemic on the Italian humanitarian cardiac surgical activity overseas and in Italy. This shocking result highlights the failure of the systems adopted so far to solve the problem of CHD in developing countries., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Giamberti, Caldaroni, Varrica, Pace Napoleone, Marianeschi, Uricchio, Vanini, Santoro, Luciani, Stellin, Gargiulo, Murzi, Filippelli, Oppido, Agati, Galletti and Frigiola.)

An easily reproducible surgical technique to switch from percutaneous minimally invasive biventricular mechanical support to cardiopulmonary bypass during heart transplantation is illustrated. After cannulation of the distal ascending aorta with a standard arterial cannula, the ProtekDuo® cannula and the ProtekSolo® Transseptal cannula were partially retracted to reach the superior and inferior vena cava, respectively, and connected to the pump circuit for the venous drainage. With this cardiopulmonary bypass configuration, orthotopic heart transplantation was routinely performed and, at the end of the procedure, the 2 cannulas were uneventfully removed., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation., Methods: Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers., Results: Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patients, especially in the northern area. Two hundred sixty-three surgical procedures were performed: 20% elective, 62% urgent, 10% emergency and 3% life-saving. Hospital mortality was 0.4%. Compared to 2019, the reduction in surgical activity was 52%. No patients operated on had positive test results before surgery for severe acute respiratory syndrome coronavirus 2, the virus responsible for COVID-19. Three patients were infected during the postoperative period. Twenty-nine nurses and 12 doctors were infected. Overall, 80% of our infected healthcare providers were in northern centres., Conclusions: Our study shows that the pandemic had a different impact on the various Italian congenital cardiac surgery centres based on the different patterns of spread of the virus across the country. During the lockdown, the system was able to satisfy all emergency clinical needs with excellent results., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device., Methods: From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects., Results: Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months-12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV., Conclusions: An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Cangrelor is an intravenously administered P2Y 12 receptor antagonist, which has been approved for adult patients undergoing percutaneous coronary intervention and, due to its unique pharmacokinetics, it allows effective and controllable peri-procedural platelet inhibition. We report the case of a 6-year-old child with anomalous origin of right coronary artery from aortic left coronary sinus, who underwent elective surgical replacement of stenotic and calcified conduit between the right ventricle and the main pulmonary artery. The surgery was complicated by acute myocardial infarction secondary to coronary extrinsic compression. The patient was successfully treated with urgent percutaneous coronary intervention (simultaneous V-stenting) and cangrelor infusion, subsequently switched to clopidogrel therapy.

Objective: By the end of February 2020, the COVID-19 pandemic infection had spread in Northern Italy, with thousands of patients infected. In Lombardy, the most affected area, the majority of public and private hospitals were dedicated to caring for COVID-19 patients and were organized following the 'Hub-and-Spoke' model for other medical specialties, like cardiac surgery and interventional procedures for congenital cardiac disease (CHD). Here, we report how the congenital cardiac care system was modified in Lombardy and the first results of this organization., Methods: We describe a modified 'Hub-and-Spoke' model - that involves 59 birthplaces and three specialized Congenital Cardiac Centers -- and how the hub center organized his activity. We also reported the data of the consecutive cases hospitalized during this period., Results: From 9 March to 15 April, we performed: a total of 21 cardiac surgeries, 4 diagnostic catheterizations, 3 CT scans, and 2 CMR. In three cases with prenatal diagnosis, the birth was scheduled. The spoke centers referred to our center six congenital cardiac cases. The postop ExtraCorporeal Membrane Oxygenation support was required in two cases; one case died. None of these patients nor their parents or accompanying person was found to be COVID-19-positive; 2 pediatric intensivists were found to be COVID-19-positive, and needed hospitalization without mechanical ventilation; 13 nurses had positive COVID swabs (4 with symptoms), and were managed and isolated at home., Conclusion: Our preliminary data suggest that the model adopted met the immediate needs with a good outcome without increased mortality, nor COVID-19 exposure for the patients who underwent procedures.

Objectives: Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique., Methods: This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV., Results: We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7-11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1-21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P = 0.006) and PV preservation was a significant protective factor against postoperative complications (P = 0.009, odds ratio 0.5) and late adverse events (P = 0.022)., Conclusions: Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom., Methods: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units., Results: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons., Conclusions: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Implantable cardioverter-defibrillator (ICD) is the gold standard therapy for the prevention of sudden cardiac death. Nevertheless, ICD placement in the paediatric population is still limited because of several technical difficulties. Several implantation techniques have been proposed but experience in infants with very low weight and less than 6 months is very limited. We herein describe a case of a minimally invasive ICD epicardial implantation in a 4-month-old infant weighing 5 kg. A diagnosis of arrhythmic cardiomyopathy with left ventricular non-compaction disease with ventricular tachycardia storms, QT prolongation and Wolff-Parkinson-White pattern was made. Antiarrhythmic drugs, radiofrequency ablation and sympathetic denervation were not effective. ICD implantation was successful allowing the infant to survive and bridging to heart transplantation., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We report the case of an unusual variation of a Kommerell's diverticulum in a left aortic arch with an aberrant left subclavian artery, associated with dilatation of the distal aortic arch, surgically treated without the use of extracorporeal circulation., (© 2016 Wiley Periodicals, Inc.)

A newborn was suspected of having situs inversus with levocardia based on chest X-ray. Echocardiography ruled out this hypothesis but revealed a giant cardiac mass that was confirmed by magnetic resonance imaging. Coronary angiography showed that the right coronary artery ran on the surface of the mass, and only partial debulking surgery was performed to relieve right heart compression. Histological examination classified the mass as cardiac fibroma. Complex diagnostic work-up allowed correct anatomic definition of the mass as well as its relationship with adjacent structures, and helped guide surgical planning.

Dextro-transposition of the great arteries with intact ventricular septum (d-TGA) is the most frequent cyanotic congenital heart disease in neonates. In newborns affected by d-TGA, the pulmonary and systemic circulations are in parallel instead of being in series. The survival of babies affected by d-TGA is related to the level of mixing at the patent foramen ovale. The diagnosis of d-TGA is an indication for treatment due to scarce survival beyond the neonatal period if not corrected. The current surgical approach is the anatomical correction with the arterial switch operation, which has excellent early and medium-term results. In this review, we discuss treatment of d-TGA and the arterial switch operation, the medium- and long-term results following surgery, and the reasons that have led the arterial switch operation to its popularity compared to the former physiological correction of d-TGA, i.e. the Senning and Mustard procedures.

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., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Several patterns of anomalous pulmonary venous drainage have been described in the literature, and bilateral partial pulmonary anomalous vein connection (PAPVC) has been described as a rare congenital cardiac anomaly. We report an unusual type of bilateral PAPVC, involving both the superior right and left pulmonary veins draining into the left brachiocephalic vein in a young adult who was symptomatic with dyspnea and a dry cough., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

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.

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.

We report the case of a 55-year-old female patient who underwent reoperation because of left ventricular to coronary sinus fistula following previous mitral valve replacement. The fistula was closed surgically with a patch of heterologous bovine pericardium. The postoperative course was uneventful and the patient is recovering well 12 months after the operation.

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.

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.

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.

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.

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.

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.

Background: Moderate to severe Ebstein's malformation remains a surgical challenge. Although the various approaches that have been used are appropriate and successful in many patients, there are many for which these approaches are suboptimal. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches are necessary., Methods: From December 1995 to October 1997, 10 patients (median age, 9 years) with moderate or severe Ebstein's malformation and mild to severe tricuspid regurgitation had partial biventricular repair with reduction of right ventricular volume load. All patients were symptomatic in New York Heart Association functional class II (n = 9) or III (n = 1). In addition to bidirectional cavopulmonary anastomosis and closure of intracardiac defects in all patients, 6 underwent tricuspid valve repair using a variety of procedures, most often simple horizontal annuloplasty., Results: There were no deaths. Early reoperation was required in 1 patient (atrial septostomy on the day after operation for right ventricular failure) and another required revision of the tricuspid valve repair 10 months postoperatively for recurrent regurgitation. At follow-up ranging from 2 to 24 months, all patients are in New York Heart Association class I and have trivial tricuspid regurgitation, including the 4 who had no tricuspid valvuloplasty performed., Conclusions: We have presented an alternative approach to the management of severe Ebstein's malformation that focuses on both the tricuspid valve and the right ventricle. Just as tricuspid valve repair and reduction of regurgitation will likely improve right ventricular performance, reducing the volume load on the ventricle may improve both ventricular (right and left) and tricuspid valve function. All patients have demonstrated improved exercise tolerance and right heart function at follow-up ranging to 24 months. Additional experience will be necessary to evaluate this strategy more completely.

Background: The bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functional single-ventricle heart. However, there remain a number of unresolved issues regarding this procedure, many of which concern the response of the pulmonary vasculature to this unique circulatory physiology. Among the issues of debate are the role and effects of an additional source of pulmonary blood flow., Methods: Between January 1990 and April 1997, 160 patients underwent bidirectional cavopulmonary anastomosis. Median age at operation was 7.8 months, and age ranged from 24 days to 43 years. An additional source of pulmonary blood flow was included in 93 patients (58%). A retrospective review of our experience with this cohort was performed with a focus on the role of additional pulmonary blood flow., Results: Eight patients (5%) died in the early postoperative period, and the overall early failure rate (death or take-down) was 7.5% (n=12). Eleven other patients underwent early reoperation to decrease (n=8) or increase (n=3) the amount of pulmonary blood flow. Early survivors were followed up for a median of 23 months, during which time 5 patients died and 30 patients underwent Fontan completion. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively., Conclusions: The bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate-term management of patients with a functional univentricular heart. However, there is much still to be learned about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the bidirectional cavopulmonary shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome.

Background: Pulmonary arteriovenous malformations can occur in a variety of clinical situations, including liver disease, systemic disorders, or after palliation of congenital heart disease, with serious clinical consequences., Methods: We reviewed the potential mechanisms of this condition, diagnostic tools, and clinical management., Results: Contrast echocardiography is an important diagnostic modality, which has been shown to be more sensitive than pulmonary arteriography, especially when rapid contrast injection is used. The finding that pulmonary capillary vasodilation is observed in hepatopulmonary syndrome, in cirrhotic patients, and after congenital heart repair is strongly suggestive that an unidentified hepatic factor is involved in inhibiting the development of pulmonary arteriovenous malformations., Conclusions: Prompt detection and treatment of pulmonary arteriovenous malformations is of utmost importance, to prevent serious clinical consequences. It may very well be the case that the etiology of arteriovenous malformations is multifactorial. We are now investigating the role of alterations of gene expression in the vascular remodeling that results in formation of pulmonary arteriovenous malformations.

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.

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.

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.

The accumulation of clear fluid leaking from a tubular prosthesis and confined within a fibrous pseudomembrane has been defined as a "seroma". Both the literature and our experience on this complication have been reviewed. Fifty-three cases of seroma have been reported after implantation of a tubular prosthesis. In our experience, from June 1982 to September 1988 we observed 11 cases (11/108 = 10.1%) of seroma complicating a modified Blalock-Taussig procedure. The mean age of these patients was 2 years 2 months. The diameter of the implanted tubular prostheses was always 5 mm. Among these cases, we report the details of a child, 1 year 10 months old, in whom the seroma significantly complicated the surgical history. According to the data derived from the literature and from our experience, we derived the following risk factors for the occurrence of seroma: age of the patient, size of the prosthesis, surgical manipulations, arterial-venous gradient, transprosthetic flow.