Myocardial Expression of Estrogen Receptor-mRNA Is Associated With Lower Markers of Post-operative Organ Damage in Young Patients With Congenital Cardiac Defect

Background: Estrogen receptors (ERs) relate to cardio-protection in adults, but their role in younger patients is not known. We aimed to assess the myocardial expression of ERα- and ERβ- mRNA in young patients with congenital cardiac disease and to analyze their putative protective role.Patients and Methods: Twenty children and young adults (seven females and 13 males) with a median age of 13.8 years (interquartile range: 12.3 years) were enrolled in this prospective study. The myocardial expression of ER-mRNA and genes involved in inflammation, growth, and stress response was assessed by real-time PCR and was correlated to post-operative (po) outcome.Results: ER-mRNA was detected in the myocardium of all patients, independently of gender and age. The expression of ER-mRNA correlated with that of mRNA coding for brain natriuretic peptide and for all cytokines tested. A higher ERα-mRNA expression correlated with lower troponin T concentrations at 24 h po (p = 0.032), higher PaO2/FiO2 ratio at 4 h po (p = 0.059), lower fluid retention at 4 h po (p = 0.048), and lower aspartate aminotransferase (AST) levels at 24 h po (p = 0.047). A higher ERβ-mRNA expression was also correlated with lower fluid retention at 24 h po (p = 0.048).Patients in whom the levels of ERα- and ERβ-mRNA were >P50 had lower troponin T (p = 0.003, respectively) and lower AST concentrations at 24 h po (p = 0.043, respectively) than the others.Conclusions: The expression of ERα- and ERβ-mRNA is present in the myocardium of children and young adults with congenital cardiac defect and is associated with lower markers of po organ damage. This suggests that ERs may provide perioperative organ protection in this population.

The Vein Of Galen Aneurysmal Malformation Associated With Sinus Venosus Defect And Anomalous Pulmonary Veins

he Vein of Galen Aneurysmal Malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM is safer in infancy, even it may be undertaken in the neonatal period if necessary.

Isolated Right Superior Cava Drainage to the Left Atrium: An Uncommon Etiology of Cyanosis

Anomalous superior vena cava drainage is a rare congenital cardiac defect where the right superior vena cava anomalously drains into the left atrium causing a right to left shunt. We present a case of a 17-day-old male who presented with cyanosis without respiratory distress and was found to have a right superior vena cava draining anomalously into the left atrium.

Incidence and Clinical Significance of Cardiac Murmur in Neonates in a Tertiary Hospital

Background: Congenital cardiac defect is the major cause of mortality and morbidity in our country but maximum cases are undetected due to lack of skill, shortage of man power, modern equipment in peripheral hospitals. But simply observing murmur can give us the clue for underlying congenital heart diseases.Objectives: The purpose of this study is to determine the incidence and clinical significance of cardiac murmur in neonates in a tertiary level hospital in Rajshahi.Methods: This cross-sectional descriptive study was done in neonates (age ≤ 28 days) in Rajshahi Medical College Hospital from January 2014 to December 2015.Results: during this study period total 4120 neonates were admitted in neonatal ward and only 52 cases were presented with cardiac murmur clinically. Incidence rate of murmur was 1.26%. Among 52 cases 40 cases were having pathological murmur and 12 cases were having innocent murmur. After doing echocardiogram 34 cases were showing structural cardiac defect out of 40 pathological murmur and 4 cases were showing structural cardiac defect out of 12 innocent murmur. Total cases of structural cardiac defect (congenital heart disease) were 38(34+4). So, incidence of congenital heart disease was 9.22/ 1000 live births.Conclusion: Congenital cardiac defect is the major cause of mortality and morbidity in our country but maximum cases are undetected during neonatal period but simply observing murmur can give us the clue for underlying congenital heart diseases in 73.08% cases (38 were congenital heart disease out of 52 cases). If congenital heart disease is early detected and properly managed many valuable lives of neonates can be saved and their growth and development will be smooth.TAJ 2017; 30(2): 54-59

Can device closure of AP window be made easy?

Aortopulmonary window is an uncommon congenital cardiac defect. Most infants presenting with aortopulmonary window will undergo conventional surgical repair. Experience with transcatheter closure of aortopulmonary window is limited. We report the case of a 5-year-old boy with aortopulmonary window, in whom transcatheter closure was performed successfully using Amplatzer ductal occluder device.