The evaluation of the patent ductus arteriosus (PDA) in the very premature neonate is a challenge. Echocardiography provides an interpretation of the hemodynamic condition. It is however, only a ...snapshot. Biomarkers may represent a physiological response to the hemodynamic alterations brought on by the PDA and may add to the identification of the clinical significant PDA.
To investigate the association between mid regional proadrenomodulin (MR-proADM), N-terminal pro b-type natriuretic peptide (NT-proBNP), mid regional pro-atrial natriuretic peptide (MR-proANP), C-terminal pro endothelin-1 (CT-proET1) and copeptin and echocardiographic measures of PDA.
Cohort study with echocardiography performed on day 3 and 6. Blood samples from day 3.
139 consecutive neonates born at a gestational age <32 weeks.
The main outcomes were presence of a PDA day 3 and 6, PDA diameter, left atrium to aorta ratio (LA:Ao-ratio), and descending aorta diastolic flow (DADF).
Adjusted plasma levels of all investigated biomarkers, except CT-proET1, were found to be associated with both PDA diameter and LA:Ao-ratio, and also the presence of a large PDA. CT-proET1 and copeptin was found to be associated with abnormal DADF. Using pre-specified cut-off values NT-proBNP and MR-proANP day 3 seemed to be of value in identifying a large PDA day 3 and 6 in very preterm neonates.
Among the investigated biomarkers NT-proBNP and MR-proANP performed best in relation to echocardiographic markers of PDA severity in very preterm neonates.
•The assessment of the patent ductus arteriosus (PDA) in the preterm neonate is a challenge.•We investigated 5 candidate biomarkers association with PDA in 139 very preterm neonates.•NT-proBNP and MR-proANP may be of value in the detection of a large PDA in preterm neonates.
Background
Extra-renal involvement in hemolytic uremic syndrome (HUS) includes gastrointestinal, pancreatic, hepatic, neurological and cardiac manifestations. The current 3–5% mortality rate in HUS ...patients is primarily attributed to complications related to the central nervous system and the heart. In this brief report, we illustrate that severe cardiac involvement in a patient with HUS is potentially reversible using cardiopulmonary bypass as rescue.
Case-Diagnosis/Treatment
A 12-year-old boy was diagnosed with enterohemorrhagic
Escherichia coli
-induced HUS related to
E. coli
serotypes O55:H7 and O121:H19. The patient developed anuria and hypertension of 150/105 mmHg and had neurological symptoms, with lethargy, confusion and later a tonic-clonic seizure successfully treated with midazolam. Laboratory tests on blood samples revealed acute renal failure, with a creatinine level of 3.98 mg/dL, thrombocytopenia of 47 × 10
9
/L, lactate dehydrogenase level of 3620 IU/L, low haptoglobin (<20 mg/dL), anemia (10.0 g/dL) and schistocytes on blood smears. Peritoneal dialysis was initiated without complications. Serum potassium level was normal. At day 3, the patient suffered cardiac arrest on two separate occasions. Troponin-T, creatine kinase and creatine kinase-MB levels were significantly increased. The second episode of cardiac arrest could not be reversed with advanced cardiopulmonary resuscitation, and a cardiopulmonary bypass circuit was established. Declining cardiac pump function to a near non-contractile state with an ejection fraction of <10% was observed on echocardiography. This persisted during the following days. After the patient had been on the cardiopulmonary bypass (CPB) circuit for 7 days, the myocardium slowly recovered function. Three days later, the CPB was successfully discontinued; the echocardiography showed near-normal ejection fraction, and electrocardiography (ECG) showed sinus rhythm.
Conclusions
Fatal outcome in patients with HUS may be the result of severe cardiac involvement. The present case illustrates the need for intensive supportive care, including the use of CPB, as the cardiac symptoms in HUS patients may be reversible. We suggest the monitoring of cardiac-specific enzymes, ECG and echocardiography in high-risk patients.
Aim: Describe the relation between levels of total serum bilirubin (TsB) ≥450 μmol/L and acute intermediate, acute advanced and chronic bilirubin encephalopathy.
Material and methods: All infants ...born at gestational age ≥35 weeks in Denmark between 2000 and 2007 with a TsB ≥450 μmol/L according to the national laboratory information system. Infants diagnosed with bilirubin encephalopathy were found in the Danish National Registry of Patients.
Results: 502 766 infants at gestational age ≥35 weeks were identified. Two hundred twenty‐four developed a TsB ≥450 μmol/L, equivalent to an incidence of 45/100 000/year, and it increased during the period. Incidence of infants with peak TsB of 450–499, 500–599 and 600–1000 μmol/L were 29.6, 12.7 and 2.2 per 100 000, respectively. Three infants had acute advanced bilirubin encephalopathy and got severe sequelae, whereas the two infants with acute intermediate encephalopathy developed normally. Their peak TsB was ≥544 μmol/L. Having a peak TsB 600–1000 μmol/L, the risk of acute advanced and chronic bilirubin encephalopathy was 27% (95% CI 6;61), and the incidence of these conditions was 0.6 (95% CI 0.1;1.7) per 100 000.
Conclusion: The incidence of infants with TsB ≥450 μmol/L was 45/100 000/year. Infants with a TsB ≥600 μmol/L had a substantial risk of developing acute advanced and chronic bilirubin encephalopathy, and the incidence of these conditions was 0.6 per 100 000.
Radiofrequency catheter ablation (RFA) is the treatment of choice for a variety of cardiac arrhythmias in adults. RFA is considered effective and is associated with few complications. We aimed to ...review the characteristics and outcomes of invasive electrophysiological study (EPS) and RFA in children with supraventricular tachyarrhythmia.
Consecutive patients younger than 16 years of age undergoing EPS and possible RFA from January 2009 to September 2018 at Aarhus University Hospital (uptake three million people) were reviewed retrospectively. Procedural and outcome data were collected from patient charts and from the Danish Ablation Database. Numbers (%) or median (range) are reported.
A total of 304 patients (148 girls) underwent EPS (352 procedures). RFA was performed in 246 patients (279 procedures), aged 13 (1-15) years and weighing 46 (6-99) kg. Treatment success was achieved in 195 (79%) of the initial procedures. Using more than one procedure, 227 (92%) patients were free from arrhythmia after 89 (26-143) months of follow-up. The procedure time was 60 (22-222) min. and ablation time 2 (1-23) min. Major complications occurred in two cases. One patient developed transient superior vena cava syndrome and one patient developed an atrioventricular block requiring pacemaker implantation.
RFA may be performed in children with a high success rate and a low but not negligible risk of complications.
none.
Approval was obtained from the Danish Data Protection Agency (1-16-02-430-13).
Left ventricular aneurysm is a potentially serious but rare condition in children. This case describes delayed but fatal rupture of an occult posttraumatic left ventricular aneurysm in an 11-year-old ...boy with a history of blunt chest trauma from a high-impact automobile collision 7 months earlier. (Level of Difficulty: Intermediate.)
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Infants with Noonan Syndrome and hypertrophic cardiomyopathy have a poor prognosis and a high mortality especially when diagnosed before six months of age. As for the majority of the RASopathies, no ...medical treatment has been approved for Noonan Syndrome. Meanwhile, several approved agents targeting the same RAS/MAPK signaling pathway are used in cancer treatment. In this case report we describe a child with Noonan Syndrome caused by a pathogenic RIT1 variant, who developed severe early-onset hypertrophic cardiomyopathy and pulmonary valve stenosis. She received off-label treatment with the MEK-inhibitor trametinib which resulted in complete remission of the cardiac hypertrophy and a significant improvement of the pulmonary valve stenosis. Our case emphasizes the potential of existing cancer agents targeting the RAS/MAPK signaling pathway as successful treatment for RASopathy manifestations.
A patent ductus arteriosus (PDA) is frequently found in very preterm neonates and is associated with increased risk of morbidity and mortality. A shunt across a PDA can result in an unfavorable ...distribution of the cardiac output and may in turn result in poor renal perfusion. Urinary Neutrophil Gelatinase-associated Lipocalin (U-NGAL) is a marker of renal ischemia and may add to the evaluation of PDA. Our primary aim was to investigate if U-NGAL is associated with PDA in very preterm neonates. Secondary, to investigate whether U-NGAL and PDA are associated with AKI and renal dysfunction evaluated by fractional excretion of sodium (FENa) and urine albumin in a cohort of very preterm neonates.
A cohort of 146 neonates born at a gestational age less than 32 weeks were consecutively examined with echocardiography for PDA and serum sodium, and urine albumin and sodium were measured on postnatal day 3 and U-NGAL and serum creatinine day 3 and 6. AKI was defined according to modified neonatal Acute Kidney Injury Network (AKIN) criteria. The association between U-NGAL and PDA was investigated. And secondly we investigated if PDA and U-NGAL was associated with AKI and renal dysfunction.
U-NGAL was not associated with a PDA day 3 when adjusted for gestational age and gender. A PDA day 3 was not associated with AKI when adjusted for gestational age and gender; however, it was associated with urine albumin. U-NGAL was not associated with AKI, but was found to be associated with urine albumin and FENa.
Based on our study U-NGAL is not considered useful as a diagnostic marker to identify very preterm neonates with a PDA causing hemodynamic changes resulting in early renal morbidity. The interpretation of NGAL in preterm neonates remains to be fully elucidated.
To describe the impact of CHD surgery in early childhood on quality of life in children aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and ...Tetralogy of Fallot.
A cross-sectional survey study of quality of life survey on 161 children and adolescents aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. The international Paediatric Quality of Life 4.0 quality of life questionnaires were applied and collected for assessment from patients and parents. The endpoints were total, physical, emotional, social, and school quality of life scores.
The quality of life total and school scores was significantly lower in children with CHD than their healthy peers. There was no significant difference in quality of life between the three CHD groups. All three CHD groups had a significantly lower total (7.7-13.2%, p<0.001) and school scores (21.1-31.6%, p<0.001) than the control group. The tetralogy of Fallot group was the only group that had significantly lower scores in the physical subscale (p<0.001) than the controls.
Children and adolescents with surgically corrected CHD show losses in quality of life in total and school scores compared to healthy controls. The tetralogy of Fallot group was the only CHD group that had significantly lower physical score than the controls.
Children with complex CHD are at risk for psychopathology such as severe attention-deficit/hyperactivity disorder symptoms after congenital heart surgery.
The aim of this study was to investigate if ...children with Ventricular Septal Defect, Transposition of Great Arteries, or Tetralogy of Fallot have an increased occurrence of attention-deficit/hyperactivity disorder symptoms compared with the background population and to investigate differences between the three CHDs in terms of occurrence and appearance of attention-deficit/hyperactivity disorder symptoms.
A national register-based survey was conducted, including children aged 10-16 years with surgically corrected CHDs without genetic abnormalities and syndromes. The Attention-Deficit/Hyperactivity Disorder-Rating Scale questionnaires were filled in by parents and school teachers.
In total, 159 out of 283 questionnaires were completed among children with CHDs and compared with age- and sex-matched controls. Children with CHDs had significantly increased inattention scores (p = 0.009) and total attention-deficit/hyperactivity disorder scores (p = 0.008) compared with controls. Post hoc analyses revealed that children with Tetralogy of Fallot had significantly higher inattention scores compared with children both with Ventricular Septal Defect (p = 0.043) and controls (p = 0.004).
Attention-deficit/hyperactivity disorder symptoms and inattention symptoms were significantly more frequent among children aged 10-16 years with CHDs, in particular in children with corrected Tetralogy of Fallot.
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