A History of Neonatal Uterine Bleeding and Its Significance

Bleeding in newborns and young girls fascinated writers for more than a millennium. Initially, there was confusion between neonatal bleeding, early menstruation due to precocious puberty, and hemorrhage due to disease. During the 19th century descriptions appeared of what is referred to today as ‘neonatal menstruation’ or ‘neonatal uterine bleeding’. By the turn of the century, Halban linked bleeding to active substances present during pregnancy and hypothesized that, while the maternal uterus reacts with decidua formation, the “weaker” fetal uterus reacts only with menstrual-like changes. Despite this clear description, several alternative theories endured for decades. Bleeding was believed to be due to a ‘catarrhal’ or neoplastic state of the genital tract, pulmonary circulatory disorder, congenital heart malformations, closure of the umbilical cord or affections of the intestine. During the 1950s, progesterone response and resistance were proposed to explain the pathogenesis of bleeding and its low incidence. The fetal endometrium is resistant to the high circulating progesterone. A decidual response is infrequent and results in menstrual shedding upon progesterone withdrawal after birth. Further research linked fetal stress consequent to pregnancy complications and post-maturity to increased incidence and preterm birth to reduced incidence of neonatal uterine bleeding.

Ascertaining the Prevalence of Heart Malformations in Neonates: A Novel Clinically Approved Solution

Background: It is vital to screen all critical congenital heart defects as early as possible. Screening for congenital heart diseases in neonates is essential for saving children's lives and reducing undetected adult congenital heart diseases. Unfortunately, over 50% of neonates with heart malformations are unrecognized at birth. Accurate screening for congenital heart malformations can be accomplished using the certified and internationally patented digital intelligent phonocardiography machine. The objective of this study is 2-fold: assess the actual prevalence of newborn heart defects in our well-baby nursery and evaluate the digital intelligent phonocardiogram screening for critical congenital heart diseases. Methods: In this study, we have accomplished "The Neonates Cardiac Monitoring Research Project" (ethics approval IR-IUMS-FMD. REC.1398.098) at Shahid Akbarabadi Maternity Hospital. Eight hundred forty neonates from our well-baby nursery have undergone both the standard clinical examinations at birth and the digital intelligent phonocardiogram examination in a double-blinded format. The median age of the neonates was 30,85 hours, and the median weight was 3.12 Kg. In addition, the pediatric cardiologist has performed echocardiography diagnosis for each neonate classified as abnormal either by the intelligent machine or by the standard medical examinations of a neonate. Detailed dataset of the eight hundred neonates’ examinations and printouts of the intelligent machine are available online.Results: We have found that the prevalence of heart malformations in our well-baby nursery is 5%. 44% of the heart malformations are unrecognized at birth through the standard medical examination of the hospital, including neonates with CCHD, Whereas the intelligent phonocardiogram has recognized 100% of neonates with heart malformations.Conclusions: We have accomplished screening for congenital heart malformations in all neonates accurately and cost-effectively in our hospital using the digital intelligent phonocardiogram. The intelligent machine has detected CCHD, where we have not detected it through the hospital's standard medical examinations, including pulse oximetry and the heart sound auscultation. In addition, the intelligent machine interprets innocent murmurs as healthy heart sounds.

Acyanotic Truncus Arteriosus Type 4 in a Sudden Infant Death: A Case Report

A congenital cyanotic heart defect such as Truncus Arteriosus (TA) is rare and is represented by a single arterial trunk that develops from the heart and gives rise to the pulmonary trunk in several manners. TA comprises 0.7% of all complex congenital heart malformations caused by numerous aetiologies, with the majority being genetic in origin. This malformation can be recognised in the prenatal period so that further medical intervention can be advocated. There are four types of the defect based on the Collett-Edwards classification. Type 4 is a rare form in which the pulmonary arteries are non-existent. Therefore, the lungs are supplied by major aortopulmonary collateral arteries. It may also describe this anatomical picture as pulmonary atresia with VSD. We report here a unique case of truncus arteriosus type 4 in an apparently well-thriving and acyanotic 6-month-old male infant. The deceased was presented with a brief history of inconsolable cry shortly before his demise and the underlying cardiac abnormalities were discovered at autopsy. A meticulous post-mortem examination elucidates a better diagnosis approach. Genetic counselling can be offered to parents in recurrent cases, such as cases of consanguinity.

Surgery versus intervention in congenital heart disease as a risk factor for executive dysfunction: a case control study

Background Improving surgical and interventional modalities for treating congenital heart disease increased these children's living expectancy, a leading cause of cognitive and behavioral problems. This study compared executive functions among surgically, interventionally treated congenital heart disease, and aged match control. Methods This was a retrospective cross-sectional study. We enrolled thirty children treated with surgery, 30 interventionally treated patients and 30 healthy controls. We compared these participants in the executive functions domains by Stroop test, trail making test, cancellation test, visual and auditory memory span test, and behavioral problems using the parent form strength and difficulty questionnaire. Executive performance in memory span and sustained attention was lower in the surgical group than in the intervention group (P˂0.05). Results Both groups of patients had lower flexibility, memory recall, simple and complex working memory, visual attention, and simple and complex selective attention performance (P < 0.05). The effect of behavioral problems on daily life was higher in surgical patients than in the intervention group (P˂0.0001). Both groups of patients had higher emotional problems, conduct problems, and communication problems than the normal group (P˂0.0001). Conclusion Interventionaly treated patients had better performance in comparison to surgically-treated patients. Regardless of the severity of the disease or treatment, patients with congenital heart malformations had poor performance compared to normal subjects and suffered from behavioral disorders affecting their daily lives. It is essential to include diagnostic and therapeutic interventions in treating these patients' executive function and behavioral problems.

Glypican-6 deficiency causes dose-dependent conotruncal congenital heart malformations through abnormal remodelling of the endocardial cushions

Tetralogy of Fallot (TOF) is considered to be the commonest type of cyanotic congenital heart disease (CHD). A previous GWAS showed significant association between TOF and single nucleotide polymorphisms in chromosome 13q31. Here through integration of population genomic and chromosomal interaction data we identify the heparan sulfate proteoglycan glypican-6 (GPC6) as the potentially responsible gene at the associated locus. We showed that GPC6 is expressed in the endocardial cushions at the appropriate time in development to contribute to TOF risk. We generated mice homozygous for a Gpc6 KO allele, which exhibit 100% neonatal lethality with severe cardiac malformations, namely TOF-type double outlet right ventricle (DORV) with rightward mal-positioned aorta and perimembranous ventricular septal defect (VSD), together with right ventricular (RV) hypertrophy and narrowing of the pulmonary artery. We established a dose-response relationship between Gpc6 expression and the anatomical severity of cardiac malformations. We showed the mouse knockout phenotype arises from abnormal morphology of the endocardial cushions, and tissue-specific knockout of Gpc6 in endothelial and neural crest cell lineages produces a phenotype featuring VSD and aortic malposition analogous to human TOF. This successful identification of a CHD gene from GWAS data suggests that larger GWA studies may find additional causative genes.

Ascertaining The Prevalence of Heart Malformations in Neonates: A Novel Clinically Approved Solution

Background: Screening of all congenital heart malformations (CHD & CCHD) , as early as possible, is essentials to save children’s lives and, vanishing undetected CHD before becoming adult congenital heart disease (ACHD). As it is known, over 50% of neonates with heart malformations are not recognized at birth. Accurate screening of congenital heart malformations can be accomplished by a certified and patented passive intelligent machine known as Pouya Heart. The objective of this study is 2-fold: to evaluate true prevalence of neonates heart malformations and to evaluate screening of critical congenital heart diseases (CCHD) by the intelligent machine.Methods: 840 neonates with a median age of 31.16 hours and a median weight of 3.11 Kg from the well-baby nursery have been screened by the passive intelligent machine. 20 seconds of heart sounds are recorded into the intelligent machine from two thoracic sites. Each neonate is then classified as normal or abnormal. Echocardiography diagnosis is carried out for verification of each abnormal case.Results: The prevalence of heart malformations (CHD & CCHD), in our well-baby nursery, is 5%. Where, 44% of the heart malformations have not been recognized at birth through the standard medical examination routine.Conclusions: Screening of congenital heart malformations, in all neonates, can be accomplished accurately and cost-effectively by using the Pouya Heart. It detects even CCHD in which it has not been detected through standard medical routine. The innocent murmurs are interpreted as healthy heart sounds.

Clinical scope of early fetal echocardiography

The chances of detecting congenital heart disease are improved following structured protocols. Fetal heart disease is one of the main serious congenital malformations. The objective of this review is to present the benefits of sequential and routine cardiac evaluation in the first and early second trimesters and to identify the structures and pathologies that can be detected at this gestational age. The databases of PubMed, Medline, MD consult, Embase, Clinical Key, Scielo, and ScienceDirect, as well as specialized texts in Spanish and English were searched for diagnostic studies, systematic reviews, and meta-analysis related to the terms “early fetal echocardiography” and “congenital heart malformations” published between 2000 and 2019. Technological advances have revolutionized the fetal echocardiographic examination making possible the diagnosis of congenital heart disease from the first and early second trimester of pregnancy. However, it should be recognized that early fetal echocardiography has limitations such as the evaluation of pulmonary veins and cardiac lesions that are progressive. The benefit found was earlier detection of pathologies with high sensitivity and specificity. A fetal cardiac evaluation sequence was included in these early fetal echocardiographic examinations due to the awareness of fetal anatomical and hemodynamic differences in the first trimester and the beginning of the second trimester. Early fetal echocardiography is a very useful tool for the detection of congenital heart disease; it allows parents to be informed about the prognosis and possible treatments early. Fetal cardiac examination in the first trimester is safe and can detect the most of heart congenital malformation.