Atrial Septum Perforation Via Levoatriocardinal Vein in a Newborn With Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome with intact atrial septum has high perioperative mortality. An interventional septum perforation from the right atrium may be technically challenging in newborns with high risk of tamponade. However, neonates with a levoatriocardinal vein provide an alternative approach for septostomy from the left atrium side. (Level of Difficulty: Advanced.).

Heart Rate Variability in Individuals with Down Syndrome: A Scoping Review with Methodological Considerations.

Individuals with Down syndrome (DS) present similar heart rate variability (HRV) parameters at rest but different responses to selected movement maneuvers in comparison to individuals without DS, which indicates reduced vagal regulation. The present study undertakes a scoping review of research on HRV in individuals with DS, with special attention paid to the compliance of the studies with standards and methodological paper guidelines for HRV assessment and interpretation. A review was performed using PubMed, Web of Science and CINAHL databases to search for English language publications from 1996 to 2020 with the MESH terms "heart rate variability" and "down syndrome", with the additional inclusion criteria of including only human participants and empirical investigations. From 74 studies, 15 were included in the review. None of the reviewed studies met the recommendations laid out by the standards and guidelines for providing the acquisition of RR intervals and necessary details on HRV analysis. Since authors publishing papers on this research topic do not adhere to the prescribed standards and guidelines when constructing the methodology, results of the research papers on the topic are not directly comparable. Authors need to design the study methodology more robustly by following the aforementioned standards, guidelines and recommendations.

Electrocardiographic Parameters Associated with Adverse Outcomes in Children with Cardiomyopathies.

Cardiomyopathies have a low prevalence in children and thus may lead to malignant ventricular arrhythmias or the progression of heart failure, resulting in death. In adults, the QRS-T angle derived from ECG has been associated with adverse outcomes in patients with hypertrophic and dilated cardiomyopathies. We aimed to assess the electrocardiographic parameters, including QRS-T angle, associated with adverse cardiac events in children with cardiomyopathies. Forty-two children with cardiomyopathies were included in this study: 19 with dilated cardiomyopathy, 17 with hypertrophic cardiomyopathy, and 6 with left ventricular non-compaction. Additionally, 19 control subjects were recruited. In terms of ECG parameters, the QRS-T angle was significantly greater among patients with adverse outcomes compared to patients without the end points of the study (133° vs. 65°, p < 0.001). On Kaplan−Meier survival curves, QRS-T angle > 120°, increased serum concentrations of NT-proBNP and troponin I levels as well as greater NYHA or Ross scale were associated with the greatest risk of unfavorable outcome. The QRS-T angle appears to be a valuable component of 12-lead ECG interpretation, and might be helpful in outlining patients with the greatest cardiovascular risk. Additionally, serum biomarkers such as NT-proBNP (p = 0.003) and troponin (p < 0.001) are useful in outlining patients with the worst survival.

Pediatric Inflammatory Multisystem Syndrome (PIMS) Did Occur in Poland during Months with Low COVID-19 Prevalence, Preliminary Results of a Nationwide Register.

Pediatric inflammatory multisystem syndrome (PIMS) is a new entity in children, likely associated with previous coronavirus disease 19 (COVID-19) infection. Most of the reports about PIMS come from countries particularly hit by the COVID-19 pandemic. Our aim was to investigate the nature of inflammatory syndromes in Poland (country with low COVID-19 prevalence) and to perceive the emergence of PIMS in our country. On 25 May 2020, we launched a nationwide survey of inflammatory syndromes in children for retrospective (since 4 March 2020) and prospective data collection. Up to 28 July, 39 reported children met the inclusion criteria. We stratified them according to age (<5 and ≥ 5 years old) and COVID-19 status. The majority of children had clinical and laboratory features of Kawasaki disease, probably non-associated with COVID-19. However, children ≥5 years of age had PIMS characteristics, and nine children had COVID-19 confirmation. This is, to our knowledge, the first report of the PIMS register from a country with a low COVID-19 prevalence, and it proves that PIMS may emerge in any area involved in the COVID-19 pandemic. In a context of limited COVID-19 testing availability, other risk factors of PIMS, e.g., older age, should be considered in the differential diagnosis of inflammatory syndromes in children.

How Normal is a 'Normal' Heart in Fetuses and Infants with Down Syndrome?

BACKGROUND: Congenital heart disease is present in 44-56% of fetuses with Down syndrome (DS). There are, however, signs that hearts in DS without apparent structural heart defects also differ from those in the normal population. We aimed to compare the atrioventricular (AV) septum and valves in 3 groups: DS without AV septal defect (DS no-AVSD), DS with AVSD (DS AVSD) and control hearts. METHODS: The ventricular septum, membranous septum and AV valves were examined and measured in histological sections of 15 DS no-AVSD, 8 DS AVSD and 34 control hearts. In addition, the ventricular septum length was measured on ultrasound images of fetal (6 DS AVSD, 9 controls) and infant (10 DS no-AVSD, 10 DS AVSD, 10 controls) hearts. RESULTS: The membranous septum was 3 times larger in DS no-AVSD fetuses compared to control fetuses, and valve dysplasia was frequently (64%) observed. The ventricular septum was shorter in patients with DS both with and without AVSD, as compared to the control group. CONCLUSION: DS no-AVSD hearts are not normal as they have a larger membranous septum, shorter ventricular septum and dysplasia of the AV valves as compared to control hearts.

[Post-mortem stereomicroscopic examination of embryonic and fetal hearts in the 1st trimester of pregnancy]. / Badanie autopsyjne serc zarodków i plodów w I trymestrze ciazy metoda stereomikroskopowa.

UNLABELLED: Post-mortem examination of embryos and fetuses becomes increasingly important due to the development of prenatal diagnostic methods. Possibilities of visualization of embryonic and fetal heart in echocardiography and autopsy develops rapidly and due to learning curve there is a need for confirmation of results. There are different methods for visualization of cardiac anatomy. Some morphologists prefer classical histology while others use stereomicroscopy OBJECTIVES: To develop our own autopsy routine for embryonic and small fetal hearts. MATERIAL AND METHODS: Twenty five normal and malformed fetal hearts aged from 7 to 12 weeks, fixed and unfixed, were submerged in saline solution and dissected using microsurgical instruments. nikon SMZ 1500 microscope, Nikon DS-Fi1 camera and NIS-Elements 3.00 software were used for observation and documentation. The chest was opened by median sternotomy the heart was dissected in situ and sequential segmental analysis was applied. RESULTS: Detailed anatomy of atriums, interatrial septum, pulmonary and systemic veins drainage were examined. In all cases it was possible to assess morphology of chambers, atrioventricular and ventriculoarterial connections. Congenital heart defect was diagnosed in only 3 out of 25 cases: one in the 10th week of gestation (complete atrioventricular septal defect--CAVSD) and two in the 12th week of gestation (1 case of CAVSD with trisomy 21 confirmed cytogenetically and one of tetralogy of Fallot). CONCLUSION: Microautopsy of fetal heart in the 1st trimester of pregnancy is possible using stereomicroscopy and allows us to diagnose congenital heart diseases. Detailed structures are best visible when specimen is unfixed and submerged in saline solution.