Platelets in Pulmonary Hypertension: a Causative Role or a Simple Association?

Pathophysiology of pulmonary arterial hypertension is based on three basic mechanisms: thrombotic pulmonary vascular lesions, vasoconstriction and vascular remodeling. Platelets are related to all of these mechanisms by their aggregation, production, storage and release of several mediators. The role of platelets is more prominent in some types of pulmonary arterial hypertension, including those which are secondary to inflammatory and infectious diseases, hemoglobinopathies, essential thrombocythemia, drugs, thromboembolism, and cardiac surgery. Most pulmonary antihypertensive drugs have a negative effect on platelets. In this review, the mechanisms of platelets association with pulmonary arterial hypertension, those types of pulmonary arterial hypertension with greatest platelet contribution to their pathophysiology, and the effects of pulmonary antihypertensive drugs on platelets are summarized

Aortopulmonary Window Closure by Duct Occluder: Report of Two Cases

Aortopulmonary window is usually treated surgically. However, small windows far from the coronary and pulmonary branch arteries can be occluded by transcatheter approache. The key step is the precise sizing of the defect. We report two successful transcatheter closures. We used CT angiography for precise measurement of the defect in the first patient, and balloon sizing in the other. We used duct occluders in both patients

Transcatheter Atrial Septal Defect Closure Using Occlutech Figulla Device: A Two-Center Experience

Background: Despite several reports regarding the use of the Occlutech Figulla® Flex septal occluder (OFFSO) in adults, there are few reports on its use in children. We sought to study the result of the transcatheter closure of atrial septal defect (ASD) using the OFFSO in children ≤ 12 years. Methods: We enrolled 45 consecutive patients, ranging from 2.5 to 12 years of age, in two large pediatric cardiovascular centers. All the children underwent complete echocardiographic examination before the procedure. Defect/device ratio and device/weight ratio were measured. The device diameter to the cardiac diameter ratio (DD/CD ratio) in anteroposterior projection after device release and the DD/CD index were calculated by dividing the DD/CD ratio by the body surface area. Results: Of the 45 enrolled patients, 25 (55%) were female. The range and mean ± standard deviation (SD) of age were2.5 to 12 years and 6.8 ± 2.5 years, respectively. The range and mean ± SD weight were 8.5 to 37.0 kg and 19.7 ± 7.2 kg, respectively. Successful implantation was performed in all the patients. No major complications occurred in any of the subjects. We encountered one cobra head deformity in one patient. Neither residual shunt nor conduction abnormality was observed in any of the cases. Conclusion: Transcatheter ASD closure using the OFFSO was effective in our pediatric patients. Although this device needs relatively larger delivery sheaths, its use is safe while closing even large defects in children

Right Ventricular Myocardial Tissue Velocities, Myocardial Performance Index, and Tricuspid Annular Plane Systolic Excursion in Totally Corrected Tetralogy of Fallot Patients

Background: Longer survival after the total repair of the Tetralogy of Fallot increases the importance of late complications such as right ventricular dysfunction. This is a prospective study of the right ventricular function in totally corrected Tetralogy of Fallot patients versus healthy children. Methods: Thirty-two healthy children were prospectively compared with 30 totally corrected Tetralogy of Fallot patients. Right ventricular myocardial tissue velocities, right ventricular myocardial performance index, and tricuspid annular plane systolic excursion were investigated as well as the presence and severity of pulmonary regurgitation. Results: The two groups were age-and sex-matched. Mean systolic peak velocity (Sa) and tricuspid annular plane systolic excursion were significantly decreased, while myocardial performance index and early to late diastolic velocity (Ea/Aa) were significantly increased in the Tetralogy of Fallot patients. Early diastolic velocity (Ea) showed no significant difference between the two groups. Sa correlated significantly with tricuspid annular plane systolic excursion in both the normal children and totally corrected Tetralogy of Fallot patients. Myocardial performance index was significantly higher in the patients with moderate to severe pulmonary regurgitation than in those with mild regurgitation. However, there was no significant correlation between this index and right ventricular myocardial tissue velocities. Conclusion: In this study, systolic right ventricular function indices (Sa and tricuspid annular plane systolic excursion) were impaired in the totally corrected Tetralogy of Fallot patients. Myocardial performance index was affected by the severity of pulmonary regurgitation

Transcatheter Atrial Septal Defect Closure under Transthorasic Echocardiography in Children

Objective:Atrial septal defect (ASD) device closure is routinely done under the guide of transesophageal or intracardiac echocardiography which are expensive techniques and not easily affordable in developing countries. Methods: Using metallic devices, we attempted 32 ASD device closures under transthoracic echocardiography. Findings: Of those, 30 procedures were successful (94 %). In two patients with relatively large ASD we encountered difficulty in positioning the device. These patients were referred for surgical closure. Conclusion: ASD device closure can be carried out successfully in most patients under transthoracic echocardiography in echocardiographies are not available or affordable. situations where transesophageal or intravenous echocardiographies are not available or affordable

Left ventricular function assessment in Kawasaki disease by two-dimensional global longitudinal systolic strain with automated function imaging

Abstract Background Kawasaki disease is an acute febrile vasculitis of childhood mainly affecting children under 4 years of age. In the acute stage of the disease, heart function decreases and gradually returns to normal after treatment. However, subendocardial involvement may persist, which cannot be assessed by M-mode echocardiography. Strain echocardiography is a recently developed technique to assess subendocardial involvement of myocardial deformation. We aimed to study the stratified strain of left ventricular function in a Kawasaki patient at least 6 months after the acute stage of the disease with special conditions for entering the study using two-dimensional speckle-tracking imaging. Between September 2020 and October 2022, 27 healthy children and 27 children with a history of Kawasaki disease more than 6 months ago were evaluated using two-dimensional global longitudinal peak systolic strain with automated function imaging technology. Results The mean age of patients was 5.6 years. With M-mode echocardiography, ejection fraction of each group was in the normal range. Mean (± standard deviation) global longitudinal peak strain in four-chamber view of girls with Kawasaki disease was − 23.74 ± 2.77, and that in boys with Kawasaki disease was − 20.93 ± 2.06 (P value = 0.008). GLPS (global longitudinal peak strain) was compared as an overall average and as in a separate segment, which showed significant difference in two comparisons. In our study, a decrease in the function of some cardiac segments is reported. Global longitudinal peak strain in four-chamber view was significantly lower in boys. Comparing different segments, a difference in global left ventricular long-axis strain was found between the two groups. On the other hand, there was a major difference between the two groups in the basal inferolateral, basal anterolateral, and mid-inferolateral, which receives blood from Left Circumflex artery. Conclusion Using stain echocardiography to detect continued subendocardial involvement in asymptomatic children with a history of Kawasaki disease for a better understanding of the condition, effective management and follow-up is recommended

COVID-19 in Children with Congenital Heart Diseases: A Multicenter Case Series from Iran

Background. Promptly discovering and counteracting COVID-19 is critical as it could have catastrophic effects. As an asymptomatic group, children are highly susceptible to be misdiagnosed, especially those suffering from underlying diseases. Furthermore, discriminating the direct effects of the virus from those of the underlying diseases can pose a dilemma to physicians. This case series aims to determine the relationship between COVID-19 and various types of congenial heart disease among children. Patients and Methods. Seven patients from three different medical centers were enrolled. Their detailed demographic information, past medical history, symptoms, type of congenital heart diseases, imaging tests, laboratory tests, medications, and outcomes were analyzed. Results. The patients included 4 infants, 1 child, and 2 adolescents, with a median age of 9 months and a majority of boys. All of them had either obstructive lesions (right or left ventricular outflow tracts) or significant pulmonary hypertension. The more common clinical symptoms were cough, dyspnea, and fever. Two patients did not survive the illness. Conclusion. Prompt treatment of patients with a combination of COVID-19 and severe obstructive cardiac pathology or pulmonary hypertension is essential due to a risk for serious and/or fatal consequences