Color tissue doppler imaging of tricuspid annular plane systolic and diastolic excursion in children: A comparison of normal, volume-overloaded and pressure overloaded right ventricles.

BACKGROUND: Tricuspid annular plane (TAP) systolic excursion (TAPSE) is a reproducible M-mode parameter for the measurement of longitudinal shortening of the right ventricle (RV). To date, all attention has been focused on the systolic excursion of TAP and the diastolic excursion of the annular plane back to the base has been ignored. This study aims to compare the quantitative (excursion, slope, and duration) and qualitative (velocity, acceleration, and indentation) characteristics of TAP systolic and diastolic excursion, using color tissue Doppler imaging, in three groups of children with normal RV (NORV), volume overloaded RV (VORV), and pressure overloaded RV (PORV) and normal pulmonary arterial pressure. SUBJECTS AND METHODS: A prospective case-control study was performed in three groups of children with normal heart, VORV and PORV. TAPSE and tricuspid annular plane diastolic excursion (TAPDE) were quantitatively and qualitatively analyzed and compared between the three groups. Statistical analysis was performed using IBM SPSS Statistics for Windows. RESULTS: TAPSE, TAPDE, TAPSE slope, TAPSE slope/TAPDE slope, TAPDE duration and TAPDE duration/RR interval were lower in PORV (TAPSE: PORV: 14.45 ± 4.30, NORV: 20.45 ± 5.46, P = .003, TAPDE:PORV: 14.39 ± 4.61, NORV: 20.28 ± 5.65, P = .004, TAPSE slope:PORV: 4.79 ± 1.40, NORV: 7.15 ± 1.98, P = .001, .001, TAPDE duration:PORV: 201.1 ± 87.9 ms, NORV: 292.1 ± 97.9, P = .006, TAPDE duration/RR interval: PORV: .37 ± .09, NORV: .48 ± .08, P = .0002). CONCLUSION: Pressure-overload on RV produced more impairment of TAPSE and TAPDE patterns than volume overload. Values of TAPSE and TAPDE in patients with VORV and PORV stay in two ends of the normal spectrum. The harmful impact of pre-tricuspid volume overload seems to be less than the post-tricuspid volume overload.

Postoperative normalization of left ventricular noncompaction and new echocardiographic signs in aorta to left ventricular tunnel.

We report postoperative normalization of left ventricular noncompaction in a neonate undergoing successful neonatal surgery for type II aorta to left ventricular tunnel (ALVT) associated with a large patent ductus arteriosus, floppy and extremely redundant anterior mitral leaflet, right coronary artery arising directly from the tunnel, and severe left ventricular noncompaction. We also described 2 novel echocardiographic findings in ALVT including "triple wavy line sign" on M-mode echocardiography which disappeared 1 month after operation and "abnormally increased left ventricular posterior wall motion" on M-mode of standard parasternal long-axis view on color tissue Doppler imaging (TDI) that also normalized postoperatively. We showed that proper definition of endocardial border is extremely important in strain and strain rate imaging in the context of left ventricular noncompaction. Preoperative longitudinal strain and strain rate were significantly decreased in comparison to radial strain and strain rate. Circumferential strain and strain rate were normal.

Whole Exome Sequencing Identified the Causative Mutation in a 4-Year-Old Female with Mulibrey Nanism: A Case Report.

Mulibrey Nanism is a rare multisystem disorder inherited in an autosomal recessive manner caused by mutations in the TRIM37 gene. Most of the reported cases are from Finland, but this condition has rarely occurred in other countries. Although the clinical diagnosis of Mulibrey nanism is a challenge during the first months of life, the disease can be suspected clinically due to the distinctive features of the patients. A 4-year-old female with pneumonia, cardiomyopathy, growth retardation, peripheral edema, and characteristic craniofacial features was referred to Tehran Hope Generation Foundation Genetic diagnosis Center, in October 2021. Genomic DNA was isolated from peripheral blood samples of the patient and her parents and Whole exome sequencing was performed for the patient. Whole exome sequencing revealed a homozygous G>A splice site variant (TRIM37; c.370-1G>A). Sanger sequencing confirmed the segregation of the variant with phenotype in this family. Whole exome sequencing can be helpful in the diagnosis of the patients suspecting to Mulibrey nanism and lacking sufficient clinical presentation according to the diagnostic algorithm.

Ross operation in a neuro-cardio-facial-cutaneous syndrome patient.

Congenital heart diseases are a major part of Costello and cardio-facio-cutaneous syndromes. Subaortic stenosis was reported rarely and Ross operation never in these syndromes.We reported a girl patient whose manifestations were consistent with these syndromes. Distinction between these syndromes was not possible as genetic testing was not carried out. She developed severe neoaortic regurgitation 2.5 years after the Ross operation and died due to the complications of aortic valve replacement.Ross operation may be an unsuitable option in these syndromes due to the possibility of subtle pulmonic valve pathology.

Exercise-induced electrocardiographic changes after treadmill exercise testing in healthy children: A comprehensive study.

Background: Treadmill exercise testing is a crucial diagnostic tool for evaluating congenital and acquired heart disease in the pediatric population. This study aimed to perform a comprehensive evaluation of exercise-induced electrocardiographic (ECG) changes in children. Although there are numerous studies on exercise testing in various cardiac pathologies, studies on exercise-induced ECG changes in normal children with coverage of all ECG parameters of atrial and ventricular depolarization and repolarization are very scant, if any. Aims and Objectives: This study aimed to investigate the exercise-induced ECG changes in healthy children and evaluate the effects of gender and four different formulas of heart rate correction of Bazett, Fridericia, Framingham and Hodges on ventricular repolarization parameters pre-and post-exercise. Materials and Methods: Between April 2019 and April 2020, all children with normal electrocardiogram, echocardiogram and exercise test, high-quality ECG tracings and consent for participation were enrolled in this prospective study. Twenty electrocardiographic parameters were measured and 25 indices were calculated. P-value < 0.05 was considered significant. Results: Seventy-four healthy children were studied. Amplitudes of P, S, and T waves increased significantly after the exercise. All durations, except P wave time to peak and T peak -T end /QT (Tp-e/QT) interval decreased significantly with exercise. Generally, the parameters of ventricular repolarization were not statistically significant between males and females. There were significant differences among the heart-rate corrected values of intervals of QTc, QoTc, JTc, J point to peak T and Tp-e/QTc by various formulas. There was no U wave either at pre-exercise or post-exercise. QT interval was shortened by 24.6 % ± 12.1 % with exercise. The ECG-derived estimated duration of mechanical systole and diastole decreased with exercise. The percentage of decrease in diastole was more than systole (43.79 %± 13.31% versus 33.74% ±15.79 %, respectively, P-value < 0.001). Conclusion: Diastolic time decreased more than systolic time with exercise and systolic time to diastolic time increased with exercise. Hodges' and Fridericia's formulas resulted in the longest and shortest QT and QoT, JT, and JTP, respectively. Thus, using a single value as the cut-off for long QT syndrome can lead to under or over-diagnosis. Nomograms incorporating data on age, heart rate, and heart rate correction formula are indispensable for accurate long QT diagnosis. Furthermore, gender differences in ventricular repolarization parameters are not generally present in 5 to 14-year-old healthy children. The lack of U wave in this study may implicate the need for more careful investigation in the presence of U wave in the treadmill exercise testing of healthy children.

Effects on knowledge and attitudes of using stages of change to train general practitioners on management of depression: a randomized controlled study.

OBJECTIVE: To assess the impact on knowledge and attitudes of a tailored educational intervention on depression using a modified version of the Prochaska stages of change model, compared with standard continuing medical education, for general practitioners (GPs) in primary care in Iran. METHOD: Using a randomized controlled trial, a total of 192 GPs were evenly randomized to intervention or control arm. The topic for the educational intervention was depressive disorders. The participants were divided in to small and large groups, depending on their initial stage of change. The GPs' knowledge and skills regarding management of depressive disorders were assessed through a questionnaire with 7 multiple choice questions, 11 Likert statements, 3 case vignettes, and 1 essay question. Attitudes toward management of depressive disorders were also assessed. Both questionnaires were validated. RESULTS: There was a significant improvement in knowledge mean scores regarding multiple choice and Likert questions (intervention effect 6%; P = 0.002), as well as for the case vignettes and essay question (intervention effect 12%; P = 0.011) in the intervention arm, in comparison with the control arm. There were significant changes in mean attitude scores in both study arms, but no difference between them. CONCLUSIONS: A theoretical model of medical learning and behavioural change can be used to devise educational formats that suit different stages of learning. Such tailored educational formats can improve GPs' knowledge and skills regarding management of depressive disorders.

Cardiac decompensation in a patient with Eisenmenger syndrome undergoing T5-T7 levels laminectomy in the sitting position.

Although several different anesthetic techniques have been employed for noncardiac surgery in Eisenmenger syndrome (ES), the best anesthetic technique is still controversial. A17-year-old male with ES and an epidural abscess at the T5- T7 levels was scheduled to undergo laminectomy in the sitting position. Anesthesia was induced by ketamane and maintained with an opioid-isoflurane technique. A few minutes after extubation, respiratory distress appeared and the patient was reintubated. He was transferred to the intensive care unit (ICU) and maintained on spontaneous intermittent mandatory ventilation and pressure support for 24 hours, and discharged fully recovered on the 4th postoperative day. To our knowledge this is the first case where the sitting position has been adopted for evacuation of an epidural abscess at the T5-T7 levels in a patient with ES. We recommend late extubation and an overnight observation in the ICU for such patients to prevent dangerous sequelae.

Atrial and ventricular ejection force of the fetal heart: Which of the four chambers is the dominant?

BACKGROUND/AIM: This study aimed to measure and compare the ejection force of the cardiac chambers in healthy singleton fetuses and to investigate the relationship of ejection force of cardiac chambers with gestational age, fetal sex, and fetal heart rate. PATIENTS AND METHODS: A prospective study was performed on 68 singleton fetuses with a gestational age of 17-34 weeks. Atrial and ventricular ejection force was measured. Measurements were repeated in 18 of the fetuses to assess intraobserver reliability. RESULTS: The right atrium had the highest ejection force of all the cardiac chambers. Ejection force of both atria and ventricles increased with gestational age. CONCLUSION: The right atrium is the dominant chamber of the fetal heart in 17-34 weeks of gestation. Comparison of our values with previous studies indicates that left atrial ejection force almost doubles in the 1st month after birth. This study highlights the crucial role of the right atrium in the fetal cardiac function during 17-34 weeks of gestation.

Efficacy of Traditional Medicine Product Henna and Hydrocortisone on Diaper Dermatitis in Infants.

BACKGROUND: Diaper dermatitis (DD) is a common inflammatory disorder in infants, including newborns. OBJECTIVES: This study aimed to compare the effects of a traditional medicine product (containing natural henna oil 25%) and hydrocortisone 1% cream on DD in infants. PATIENTS AND METHODS: In a triple-blind, randomized trial, 82 children aged two years or less were randomly divided into two groups of 41 children each to receive either hydrocortisone ointment or henna medicinal product. Infants were treated 3 times a day for 5 days. The severity of dermatitis was assessed on the first, third, and fifth days using a six-point scale. The study was conducted in 2013 in a children teaching hospital in Qazvin, Iran. RESULTS: Both groups showed an improvement in the severity of DD (mean DD severity on the first, third and fifth days, respectively, was 3.20, 1.39, and 1.20 in the henna group versus 3.20, 2.05, and 1.90 in the hydrocortisone group; P < 0.001). The henna group showed a better response when compared with the hydrocortisone group: the rate of improvement on the fifth day of treatment was 90.2% (37 of 41 children without erythema) in the former versus 61% (25 of 41 patients) in the latter (P = 0.042). No significant side effects were observed in both the groups. CONCLUSIONS: Henna, a traditional medicine product, can be considered an effective and appropriate treatment for DD in infants and children.

Echocardiographic abnormalities and their correlation with bronchiectasis score in primary antibody deficiencies.

BACKGROUND: Primary antibody deficiencies are characterized by defective antibody production and recurrent infections. Patients usually present with recurrent respiratory tract infections with consequent chronic pulmonary damage and bronchiectasis, which could potentially influence cardiac function. Our aim was to assess noninvasively the cardiac complications due to pulmonary disease in patients with primary antibody deficiency. METHODS: A cross-sectional series of patients with primary antibody deficiency syndromes from our referral immunology center were recruited. Individuals undergoing high-resolution computed tomography (HRCT) and transthoracic echocardiography were reviewed. RESULTS: Thirty primary immunodeficient patients aged 5-55 years of age (21 males and 9 females) were enrolled in this study. Half of the patients (50%) were found to have bronchiectasis in HRCT imaging. In echocardiographic examination, 20 patients (67%) had at least one abnormality; among which pulmonary hypertension was the most common (33%). Patients with bronchiectasis had higher pulmonary artery pressures and HRCT bronchiectasis score was strongly correlated with pulmonary artery pressure (regression R = 0.59, P value = 0.001). CONCLUSION: Echocardiographic evaluation of right ventricular function and noninvasive estimation of pulmonary artery pressure could have an important diagnostic role in the follow-up and therapeutic management of patients with primary immune deficiency.