Infants with truncus arteriosus typically undergo repair by repurposing the truncal valve as the neo-aortic valve and using a valved conduit homograft for the neo-pulmonary valve. In cases where the native truncal valve is too insufficient for repair, it is replaced, but this is a rare occurrence with a paucity of data, especially in the infant population. Here, we conduct a meta-analysis to better understand the outcomes of infant truncal valve replacement during the primary repair of truncus arteriosus.

We systematically reviewed PubMed, Scopus, and CINAHL for all studies reporting infant (<12 months) truncus arteriosus outcomes between 1974 and 2021. Exclusion criteria were studies which did not report truncal valve replacement outcomes separately. Data extracted included valve replacement type, mortality, and reintervention. Our primary outcome was early mortality, and our secondary outcomes were late mortality and reintervention rates.

Sixteen studies with 41 infants who underwent truncal valve replacement were included. The truncal valve replacement types were homografts (68.8%), mechanical valves (28.1%), and bioprosthetic valves (3.1%). Overall early mortality was 49.4% (95% CI: 28.4–70.5). The pooled late mortality rate was 15.3%/year (95% CI: 5.8–40.7). The overall rate of truncal valve reintervention was 21.7%/year (95% CI: 8.4–55.7).

Infant truncal valve replacement has poor early and late mortality as well as high rates of reintervention. Truncal valve replacement therefore remains an unsolved problem in congenital cardiac surgery. Innovations in congenital cardiac surgery, such as partial heart transplantation, are required to address this.

Infradiaphragmatic partial anomalous pulmonary venous connection is occasionally diagnosed in adulthood. Management of infradiaphragmatic PAPVC depends on anatomy and clinical presentation.

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.

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.

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.

Neonatal enteroviral myocarditis is a rare but potentially fatal illness. We sought to identify echocardiographic markers at diagnosis that could help risk-stratify infants for poor outcome and to characterise late sequelae.

We reviewed data for infants <30 days of age diagnosed with enteroviral myocarditis between 1999 and 2019 at Children’s Wisconsin. Echo measures were collected retrospectively from the initial neonatal study including left ventricular ejection fraction, shortening fraction, diastolic and systolic dimensions, and peak global circumferential and longitudinal strain.

Fourteen neonates were diagnosed at an average age of 11 days. All had abnormal left ventricular ejection fraction (mean 38%; range 22–53%) at diagnosis. Three infants died, and one required transplantation during initial hospital. The 10 transplant-free survivors had significantly better global circumferential strain and global longitudinal strain at the initial echo compared to the 4 who died or needed transplant (global circumferential strain −13.2% versus −6.8%, p = 0.005; global longitudinal strain −8.8% versus −4.7%, p = 0.016). All other measures of left ventricular systolic function/dimensions were similar between the two groups. Follow-up data were available for 8/10 survivors; 5/8 had a persistently abnormal echo at an average interval of 8.3 years. 4/8 developed a left ventricular aneurysm that was consistently localised to the posterior basal wall.

Neonatal enteroviral myocarditis carries a high risk of early mortality and late morbidity. Echo-derived left ventricular strain measures have utility in risk stratifying infants with enteroviral myocarditis. Most survivors continue to have late dysfunction necessitating cardiology surveillance and medical therapy.

Mortality between stages 1 and 2 single-ventricle palliation is significant. Home-monitoring programmes are suggested to reduce mortality. Outcomes and risk factors for adverse outcomes for European programmes have not been published.

To evaluate the performance of a home-monitoring programme at a medium-sized United Kingdom centre with regards survival and compare performance with other home-monitoring programmes in the literature.

All fetal and postnatal diagnosis of a single ventricle were investigated with in-depth analysis of those undergoing stage 1 palliation and entered the home-monitoring programme between 2016 and 2020. The primary outcome was survival. Secondary outcomes included multiple parameters as potential predictors of death or adverse outcome.

Of 217 fetal single-ventricle diagnoses during the period 2016–2020, 50.2% progressed to live birth, 35.4% to stage 1 and 29.5% to stage 2. Seventy-four patients (including 10 with postnatal diagnosis) entered the home-monitoring programme with six deaths making home-monitoring programme mortality 8.1%. Risk factors for death were the hybrid procedure as the only primary procedure (OR 33.0, p < 0.01), impaired cardiac function (OR 10.3, p < 0.025), Asian ethnicity (OR 9.3, p < 0.025), lower mean birth-weight (2.69 kg versus 3.31 kg, p < 0.01), and lower mean weight centiles during interstage follow-up (mean centiles of 3.1 versus 10.8, p < 0.01)

Survival in the home-monitoring programme is comparable with other home-monitoring programmes in the literature. Hybrid procedure, cardiac dysfunction, sub-optimal weight gain, and Asian ethnicity were significant risk factors for death. Home-monitoring programmes should continue to raise awareness of these factors and seek solutions to mitigate adverse events. Future work to generalise home-monitoring programme and single-ventricle fetus to stage 2 outcomes in the United Kingdom will require multi-centre collaboration.

Intraoperative imaging determines the integrity of surgical repairs. Transoesophageal echocardiography represents standard care for intraoperative imaging in CHD. However, some conditions preclude its use, and epicardial echocardiography is used alternatively. Minimal literature exists on the impact of epicardial echocardiography versus transoesophageal echocardiography. We aimed to evaluate accuracy between the two modalities and hypothesised higher imaging error rates for epicardial echocardiography.

We retrospectively reviewed all epicardial echocardiograms performed over 16 years and compared them to an age- and procedure-matched, randomly selected transoesophageal echocardiography cohort. We detected un- or misidentified cardiac lesions during the intraoperative imaging and evaluated patient outcomes. Data are presented as a median with a range, or a number with percentages, with comparisons by Wilcoxon two-sample test and Fisher’s exact test.

Totally, 413 patients comprised the epicardial echocardiography group with 295 transoesophageal echocardiography matches. Rates of imaging discrepancies, re-operation, and incision infection were similar. About 13% of epicardial echocardiography patients had imaging discrepancies versus 16% for transoesophageal (p = 0.2352), the former also had smaller body sizes (p < 0.0001) and more genetic abnormalities (33% versus 19%, p < 0.0001). Death/mechanical support occurred more frequently in epicardial echocardiography patients (16% versus 6%, p < 0.0001), while hospitalisations were longer (25 versus 19 days, p = 0.0003).

Diagnostic accuracy was similar between patients undergoing epicardial echocardiography and transoesophageal echocardiography, while rates of death and mechanical support were increased in this inherently higher risk patient population. Epicardial echocardiography provides a reasonable alternative when transoesophageal echocardiography is not feasible.

Systemic-to-pulmonary shunts are used as a source of pulmonary blood flow in palliated Congenital Heart Disease in neonates and young infants. Shunt thrombosis, often requiring shunt interventions during index hospitalisation, is associated with poor outcomes. We hypothesised that extensive use of perioperative pro-coagulant products may be associated with shunt thrombosis.

Children (≤18 years) undergoing systemic-to-pulmonary shunts with in-hospital shunt reinterventions between 2016 and 2020 were reviewed retrospectively. Perioperative associations to shunt thrombosis were examined by univariate logistic regression and Wilcoxon rank sum tests as appropriate. Cox and log transformed linear regression were used to analyse postoperative ventilation duration, length of stay, and cost.

Of 71 patients requiring in-hospital shunt intervention after systemic-to-pulmonary shunts, 10 (14%) had acute shunt thrombosis, and among them five (50%) died. The median age was four (interquartile range: 0-15) months. There were 40 (56%) males, 41 (58%) had single ventricle anatomy, and 29 (40%) were on preoperative anticoagulants. Patients with acute shunt thrombosis received greater volume of platelets (p = 0.04), cryoprecipitate (p = 0.02), and plasma (p = 0.04) postoperatively in the ICU; experienced more complications (p = 0.01) including re-exploration for bleeding (p = 0.008) and death (p = 0.02), had longer hospital length of stays (p = 0.004), greater frequency of other arterial/venous thrombosis (p = 0.02), and greater hospital costs (p = 0.002).

Patients who develop acute shunt thrombosis receive more blood products perioperatively and experience worse hospital outcomes and higher hospital costs. Future research on prevention/early detection of shunt thrombosis is needed to improve outcomes in infants after systemic-to-pulmonary shunt surgery.

The optimal visceral preservation method during aortic arch reconstruction is still controversial. It has been thought that double aortic cannulation is effective. Herein, it was aimed to evaluate this technique in providing distal perfusion.

A total of 74 patients who underwent arch reconstruction between 2011 and 2019 were included. Patients were grouped according to ventricular physiology and cannulation strategies. Group 1 were univentricle patients, and all had double aortic cannulation. Group 2 were biventricular patients. Group 2A double aortic cannulation-done and Group 2B non-double aortic cannulation were included. Lactate, urea, creatinine values, renal functions, and need for peritoneal dialysis of patients were evaluated.

There were no complications observed due to descending aortic cannulation in any of the patients. A delayed sternal closure and the need for peritoneal dialysis were more common in the Group 1 (p < 0.01). The preoperative and postoperative 1st- and 2nd-day lactate, urea, and creatinine values in the Group 1 were higher (p < 0.05) when compared with the Group 2A and 2B. The same values were higher in Group 2A than the Group 2B (p < 0.05).

The positive effect of double aortic cannulation on renal dysfunction could not be demonstrated. This may be associated with a <1 month of age, low weight, complex surgical procedure, and high preoperative lactate, urea, and creatinine values in patients with double aortic cannulation.

In this single-centre prospective study, we aimed to evaluate the role of growth differentiation factor-15 in children with acute rheumatic fever.

The study group included 25 children with acute rheumatic fever, and the control group included 25 healthy children. In addition to routine laboratory tests used in the diagnosis and treatment of acute rheumatic fever, growth differentiation factor-15 levels of the study group (at the time of diagnosis and after the treatment) and the control group were assessed and compared.

The mean growth differentiation factor-15 level of the study group at the time of diagnosis (918.40 ± 605.65 pg/ml) was significantly higher than the mean post-treatment level (653.08 ± 330.92 pg/ml) (p = 0.015). Similarly, the mean growth differentiation factor-15 level of the study group at the time of diagnosis was significantly higher than the control group (p = 0.04). However, mean growth differentiation factor-15 levels were similar between the groups after the treatment. Growth differentiation factor-15 was positively correlated with both C-reactive protein (p < 0.001) and erythrocyte sedimentation rate (p = 0.001) at the time of diagnosis.

Growth differentiation factor-15 levels are significantly increased in children with acute rheumatic fever at the time of diagnosis and return to similar levels with healthy children after treatment. Growth differentiation factor-15 is positively and significantly correlated with erythrocyte sedimentation rate and C-reactive protein at the time of diagnosis.

The incidence of prosthetic valve implantation is increasing in the paediatric population. Prosthetic valve thrombosis leading to obstruction could potentially be a life-threatening complication. There is a debate regarding optimal management of this complication, and there is limited use of thrombolytic therapy in childhood in the setting of valve thrombosis.

We aim to share our experience of successfully using fibrinolytic therapy in terms of alteplase for paediatric prosthetic mitral valve thrombosis and to propose a management algorithm.

This retrospective analysis of the database was conducted at our hospital including patients who underwent thrombolysis (alteplase) for prosthetic mitral valve thrombosis from June, 2011 to June, 2021. A total of 10 patients with 20 attempts of alteplase infusion were found in our record.

Alteplase was successful in 19 attempts to relieve valve thrombosis. The safe and effective dose of alteplase was between 0.1 and 0.3 mg/kg/hour. There were no associated major bleeding complications and alteplase was administered either by central or peripheral line.

Thrombolysis by alteplase infusion was found to be successful in relief of prosthetic mitral valve thrombosis in paediatric population without major bleeding complications.

Electroanatomic mapping systems are increasingly used during ablations to decrease the need for fluoroscopy and therefore radiation exposure. For left-sided arrhythmias, transseptal puncture is a common procedure performed to gain access to the left side of the heart. We aimed to demonstrate the radiation exposure associated with transseptal puncture.

Data were retrospectively collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry. Patients with left-sided accessory pathway-mediated tachycardia, with a structurally normal heart, who had a transseptal puncture, and were under 22 years of age were included. Those with previous ablations, concurrent diagnostic or interventional catheterisation, and missing data for fluoroscopy use or procedural outcomes were excluded. Patients with a patent foramen ovale who did not have a transseptal puncture were selected as the control group using the same criteria. Procedural outcomes were compared between the two groups.

There were 284 patients in the transseptal puncture group and 70 in the patent foramen ovale group. The transseptal puncture group had a significantly higher mean procedure time (158.8 versus 131.4 minutes, p = 0.002), rate of fluoroscopy use (38% versus 7%, p < 0.001), and mean fluoroscopy time (2.4 versus 0.6 minutes, p < 0.001). The acute success and complication rates were similar.

Performing transseptal puncture remains a common reason to utilise fluoroscopy in the era of non-fluoroscopic ablation. Better tools are needed to make non-fluoroscopic transseptal puncture more feasible.

Permanent pacing in children with isolated congenital complete atrioventricular block may cause left ventricular dysfunction. To prevent it, alternative pacing sites have been proposed: left ventricular epicardial or selective right ventricular endocardial pacing.

To compare the functional outcome (left ventricular systolic function and synchrony) in paediatric patients with congenital complete atrioventricular block and left ventricular apical epicardial or right ventricular transvenous mid-septal pacing.

Retrospective study. Epicardial leads were implanted by standard surgical technique, transvenous leads by 3D electroanatomic mapping systems. 3D mapping acquired 3D right ventricular local pacing map and defined the narrowest paced QRS site. 3D mapping guided screw-in bipolar leads on that ventricular site. Electrocardiogram (ECG) (QRS duration) and echocardiographic data (synchrony: interventricular mechanical delay, septal to posterior wall motion delay, systolic dyssynchrony index; contractility: global longitudinal strain, ejection fraction) were recorded. Data are reported as median [interquartile ranges]. p < 0.05 was significant.

There were 19 transvenous systems (age 8.8 [6–14] years; right ventricular mid-septum) and 17 epicardial systems (0.04 [0.001–0.6] years; left ventricular apex). Post-implantation QRS significantly widened either in endocardial or in epicardial patients. Most patients reached 4-year follow-up. One-year and 4-year ejection fraction and global longitudinal strain were mostly within normal limits and did not show significant differences between the two groups and between the same endocardial/epicardial group. Synchrony parameters were within normal limits in the two groups.

Left ventricular apical epicardial pacing and 3D mapping-guided right ventricular mid-septal pacing preserved left ventricular contractility and synchrony in children and adolescents with congenital complete atrioventricular block at short-/mid-term follow-up, without relevant significant differences between the two groups.

Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life.

In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life.

Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 – 176 days, 3rd = 178 – 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year.

Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

To investigate the correlation between ventricular pre-excitation-related dyssynchrony, on cardiac dysfunction, and recovery.

This study included 76 children (39 boys and 37 girls) with a median age of 5.25 (2.67–10.75) years. The patients with pre-excitation-related cardiac dysfunction (cardiac dysfunction group, n = 34) had a longer standard deviation of the time-to-peak systolic strain of the left ventricle and larger difference between the maximum and minimum times-to-peak systolic strain than those with a normal cardiac function (normal function group, n = 42) (51.77 ± 24.70 ms versus 33.29 ± 9.48 ms, p < 0.05; 185.82 ± 92.51 ms versus 111.93 ± 34.27 ms, p < 0.05, respectively). The cardiac dysfunction group had a maximum time-to-peak systolic strain at the basal segments of the anterior and posterior septa and the normal function group at the basal segments of anterolateral and posterolateral walls. The prevalence of ventricular septal dyssynchrony in the cardiac dysfunction group was significantly higher than that in the normal function group (94.1% (32/34) versus 7.7% (3/42), p < 0.05). The patients with ventricular septal dyssynchrony (n = 35) had a significantly higher prevalence of intra-left ventricular systolic dyssynchrony than those with ventricular septal synchrony (n = 41) (57.1% (20/35) versus 14.6% (6/41), p < 0.05). During follow-up after pathway ablation, the patients who recovered from intra-left ventricular dyssynchrony (n = 29) had a shorter left ventricular ejection fraction recovery time than those who did not (n = 5) (χ2 = 5.94, p < 0.05). Among the patients who recovered, 93.1% (27/29) had a normalised standard deviation of the time-to-peak systolic strain and difference between the maximum and minimum times-to-peak systolic strain within 1 month after ablation.

Ventricular pre-excitation may cause ventricular septal dyssynchrony; thus, attention must be paid to intra-left ventricular dyssynchrony and cardiac dysfunction. Whether intra-left ventricular systolic dyssynchrony can resolve within 1 month may be a new early predictor of patient prognosis.

Surgical care for CHD is increasingly available in low- and middle-income countries, and efforts to optimise outcomes are growing. This study characterises cardiac imaging and prenatal diagnosis infrastructure in this setting.

An infrastructure survey was administered to sites participating in the International Quality Improvement Collaborative for CHD. Questions regarding transthoracic, transesophageal and epicardial echocardiography, cardiac CT, cardiac magnetic resonance, prenatal screening and fetal echocardiography were included. Associations with in-hospital and 30-day mortality were assessed.

Thirty-seven sites in 17 countries responded. Programme size and geography varied considerably: < 250 cases (n = 13), 250–500 cases (n = 9), > 500 cases (n = 15); Americas (n = 13), Asia (n = 18), and Eastern Europe (n = 6). All had access to transthoracic echo. Most reported transesophageal and epicardial echocardiography availability (86 and 89%, respectively). Most (81%) had cardiac CT, but only 54% had cardiac magnetic resonance. A third reported impediments to imaging, including lack of portable machines, age/size-appropriate equipment and advanced cardiac imaging access and training. Only 19% of centres reported universal prenatal CHD screening in their catchment area, and only 46% always performed fetal echocardiography if screening raised concern for CHD. No statistically significant associations were identified between imaging modality availability and surgical outcomes.

Although access to echocardiography is available in most middle-income countries; advanced imaging modalities (cardiac CT and magnetic resonance) are not always accessible. Prenatal screening for CHD is low, and availability of fetal echocardiography is limited. Imaging infrastructure in low- and middle-income countries and associations with outcomes merits additional study.