Corrigendum: Case Report: Epstein-Barr virus and constrictive pericarditis-an unusual combination.

[This corrects the article DOI: 10.3389/fped.2023.1215928.].

Case report: Epstein-Barr virus and constrictive pericarditis-An unusual combination.

Constrictive pericarditis is a chronic inflammatory process that can lead to heart failure if not diagnosed and treated correctly. Although Epstein-Barr virus (EBV)-related pericarditis is a very rare condition, it should still be considered for a differential diagnosis. We report the case of an 18-year-old male, who was surgically treated for constrictive pericarditis, in which in situ hybridization to Epstein-Barr virus-encoded RNA (EBER) probe of the excised pericardium led to the subsequent etiological diagnosis of chronic pericarditis caused by EBV.

The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches to its management. Unifocalization and rehabilitation focus on mobilization of collateral arteries and growth of native pulmonary arteries, respectively, with the ultimate surgical goal of achieving separated systemic and pulmonary circulations with the lowest possible right ventricular pressure. Regardless of the strategy, outcomes have altered the natural history of the disease, with a complete repair rate of approximately 80% and low early and late mortality rates. Given this heterogeneity of pulmonary vasculature, a tailored approach should be adopted for each patient, using all diagnostic methods currently offered by technical developments.

Blood flow characteristics after aortic valve neocuspidization in paediatric patients: a comparison with the Ross procedure.

AIMS: The aortic valve (AV) neocuspidization (Ozaki procedure) is a novel surgical technique for AV disease that preserves the natural motion and cardiodynamics of the aortic root. In this study, we sought to evaluate, by 4D-flow magnetic resonance imaging, the aortic blood flow characteristics after AV neocuspidization in paediatric patients. METHODS AND RESULTS: Aortic root and ascending aorta haemodynamics were evaluated in a population of patients treated with the Ozaki procedure; results were compared with those of a group of patients operated with the Ross technique. Cardiovascular magnetic resonance studies were performed at 1.5 T using a 4D flow-sensitive sequence acquired with retrospective electrocardiogram-gating and respiratory navigator. Post-processing of 4D-flow analysis was performed to calculate flow eccentricity and wall shear stress. Twenty children were included in this study, 10 after Ozaki and 10 after Ross procedure. Median age at surgery was 10.7 years (range 3.9-16.5 years). No significant differences were observed in wall shear stress values measured at the level of the proximal ascending aorta between the two groups. The analysis of flow patterns showed no clear association between eccentric flow and the procedure performed. The Ozaki group showed just a slightly increased transvalvular maximum velocity. CONCLUSION: Proximal aorta flow dynamics of children treated with the Ozaki and the Ross procedure are comparable. Similarly to the Ross, Ozaki technique restores a physiological laminar flow pattern in the short-term follow-up, with the advantage of not inducing a bivalvular disease, although further studies are warranted to evaluate its long-term results.

Aortic Valve Neocuspidalization May Be a Viable Alternative to Ross Operation in Pediatric Patients.

The aim of the study was to evaluate the medium-term results of aortic valve neocuspidalization according to Ozaki compared to Ross procedure for treatment of isolated aortic valve disease in pediatric age. Thirty-eight consecutive patients with congenital or acquired aortic valve disease underwent either Ozaki (n = 22) or Ross (n = 16) operation between 01/2015 and 05/2020. The primary outcome was progression of aortic valve disease and aortic ring and root dimension, whereas secondary outcome was freedom from reintervention or death by type of operation. Median age was 12.4 (8.8-15.8) years and the prevailing lesion was stenosis in 20 cases (52%) and incompetence in 18 (48%). One death occurred in the Ross group in the early postoperative period, while there were no deaths in the Ozaki group. Effective treatment of aortic valve stenosis or regurgitation occurred in both groups and remained stable over a median follow-up of 18.2 (5-32) months. In Ozaki group, 3 patients required aortic valve replacement at 4.9, 3.5, and 33 months, respectively. In Ross group, 1 patient required Melody pulmonary valve replacement, whereas none required aortic valve surgery. Finally, significantly higher aortic transvalvular gradient at follow-up was recorded in Ozaki group compared to Ross group. Overall, there was no significant difference in freedom from reoperation or death between the two groups. The medium-term outcome of Ozaki and Ross in pediatric patients is similar, despite an increased tendency of the former to develop aortic transvalvular gradient in the follow-up. Future larger multicenter studies with longer follow-up are warranted to confirm these results.

Aortic valve neocuspidalization in paediatric patients with isolated aortic valve disease: early experience.

OBJECTIVES: There is growing interest in the aortic valve (AV) neocuspidalization technique for the treatment of aortic valve disease (AVD). We report our medium-term results with this procedure performed in a paediatric patient population. METHODS: Between July 2016 and May 2020, 22 patients with both congenital and acquired isolated AVD were treated with neocuspidalization. The primary outcome was progression of the preoperatively assessed AVD in the immediate postoperative course and at follow-up. Secondary outcome was freedom from reintervention by material used. Potential predictors of failure were analysed in relation to the primary outcome. RESULTS: The median age at operation was 13.9 (interquartile range, 9.8-16.2) years, and the prevailing AV defect was stenosis in 10 cases (45%) and incompetence in 12 (55%). Pre-treated autologous pericardium was used in 13 patients whereas bovine pericardium in 9. Effective treatment of AV stenosis or regurgitation was achieved and remained stable over a median follow-up of 11.3 (4.7-21) months. Three patients required AV replacement at 4.9, 3.5 and 33 months. At follow-up, an upward trend of both median indexed vena contracta jet widths and aortic peak and mean gradients were recorded, the latter associated with a failure to grow the aortic annulus. Predictor of such outcome turned out to be the use of bovine pericardium. A significant inverse linear correlation between AV peak gradient at follow-up and preoperative aortic annular size (P = 0.008) was also demonstrated. CONCLUSIONS: The Ozaki procedure is safe and effective in paediatric patients with AV disease. The use of heterologous pericardium should probably be minimized. Moreover, preoperative small aortic annuli should probably be promptly treated by means of an associated ring enlargement procedure.

Midterm Echocardiographic Assessment of Right Ventricular Function After Midline Unifocalization.

BACKGROUND: Patients with an open ventricular septal defect (VSD) after repair of pulmonary atresia (PA), VSD, and major aortopulmonary collaterals (MAPCAs) are the most vulnerable subgroup. We analyzed the impact of concomitant versus delayed VSD closure on survival and intermediate-term right ventricular (RV) function. METHODS: Between October 1996 and February 2017, 96 patients underwent a pulmonary flow study-aided repair of PA/VSD/MAPCAs. For patients who underwent either concomitant or delayed intracardiac repair, echocardiographic RV systolic function was retrospectively calculated to assess (1) RV fractional area change (RVFAC) and (2) two-dimensional RV longitudinal strain (RVLS) of the free wall of the right ventricle. QLAB cardiac analysis software version 10.3 (Philips Medical Systems, Andover, MA) was used for analysis. RESULTS: A total of 64 patients underwent concomitant VSD closure at the time of unifocalization, and 16 patients underwent delayed VSD closure at a median of 2.3 years (range: 3 days to 7.4 years). At a median follow-up of 8.1 years (range: 0.1 to 19.5 years) for the concomitant repair group versus 7.4 years (range: 0.01 to 15.3 years) for the delayed repair group, no differences in RVFAC and RVLS were observed (RVFAC: 41.0% ± 6.2% versus 41.2% ± 7.6%, p = 0.91; RVLS: -18.7 ± 4.3 versus -18.9 ± 4.0, p = 0.87). CONCLUSIONS: Patients (83%) with PA/VSD/MAPCAs underwent complete repair at intermediate-term follow-up with preserved RV function. Delayed VSD closure was accomplished in 50% of the patients initially deemed unsuitable for repair. Delayed VSD closure did not affect survival and did not portend impaired RV systolic function.

Bronchial Mismatch as a Predictor of Respiratory Failure After Congenital Tracheal Stenosis Repair.

BACKGROUND: The purpose of this study was to identify predictors of postoperative respiratory failure after surgical tracheoplasty for congenital tracheal stenosis. METHODS: Between February 2011 and April 2017, 16 infants underwent congenital tracheal stenosis repair. Preoperative computed tomography evaluation of the trachea and mainstem bronchi was performed. The primary outcome was midterm freedom from postoperative respiratory failure defined as persistent need for mechanical ventilation or surgical tracheobronchial reoperation. Bronchial mismatch, defined as [1 - (smaller bronchus diameter / larger bronchus diameter)] × 100, was analyzed in relation to the primary outcome. RESULTS: Median age was 106 days (range, 1 to 406) and median weight was 5.3 kg (range, 2.6 to 8 kg). Four patients were neonates (25%) and 6 had genetic abnormalities (37.5%). There were no early nor late deaths. Median ventilation time was 5.5 days (range, 3 to 45). Mean follow-up time was 2.2 years (range, 0.1 to 4.5). Four patients with bronchial mismatch greater than 20% had postoperative respiratory failure (p = 0.002). Two of them underwent tracheostomy and were discharged with ventilation home care support. One underwent successful reoperation consisting of bilateral bronchial plasty with autologous cartilage rib grafts, and the other underwent successful right bronchial and tracheal reconstruction. One patient with bilateral bronchial hypoplasia underwent slide tracheoplasty associated with preemptive bilateral bronchial plasty and made a full recovery. CONCLUSIONS: Surgical treatment of congenital tracheal stenosis in neonates and infants portends a good outcome. Bronchial mismatch greater than 20% can identify a subset of patients at increased risk for surgical reintervention and chronic respiratory failure. Slide tracheoplasty with preemptive bronchial reconstruction may prevent postoperative respiratory failure.

Long-Term Outcomes After Extracardiac Fontan Takedown to an Intermediate Palliative Circulation.

BACKGROUND: Acute failure of the Fontan circulation is rare but remains associated with high morbidity and mortality rates. Little is known about the long-term outcome of patients who underwent Fontan takedown to an intermediate palliative circulation and their potential candidacy for redo Fontan completion. METHODS: Patients followed up at a single institution who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 2 months of extracardiac Fontan completion were reviewed. RESULTS: Between October 1990 and December 2015, 18 patients underwent Fontan takedown to a superior cavopulmonary connection (with or without an additional shunt) at a median age of 3.3 years (range, 1.8 to 8.0) and median weight of 13.8 kg (range, 8.0 to 27.0 kg). Takedown was required during the Fontan procedure itself in 2 patients, and within the first 2 postoperative months in 16 patients (median time to takedown, 3 days). Seventeen patients survived the post-takedown period and 3 ultimately underwent successful redo Fontan. Four patients required heart transplantation, with 2 deaths. In patients with extended intermediate palliation, median arterial oxygen saturation was 84% (range, 76% to 92%) at a median follow-up of 6.3 years (range, 0.7 to 25.9). CONCLUSIONS: Takedown to a superior cavopulmonary connection is an effective treatment option and, in some patients, acts as a bridge to subsequent redo Fontan completion or heart transplantation. An extended intermediate palliative circulation is tolerated for several years with reasonable oxygen saturation levels at rest. In our experience, an early takedown strategy to a superior cavopulmonary connection is the treatment of choice for acute Fontan failure.

Edwards Sapien 3 Valve for Mitral Replacement in a Child After Melody Valve Endocarditis.

We present the surgical implantation in mitral position of the Edwards Sapien 3 valve for prosthetic valve endocarditis in a severely ill child after multiple valve replacements. The procedure was safely performed and provided adequate hemodynamic results in the short term. This report highlights the excellent potential of Sapien 3 valve for the treatment of mitral valve disease in children with a mitral valve annulus of appropriate size. Of note, the use of the Sapien 3 valve should be considered when a bioprosthesis is required at the time of valve re-replacement.

Impact of Pulmonary Flow Study Pressure on Outcomes After One-Stage Unifocalization.

BACKGROUND: The purpose of this study was to evaluate the accuracy of the pulmonary flow study in (1) predicting the feasibility of concomitant intracardiac repair after one-stage unifocalization; and in (2) predicting long-term survival and the onset of right ventricular dysfunction after surgery. METHODS: Between October 1996 and July 2015, a flow study was obtained in 95 patients undergoing complete one-stage unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. The ability to achieve 100% flow (approximately 2.5 L · min-1 · m-2) into the pulmonary bed at a mean pressure of 30 mm Hg or less was utilized as an indicator for acceptability of ventricular septal defect closure. RESULTS: Overall survival was 78% ± 6% at 15 years. Sixty-four patients underwent successful one-stage intracardiac repair. The flow study accurately predicted suitability for VSD closure (area under the curve = 0.855). After one-stage ventricular septal defect closure, no difference in survival was observed after stratification according to flow study pressures (25 mm Hg or less versus greater than 25 mm Hg, log rank p = 0.20). At a median follow-up of 7 years, no association was found between flow study pressure and the onset of right ventricular dysfunction (p = 0.21). Overall, the inability to achieve final intracardiac repair was a strong predictor of death (hazard ratio 9.14, 95% confidence interval: 1.98 to 42.07, p < 0.0001). CONCLUSIONS: Suitability for ventricular septal defect closure is reliably defined by the flow study with a cutoff of 30 mm Hg. Flow study pressure values do not affect long-term outcomes. The ability to obtain intracardiac repair (in either one or more stages) is the strongest predictor of survival.

Neonatal Repair of Persistent Fifth Aortic Arch Coarctation and Interrupted Fourth Aortic Arch.

Persistent left fifth aortic arch is a rare anomaly often associated with aortic coarctation. We report the case of a newborn presenting with signs of duct-dependent aortic coarctation. Echocardiography showed an interrupted fourth aortic arch, persistent left fifth aortic arch associated with aortic coarctation, and a restrictive arterial duct. Arch repair was accomplished using the fifth aortic arch as an in situ flap to enlarge the hypoplastic fourth aortic arch associated with coarctectomy and extended end-to-end anastomosis. We believe that this newly reported surgical approach is safe and effective and allows for adequate enlargement of the entire fourth aortic arch.

Intraoperative coronary revision but not coronary pattern is associated with mortality after arterial switch operation.

OBJECTIVES: We sought to determine differences in baseline characteristics and clinical outcomes in a consecutive series of patients undergoing arterial switch operation (ASO), assessing the effect of coronary anatomy on postoperative mortality, both overall and adjusted for surgical era. METHODS: From January 2000 to May 2015, 283 consecutive patients underwent ASO for transposition of the great arteries. A total of 103 patients (36.4%) had an associated ventricular septal defect and 23 (8.1%) had an aortic arch obstruction. Coronary anomalies were present in 119 (42%) patients. RESULTS: Overall survival was 93.0 ± 1% at 15 years (19 events). Intraoperative revision of the coronary anastomosis was required in 8 patients (2.8%) due to inability to wean from cardiopulmonary bypass (CPB) or post-CPB myocardial ischaemia. Surgical revision was neither related to the underlying coronary anatomy ( χ 2 = 0.681, P = 0.4091) nor to the surgical era ( χ 2 = 0.682, P = 0.4090). Univariable analysis suggested decreased mortality rate for infants who underwent surgery during 2008 or later ( P = 0.01). In multivariable analysis, intraoperative coronary button revision was the only predictor of mortality [ P < 0.001, hazard ratio (HR) 12.01, 95% confidence interval (CI) 3.85-37.49], whereas surgical era (before 2008) failed to achieve statistical significance ( P = 0.057, HR 2.19, 95% CI 0.96-12.04). CONCLUSIONS: In the recent era, unusual coronary patterns do not affect survival following ASO, whereas intraoperative coronary button revision is a predictor of mortality. Preventive strategies aimed towards intraoperative detection of impaired coronary perfusion are useful tools that might be considered for all patients in order to improve outcomes.

Primitive intrapericardial teratoma associated with yolk sac tumour.

An intrapericardial vacuolated mass compressing and displacing the heart was diagnosed by echocardiography in a foetus of 22 weeks gestation. The birth was induced for early signs of foetal distress at 29 weeks and, after two initial pericardial evacuation procedures, the tumour was resected radically 7 days after birth at a weight of 1.55 kg. Mass histology showed teratoma associated with yolk sac tumour. We comment on the overall approach adopted after foetal diagnosis and the histopathological features of the tumour, and try to draw conclusions on patient outcome data.

Managing major vessel injuries with a Fogarty catheter during chest re-opening in children.

Injury of structures, leading to a major bleeding during chest opening, is a severe and potentially life-threatening complication, especially in redo cardiac surgery, both in adults and children. In three paediatric redo operations performed via midline sternotomy, we managed this complication successfully and uneventfully by using an inflated Fogarty catheter to plug the blood leak from the injured vessel before repairing the lesion under direct vision in a bloodless surgical field. Herein we report in detail the technique used and a comment on our experience.

Surgical treatment of transposition of the great arteries with bilateral intramural coronary arteries.

A 33-day-old neonate with transposition of the great arteries, ventricular septal defect, and intramural course of both coronary arteries underwent successful arterial switch repair. At a follow-up time of 36 months, left ventricular function and coronary arterial anatomy at angiography were normal. Optimal treatment of such rare coronary arterial abnormality may allow functional and anatomic results comparable to usual coronary arterial patterns in transposition of the great arteries.

Effects of ascending aorta replacement on aortic root dilatation.

OBJECTIVE: Because of an increase of aortic root wall stress, prosthetic replacement of the ascending aorta might be a risk factor for the progressive increase of the aortic root dimension. Aim of the present study was to evaluate the aortic root diameter change and the progression of aortic valve regurgitation late after ascending aorta replacement for different ethiology. METHODS: Sixty-three late survivors after supracoronary ascending aortic replacement were evaluated. Forty-one patients were operated on for acute aortic dissecting aneurysm (group I) and 22 for chronic atherosclerotic non-dissecting aneurysm (group II). Aortic root diameter and aortic valve regurgitation were assessed echocardiografically after a mean follow-up of 63+/-31 months and were compared with those early after surgery. RESULTS: Seven patients of group I (17%) needed reoperation for aortic root dilatation or dissection. Twenty-five percent of the patients (15 of group I and 1 of group II) showed at least a 10% increase in aortic root diameter at follow-up (46.8+/-6.1 vs. 38.1+/-6.1mm, P<0.0001). Aortic root diameter increased almost exclusively in patients operated on for acute dissecting aneurysm. A significant worsening of aortic valve insufficiency with time was evident only in patients operated on for acute dissecting aneurysm with an higher incidence in those with progressive root dilatation. CONCLUSIONS: Both the increase of aortic root diameter and the progressive worsening of aortic valve insufficiency seem to justify a more aggressive treatment of the aortic root at the time of surgery for acute aortic dissecting aneurysm but not for chronic atherosclerotic non-dissecting aneurysms.