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The Thoracic and Cardiovascular Surgeon Jun 2024In 2021 a total of 5439 patients were included by 22 participating centers. In total 3721 surgical, 3413 interventional and 34 hybrid procedures were performed during...
In 2021 a total of 5439 patients were included by 22 participating centers. In total 3721 surgical, 3413 interventional and 34 hybrid procedures were performed during 6122 hospital stays. 2220 cases (36.3%) could be allocated to the 15 index procedures. The mean unadjusted in-hospital mortality ranged from 0.4% among interventional and 2% among surgical cases up to 6.2 % in cases with multiple procedures. In-hospital mortality among index procedures accounted for 2.3% in TCPC, 20.3% in Norwood procedures and 0.4% following interventional closure of patent ductus arteriosus. For the remaining 7 surgical and 5 interventional index procedures no in-hospital deaths were recorded. The 10 years longitudinal evaluation of 1795 patients after tetralogy of Fallot repair revealed repeat interventional or surgical procedures in 21% of the patients. Over the same period 31.1% of 2037 patients, following initial treatment of native coarctation, required at least one additional hospital admission, 39.4% after initial interventional and 21.3% after initial surgical therapy. Conclusion The annual report 2021 of the German Registry for Cardiac Operations and Interventions in CHD shows continuously good results in accordance with previous data of the registry. Compared to international registries on CHD it can be ascertained that in Germany invasive treatment of CHD is offered on a high medical level with excellent quality. The proven fact that patients with various malformations like tetralogy of Fallot and coarctation of the aorta require repeat procedures during follow-up confirms the urgent requirement of longitudinal assessment of all patients presenting with complex lesions.
PubMed: 38914128
DOI: 10.1055/a-2350-7374 -
Cureus May 2024Cyclin-dependent kinase 13 (CDK13)-related disorder is a rare autosomal dominant disease caused by pathogenic variants in the gene. This disorder was found to be...
Cyclin-dependent kinase 13 (CDK13)-related disorder is a rare autosomal dominant disease caused by pathogenic variants in the gene. This disorder was found to be related to several clinical features, including structural cardiac anomalies, developmental delay, anomalies of the corpus callosum, and a variety of facial dysmorphisms. In addition, feeding difficulties and neonatal hypotonia might also present. The diagnosis of this disorder is based on molecular genetic testing to detect the causative pathogenic variants. Here, we report a case of a one-year-old girl from Yemen, residing in Bahrain, with a CDK13-related disorder who was found to have an unusual association of abdominal situs inversus along with multiple structural cardiac anomalies, including atrial septal defect, ventricular septal defect, patent ductus arteriosus, interrupted inferior vena cava, bilateral superior vena cava, mild coarctation of the aorta, dilated coronary sinuses, and mild regurgitation in the tricuspid valve. Moreover, facial dysmorphism including medial epicanthal folds, posteriorly rotated ears, and a depressed nasal bridge was also noted. Further assessment showed a delay in reaching developmental milestones, including speech and motor delay. The patient also presented with recurrent episodes of upper respiratory tract infections, acute bronchiolitis, and lobar pneumonia which required admission to the intensive care unit and ventilation. The last infection episode was at the age of one year. Thereafter, the patient underwent cardiac repair of the ventricular septal defect followed by no more infection episodes until the age of one year and two months. The diagnosis of CDK13 was confirmed by a whole exome sequencing test which demonstrated a novel missense variant in exon 14 of the gene as a variant of uncertain significance in a heterozygous state.
PubMed: 38910624
DOI: 10.7759/cureus.60970 -
Cureus May 2024Congenital complete heart block (CCHB) is a rare and potentially life-threatening condition, often associated with maternal autoantibodies. We present the case of a...
Congenital complete heart block (CCHB) is a rare and potentially life-threatening condition, often associated with maternal autoantibodies. We present the case of a one-year-old girl with recurrent respiratory symptoms, ultimately diagnosed with CCHB and congenital heart disease. She exhibited bradycardia and signs of congestive heart failure. A diagnostic workup revealed significant cardiac abnormalities, including dilated chambers, ventricular septal defect, and patent ductus arteriosus. Serological tests for maternal autoantibodies were negative. The child's parents opted for discharge without surgical intervention. This case underscores the importance of comprehensive evaluation and management strategies in patients with congenital heart block, particularly in resource-limited settings.
PubMed: 38903295
DOI: 10.7759/cureus.60720 -
Journal of Clinical Medicine May 2024: Transcatheter interventions are increasingly used in children with congenital heart disease. However, these interventions can affect cardiac output and cerebral...
: Transcatheter interventions are increasingly used in children with congenital heart disease. However, these interventions can affect cardiac output and cerebral circulation. In this pilot study, we aimed to investigate the use of NeoDoppler, a continuous transfontanellar cerebral Doppler monitoring system, to evaluate the impact of transcatheter interventions on cerebral circulation. : Nineteen participants under one year of age (mean age 3.5 months) undergoing transcatheter cardiac interventions were prospectively included. Transfontanellar cerebral Doppler monitoring with the NeoDoppler system was initiated after intubation and continued until the end of the procedure. : Instant detection of changes in cerebral blood flow were observed across a spectrum of transcatheter interventions. Balloon aortic valvuloplasty demonstrated temporary cessation of cerebral blood flow during balloon inflation. Increase in cerebral diastolic blood flow velocity and decreased pulsatility were observed during patent ductus arteriosus occlusion. Changes in cerebral blood flow patterns were detected in two patients who encountered complications during their transcatheter interventions. There was no significant change in Doppler parameters before and after the interventions for the entire patient group. High quality recordings were achieved in 87.3% of the monitoring period. : Continuous transfontanellar cerebral Doppler is feasible in monitoring cerebral hemodynamic trends and shows instantaneous changes associated with interventions and complications. It could become a useful monitoring tool during transcatheter interventions in infants.
PubMed: 38892827
DOI: 10.3390/jcm13113115 -
Nutrients May 2024The nutrition of preterm infants remains contaminated by wrong beliefs that reflect inexactitudes and perpetuate old practices. In this narrative review, we report... (Review)
Review
The nutrition of preterm infants remains contaminated by wrong beliefs that reflect inexactitudes and perpetuate old practices. In this narrative review, we report current evidence in preterm neonates and in preterm neonates undergoing surgery. Convictions that necrotizing enterocolitis is reduced by the delay in introducing enteral feeding, a slow advancement in enteral feeds, and the systematic control of residual gastric volumes, should be abandoned. On the contrary, these practices prolong the time to reach full enteral feeding. The length of parenteral nutrition should be as short as possible to reduce the infectious risk. Intrauterine growth restriction, hemodynamic and respiratory instability, and patent ductus arteriosus should be considered in advancing enteral feeds, but they must not translate into prolonged fasting, which can be equally dangerous. Clinicians should also keep in mind the risk of refeeding syndrome in case of high amino acid intake and inadequate electrolyte supply, closely monitoring them. Conversely, when preterm infants undergo surgery, nutritional strategies are still based on retrospective studies and opinions rather than on randomized controlled trials. Finally, this review also highlights how the use of adequately fortified human milk is strongly recommended, as it offers unique benefits for immune and gastrointestinal health and neurodevelopmental outcomes.
Topics: Humans; Infant, Newborn; Infant, Premature; Infant Nutritional Physiological Phenomena; Milk, Human; Enteral Nutrition; Enterocolitis, Necrotizing; Parenteral Nutrition; Food, Fortified
PubMed: 38892652
DOI: 10.3390/nu16111719 -
International Journal of Cardiology Jun 2024The purpose of this study was to analyse the association between stannum exposure during pregnancy and congenital heart diseases in offspring.
BACKGROUND
The purpose of this study was to analyse the association between stannum exposure during pregnancy and congenital heart diseases in offspring.
METHODS
Based on a prospective birth cohort study conducted in Gansu Maternal and Child Health Hospital from 2010 to 2012, 14,359 pregnant women were followed up using a nested case-control study method. 97 pregnant women whose offspring were diagnosed with CHDs were used as the case group, and 194 pregnant women whose offspring did not suffer from congenital heart diseases were used as the control group in a ratio of 1:2 according to their age and place of birth. Inductively coupled plasma mass spectrometry was used to determine elemental stannum in blood samples from pregnant women hospitalized for delivery and in fetal cord blood samples. Multifactorial logistic regression analysis was used to assess the association between stannum and offspring CHDs.
RESULTS
There was a moderate positive correlation between the concentration of stannum in pregnant women's blood and that in umbilical cord blood. A higher concentrations of maternal blood stannum level was associated with a greater risk of CHDs (aOR 3.409, 95%CI 1.785-6.826), isolated CHDs (aOR 4.044, 95%CI 1.803-9.070), multiple CHDs (aOR 2.625, 95%CI 1.137-6.061), patent ductus arteriosus (aOR 2.882, 95%CI 1.443-5.756), atrial septal defects (aOR 3.067, 95%CI 1.406-6.690), ventricular septal defects (aOR 7.414, 95%CI 1.414-38.874). There was a correlation between the maternal and cord blood sample suggesting stannum crosses the placenta.
PubMed: 38880423
DOI: 10.1016/j.ijcard.2024.132270 -
BMC Pregnancy and Childbirth Jun 2024To investigate the prognosis of the remaining fetus in twin pregnancy after experiencing one fetal demise in the first trimester according to the location of the demised...
BACKGROUND
To investigate the prognosis of the remaining fetus in twin pregnancy after experiencing one fetal demise in the first trimester according to the location of the demised fetus.
METHODS
This was a retrospective study of twin pregnancies with one fetal demise after the first trimester (14 weeks of gestation) delivered between September 2004 and September 2022. The study population was divided into two groups based on the location of the demised fetus as determined by the last recorded ultrasonography results: Group 1 included twin pregnancies where the presenting fetus was demised (n = 36) and Group 2 included twin pregnancies where the non-presenting fetus was demised (n = 44). The obstetric and neonatal outcomes were also reviewed.
RESULTS
A total of 80 pregnant women were included. The median gestational age for the diagnosis of fetal demise was 24.1 weeks. The gestational age of the demised fetus was not different between Groups 1 and 2; however, the gestational age of the remaining fetus at delivery was significantly earlier in Group 1 than it was in Group 2 (33.8 vs. 37.3 weeks, P = .004). The rate of preterm birth before 28 weeks was almost five times higher in Group 1 than in Group 2 (22.2% vs. 4.5%, P = .037). Regression analysis demonstrated significant differences between Groups 1 and 2. Respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus, retinopathy of prematurity, and jaundice were more common in Group 1 than in Group 2; however, the association was not significant after adjusting for gestational age at delivery.
CONCLUSIONS
When the presenting fetus is demised in a twin pregnancy, the remaining fetus tends to be delivered earlier than when the non-presenting fetus is demised.
Topics: Humans; Female; Pregnancy; Pregnancy, Twin; Retrospective Studies; Adult; Fetal Death; Gestational Age; Prognosis; Infant, Newborn; Premature Birth; Pregnancy Outcome; Pregnancy Trimester, First; Ultrasonography, Prenatal; Fetus
PubMed: 38877391
DOI: 10.1186/s12884-024-06621-w -
Radiology Case Reports Aug 2024Ventricular septal defect is the most common congenital heart disease in children and is associated with patent ductus arteriosus in 1%-7% of cases. The coexistence of...
Diagnostic approach and treatment of ventricular septal defect associated with PDA, coarctation of aorta, hypoplastic aortic arch and multiple valvular heart disease in a tertiary center: An infrequent association.
Ventricular septal defect is the most common congenital heart disease in children and is associated with patent ductus arteriosus in 1%-7% of cases. The coexistence of both malformities with hypoplastic aortic arch and aortic coarctation is even rarer. We present the case of a 6-year-old girl referred to our hospital because of dyspnea on feeding, recurrent respiratory infections, poor weight gain, and a heart murmur. The image studies revealed a ventricular septal defect, patent ductus arteriosus, severe hypoplasia of the aortic arch with critical stenosis of the proximal portion, severe dilatation of the pulmonary artery and pulmonary, mitral, tricuspid, and aortic regurgitation. We will discuss the diagnostic approach and treatment in a tertiary reference center for patients with cardiovascular diseases.
PubMed: 38872743
DOI: 10.1016/j.radcr.2024.05.034 -
The Journal of Invasive Cardiology Jun 2024
PubMed: 38870444
DOI: 10.25270/jic/24.00139 -
The Journal of Pediatric Pharmacology... Jun 2024Acetaminophen (APAP) is an alternative to indomethacin and ibuprofen for treatment of patent ductus arteriosus (PDA). The side effect profile of non-steroidal...
OBJECTIVE
Acetaminophen (APAP) is an alternative to indomethacin and ibuprofen for treatment of patent ductus arteriosus (PDA). The side effect profile of non-steroidal anti-inflammatory drugs (NSAIDs) presents enteral feeding safety concerns; however, the safety of enteral feeding on APAP is largely unknown. Optimal feeding strategies during pharmacological PDA treatment are unknown, leading to practice variation. This study aims to assess the incidence of adverse gastrointestinal (GI) outcomes in neonates treated with APAP for PDA closure while receiving enteral feedings.
METHODS
Single-center retrospective cohort study of 59 extremely low birth weight (ELBW), premature neonates who received APAP for PDA treatment divided into Low Volume (LV; ≤ 20 mL/kg/day) and High Volume (HV; > 20 mL/kg/day) enteral feeding groups. The primary outcome was the incidence of any suspected or confirmed necrotizing enterocolitis (NEC). Timing of nutrition milestones, parenteral nutrition (PN) days, and adverse outcomes (feeding intolerance, liver dysfunction, death prior to discharge) were evaluated.
RESULTS
The incidence of suspected or confirmed NEC was 19.5% in the LV group and 13.3% in the HV group (p = 0.593). The HV group reached full feeds 6 days sooner (18 vs 24 days, p = 0.024) and had fewer PN days (17 vs 23.5 days, p = 0.044) with no difference in adverse outcomes.
CONCLUSIONS
Provision of > 20 mL/kg/day of enteral feeds during APAP treatment of PDA decreased time to full feeds and PN days compared to trophic feedings (≤ 20 mL/kg/day) with no difference in adverse GI outcomes. Continuing enteral feeding during APAP PDA treatment appears safe while improving achievement of nutritional milestones.
PubMed: 38863856
DOI: 10.5863/1551-6776-29.3.278