Postoperative Outcomes of Fontan Operation in a Multicenter Italian Study. How Far Have We Gone? Early Outcomes After Fontan Operation.

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.

Infradiaphragmatic partial anomalous pulmonary venous connection in adulthood. Considerations for management: a single centre experience.

BACKGROUND AND OBJECTIVES: Infradiaphragmatic partial anomalous pulmonary venous connection is occasionally diagnosed in adulthood. Management of infradiaphragmatic PAPVC depends on anatomy and clinical presentation. METHODS: Over a 10-year period, we observed seven adult patients (median age 29 years) with partial anomalous pulmonary venous connection. We classified our patients in two groups. Group I: isolated partial anomalous pulmonary venous connection from one pulmonary lobe to the inferior vena cava, three patients. Group II: partial anomalous pulmonary venous connection of the entire right lung to IVC, four patients. RESULTS: The mean term follow-up was 5.4 years. Patients in Group I have been managed conservatively, as they were asymptomatic, without a significant shunt. Patients in Group II were surgically corrected using long right intra-atrial baffles. After 6 months of follow-up, the first two cases were diagnosed with complete tunnel thrombosis and loss of right lung function. Oral anticoagulation failed to recanalize the tunnel. Considering this serious complication, the other two patients were empirically and preventively treated with anticoagulation after surgery, with good outcome on long-term follow-up. CONCLUSIONS: Conservative management should be considered for asymptomatic patients, without a significant shunt. Surgical treatment of infradiaphragmatic partial anomalous pulmonary venous connection of the entire right lung in inferior vena cava is challenging. Slow blood flow inside the long intra-atrial baffles inclines to thrombosis and occlusion, as we observed in two cases. Therefore, oral anticoagulation should be considered for long baffles with slow blood flow.

Tetralogy of Fallot and absent pulmonary valve syndrome.

Absent pulmonary valve syndrome is a relatively rare condition, representing a subset of about 3% of cases of tetralogy of Fallot in both autopsy and clinical reviews (1). The syndrome is characterized by a ringlike and usually stenotic malformation rather than by the absence of the pulmonary valve, with failure of development of the valve cusps. Another important feature of absent pulmonary valve syndrome is the marked aneurysmal dilatation of the proximal pulmonary arteries, which causes extrinsic compression of the tracheobronchial tree, leading to respiratory symptoms of variable severity (2). The surgical goals are to correct any intracardiac anomaly, prevent right-sided heart failure, and alleviate or prevent bronchial compression and peripheral lung damage. In this video tutorial, we present the straightforward correction of tetralogy of Fallot with absent pulmonary valve with a transannular patch combined with size reduction arterioplasty of bilateral aneurysmal pulmonary arteries. This patient also had a subaortic membrane that was completely removed concomitantly. Our technique shows right ventricular outflow tract reconstruction without using a valved conduit or creating a monocusp patch in order to reduce the incidence of a right ventricular outflow track reoperation. Nonapplication of the right ventricle-pulmonary artery conduit did not affect the early postoperative course or the immediate postoperative outcome.

Aortopulmonary window and coronary anatomy - still a pre-operative diagnostic trouble!

We present a case of aortopulmonary window in which the diagnosis of anomalous left coronary artery originating from pulmonary artery was made intra-operatively even if the coronary arteries anatomy was correctly studied pre-operatively with echocardiography. No evidence of coronary anomalies or indirect sings of coronary anomalies has been noted. Should we improve our pre-operative diagnostic accuracy and how?

Aortic arch reconstruction in neonates using an autologous pericardial patch.

This video tutorial illustrates the surgical correction of aortic coarctation associated with severe hypoplastic aortic arch and a large subaortic ventricular septal defect using a fresh autologous pericardial patch for arch reconstruction under hypothermic cardiopulmonary bypass and selective cerebral perfusion.

Aortic arch uncrossing using a tracheal resection procedure.

One category of vascular rings is the right aortic arch associated with the diverticulum of Kommerell from which the left subclavian artery usually originates. In some cases, the right aortic arch crosses behind the trachea and the esophagus from right to left. The trachea and esophagus are compressed by the right aortic arch, the left ligamentum, and the posterior crossing aorta, which causes the typical symptoms of noisy breathing, dyspnea on exertion, dysphagia, and frequent upper respiratory tract infections. Division of the atretic arch segment between the diverticulum of Kommerell and the left common carotid artery may relieve the symptoms temporarily but does not relieve the compression produced by this vascular abnormality. Indeed, at the age of 10 months, this patient underwent anterior arch division and posterior aortopexy via a posterolateral thoracotomy in order to relieve the compression caused by the vascular ring. Several months after the initial operation, the patient had recurrent respiratory symptoms as a result of residual vascular compression from the circumflex arch. A CT scan and airway endoscopy confirmed tracheal compression; in addition, the tracheoscopy showed tracheomalacic changes in the compressed segment of the trachea. To relieve the symptoms and the compression, we decided to resect the tracheomalacic segment of the trachea and translocate the aortic arch anterior to the trachea and esophagus.

Procalcitonin and Early Postoperative Infection After Pediatric Cardiopulmonary Bypass Surgery.

OBJECTIVES: Systemic inflammation and bacterial infections are critical occurrences after pediatric cardiac surgery. Elevated white blood cell count and C-reactive protein cannot discriminate between these two conditions in the early postoperative period. The aim of this study was to understand whether procalcitonin (PCT) values within 48 hours of surgery could be a useful marker of postoperative infection. DESIGN: Retrospective observational study. SETTING: The study was performed in a teaching hospital. PARTICIPANTS: All patients ≤six years of age. INTERVENTIONS: Cardiac surgery on cardiopulmonary bypass from January 1, 2017 to January 1, 2020. MEASUREMENT AND MAIN RESULTS: PCT, white blood cell count, and C-reactive protein values were measured at intensive care unit admission and at 24 and 48 hours after surgery. All positive cultures in the first seven days after surgery were recorded. Out of 177 consecutive patients, 22 (12%) developed infections. PCT at 48 hours after surgery was the only laboratory predictor of infections in the first seven days after surgery (p = 0.02). Receiver operating curve analyses on PCT values at 48 hours identified an optimal cut-off value of 1.85 ng/mL in the overall population. Area under the curve was 0.63, sensitivity 63%, and specificity 69%. CONCLUSIONS: In light of this preliminary result, the clinical relevance and predictive accuracy of PCT are promising in patients with increasing values of PCT but need to be confirmed in a larger sample.

Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature.

Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin in a 9-year-old girl, treated with debulking surgery, adjuvant chemotherapy, and radiotherapy.

Isolated Congenital Coronary Ostial Stenosis in a Young Infant: A Case Report.

Coronary ostial stenosis is a rare congenital cardiac anomaly, frequently associated with hypoplasia of the proximal coronary artery. This condition is potentially life-threatening, as it may present with myocardial ischemia and sudden death. We present a case of left coronary ostial stenosis in a 48-day-old infant symptomatic for sudden cardiac arrest, who successfully underwent surgical angioplasty. Any cardiac arrest in a neonate or young infant should raise suspicion of coronary ostial stenosis/atresia, considering the difficulty in diagnosing this congenital heart defect.

Transposition of the great vessels and intact ventricular septum: is there an age limit for the arterial switch? Personal experience and review of the literature.

OBJECTIVE: Prognosis of the transposition of the great arteries has completely changed since the introduction of the arterial switch. Time limit to perform this intervention is still controversial. The aim of this study is to demonstrate the early and late outcome of primary arterial switch operation beyond the age of months. METHODS: We included all patients with the diagnosis of transposition of the great arteries with intact ventricular septum beyond the age of 8 weeks who underwent primary arterial switch operation. The procedures were performed by the same surgeon, in two different institutes. Patients who had transposition of the great arteries and associated anomalies (except atrial septal defect and persistent arterial duct) were excluded. Ventricular shape, geometry, and mass were not considered during the decision on procedure type. RESULTS: In the study, 11 patients with the diagnosis of simple d-transposition of the great arteries beyond 8 weeks were undergone primary arterial switch operation with a mean age of 90.63 days (60-137 days), and 7 patients had a Rashkind procedure. All patients had squashed left ventricle shape with preserved function. The sternum was left open in 10 patients. Extracorporeal membrane oxygenation support was necessary in 45.45% of cases. The mean mechanical ventilation time was 7.27 days (1-16 days). No mortality was recorded until now. Post-operatory left ventricular function was preserved in 90.9% of the patients. Only one patient had mild myocardial dysfunction at the time of discharge. CONCLUSIONS: Primary arterial switch procedure can still be the best surgical option in patients with the diagnosis of transposition of the great arteries with intact ventricular septum beyond 8 weeks of age, providing that mechanical circulatory support and an expert cardiac intensive care unit service are available.

Aorto-Right Ventricular Tunnel: A Case Series and Literature Review.

Aorto-right ventricular tunnel (ARVT) is a rare cardiac congenital anomaly where an extracardiac channel connects the ascending aorta above the sinutubular junction to the right ventricle. This defect is caused by an abnormal development of the cushions of the aorto-pulmonary outflow tract. A case series and literature review are described. Two cases of ARVT are described. A literature review was conducted, in which 31 cases were reported. In our 2 cases, both ARVTs connected the ascending aorta above the left aortic sinus to the right ventricle (one to the right ventricular outflow tract and one to the right ventricular apex). Both patients underwent successful surgical correction by patch closure of both tunnel orifices, with uneventful postoperative course. Of the 31 ARVT cases described in our review, only 10 patients (32.3%) had an anatomy similar to the 2 cases described. Coronary artery anomalies can be associated, as reported in our 2 patients and in 16 cases (51.6%) in the review. Surgical correction can be achieved by direct closure or, more often, by patch closure of one or both tunnel orifices, depending mostly on coronary anatomy. Two cases of transcatheter device closure were described in literature, in favorable anatomy cases. Careful attention is required during repair to avoid coronary lesions, due to the high incidence of comorbid coronary anomalies.

Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case.

BACKGROUND: Double-outlet right atrium (DORA) is a rare congenital cardiac defect, where the right atrium (RA) is connected to both the right ventricle (RV) and the left ventricle (LV). Double-outlet right atrium is classified into two types, each containing two subtypes: malaligned atrial septum with common or single atrioventricular (AV) valve and malaligned ventricular septum (VS) with adequate or inadequate RV. The VS type is characterized by straddling right AV valve (RAVV) with intact VS, resulting in two RAVV orifices. METHODS: We report a case of "acquired DORA" in a 17-year-old male patient previously treated with subaortic VSD closure. At admission, we diagnosed DORA with VS malalignment and adequate RV. The patient had 2 orifices within the RAVV, connecting the RA to both the RV (via RAVV orifice 1) and the LV (via RAVV orifice 2). The latter was insufficient, with severe LV to RA shunt. A review of the literature indexed in PubMed and Scopus databases was undertaken. RESULTS: The patient underwent biventricular repair through closure of the RAVV orifice 2 with a pericardial patch. Pacemaker implantation for complete AV block was necessary. Postoperative course and follow-up were regular. The literature review showed 39 cases of DORA, of which 8 had a malaligned VS and an adequate RV, as in our case. CONCLUSION: Acquired DORA is an interesting post-surgical variant of a rare congenital heart defect. Biventricular repair is a feasible and viable option, which involves closing the third AV orifice. Careful attention is required in order to avoid injury to the conduction system.

Antibiotic Management for Delayed Sternal Closure Following Pediatric Cardiac Surgery: A Systematic Review of Recent Literature.

Delayed sternal closure after pediatric cardiac surgery is a management option for the treatment of patients with severely impaired heart function. The optimal antimicrobial treatment strategy for this condition is unknown. The aim of this systematic review was to evaluate the current antibiotic administration attitudes in pediatric cardiac surgery patients needing an open chest in terms of infection with a focus on surgical site infection rate. The authors performed a systematic review and meta-analysis of all articles, which described the antibiotic administration strategy and surgical site infection rate in pediatric patients with an open chest after cardiac surgery. The authors performed a subgroup analysis on "standard" versus "non-standard" (defined as any antimicrobial drugs different from the adult guidelines recommendations) therapy for one-proportion meta-analysis with a random effect model. The authors identified 12 studies published from January 1, 2000 to July 1, 2019 including a total of 2,203 patients requiring an open chest after cardiac surgery, 350 of whom (15.9%) developed infections and 182 (8.3%) developed a surgical site infection. The surgical site infection rate in patients with "non-standard" strategy was higher than in patients with "standard" strategy: 8.8% (140 reported infections/1,582 patients) versus 6.8% (42 reported infections/621 patients), p = 0.001. The "standard" antibiotic management proposed by guidelines for adult cardiac surgery patients could be used an acceptable strategy to treat pediatric patients with an open chest after cardiac surgery.

Intensivists Performed Percutaneous Bicaval Double-Lumen Echo-Guided Extracorporeal Membrane Oxygenation Cannulation at Bedside in Newborns and Children: A Retrospective Analysis.

OBJECTIVES: To describe a single institution experience on echo-guided percutaneous bicaval double lumen extracorporeal membrane oxygenation cannulation performed at the bedside by intensivists. DESIGN: Retrospective observational study. SETTING: Extracorporeal membrane oxygenation team of a tertiary care children's hospital. PATIENTS: All patients 0-14 years old undergoing venovenous extracorporeal membrane oxygenation from January 1, 2013, to January 1, 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Thirty children underwent 32 extracorporeal membrane oxygenation runs. Median age at enrollment was 2 months (interquartile range, 0-20.5 mo), 65.6% of the runs (21 patients) were performed in newborns (n = 13, 40.6%) or infants (n = 8, 25%). Median preextracorporeal membrane oxygenation index was 66.9 (interquartile range, 50-85.6). Major comorbidities were present in 50% of patients. All patients were cannulated percutaneously. In two cases cannulation occurred from the left internal jugular vein. Extracorporeal membrane oxygenation was effective in increasing pH, arterial oxygen saturation, PaO2, and lowering PaCO2. The overall differences in pre and postextracorporeal membrane oxygenation values were statistically significant, while stratifying patients according to the cannula diameter (mm)/major diameter of the cannulated internal jugular vein (mm) ratio (> 0.67 or ≤ 0.67), statistical significance was reached only for the highest ratio. Complications were observed in three runs: two cannula tip dislocations in the right atrium and one limited flow in the only case in which an Avalon cannula was not used. In 20 cases (62.5% of 32 runs), the cannulated vessel was patent at follow-up or autopsy. A ratio less than or equal to 0.67 or greater than 0.67 did not influence the occurrence rate of complications, nonpatency of the internal jugular vein, death for intracranial bleeding and death at 30 days from extracorporeal membrane oxygenation discontinuation. Overall cumulative survival at 30 days from extracorporeal membrane oxygenation discontinuation was 60% (95% CI, 40-75), with a survival advantage in the case of ratio greater than 0.67 (65%; 95% CI, 44-80 vs 25%; 95% CI, 0-60). CONCLUSIONS: The described technique proved to be feasible, safe, and effective. Further investigation is needed.

International cooperation in healthcare: model of IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association for congenital heart diseases.

Cooperation activity in training programmes promoted by IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association is a model of cooperation between people as an alternative intervention in promoting the right to healthcare, especially offering programmes of training and medical care on cardiovascular and congenital heart disease. This new strategy, implemented in several developing countries, has absolutely contributed to the improvement of the medical services concerning the diagnostic and surgical approach in the treatment of paediatric and adult cardiovascular disease. To strengthen this kind of activity, both IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo have introduced a global perspective aiming at the realization of surgical missions 'in situ', building new cardiac surgery units in collaboration with the local partners, which are mainly university hospitals. They, furthermore, support financially the scholarship and accommodation in favour of medical and paramedical staff.

Porcine bioprosthetic valve in the pulmonary position: mid-term results in the right ventricular outflow tract reconstruction.

Pulmonary valve replacement (PVR) for pulmonary valve insufficiency (PVI) currently represents the most frequent reoperation performed for adults with congenital heart disease. A variety of pulmonary valve substitutes have been used, but none has proved to be ideal. This report reviews the authors' experience using a porcine prosthetic valve in the pulmonary position. Between January 2001 and December 2011, 76 patients (mean age, 36 years; range, 18-64 years) underwent PVR for chronic PVI using a porcine bioprosthesis. All the patients had previously undergone surgery: 65 for repair of tetralogy of Fallot and 11 for pulmonary surgical valvotomy. Magnetic resonance imaging (MRI) evaluations before surgery and at the 1-year postoperative follow-up evaluation were compared. Aside from the PVR, 59 patients (59/65, 78 %) received 94 associated cardiac surgical procedures. Two hospital deaths occurred. The mean hospital stay was 13 days (range, 7-48 days). At the 1-year control MRI, pulmonary regurgitation fraction, right ventricular end diastolic volume (RVEDV), and RV/LV EDV had improved significantly. During a mean follow-up period of 52 months (range, 6-132 months), one patient died. All the patients were categorized as New York heart association (NYHA) functional class 1. No episodes of structural valve deterioration, endocarditis, or thromboembolic event were noted. Echocardiography showed trivial or no PVI in all the patients. The porcine bioprosthetic valves demonstrated excellent midterm results in the RV outflow tract reconstruction. The hemodynamic characteristics of this valve are comparable with those of homografts or valved conduits. It is easy to implant and allows for avoiding extensive dissection, especially of the pulmonary arteries.

Cor triatriatum dexter: unusual features in utero and after birth.

Cor triatriatum dexter is a rare congenital anomaly, caused by the persistence of the right valve of the sinus venosus, which divides the right atrium into two chambers and its diagnosis is rather difficult, due to the features often mimicking Ebstein's anomaly. We describe a case followed from the foetal age for a relative right ventricular hypoplasia and suspicious pulmonary stenosis and diagnosed after birth to have cor triatriatum dexter with an obstructing membrane in the right atrium, needing surgical correction, done successfully.

Morbidity and mortality risk factors in adults with congenital heart disease undergoing cardiac reoperations.

BACKGROUND: Reoperations represent relatively frequent events in adults with congenital heart disease (ACHD). Cardiac operations in these patients present major difficulties in management and technique. Although reoperations in ACHD are becoming increasingly frequent, limited knowledge exists regarding perioperative risk factors. METHODS: The study included 164 ACHD patients who underwent cardiac reoperations between January 2002 and December 2007 at our institution. Preoperative and intraoperative data were analyzed to identify morbidity and mortality risk factors. RESULTS: Reoperations included pulmonary valve implantation or conduit replacement in 60, aortic valve/root procedures in 36, residual atrial or ventricular septal defect closure in 19, and Fontan operation/conversion in 19. Hospital mortality was 3.6%. The mean mechanical ventilation time was 26 hours. Mean intensive care unit stay was 3.1 days. Severe postoperative complications occurred in 24 (15.1%). Cardiopulmonary bypass time (p = 0.001), Fontan operation/conversion (p = 0.001), preoperative hematocrit (p = 0.004), previous number of operations (p = 0.001), and preoperative congestive heart failure (p = 0.021) were associated with severe morbidity. No factor was associated with death. CONCLUSIONS: Reoperations in ACHD are mostly due to right ventricular outflow tract lesions and were associated with a low mortality rate if performed in a center with a considerable activity and a dedicated program. Severe morbidity is relatively frequent and is generally associated with the preoperative (high hematocrit due to cyanosis, congestive heart failure, and the number of previous operations) and operative (Fontan operation/conversion and cardiopulmonary bypass duration) conditions of the patient.