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Background: Abnormal diameters of foetal vessels are common findings in congenital heart defects. However, it is difficult for pathologist to assess whether the diameters of the vessels are normal or not and to compare with echocardiographic data. The Z-score is a dimensionless quantity representing the distance between the raw score and population mean in standard deviation units. Using Z-score, we can compare single cases to average standards in the population. Aim: To compare diameters of great arteries measured in specimens fixed in 10% formalin solution to normal values obtained on echocardiography using Z-scores. Material and Methods: The study was performed on 43 formalin-fixed normal foetal hearts aged from 19 to 40 weeks of gestation. Each specimen was dissected and the following diameters were measured: aortic valve (AoV), ascending aorta (AAo), descending aorta (DAo), aortic isthmus (AoI), ductus arteriosus (DA), pulmonary trunk valve (PV), and pulmonary arteries (RPA and LPA). Z-score parameters were calculated for all structures using the Foetal Echo Z-score Calculator. Results: Z-score values of all measured foetuses were mostly found to fit within the echocardiographic norms, although the most reliable results were obtained for foetuses aged 23 weeks. This group was represented by the highest number of specimens. The minimal and maximal Z-score values were, respectively: AoV -3.45, 1.4; AAo -4.24, -0.11; DAo -2.12, 1.29; PV -3.21, 0.69; RPA -3.4, 0.84; LPA -2.25, 0.31; AoI -1.97, 0.96; DA -3.56, -1.52. Conclusions: The diameters of great arteries measured on autopsy are comparable to the results obtained from echocardiographic measurement. Z-score values for foetal echocardiography can be applied to post-mortem examination. [ABSTRACT FROM AUTHOR]

Background:In some cases of epistaxis, anterior packing and/or electrocoagulation are not sufficient and suturing is needed. However, standard suturing techniques are sometimes ineffective. This paper reports the use of a new kind of trans-septal mattress suture, based on regional vascular anatomy, in this clinical context.Methods:Four adult patients with haemorrhage in Little's area underwent standard suturing, but bleeding persisted. These patients were successfully treated with trans-septal mattress suturing, using four punctures around the haemorrhage site.Results:No relapses or complications were observed over a six-month follow-up period.Conclusion:In cases of persistent bleeding from Little's area, suturing should surround the bleeding area. The described suturing method is simple, efficacious and cheap. [ABSTRACT FROM PUBLISHER]

Conventional diastolic dysfunction parameters seem to be imperfect when applied to the pediatric cardiomyopathy population. The aim of this pilot study was to search for novel echocardiographic parameters associated with adverse outcomes in children with the most common cardiomyopathies. Fifty-six patients with pediatric cardiomyopathies (28 with dilated, 21 with hypertrophic, 7 with left ventricular non-compaction cardiomyopathy) and 28 healthy subjects were included in the study. Left atrial reservoir (LASr), conduit (LAScd) and contraction (LASct) strain, left atrial stiffness index (LASI), as well as conventional diastolic dysfunction parameters were measured using echocardiography. Adverse outcomes were defined as heart failure (including heart transplant) and arrhythmic endpoints. Patients with adverse outcomes presented with significantly lower LASr (16.68% ± 8.64% vs. 33.97% ± 9.99%, p-value < 0.001), lower LAScd (- 10.37% ± 5.83% vs. - 25.50% ± 9.24%, p-value < 0.001) and higher values of LASI (0.69 [IQR 0.34; 1.11] vs. 0.21 [IQR 0.16; 0.31], p-value < 0.001). LASr < 20%, LAScd ≥ - 12%, and LASI ≥ 0.26 were all associated with reduced survival. LASr, LAScd and LASI seem to be promising parameters in predicting adverse outcomes in the most common pediatric cardiomyopathies. Left atrial strain parameters and LASI are helpful in differentiating healthy control subjects from children with hypertrophic and dilated cardiomyopathies., (© 2024. The Author(s).)

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. )., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

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.

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) 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.

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., (© 2015 S. Karger AG, Basel.)

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.