Regression of Cardiac Rhabdomyomas Producing a Severe Aortic Stenosis: Case Report and Discussion of the Literature.

We describe a case of a voluminous rhabdomyoma (R) detected by fetal echocardiography at 32 weeks' gestation (w.g.) obstructing the left ventricular inflow and aortic outflow tract, with a moderate aortic gradient at birth, not needing immediate surgery. At follow-up, the mass progressively regressed, leaving the aortic valve partly damaged, with a gradient that increased to a maximum of 100 mmHg at 9 years. The girl was then operated on successfully by a plasty of the aortic valve. The literature regarding R is discussed.

Efficacy of Polyhexamethylene Biguanide in Reducing Post-Operative Infections: A Systematic Review and Meta-Analysis.

Background: Post-operative infections are a substantial cause of morbidity and mortality worldwide. Polyhexamethylene biguanide (PHMB) is an antimicrobial agent that has been used in various surgical settings to prevent infections. However, the literature on its efficacy in reducing post-operative infections remains unclear. Materials and Methods: We conducted a systematic review and meta-analysis of randomized controlled trials (RCTs) to evaluate the efficacy of PHMB in reducing post-operative infections. The risk of bias and methodologic quality of the included studies were also assessed. Results: The systematic review included nine RCTs, and eight were included in the meta-analysis that showed that the use of PHMB was associated with a reduction in the rate of post-operative infections. The overall effect size was statistically significant, with moderate heterogeneity across the included studies (log Peto's odds ratio [OR], -0.890; 95% confidence interval [CI], -1.411 to -0.369; I2 = 41.89%). However, the diversity in the application of PHMB and the potential influence of other factors, such as adherence to infection prevention protocols and organizational-level variables, underscore the need for further primary studies. Conclusions: Polyhexamethylene biguanide appears to be a promising intervention for reducing post-operative infections. However, more high-quality, well-designed RCTs are needed to confirm these findings and to explore the most effective ways to use PHMB within specific infection prevention bundles. Future research should also aim to control for potential confounding factors to provide a more comprehensive understanding of the efficacy of PHMB in reducing post-operative infections.

Lombardy regional urgent reorganization for congenital cardiac patients following the Covid-19 pandemic.

OBJECTIVE: By the end of February 2020, the COVID-19 pandemic infection had spread in Northern Italy, with thousands of patients infected. In Lombardy, the most affected area, the majority of public and private hospitals were dedicated to caring for COVID-19 patients and were organized following the 'Hub-and-Spoke' model for other medical specialties, like cardiac surgery and interventional procedures for congenital cardiac disease (CHD). Here, we report how the congenital cardiac care system was modified in Lombardy and the first results of this organization. METHODS: We describe a modified 'Hub-and-Spoke' model - that involves 59 birthplaces and three specialized Congenital Cardiac Centers -- and how the hub center organized his activity. We also reported the data of the consecutive cases hospitalized during this period. RESULTS: From 9 March to 15 April, we performed: a total of 21 cardiac surgeries, 4 diagnostic catheterizations, 3 CT scans, and 2 CMR. In three cases with prenatal diagnosis, the birth was scheduled. The spoke centers referred to our center six congenital cardiac cases. The postop ExtraCorporeal Membrane Oxygenation support was required in two cases; one case died. None of these patients nor their parents or accompanying person was found to be COVID-19-positive; 2 pediatric intensivists were found to be COVID-19-positive, and needed hospitalization without mechanical ventilation; 13 nurses had positive COVID swabs (4 with symptoms), and were managed and isolated at home. CONCLUSION: Our preliminary data suggest that the model adopted met the immediate needs with a good outcome without increased mortality, nor COVID-19 exposure for the patients who underwent procedures.

Novel JAG1 Deletion Variant in Patient with Atypical Alagille Syndrome.

Alagille syndrome (AGS) is an autosomal-dominant disorder characterized by various degrees of abnormalities in the liver, heart, eyes, vertebrae, kidneys, face, vasculature, skeleton, and pancreas. This case report describes a newborn child exhibiting a congenital neural tube defect and peculiar craniofacial appearance characterized by a prominent forehead, deep-set eyes, bulbous nasal tip, and subtle upper lip. Just a few hours after birth, congenital heart disease was suspected for cyanosis and confirmed by heart evaluation. In particular, echocardiography indicated pulmonary atresia with ventricular septal defect with severe hypoplasia of the pulmonary branches (1.5 mm), large patent ductus arteriosus and several major aortopulmonary collateral arteries. Due to the association of peculiar craniofacial appearance and congenital heart disease, a form of Alagille syndrome was suspected. In addition, on the fifth day after birth, the patient developed jaundice, had acholic stools, and high levels of conjugated bilirubin and gamma-glutamyltransferase (GGT) were detected in the blood. Genetic testing revealed the novel variant c.802del in a single copy of the JAG1 gene. No variants in the NOTCH2 gene were detected. To the best of our knowledge, this is the first clinical description of a congenital neural tube defect in a molecularly confirmed Alagille patient. This work demonstrates a novel pathogenic heterozygous JAG1 mutation is associated with an atypical form of Alagille syndrome, suggesting an increased risk for neural tube defects compared to other Alagille patients.

Giant Cardiac Mass Detected to an Infant with Normal Fetal Echography and No Systolic Murmur in Early Postnatal Evolution.

Infective endocarditis is rare in children and is rarer on a normal structural heart in an infant without any surgical intervention. Most cases are related to a pre-existing congenital lesion, the most frequent etiology are Gram-positive cocci and the most feared are fungal agents. This report presents a 7-monthold infant with fungal endocarditis on a normal structural heart. The diagnosis was suspected on clinical examination and was confirmed by echocardiography and positive blood cultures. His particular clinical evolution after medical and surgical treatment illustrates a severe disease with poor prognosis which may be a complication of neonatal intensive care procedures. Unusual cause of fungal endocarditis in a previously healthy infant: neonatal hospitalization in intensive care unit.

Comparative Study between the Quality Management Indicators, Marker of Major Psychiatric Disorders in Evolution.

UNLABELLED: Indicators of quality management are represented by: accounting hospitalization days (duration of stay); mortality rate; the rate of nosocomial infections; patients readmitted in 30 days; the percentage patients transferred; inconsistent diagnoses. The hospitalization period is a marker of evolution. The present study reflects comparative data between duration and the number of hospitalisations in patients with major psychiatric disorders. INTRODUCTION: Medical Psychiatry as a discipline has developed a specialized approach in the treatment of mental diseases, a phenomenon linked to the progress of knowledge ofpsychopathology and the increasing complexity of methods of diagnosis and treatment. The history of psychiatric patients reflects not only changes in scientific understanding of mental disorder, but also the beliefs of political, social and economic interests of that period [1,2]. OBJECTIVES: We analised the duration and number of hospitalisation in patients with schizophrenia, psychotic disorders compared to those with bipolar disorder and major depressive disorder in Psychiatric clinic from Craiova between 2006-2010. METHOD: Retrospective study during 2006-2010 took into account 8234 patients aged between 20-65 years from the Psychiatric Clinic Craiova constituting four groups: group N1=989 patients belonging to the schizophrenia group; group N2=1222 patients with psychotic disorders, group N3=918 patients with bipolar disorder and group N4=5101 with major depressive disorder.For data processing we used Microsoft Excel (Microsoft Corp., Redmond, WA, USA), together with the suite for MS Excel XLSTAT (Addinsoft SARL, Paris, France) and IBM SPSS Statistics programme 20.0 (IBM Corporation, Armonk, NY, USA). RESULTS: Schizophrenia being defined as a chronic disease was noted that 85.4 % of the patients from the first group received at least two admissions during the trial. Females have better prognosis, having a lower share in the group with multiple hospital admissions. The duration of the stay was longer than two weeks for 61,7% in the N1 group. Group N2 patients, bipolar disorder, have received at least two admissions, the share between the sexes is similar, the duration of hospitalisation not exceeding 7 days for 54,2% of patients with bipolar disorder. Women with more than 14 hospitalisation days had a share of 21.6%. Duration of hospitalization with the N3 group was of maximum two weeks at 71,4%, the group of major depressive disorders N4 had a number of short admissions of 44,4%. CONCLUSION: Statistically significantly high differences (p =0,000233<0.001) in favor of the schizophrenia and psychotic disorders group compared with the group of disorders of affectivity, in terms of the number and the duration of hospitalizations, marker of the evolution on the long term.