Wearable cardioverter-defibrillator as bridging to ICD in pediatric hypertrophic cardiomyopathy with myocardial bridging - a case report

Background: There is only limited experience with wearable cardioverter-defibrillators (WCD) in pediatric patients. We report on the successful application of a WCD in an adolescent patient with hypertrophic cardiomyopathy and myocardial bridging. Case presentation: A 15-year-old girl presented with a history of recurrent syncope, dyspnea, and vertigo with exercise. Diagnostic work-up revealed non-obstructive hypertrophic cardiomyopathy and signs of myocardial ischemia with exercise. Given this high-risk constellation, the patient was scheduled for prophylactic implantation of an implantable cardioverter-defibrillator (ICD). One month after initial presentation and days prior to the planned ICD implantation, the patient collapsed during an episode of sustained ventricular tachycardia (VT) while running. VT was terminated by WCD shock delivery. Following this event, computerized tomography scan revealed myocardial bridging of the left anterior descending coronary artery causing a 90% stenosis in systole. After coronary surgery, life threatening arrhythmias have not recurred, but due to progressive heart failure, the patient underwent successful heart transplantation after 2 years. Conclusions: The reported case highlights the importance and applicability of WCDs and the potentially malign nature of myocardial bridging in pediatric high-risk patients

Clinical NEC prevention practices drive different microbiome profiles and functional responses in the preterm intestine

Preterm infants with very low birthweight are at serious risk for necrotizing enterocolitis. To functionally analyse the principles of three successful preventive NEC regimens, we characterize fecal samples of 55 infants (less than 1500 g, n = 383, female = 22) longitudinally (two weeks) with respect to gut microbiome profiles (bacteria, archaea, fungi, viruses; targeted 16S rRNA gene sequencing and shotgun metagenomics), microbial function, virulence factors, antibiotic resistances and metabolic profiles, including human milk oligosaccharides (HMOs) and short-chain fatty acids (German Registry of Clinical Trials, No.: DRKS00009290). Regimens including probiotic Bifidobacterium longum subsp. infantis NCDO 2203 supplementation affect microbiome development globally, pointing toward the genomic potential to convert HMOs. Engraftment of NCDO 2203 is associated with a substantial reduction of microbiome-associated antibiotic resistance as compared to regimens using probiotic Lactobacillus rhamnosus LCR 35 or no supplementation. Crucially, the beneficial effects of Bifidobacterium longum subsp. infantis NCDO 2203 supplementation depends on simultaneous feeding with HMOs. We demonstrate that preventive regimens have the highest impact on development and maturation of the gastrointestinal microbiome, enabling the establishment of a resilient microbial ecosystem that reduces pathogenic threats in at-risk preterm infants

Respiratory syncytial virus hospitalizations in infants of 28 weeks gestational age and less in the palivizumab era

Objective: To obtain data on respiratory syncytial virus (RSV) associated hospitalization rates in preterm infants of 28 weeks gestational age and less in the era of palivizumab prophylaxis. Methods: Retrospective single-center cohort study including all preterm infants up to 28 weeks + 6 days gestational age and born between 2004 and 2012 at a tertiary care university hospital. Data on RSV related hospitalizations over the first two years of life covering at least two RSV seasons (November-April) were analyzed. Results: Ninety-one of 287 (32%) infants were hospitalized due to respiratory illness, and a total of 17 infants (5.9%) tested RSV positive during the first 2 years of life. Fourteen infants (4.9%) were hospitalized during the first RSV season. RSV hospitalization rate in infants with BPD was 4.5% (2/44) compared to 4.9% (12/243) without BPD. Palivizumab prophylaxis was documented in 74.6% of the infants. Infants with RSV compared to other respiratory tract infection were of younger age (6.8 vs. 9.1 months; p = 0.049), had longer hospital stays (median 11 vs. 5 days; p = 0.043) and more severe respiratory illness (median LRI score 3 vs. 2; p = 0.043). Conclusions: Despite palivizumab prophylaxis the burden of RSV disease and all cause respiratory illness was still remarkable in this vulnerable preterm population and mainly limited to the first season

Surfactant Protein B Deficiency Caused by Homozygous C248X Mutation—A Case Report and Review of the Literature

Abstract Objective Surfactant protein B (SP-B) deficiency is a rare autosomal recessive disorder that is usually rapidly fatal. The c.397delCinsGAA mutation (121ins2) in exon 4 is found in more than two-thirds of patients. Design We report on a fatal case of SP-B deficiency caused by a homozygous C248X mutation in exon 7 of the SP-B gene. In addition, we provide an update of the current literature. The EMBASE, MEDLINE, and CINAHL databases were systematically searched to identify all papers published in the English and German literature on SP-B deficiency between 1989 and 2013. Results SP-B deficiency is characterized by progressive hypoxemic respiratory failure generally in full-term infants. They present with symptoms of respiratory distress and hypoxemia; chest X-ray resembles hyaline membrane disease. Prenatal diagnosis is possible from amniotic fluid or chorionic villi sampling. Conclusion Thirty-four mutations have been published in the literature. Treatment options are scarce. Gene therapy is hoped to be an option in the future

First clinical experience with the Kora pacemaker system in congenital complete heart block in newborn infants

Abstract Background To report first clinical experience on three cases of congenital complete heart block and the use of a pacemaker system with a maximum lower rate interval of 95 beats per minute. Methods We retrospectively analyzed three patients treated with a pacemaker system with a maximum lower rate interval of 95 beats per minute suffering from congenital complete heart block. We report a follow up period of 2.9 years, focusing on the patients’ growth, development, and adverse events, as well as pacemaker function. Results In all three patients pacemaker function was impeccable, including minute ventilation sensor rate adaption. All patients showed limited growths as expected, adequate development, good feeding tolerability and circadiane heart rate adaption. One patient experienced skin traction and revision. All patients showed high aortic velocity time integral values after birth. Conclusion The use of a pacemaker system with a maximum lower rate interval of 95 beats per minute in infants suffering from congenital complete heart block and showing high aortic VTI values seems to be feasible and to result in limited growths but adequate development

Editorial: Cardio-circulatory support of neonatal transition

Peer reviewed: Tru

Changes of Locoregional Skin Temperature in Neonates Undergoing Laser Needle Acupuncture at the Acupuncture Point Large Intestine 4

Laser acupuncture bears a potential risk for the skin surface, especially in neonates whose skin has histological and physiological peculiarities. We evaluated thermal changes of skin temperature in neonates during laser acupuncture by using a thermal camera (Flir i5, Flir Systems Inc., Portland, USA). Laserneedles (Laserneedle GmbH, Glienicke/Nordbahn, Germany) were fixed to the skin at Large Intestine 4 (LI 4, Hegu), bilaterally. Before application of laser acupuncture (685 nm, 15 mW, 500 μm), as well as after 1, 5, and 10 min, thermographic pictures of both hands were taken. The measuring was carried out on the 23rd day after birth (20 neonates, mean postmenstrual gestational age 38 + 2, mean weight 2604 g). Compared to the initial temperature of 34.2°C on the right hand, the skin temperature had increased to 35.3°C (P<0.05) after 5 min and up to 36.1°C (P<0.05) after 10 min of stimulation. Equally, on the left hand, an increase of the skin temperature from 34.5°C to 35.9°C (P<0.05) and 35.9°C (P<0.05) was measured. The highest measured skin temperature after 10 min of stimulation amounted to 38.7°C, without any clinically visible changes on the skin surface

Accuracy of Algorithms Predicting Accessory Pathway Localization in Pediatric Patients with Wolff-Parkinson-White Syndrome

We aimed to assess the accuracy of determining accessory pathway (AP) localization from 12 lead ECG tracings by applying 12 different algorithms in pediatric patients diagnosed with Wolff-Parkinson-White syndrome. We compared algorithm accuracy in electrophysiologic study ECG tracings with full preexcitation and resting ECG tracings. The assessing pediatric cardiologists were blinded regarding EP study results on AP localization. For exact AP location, the algorithms published by D&rsquo;Avila et al. and Boersma et al. yielded the highest accuracy (58%). Distinguishing laterality, the median accuracy for predicting left or right-sided APs was 74%, while for septal APs it was 68%. We conclude that algorithms predicting AP location in pediatric patients with Wolff-Parkinson-White syndrome show low accuracy for exact AP localization. For laterality, however, accuracy was significantly higher