Background. Cardiac surgery offers multiple treatment options for children with congenital heart defects. However, infectious complications still remain a major cause of morbidity and mortality in ...these patients. Mediastinitis is a detrimental complication in children undergoing cardiac surgery. The risk of mediastinitis after delayed sternal closure is up to 10%. Case Presentation. We report a case of Enterococcus faecium mediastinitis in a 4-week-old female baby on extracorporeal membrane oxygenation after Norwood procedure. Although repeated antibiotic irrigation, debridement, and aggressive antibiotic treatment were started early, the pulmonary situation deteriorated. Candida parapsilosis was isolated from bronchoalveolar lavage after pulmonary hemorrhage. Disseminated C. parapsilosis infection with pulmonary involvement was treated with liposomal amphotericin B. Subsequently, inflammatory markers increased again and eventually C. parapsilosis was isolated from the central venous catheter. Conclusion. Children undergoing delayed sternal closure have a higher risk of mediastinitis. Therefore, antibiotic prophylaxis, for example, for soft tissue infection seems justified. However, long-term antibiotic treatment is a risk factor for fungal superinfection. Antifungal treatment of disseminated C. parapsilosis infection may fail in PICU patients with nonbiological material in place due to capacity of this species to form biofilms on medical devices. Immediate removal of central venous catheters and other nonbiological material is life-saving in these patients.
Aim: Midazolam like other benzodiazepines is supposed to be neurotoxic in small children and to represent a risk factor for the development of delirium. The aim of this study was to evaluate whether ...a modified analgesia and sedation protocol is feasible and effective to reduce the requirement of midazolam in neonates and young infants after cardiac surgery.
Methods: Patients aged 6 months or younger who underwent surgery for congenital heart disease with cardiopulmonary bypass were enrolled and divided into a pre-modification group (January-December 2016) and after adjusting our sedation protocol into a post-modification group (January-December 2018). We assessed the doses of midazolam, morphine and clonidine as well as sedation scores according to our nurse-driven sedation protocol every 8 h until 120 h after cardiac surgery. During weaning from analgesia and sedation, children were monitored regarding withdrawal symptoms and pediatric delirium.
Results: Sixty-five patients were included (33 patients in the pre-modification group, 32 patients in the post-modification group). The number of patients receiving midazolam and the cumulative dose of midazolam could be successfully reduced. The sedation scores were still within the desired target range for adequate sedation without any negative side effects.
Conclusions: It is feasible and safe to reduce the use of midazolam in infants after cardiac surgery maintaining sedation goals based on a modified nurse-driven analgesia and sedation protocol.
We report interventional treatment of a congenital left ventricular aneurysm in a neonate. The aneurysm was detected prenatally at 20 weeks of gestation. Postnatally, the aneurysm increased in size ...during the first few days of life and therefore we opted for interventional closure to abolish the potential risk of rupture. The aneurysm was entered with a 2.7 French microcatheter, and complete occlusion was achieved by successive placement of three detachable large volume coils (Ruby Coil PenumbraR, Alameda, California). To the best of our knowledge, this is the first report describing interventional occlusion of a congenital ventricular aneurysm in early infancy. Interventional occlusion with detachable large volume coils appears to be an attractive alternative to surgical resection in patients presenting with ventricular outpouchings and narrow base connection to the ventricle.
One of the major obstacles preventing successful percutaneous pulmonary valve implantation (PPVI) is related to the close proximity of coronary artery branches to the expected landing zone. The aim ...of this study was to assess the frequency of coronary artery anomalies (CAAs) especially those associated with major coronary branches crossing the right ventricular outflow tract (RVOT) and to describe their relevance for the feasibility of percutaneous pulmonary valve implantation (PPVI). In our retrospective single-center study 90 patients were evaluated who underwent invasive testing for PPVI in our institution from 1/2010 to 1/2020. CAAs were identified in seven patients (8%) associated with major branches crossing the RVOT due to origin of the left anterior descending (LAD) or a single coronary artery from the right aortic sinus. In 5/7 patients with CAAs balloon testing of the RVOT and selective coronary angiographies revealed a sufficiently large landing zone distal to the coronary artery branch. While unfavorable RVOT dimensions prevented PPVI in one, PPVI was performed successfully in the remaining four patients. The relatively short landing zone required application of the “folded” melody technique in two patients. All patients are doing well (mean follow-up 3 years). CAAs associated with major coronary branches crossing the RVOT can be expected in about 8% of patients who are potential candidates for PPVI. Since the LAD crossed the RVOT below the plane of the pulmonary valve successful distal implantation of the valve was possible in 4/7 patients. Therefore these coronary anomalies should not be considered as primary contraindications for PPVI.
Tetralogy of Fallot (ToF), the most frequent cyanotic congenital heart disease, is associated with a wide range of intra- and extracardiac phenotypes. In order to get further insight into ...genotype-phenotype correlation, a large cohort of 230 unselected patients with ToF was comprehensively investigated.
230 patients with ToF were studied by karyotyping, comprehensive 22q11.2 deletion testing and sequencing of TBX1, NKX2.5 and JAG1, as well as molecular karyotyping in selected patients. Pathogenic genetic aberrations were found in 42 patients (18%), with 22q11.2 deletion as the most common diagnosis (7.4%), followed by trisomy 21 (5.2%) and other chromosomal aberrations or submicroscopic copy number changes (3%). Mutations in JAG1 were detected in three patients with Alagille syndrome (1.3%), while NKX2.5 mutations were seen in two patients with non-syndromic ToF (0.9%). One patient showed a recurrent polyalanine stretch elongation within TBX1 which represents a true mutation resulting in loss of transcriptional activity due to cytoplasmatic protein aggregation.
This study shows that 22q11.2 deletion represents the most common known cause of ToF, and that the associated cardiac phenotype is distinct for obstruction of the proximal pulmonary artery, hypoplastic central pulmonary arteries and subclavian artery anomalies. Atrioventricular septal defect associated with ToF is very suggestive of trisomy 21 and almost excludes 22q11.2 deletion. We report a further patient with a recurrent polyalanine stretch elongation within TBX1 and for the first time link TBX1 cytoplasmatic protein aggregation to congenital heart defects.
Abstract
Aims
The post-approval MELODY Registry aimed to obtain multicentre registry data after transcatheter pulmonary valve implantation (TPVI) with the Melody™ valve (Medtronic plc.) in a ...large-scale cohort of patients with congenital heart disease (CHD).
Methods and results
Retrospective analysis of multicentre registry data after TPVI with the Melody™ valve. Eight hundred and forty-five patients (mean age: 21.0 ± 11.1 years) underwent TPVI in 42 centres between December 2006 and September 2013 and were followed-up for a median of 5.9 years (range: 0–11.0 years). The composite endpoint of TPVI-related events during follow-up (i.e. death, reoperation, or reintervention >48 h after TPVI) showed an incidence rate of 4.2% per person per year 95% confidence interval (CI) 3.7–4.9. Transcatheter pulmonary valve implantation infective endocarditis (I.E.) showed an incidence rate of 2.3% per person per year (95% CI 1.9–2.8) and resulted in significant morbidity and in nine deaths. In multivariable Cox proportional hazard models, the invasively measured residual right ventricle (RV)-to-pulmonary artery (PA) pressure gradient (per 5 mmHg) was associated with the risk of the composite endpoint (adjusted hazard ratio: 1.21, 95% CI 1.12–1.30; P < 0.0001) and the risk of TPVI I.E. (adjusted hazard ratio: 1.19, 95% CI 1.07–1.32; P = 0.002). Major procedural complications (death, surgical, or interventional treatment requirement) occurred in 0.5%, 1.2%, and 2.0%, respectively. Acutely, the RV-to-PA pressure gradient and the percentage of patients with pulmonary regurgitation grade >2 improved significantly from 36 interquartile range (IQR) 24–47 to 12 (IQR 7–17) mmHg and 47 to 1%, respectively (P < 0.001 for each).
Conclusion
The post-approval MELODY Registry confirms the efficacy of TPVI with the Melody™ valve in a large-scale cohort of CHD patients. The residual invasively measured RV-to-PA pressure gradient may serve as a target for further improvement in the composite endpoint and TPVI I.E. However, TPVI I.E. remains a significant concern causing significant morbidity and mortality.
Background
Advances in the treatment of pediatric congenital heart disease have increased survival rates. Despite efforts to prevent neurological injury, many patients suffer from impaired ...neurodevelopmental outcomes. Compromised cerebral autoregulation can increase the risk of brain injury following pediatric cardiac surgery with cardiopulmonary bypass. Monitoring autoregulation and maintaining adequate cerebral blood flow can help prevent neurological injury.
Aims
Our objective was to evaluate autoregulation parameters and to define the optimal blood pressure as well as the lower and upper blood pressure limits of autoregulation.
Methods
Autoregulation was monitored prospectively in 36 infants after cardiopulmonary bypass surgery for congenital heart defects between January and December 2019. Autoregulation indices were calculated by correlating invasive arterial blood pressure, cortical oxygen saturation, and relative tissue hemoglobin levels with near‐infrared spectroscopy parameters.
Results
The mean patient age was 4.1 ± 2.8 months, and the mean patient weight was 5.2 ± 1.8 kg. Optimal mean arterial pressure could be identified in 88.9% of patients via the hemoglobin volume index and in 91.7% of patients via the cerebral oxygenation index, and a lower limit of autoregulation could be found in 66.7% and 63.9% of patients, respectively. No significant changes in autoregulation indices at the beginning or end of the monitoring period were observed. In 76.5% ± 11.1% and 83.8% ± 9.9% of the 8 and 16 h monitoring times, respectively, the mean blood pressure was inside the range of intact autoregulation (below in 21.5% ± 25.4% and 11.3% ± 16.5% and above in 8.7% ± 10.4% and 6.0% ± 11.0%, respectively). The mean optimal blood pressure was 57.4 ± 8.7 mmHg and 58.2 ± 7.9 mmHg and the mean lower limit of autoregulation was 48.8 ± 8.3 mmHg and 45.5 ± 6.7 mmHg when generated via the hemoglobin volume index and cerebral oxygenation index, respectively.
Conclusions
Postoperative noninvasive autoregulation monitoring after cardiac surgery in children can be reliably and safely performed using the hemoglobin volume index and cerebral oxygenation index and provides robust data. This monitoring can be used to identify individual hemodynamic targets to optimize autoregulation, which differs from those recommended in the literature. Further evaluation of this subject is needed.
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