World Journal for Pediatric and Congenital Heart Surgery

Atrioventricular valve (AVV) regurgitation in patients with single ventricle (SV) physiology severely impacts prognosis; the appropriate timing for surgical treatment is unknown. We sought to study the results of surgical treatment of AVV regurgitation in SV patients and evaluate risk factors for mortality.

Medical records of 81 consecutive patients with moderate or severe AAV regurgitation who were submitted to AVV repair or replacement during any stage of univentricular palliation between January 2013 and May 2017 were examined. We studied demographic data and perioperative factors looking for predictors that might have influenced the results. Binary logistic regression was used to assess the impact on postoperative ventricular dysfunction and mortality.

Median age and weight were seven months (interquartile range [IQR]: 3-24) and 5.2 kg (IQR: 3.7-11.2), respectively. Seventy (86.4%) patients underwent AVV repair, and 11 (13.6%) patients underwent AVV replacement. There was an association between AVV repair effectiveness and timing of intervention (P = .004). Atrioventricular valve intervention at the time of initial surgical palliation was associated with more ineffective repairs (P = .001), while AVV replacement was more common between Glenn and Fontan procedures (P = .004). Overall 30-day mortality was 30.5% (25 patients). In-hospital mortality was 49.4%, and it was higher when AVV repair was performed concomitant with initial (stage 1) palliation (64.1% vs 35.7%; P = .01) and when an effective repair was not achieved (75% vs 41%; P = .008). Multivariable analysis identified timing concomitant with stage 1 palliation as an independent risk factor for mortality (P = .01); meanwhile, an effective repair was a protective factor against in-hospital mortality (P = .05).

Univentricular physiology with AVV regurgitation is a high-risk group of patients. Surgery for AVV regurgitation at stage 1 palliation was associated with less effective repair and higher mortality in this initial experience. On the other hand, effective repair determined better outcomes, highlighting the importance of experience and the learning curve in the management of such patients.

The aim of this study was to evaluate the predictability of postoperative pulmonary artery pressure (PAP) using intraoperative flow study in patients undergoing bidirectional Glenn operation.

Patients who underwent Glenn operation under cardiopulmonary bypass (CPB) were included in the study. During the operation, after the completion of additional procedures under CPB, an intraoperative flow study was performed prior to Glenn anastomosis. After the completion of bidirectional Glenn, the patient was separated from the CPB and PAP was measured. The relationship between this pressure and flow study measurement was analyzed.

Nine patients who underwent bidirectional Glenn operation with additional procedures under CPB between July 2018 and January 2019 were included in the study. The median PAP was 9 mm Hg (interquartile range [IQR]: 7-10 mm Hg) in the flow study and 10 mm Hg (IQR: 8-11 mm Hg) after CPB, and the median difference between these pressures was 1 mm Hg (IQR: 1-3 mm Hg). There was a strong correlation between these two measurements (r = 0.732; P = .025).

The results of this study show that PAP after the Glenn procedure can be estimated using an intraoperative flow study. We believe that this method may be useful in intraoperative decision-making for Glenn operation in single ventricular patients who require extensive pulmonary artery (PA) reconstruction due to limited PA development, branch PA stenosis, or nonconfluent PAs. Also, this method can be used as a sort of intraoperative pulmonary resistance reversibility study in patients with high preoperative pulmonary vascular resistance due to surgically correctable pulmonary venous hypertension.

Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results.

From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention.

Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients.

Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

Aortic arch reconstruction is often challenging, especially in infants, owing to its high postoperative complication risks. This study aimed to compare the effectiveness between selective antegrade cerebral perfusion (SACP) alone and SACP in combination with continuous lower body perfusion with descending aortic cannulation (DAC) in preserving renal function, and to determine the influence of perfusion strategy on the postoperative course of infants who underwent aortic arch reconstruction.

A total of 121 infants who underwent aortic arch reconstruction between January 2008 and December 2018 were included in the analysis. Patients (median age: 29 days, range: 3-270 days) were divided into the following groups: those who underwent repair with SACP (SACP group, 79 patients) and those who underwent additional lower body perfusion (DAC group, 42 patients).

Three (7.1%) and nine (11.4%) patients died in the DAC and SACP groups, respectively (P = .54). The SACP group had more patients requiring renal replacement therapy (P = .002) and higher incidence of second stage acute kidney injury (AKI) development (Kidney disease improving global outcomes (KDIGO) criteria; P = .032). The SACP group had higher frequency of open chest postoperatively than the DAC group (P = .011). The DAC group had lower vasoactive inotropic score (VIS) at the first postoperative day (P < .001) and shorter intensive care unit length of stay (P = .050). There was no difference in neurological complications between the groups (P = .061). High VIS was associated with early mortality (odds ratio [OR]: 1.79 [1.33-2.41], P < .001) and AKI (OR: 1.60 [1.35-1.91], P < .001). The DAC perfusion strategy with minimal hypothermia was associated with lower risk of AKI (OR: 0.91 [0.84-0.98], P = .016).

Antegrade cerebral perfusion with continuous lower body perfusion via DAC could effectively be used for improving early postoperative results among infants undergoing procedures that include aortic arch reconstruction.

Xenografts used for right ventricular outflow tract (RVOT) reconstruction are typically treated with glutaraldehyde. However, potential benefit of epoxy treatment was demonstrated in experimental studies. We aimed to compare diepoxy-treated bovine pericardial valved conduits (DE-PVCs) and glutaraldehyde-treated bovine pericardial valved conduits (GA-PVCs) for RVOT reconstruction in pediatric patients.

Between 2002 and 2017, 117 patients underwent RVOT reconstruction with PVC in single center: DE-PVC group, n = 39; and GA-PVC group, n = 78. After performing propensity score analysis (1:1) for the entire sample, 29 patients from the DE-PVC group were matched with 29 patients from the GA-PVC group.

There were no conduit-related deaths. In the DE-PVC group, the freedom from conduit failure was 90.9% at four years and 54.3% at eight years postoperatively. In the GA-PVC group, it was 46.3% and 33.1%, respectively. The difference was significant (P = .037). Conduit failure was typically caused by stenosis in both groups. In the DE-PVC group, the main cause of stenosis was xenograft calcification (27.6%); while in the GA-PVC group, it was mostly due to neointimal proliferation (25.0%) and, less often, calcification (14.3%). Conduit thrombosis was the cause of replacement in 6.9% of patients from the GA-PVC group.

Diepoxy-treated bovine pericardial valved conduit is a suitable alternative to GA-PVC for RVOT reconstruction in pediatric patients. Diepoxy-treated bovine pericardial valved conduits may be less prone to conduit failure and more resistant to neointimal proliferation and conduit thrombosis than GA-PVCs.

Adhesions due to previous surgeries and some anatomical difficulties may make resternotomy dangerous in children. Femoral vessels are usually small and may not be suitable for cannulation. The aim of this report is to describe our experience with cervical cannulation during risky resternotomy in children.

Between January 2014 and January 2018, cervical cannulation was performed in eight pediatric patients during sternal reentry. Their ages were between 3 months and 17 years (mean: 5.4 years). Three patients underwent stage III extracardiac Fontan operation with the diagnosis of hypoplastic left heart syndrome. Three patients had supravalvular aortic and/or pulmonary stenosis after previous arterial switch operation. One patient had proximal aortic arch stenosis and subvalvular aortic stenosis after interrupted aortic arch repair. The last patient had aortic root pseudoaneurysm and aortic insufficiency due to endocarditis. Through a separate cervical incision, a polytetrafluoroethylene graft was anastomosed to the common carotid artery and the arterial cannula was inserted into the graft. Antegrade selective cerebral perfusion (ASCP) was used in two patients.

During resternotomies, no major injury or bleeding occurred. Three-month-old patient who had previous interrupted aortic arch repair died despite extracorporeal membrane oxygenation support due to sepsis and multi-organ failure. Median intensive care unit stay and hospital stay were 3 days (1-40 days) and 17 days (7-60 days), respectively. Mean follow-up was 17.9 ± 15.8 months. All patients were in good clinical condition.

Cervical cannulation may be a useful and safe technique during high-risk resternotomy in children. This technique may also simplify the performance of ASCP if necessary.

In neonates, transfusion of platelets after hemodilution from cardiopulmonary bypass (CPB) has been standard. We hypothesize that platelet administration during the rewarming phase before termination of CPB would reduce coagulopathy, enhance hemostasis, reduce transfusion, and improve postoperative outcomes after neonatal cardiac surgery.

A prospective, randomized trial was performed in 46 neonates. Controls received platelets only at the end of bypass with other blood products to assist in hemostasis. The treatment group received 10 mL/kg of platelets during the rewarming phase of bypass after cross-clamp release. After protamine, transfusion and perioperative management protocols were identical and constant among groups.

Two neonates in each group were excluded secondary to postoperative need for extracorporeal support. Controls (n = 21) and treatment patients (n = 21) were similar in age, weight, case complexity, associated syndromes, single ventricle status, and CPB times. Compared to controls, the treatment group required 40% less postbypass blood products (58 ± 29 vs 103 ± 80 mL/kg, P = .04), and case completion time after protamine administration was 28 minutes faster (P = .016). The treatment group required fewer postoperative mediastinal explorations for bleeding (P = .045) and had a lower fluid balance (P = .04). The treatment group had shorter mechanical ventilation (P = .016) and length of intensive care unit times (P = .033). There were no 30-day mortalities in either group.

Platelet transfusion during the rewarming phase of neonatal cardiac surgery was associated with reduced bleeding and improved postoperative outcomes, compared to platelets given after coming off bypass. Further studies are necessary to understand mechanisms and benefits of this strategy.

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.

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.

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.

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.

Recent studies have shown that oral budesonide can be used to improve albumin level in patients with protein-losing enteropathy (PLE) following Fontan procedure. However, there has never been a systematic review and meta-analysis to confirm this finding. We performed a systematic review and meta-analysis to explore the therapeutic effect of budesonide in patients with PLE post-Fontan procedure.

We searched the databases of MEDLINE and EMBASE from inception to January 2019. Included studies were published studies that evaluate albumin level before and after budesonide therapy in patients with PLE following Fontan procedure. Data from each study were combined using the random-effects model.

Five studies with 36 post-Fontan operation patients with PLE were included. In random-effects model, there was a statistically significant difference in albumin level between before and after budesonide treatment (weighted mean difference = 1.28, 95% confidence interval: 0.76-1.79). No publication bias was observed on a funnel plot and Egger test with a P value of .676.

The results of this systematic review and meta-analysis show that budesonide can be used to increase albumin level in patients with PLE following Fontan operation. Further studies may focus on the impact of outcome of budesonide in this population.

Numerous attempts have been made to extend the boundaries of arterial switch operation (ASO) in children presenting late with transposition of great arteries with intact ventricular septum (TGA/IVS) and regressed left ventricle (rLV). Many children tolerate the delayed ASO uneventfully, whereas others need mechanical circulatory support (MCS) to sustain the systemic circulation while the left ventricle undergoes retraining.

In this article, we describe six consecutive children with TGA/IVS and rLV who underwent primary ASO.

Three were managed medically, while three required MCS in the form of Centrimag left ventricular assist device (LVAD). All patients survived the operation and were discharged home in a stable condition.

Primary ASO can be safely performed in children with TGA/IVS and rLV, provided the center has MCS options. Supporting the rLV with LVAD is feasible and can be achieved safely.