Impact of Drug Elution on Infectious Outcomes after Ductal Stenting in Newborns.

Ductal stenting (DS) is an interesting palliation for neonates with duct-dependent pulmonary circulation. With the introduction of drug-eluting stents (DES), which have a lower rate of restenosis and longer patency, these stents have replaced bare metal stents (BMS) in the global market. DES release Rapamycin group drugs, which have anti-proliferative and immunosuppressive effects. While the released drug amount is negligible in adult patients, it can lead to high blood levels in neonates, potentially increasing their risk of infections. We conducted a retrospective observational study on infants (under 2 months of age) with duct-dependent pulmonary circulation who underwent successful DS procedures between September 2013 and September 2023. Infants who received at least one DES were categorized into the DES group, while those receiving only BMS were categorized into the BMS group. We compared the prevalence of sepsis (both clinical and proven) and mortality between the two groups. We identified 53 infants (58.4% males), comprising 30 (56.6%) in the DES group and 23 (43.4%) in the BMS group. In the DES group, there were 11 cases (36.7%) of sepsis, including 8 clinical and 3 culture-positive cases. In contrast, the BMS group had 4 cases (13%) of sepsis, all of which were clinical (p = 0.053). Three patients (5.6%) died due to sepsis: two from the DES group and one from the BMS group. Six patients were suspected of having immunodeficiency (22q11.2 deletion or asplenia), with three in each group. Among these, only one patient in the DES group developed clinical sepsis. Our findings indicate that drug elution did not significantly increase the risk of infection following DS in newborns.

Echocardiographic estimation of pulmonary arterial and right atrial pressures in children with congenital heart disease: a comprehensive prospective study and introduction of novel equations.

BACKGROUND: Pediatric pulmonary hypertension (PH) is characterized by a mean pulmonary arterial pressure exceeding 20 mmHg. There is limited research on the suitability of adult-based methods for estimating PH in pediatric populations. Using established formulas for adults, this study aimed to evaluate the correlation between echocardiographic estimates of systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures in children with congenital heart disease (CHD). METHODS: A prospective study was conducted involving children with CHD undergoing cardiac catheterization without prior cardiac surgery. We used echocardiography to estimate pulmonary and right atrial pressures and compared these with invasively measured values. Four reliable regression equations were developed to estimate systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures. Cutoff values were determined to predict the occurrence of PH. Linear regression, Bland-Altman analysis, and receiver operating characteristic curve analysis were performed to assess the accuracy of echocardiography and establish diagnostic thresholds for PH. RESULTS: The study involved 55 children (23 with normal pulmonary arterial pressure and 32 with PH) with acyanotic CHD aged 1 to 192 months. Four equations were developed to detect high pulmonary arterial pressures, with cutoff values of 32.9 for systolic pulmonary arterial pressure, 14.95 for diastolic pulmonary arterial pressure, and 20.7 for mean pulmonary arterial pressure. The results showed high sensitivity and moderate specificity but a tendency to underestimate systolic and mean pulmonary arterial pressures at higher pressures. CONCLUSIONS: The study provides valuable insights into the use of adult-based echocardiographic formulas for estimating PH in pediatric patients with acyanotic CHD.

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

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.

Safety of Aortic Coarctation Treatment in Patients with Turner Syndrome: A Single-Country Case Series and Literature Review.

BACKGROUND: Coarctation of the aorta is a common cardiac disease in Turner syndrome. Evidence indicates that surgery and balloon angioplasty in infants and small children do not have any added risk of mortality or complication in these patients. Stenting in older patients may, however, pose higher risks of arterial wall injury and mortality. METHODS: In this case series, we describe 15 patients with coarctation of the aorta in Turner syndrome: 9 received stenting, 4 underwent surgery, and 2 were treated via balloon angioplasty. RESULTS: Dissection occurred in 2 patients after stenting: 1 in the aorta and the other in the external femoral artery. Both were managed promptly without any mortality or serious damage, one percutaneously and the other surgically. CONCLUSIONS: Awareness of increased risks and preparedness for prompt interventions in case of an acute arterial wall injury are recommended when coarctation stenting is done for a patient with Turner syndrome.

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.

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.

Complete heart block in a boy with hyperostosis-hyperphosphataemia syndrome: a case report.

BACKGROUND: Hyperostosis-hyperphosphataemia syndrome (HHS) is a rare metabolic disorder characterized by recurrent painful swelling of long bones and periosteal new bone formation. CASE SUMMARY: A 6-year-old boy was referred to our centre due to bradycardia. He was diagnosed with HHS 3 years' prior, after investigation for the cause of joint pain and genu valgum. During medical follow-up in 2013-16, the paediatric cardiologist discovered thickened and calcified mitral and aortic valves and progression of cardiac conduction disturbance from 1st degree to 3rd degree atrioventricular block (AVB). The patient died in 2017 due to multiorgan failure caused by hyperphosphataemia and ectopic calcification. DISCUSSION: Our case is unique in that ectopic calcification occurred in the aortic, mitral valve and cardiac conduction system, and AVB progressed from 1st degree to 3rd degree over time despite treatment with high-dose phosphate binders.

Multicenter Off-Label Use of Nit-Occlud Coil in Retrograde Closure of Small Patent Ductus Arteriosus.

We studied the safety and efficacy of closing patent ductus arteriosus by Nit-Occlud coils via retrograde approach. This is a retrospective study of 46 attempts to close ducts by this method in two hospitals in Egypt and Iran. Ductus arteriosus was crossed by left or right Judkins or endhole catheters. The coil was delivered via the same catheter or the provided endhole catheter after exchange. The procedure was successful in 42 out of 46 attempts. Fluoroscopy and procedural times were significantly shorter when the catheter was not exchanged. This method is effective and safe for the closure of small ducts. Crossing the duct and delivering the coil by a left Judkins catheter is the easiest and fastest way to perform this method.

A novel method for quantification of left ventricular noncompaction using two-dimensional echocardiography in children.

OBJECTIVE: Although there are several echocardiographic criteria, there is not yet a general consensus about the diagnosis of left ventricular noncompaction. The current criteria are mostly based on the areas with maximal noncompaction in the heart. The echocardiographer may miss this maximal point leading to a misdiagnosis. Accordingly, we suggested a new method to measure the percentage of myocardial noncompaction using two-dimensional echocardiography. METHODS: In this study, the new method was examined on 4 noncompaction and 26 dilated cardiomyopathies, and 25 normal subjects. The percentage of noncompaction was measured at 3 levels (apical, papillary muscle and mitral valve) and averaged. FINDINGS: The mean percentages of myocardial noncompaction were 3.59±2.27, 8.86±5.52 and 34.7±26.1 in the control, dilated cardiomyopathy and noncompaction groups, respectively. A value of 17% or greater could distinguish left ventricular noncompaction from dilated cardiomyopathy with 92% specificity and 100% sensitivity and from normal subjects with 100% specificity and sensitivity. This percentage had a statistically significant association with noncompacted to compacted myocardial thickness ratio (P<0.001). CONCLUSION: This method showed good correlations with the existing echocardiographic and magnetic resonance criteria. However, it is not dependent on finding the area of maximal involvement. Being comparable to magnetic resonance imaging in accuracy, it is easier to perform and more available.

Correlation between QRS Duration, Pulmonary Insufficiency and Right Ventricle Performance in Totally Corrected Tetralogy of Fallot.

OBJECTIVE: Despite progresses in surgical correction of Tetralogy of Fallot, pulmonary insufficiency and progressive dysfunction of the right ventricle impress its long-term prognosis. In this study we examined the correlations between QRS duration, pulmonary insufficiency and right ventricular performance index. METHODS: We enrolled 57 repaired Tetralogy of Fallot patients. QRS duration on electrocardiogram, pulmonary regurgitation index (regurgitation time to diastolic time ratio), and right ventricular myocardial performance index were measured. FINDINGS: There was a strong inverse correlation between QRS duration and pulmonary regurgitation index. However, significant correlation did not exist between QRS duration and right ventricular myocardial performance index. QRS duration ≥160 ms predicted severe pulmonary regurgitation with 100% sensitivity and 87% specificity. CONCLUSION: Increased QRS duration can predict severity of pulmonary regurgitation.

Aortic dilatation and aortopathy in congenital heart diseases.

Longer survival after corrective surgery for congenital heart diseases has rendered late complications more important. One of these complications is aortic dilatation which may occur in patients with repaired or unrepaired disease and can progress to aneurysm, dissection, and rupture. This aortic dilatation in various congenital heart diseases does not simply mean anatomical dilatation of the aortic root, but it closely relates to the aortic pathophysiological abnormality, aortic regurgitation, and aortic and ventricular dysfunction; therefore, we can recognize this complex lesion as a new concept: "aortopathy". The pathophysiology of this disease is complex and only partially understood. In this review, we first discuss history, pathophysiology, and clinical features of aortic dilatation and aortopathy of congenital heart disease. Then we provide a review of the evaluation and management of this disease.

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.

Closure of interatrial defects by immediate-release patch.

The Immediate-Release Patch is the newest version of the Transcatheter Patch, which is a bioabsorbable device for the closure of cardiac defects. Closure of 12 atrial septal defects and 1 fenestration was attempted using this device. Of the 13 devices used, 12 were implanted successfully (including that for the fenestration), whereas 1 device moved from the original position and was retrieved percutaneously. No other major adverse events occurred. After a median follow-up period of 11 months, trivial (≤ 2-mm) residual shunts remained in two patients, and a significant residual defect (7 mm) remained in 1 patient. The major advantages of this device include its wirelessness, its bioabsorbablity, potential application for defects up to 30 mm using only three sizes, its easier retrieval, and its ability to occlude defects with a deficient rim and some ostium primum and sinus venosus type defects. In contrast to the metal devices, it is bulkier and has a different application method requiring operator familiarity, and it sometimes leaves residual shunts.

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 embolization of congenital hepatic arteriovenous malformation using ethylene-vinyl alcohol copolymer (Onyx).

A male infant with high-output heart failure who had been found to have a hepatic arteriovenous malformation by ultrasound imaging was referred to our center (Department of Diagnostic and Interventional Radiology, Imam Khomeini Hospital, Tehran University of Medical Sciences, Islamic Republic of Iran) for further evaluation. Computed tomography angiography revealed a large hepatic arteriovenous malformation with feeders originating from enlarged hepatic arteries and draining to enlarged hepatic veins. We performed a transcatheter embolization of the anomaly using ethylene vinyl alcohol copolymer (Onyx) during a single session. The cardiac function of the infant rapidly improved after the procedure. Over the 19 months of follow-up, his cardiac output remained stable and within the normal limits, and no complications were detected.

Transcatheter patch occlusion of ventricular septal defect in Down syndrome.

Closure of perimembranous ventricular septal defects in patients with Down syndrome, either surgically or by Amplatzer occluders, carries a high risk of complete heart block. We report 5 closures using the transcatheter patch, a wireless bioabsorbable device without any reported heart block to date. The median defect size was 11 mm. Small devices were used in 4 patients and a medium size in 1. The patch was released after 48 hours in 3 patients and immediately in the other 2. Of the 5 patients, 3 were followed up for >5 years and 2 for 1 year. None of these patients had atrioventricular block during their follow-up. In conclusion, the transcatheter patch might be superior in terms of cardiac conduction system protection in patients with Down syndrome after ventricular septal defect closure.

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 situations where transesophageal or intravenous echocardiographies are not available or affordable.