Transcatheter Closure of Secundum Atrial Septal Defects in Pediatric Patients: A 15-Year Single-Center Experience.

PURPOSE: Transcatheter closure is the first-line treatment option for hemodynamically significant secundum atrial septal defects (ASDs). This study examines our center's experience with this procedure over the last 15 years. MATERIALS AND METHODS: Pediatric patients aged 0-18 years with secundum ASDs who were planned for transcatheter closure in our clinic between January 2007 and January 2023 were retrospectively evaluated. RESULTS: Transcatheter secundum ASD closure was planned for a total of 334 patients during the study period: 191 girls (57.2%) and 143 boys (42.8%). Their mean age was 8.08±3.9 years, and their mean weight was 30±15.6 kg. Defect diameter measured transesophageally ranged from 5 to 35 mm, with a mean of 12.56±4.02 mm. Transesophageal echocardiographic examination revealed a single secundum ASD in 319 patients (95.5%) and multiple secundum ASDs in 15 patients (4.5%). In 11 patients (3.3%), the procedure was terminated before initiating transcatheter ASD closure because of insufficient vena cava rims or a very large or multi-fenestrated defect. The 323 patients (96.7%) who underwent transcatheter ASD closure had a mean pulmonary artery pressure of 15.1±4.0 mmHg and a mean Qp/Qs ratio of 1.97±0.56. The procedure failed in four patients (1.3%) because of device embolization (n=2) or the inability to properly position the device (n=2). Major complications other than device embolization observed during or after transcatheter closure included anesthetic-induced respiratory depression (n=1) and total atelectasis of the lung (n=1). No new major complications were detected during the patients' long-term follow-up. CONCLUSION: With appropriate patient and device selection, transcatheter closure is a safe and effective treatment for secundum ASD and should be the first treatment of choice.

P-wave asystole after a tetralogy of Fallot correction surgery.

A rarely seen arrhythmia is the p-wave asystole also mentioned ventricular asystole, ventricular standstill, or third-degree atrioventricular block with no ventricular escape rhythm. It is the result of the lack of impulse formation in ventricles (absence of idioventricular automaticity) or the failure of impulse transmission to ventricles (conduction disturbance)(1). As the name implies, the ventricles stop pumping, resulting in no effective cardiac output during the phenomenon. It is a potentially fatal rhythm disorder and need immediate diagnosis and treatment. We planned to present a case of p-wave asystole, which developed after tetralogy of Fallot total correction surgery.

Evaluation of Arrhythmia Prevalence, Management, and Risk Factors in Patients with Transcatheter and Surgically Closed Secundum Atrial Septal Defects.

OBJECTIVE: Atrial septal defect (ASD) accounts for 6-10% of all congenital heart disorders. Secundum ASD closure can be performed surgically or percutaneously. We aimed to identify the various arrhythmias that occur before, during, and after the procedure and evaluate their management. METHODS: The study included a total of 427 patients aged 0-18 years who underwent transcatheter or surgical closure of isolated secundum ASD between January 2008 and January 2020. Postoperative electrocardiogram (ECG) traces, intraoperative arrhythmias, and treatments were recorded for both groups. Echocardiography and ECG were evaluated at postoperative 1 week, 1, 3, and 6 months, and annually thereafter. RESULTS: After transcatheter closure, follow-up basal ECG showed incomplete right bundle branch block pattern in 21 patients and sinus rhythm in 229 patients. After surgical closure, incomplete right bundle branch block pattern was detected in 23 patients, complete right bundle branch block in 3 patients, and complete AV-block (Atrioventricular Block) pattern in 1 patient. The other 150 patients showed sinus rhythm. At least one postoperative follow-up Holter ECG record could be obtained for 104 patients in the transcatheter group and 96 patients in the surgical group. Of 104 patients who underwent transcatheter closure, 97 (93.3%) had normal Holter ECG findings and 7 (6.7%) had arrhythmia. Of the 96 patients who underwent surgical closure, 85 (88.5%) had normal Holter ECG traces and 11 (11.5%) had arrhythmia. There was no statistically significant difference in the frequency of arrhythmia (P = 0.164). CONCLUSION: The higher frequency of arrhythmia in adult studies compared to the pediatric age group once again demonstrates the importance of early diagnosis and treatment of ASD in childhood. The similar incidence of arrhythmia in both groups supports the safety and effectiveness of both closure methods in eligible patients.

Arrhythmias in children undergoing orthotopic heart transplantation.

Introduction: Heart transplantation (HT) is the only treatment option in children with heart failure secondary to cardiomyopathies and non-reparable congenital heart diseases. Methods: We performed a retrospective clinical data review of all consecutive pediatric patients (aged 2-18 years) who underwent orthotopic HT for advanced heart failure at our institution between January 2007 and January 2023. Clinical, procedural, and follow-up data were collected and comprehensively analyzed. Results: We identified 27 children (66.7% males) with a median age of 15 years (IQR: 7-16) and a median weight of 45 kg (IQR: 22-66) at the time of the intervention. 24 patients (88.8%) were diagnosed with dilated cardiomyopathy, 2 (7.4%) with restrictive cardiomyopathy, and 1 (3.7%) with hypertrophic cardiomyopathy. On a median follow-up of 35.07 months (IQR: 13.13-111.87), arrhythmias were detected in 9 (33%) patients. Three patients developed symptomatic sinus node dysfunction at 18, 25, and 38 days and received permanent pacemakers. One patient developed a complete AV block during acute rejection at 76 months and received a temporary pacemaker. Two patients developed chronic sinus tachycardia at 4 and 16 months and were treated with Beta-blockers after eliminating all causes of sinus tachycardia. One patient developed a complete right bundle branch block at 12 months. One patient developed ventricular extrasystole at 10 months and was found to have grade 2 rejection. An Atrial extrasystole was detected in one patient at 96 months. We did not identify significant risk factors for arrhythmias post-HT. Discussion: After pediatric HT, early-onset rhythm disturbances, often attributed to surgery-related issues such as sinus node dysfunction, may necessitate invasive treatments like permanent pacemaker therapy. Close monitoring post-transplantation is crucial, and routine follow-up with Holter ECG is necessary to identify potential rhythm disorders even in the absence of symptoms. Rhythm disturbances that develop during follow-up can serve as early indicators of graft rejection and should be carefully evaluated.

Hypertrophic cardiomyopathy after Takeuchi procedure for anomalous origin of the left coronary artery from the pulmonary artery syndrome.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare condition. Surgical intervention is indicated in all diagnosed patients. Successful repair is expected to correct left ventricle (LV) functions and heart failure, reduce mitral regurgitation, and resolve infarct patterns observed on electrocardiogram. For this reason, compared to idiopathic dilated cardiomyopathy, ALCAPA is considered a curable form of dilated cardiomyopathy. In this article, we present the case of 3-month-old girl who underwent the Takeuchi procedure for ALCAPA syndrome and developed hypertrophic cardiomyopathy despite expectations of improved LV function.

Endovascular treatment of aortic coarctation with a novel BeGraft aortic stent in children and young adults: a single-centre experience with short-term follow-up results.

OBJECTIVES: We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population. METHODS: This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre. RESULTS: The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13-21) years and a median weight of 65 (46-103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12-55) mmHg before the procedure and 5 (0-17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4-25) months and none required redilation. CONCLUSIONS: Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

The use of N-terminal (1-76) pro-brain natriuretic peptide in the aetiology of severe respiratory distress in the paediatric emergency department.

INTRODUCTION: Acute respiratory distress is one of the most common reasons for paediatric emergency visits. Paediatric patients require rapid diagnosis and treatment. Our aim in this study was to use N-terminal (1-76) pro-brain natriuretic peptide to differentiate respiratory distress of cardiac and pulmonary origin in children. Our aim was to investigate the role of N-terminal (1-76) pro-brain natriuretic peptide in the detection of patients with new-onset heart failure in the absence of an underlying congenital heart anomaly. METHODS: All children aged 0-18 years who presented to the paediatric emergency department due to severe respiratory distress were included in the study prospectively. The patients' demographic characteristics, presenting complaints, clinical findings, and N-terminal (1-76) pro-brain natriuretic peptide concentrations, were investigated. In patients with severe Pediatric Respiratory Severity Score, congestive heart failure score was calculated using the modified Ross Score. RESULTS: This study included 47 children between the ages of 1 month and 14 years. The median N-terminal (1-76) pro-brain natriuretic peptide concentration was 5717 (IQR:16158) pg/mL in the 25 patients with severe respiratory distress due to heart failure and in the 22 patients with severe respiratory distress due to lung pathology was 437 (IQR:874) pg/mL (p < 0.001). In the 25 patients with severe respiratory distress due to heart failure, 8281 (IQR:8372) pg/mL in the 16 patients with underlying congenital heart anomalies, and 1983 (IQR:2150) pg/mL in the 9 patients without a congenital heart anomaly (p < 0.001). The 45 patients in the control group had a median N-terminal (1-76) pro-brain natriuretic peptide concentration of 47.2 (IQR:56.2) pg/mL. CONCLUSION: Using scoring systems in combination with N-terminal (1-76) pro-brain natriuretic peptide cut-off values can help direct and manage treatment.

Interleukin-8 is increased in chronic kidney disease in children, but not related to cardiovascular disease.

INTRODUCTION: In this study, we aimed to detect the cytokine that is involved in the early stage of chronic kidney disease and associated with cardiovascular disease. METHODS: We included 50 patients who were diagnosed with predialytic chronic kidney disease and 30 healthy pediatric patients in Ege University Medical Faculty Pediatric Clinic, Izmir/Turkey. Interleukin-8 (IL-8), interleukin-10 (IL-10), interleukin-13 (IL-13), and transforming grow factor-ß1 (TGF-ß1) levels (pg/mL) were measured by ELISA. Carotid-femoral pulse wave velocity (PWV), augmentation index (Aix), carotid intima media thickness (cIMT), and left ventricular mass index (LVMI) were evaluated as markers of cardiovascular disease. The presence of a cardiovascular disease marker was defined as an abnormality in any of the parameters (cIMT, PWV, Aix, and left ventricular mass index (SVKI)). The patient group was divided into two groups as with and without cardiovascular disease. RESULTS: Mean Aix and PWV values were higher in CKD patients than controls (Aix: CKD 32.8±11.11%, healthy subjects: 6.74±6.58%, PWV CKD: 7.31±4.34m/s, healthy subjects: 3.42±3.01m/s, respectively; p=0.02, p=0.03). The serum IL-8 levels of CKD were significantly higher than of healthy subjects 568.48±487.35pg/mL, 33.67±47.47pg/mL, respectively (p<0.001). There was no statistically significant difference between IL-8, IL-10, IL-13, TGF-1, in CKD patients with and without cardiovascular disease (p> 0.05). DISCUSSION: IL-8 is the sole cytokine that increases in pediatric patients with chronic kidney disease among other cytokines (IL-10, IL-13 and TGF-ß1). However, we did not show that IL-8 is related to the presence of cardiovascular disease.

Usefulness of N-terminal pro-B-type natriuretic peptide (NT-ProBNP) as a marker for cardiotoxicity and comparison with echocardiography in paediatric carbon monoxide poisoning.

OBJECTIVES: To demonstrate the usefulness of N-Terminal Pro-B-Type natriuretic peptide (NT-proBNP) as an early biomarker of carbon monoxide-induced myocardial injury in children. It also aimed to identify the correlation between NT-proBNP and left ventricular systolic dysfunction findings shown by echocardiography. METHODS: Prospective, observational study conducted at a paediatric emergency department between October 2017 and April 2019 which involved children aged 0-17 years. The patients were divided into three groups based on severity; mild, moderate and severe groups. The patient characteristics, carboxyhaemoglobin, CK-MB Mass (CKMB-M), troponin-T, and NT-proBNP levels were measured, and echocardiography was performed and left ventricular ejection fraction was measured. RESULTS: Sixty-nine patients and 60 healthy controls were included. Male gender, younger age, higher carboxyhaemoglobin levels, and altered mental status were found as independent predictors of carbon monoxide-induced myocardial injury. If the cut-off value for NT-proBNP level is >480 pg/ml, the sensitivity-specificity for decreased left ventricular ejection fraction, which is the strongest carbon monoxide-induced myocardial injury sign, were 100-96%, respectively. A high negative correlation was found between NT-proBNP levels and left ventricular ejection fraction (r = -0.769, p < 0.01) in the carbon monoxide poisoning group, and there was a positive correlation between the carboxyhaemoglobin and NT-proBNP levels (r = 0.583, p < 0.01). CONCLUSION: Echocardiography is an ideal tool and very sensitive, but its routine use is limited due to its non-availability. An increased level of NT-proBNP (>480pg/ml) may be useful as an ideal biomarker for early detection of carbon monoxide-induced myocardial injury sign and reduced left ventricular ejection fraction which is the most crucial point in making a decision on hyperbaric oxygen therapy.

Periodic fever syndromes: a patient diagnosed with recurrent Kawasaki disease.

Kawasaki disease, known as mucocutaneous lymph node syndrome, is a multi-system disease of unknown aetiology that occurs in young children under 5 years of age. The recurrence rate of Kawasaki disease is as rare as 1-3%. Especially in cases with coronary artery involvement, recurrent Kawasaki disease should be investigated in terms of underlying rheumatologic diseases such as periodic fever syndromes, microscopic polyangiitis, polyarteritis nodosa, and systemic-onset juvenile arthritis. In this study, we report homozygote mutations in mevalonate kinase and familial Mediterranean fever genes in a recurrent Kawasaki disease with coronary dilatation.

Native valve endocarditis due to Candida albicans in two children: Two new case reports.

Candida endocarditis (CE) is a rare and serious complication of candidemia. Using current diagnostic tools a confirmed diagnosis is often delayed and outcomes remain poor. The majority of new cases occur following cardiac valvular surgery, and/or in patients with the following risk factors: intravenous drug use, cancer chemotherapy, prolonged presence of central venous catheters, and prior history of bacterial endocarditis. It is not a common complication in non-neutropenic patients particularly with the absence of a prosthetic valve, and very few reports in the literature are available. Attempting to add to the limited data, 211 candidemia episodes from 172 nonneutropenic pediatric cases between January 2008 and December 2017 were evaluated. All patients were considered asymptomatic for underlying heart disease. However, 2 (0.9%) patients with a central venous catheter were determined as having endocarditis following echocardiography.

Two siblings with Gaucher type 3c: different clinical presentations.

Background Gaucher disease (GD) is a lysosomal storage disorder caused by autosomal recessive mutations in the glucocerebrosidase (GBA) gene, which encodes acid ß-glucosidase. GD type 3c is a rare group characterised by cardiovascular involvement, and homozygous D448H is the most frequent mutation. Case presentation We describe two patients who had homozygous D448H mutations. The index patient had hepatosplenomegaly, liver insufficiency and cardiac involvement and her sister had severe cardiac involvement with cardiomyopathy and diffuse aortic calcification. The index case's liver was transplanted at the age of 6 months from a related donor and her sister who had severe cardiovascular disease died at the age of 12 years. Conclusions Our patients had clinical variability. We need to discuss whether liver involvement could be the initial signs in patients with GD type 3c.

Parotitis coexisting with Kawasaki disease in a child: a rare presentation of Kawasaki disease.

Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa, is known to present in multiple ways. Although inflammatory changes in several systems have been reported in KD, there are few documented child patients presenting with parotitis. We report such a case in a five-year-old.

Blalock-Taussig Shunt Size: Should it be Based on Body Weight or Target Branch Pulmonary Artery Size?

The study aimed to revisit the in-hospital predictors of shunt thrombosis (ST) in the foreground of the pulmonary artery size in patients who received modified Blalock-Taussig shunt (mBTS) as the first-stage palliation. Data from 80 patients who received mBTS as their initial palliative procedure between February 2012 and January 2017 was retrospectively collected. The median age and weight of the patients at the time of their mBTS procedure was 4 days (IQR 2-22 days) and 3.2 kg (IQR 2.8-3.7 kg), respectively. Of the 80 patients in the study, 11 (13.8%) developed ST. The diameter and corresponding z scores of the pulmonary arteries were significantly lower in patients with ST. The median shunt size/shunted pulmonary artery size (S/PA) ratio was considerably higher in patients with ST. In logistic regression analysis, pulmonary artery hypoplasia (PAH) [odds ratio (OR) = 13.7 (0.06-0.21), p < 0.001], S/PA ratio ≥ 0.9 [OR = 8.1 (0.03-0.53), p = 0.03], prematurity [OR = 9.5 (0.05-0.33), p = 0.003], and shunt size/weight (S/W) ratio ≥ 1.3 [OR = 6.4 (0.04-0.67), p = 0.012] were found to have a significant impact on ST. The best combination of sensitivity and specificity of the S/W (0.73 and 0.75) and the S/PA ratio (0.73 and 0.80) were achieved at the cut-off value of 1.3 and 0.9, respectively. The Youden index of S/PA was 0.52. While the area under the curve (AUC) of the S/W ratio was 0.686 ± 0.12 (p = 0.049), the AUC of the S/PA ratio was 0.791 ± 0.08 (p = 0.002). In conclusion, instead of weight, considering the size of the target pulmonary artery and thereby, the S/PA ratio would be more instructive in determining shunt size. There were a high number of patients in our study who showed PAH having received a shunt size based on their body weight. By contrast, our results showed that the S/PA ratio of ≥ 0.9 would be a good predictor of in-hospital ST.

Correction to: Blalock-Taussig Shunt Size: Should it be Based on Body Weight or Target Branch Pulmonary Artery Size?

The original version of the article unfortunately contained an alignment error in Table 1. The correct version of Table 1 is given below.

Association of Vitamin D Deficiency with Increased Pulse Wave Velocity and Augmentation Index in Children With Chronic Kidney Disease.

INTRODUCTION: It is known that in children with chronic kidney disease (CKD), cardiovascular damage starts in the form of arterial stiffness. There are risk factors other than the traditional ones such as arterial stiffness hypertension, obesity, hypercholesterolemia, and insulin resistance. Vitamin D deficiency is rather common in CKD, and it was introduced as a risk factor for atherosclerosis; however, its relationship with arterial stiffness is not known completely. The purpose of this study was to research the relationship between 25-hydroxyvitamin D levels and arterial stiffness. MATERIALS AND METHODS: Arterial stiffness was evaluated by measuring augmentation index (AI) and pulse wave velocity (PWV) from the radial and carotid arteries with a Vicorder. The 25-hydroxyvitamin D levels were measured by an immunoassay method. RESULTS: In the 81 CKD patients (mean age, 13.21 ± 6.02 years; mean body mass index, 19.42 ± 5.12 kg/m2; and 56.8% male), the mean vitamin D level was 60.71 ± 39.52 ng/mL, the mean AI was 7.93 ± 7.77%, and the mean PWV was 9.79 ± 4.36 m/s. Serum levels of 25-hydroxyvitamin D was correlated with AI (r = -0.482, P = 0.001) and PWV (r = -0.57, P = .001). CONCLUSIONS: In this study, it was proven that vitamin D deficiency in children was related to nondiabetic and nondialysis CKD.