Aortic Pseudoaneurysm following Ventricular Septal Defect Closure in a Pediatric Patient: A Case Report and Literature Review.

Aortic pseudoaneurysm, a rare condition characterized by localized transmural disruption and dilatation of the aorta, is very rare in the pediatric population. It is primarily caused by previous cardiovascular procedures such as aortic coarctation repair, aortic valve replacement, and subaortic membrane resection. We present a unique case of aortic pseudoaneurysm following surgery to repair a perimembranous ventricular septal defect in a 19-month-old boy who presented with fever as the sole symptom. The fever started on the 30th day after the surgery, and the patient exhibited abnormal laboratory results, including a white blood cell (WBC) count of 28.3 × 109/L, neutrophil percentage of 68%, platelet count of 880 × 109/L, erythrocyte sedimentation rate (ESR) of 200 mm/hour, and 3+ positive C-reactive protein. Echocardiogram revealed a large cystic mass (5 × 4.8 cm) in the ascending aorta, compressing the superior vena cava. Based on this finding, a diagnosis of aortic pseudoaneurysm was suspected. The diagnosis was confirmed through cardiac computed tomographic angiography, and the patient underwent emergent surgery for the repair of the aortic pseudoaneurysm under deep hypothermia and circulatory arrest. Unfortunately, our patient died shortly after the surgery.

Large right ventricular hydatid cyst in a child: a case report.

BACKGROUND: Cystic Hydatid disease is a parasitic infection with a worldwide distribution. It is caused by the larval stages of a species of tapeworms known as Echinococcus granulosus. Even in endemic areas; Cardiac involvement by hydatidiosis is very rare and has atypical presentations as well as localization which make it undiagnosed in about 10% of cases. The left ventricle is the most Common chamber involved by the hydatid cyst and isolated involvement of the right ventricle is very rare, especially in children. The aim of the present study was to describe hydatid cardiac cyst of the right ventricle of a child. CASE PRESENTATION: We present a rare case of an 8 year-old boy, living in a rural area, who was diagnosed with a cardiac hydatid cyst in the right ventricle. He also had multiple pulmonary hydatid cysts and presented with dyspnea, cough and atypical chest pain. The patient underwent surgery for the resection of pulmonary cysts and, subsequently, cardiac hydatid cyst. The outcome was favorable seven weeks after surgery and there was no clinical and echocardiographic recurrence. CONCLUSION: Cardiac Echinococcosis must be suspected in endemic areas, diagnosed with appropriate imaging techniques, and treated appropriately.

Defects in Very Long-Chain Fatty Acid Oxidation Presenting as Different Types of Cardiomyopathy.

Cardiac involvement may accompany various inborn errors of metabolism (IEM) including fatty acid oxidation (FAO) disorders, presenting as rhythm disturbances, conduction abnormalities, cardiomyopathies, pericardial effusion, and sudden cardiac death. FAO disorders are rare mitochondrial diseases with variable organ involvements and clinical presentations. Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) is a FAO disorder with diverse clinical presentations. We report two VLCADD patients with cardiac involvement and diverse presentations. The first patient represents with cardiogenic shock and dilated cardiomyopathy (DCM) at childhood. The second patient represents with suspicious sepsis at early infancy and hypertrophic cardiomyopathy (HCM) at further evaluation. IEM should be thought of in every individual case with suspicious sepsis or cardiac failure regardless of age or previous history.

Echocardiographic Indices in Pediatric Chronic Kidney Disease.

INTRODUCTION: Cardiovascular disease (CVD) may accompany chronic kidney disease (CKD), resulting in additional complications and increased death rate. This study was performed to evaluate cardiac structure and function and several risk factors in hospitalized CKD children. METHODS: Seventy-four children with CKD were enrolled in this cross-sectional descriptive study. Two-dimensional and M-mode ultrasonography, Doppler flow velocity and Tissue Doppler Imaging (TDI) were used to evaluate cardiac chamber size, left ventricular mass (LVM) and echocardiographic indices of ventricular function. RESULTS: Advanced stages of CKD showed statistically insignificant increased LVM and LVM indexed to height2.7 (LVMI), and mildly reduced diastolic function. Hypertensive patients had an insignificant increase in the incidence of left ventricular hypertrophy (LVH) defined as LVMI greater than 95th percentile for age and sex and LVH2 as LVMI2 more than 95 gr/m2 for girls and more than 115gr/ m2 for boys older than 8 years. Patients with LVH had lower left ventricular ejection fraction (LVEF) and abnormal right ventricular (RV) function based on the tricuspid valve systolic velocity (TV S') survey. LVH2 cases, however, revealed decreased LV systolic function according to ejection fraction (EF) and abnormal mitral valve systolic velocity (MV S'). CONCLUSION: LVH related to hypertension and mild systolic and diastolic dysfunction were more prevalent in advanced CKD cases, however TDI showed no statistically significant difference in the prevalence of MV S' and TV S'. We recommend strict blood pressure control and prevention of renal function deterioration as effective tools for cardiac protection in CKD children.  DOI: 10.52547/ijkd.6643.

The Role of Integrative Educational Intervention Package (Monthly ITE, Mentoring, Mocked OSCE) in Improving Successfulness for Anesthesiology Residents in the National Board Exam.

BACKGROUND: National Board of Anesthesiology (NBA) pass rate is an important and critical step in clinical residency programs. OBJECTIVES: This study was designed to assess the relationship between an integrative educational intervention (IEI) and the relative annual pass rate (RAPR). RAPR is defined as ratio of NBA pass rate of Shahid Beheshti University of Medical Sciences (SBMU) to the NBA pass rate of all the anesthesiology residency programs across Iran. METHODS: In a descriptive-analytic retrospective study from 2012 to 2019, RAPR was calculated. IEI was implanted in the latter 4years period of this time interval includes: (1) individualized mentorship for residents by faculty members; (2) monthly in-training examination (ITE) in written; and (3) periodical mocked OSCE exam. Spearman's correlation coefficient was used to assess correlation between integrative educational intervention and RAPR results. P value less than 0.05 was considered statistically significant. RESULTS: There was a statistically significant relationship between "integrative educational intervention program" and the RAPR results: Spearman's correlation coefficient = 0.655 (P value = 0.039). CONCLUSIONS: The IEI package of Anesthesiology Department, SBMU showed a significant relationship with improvements in successfulness for anesthesiology residents in the National Board Exam (RAPR trend). More prolonged studies could prevail further aspects of these interventions.

Evaluation of Regional Myocardial Function by Strain and Strain Rate before and after Surgical Repair of Congenital Heart Anomalies.

Background: Tissue Doppler imaging yields useful information about regional myocardial function. The purpose of this study was to investigate myocardial function by strain and strain rate in a group of patients with congenital heart disease (CHD) before and after cardiac surgery. Methods: Three consecutive tissue Doppler echocardiographic examinations were performed on 25 patients with CHD, who underwent open-heart surgery. The study was conducted from April 2013 to April 2014 in a university hospital, and the assessments were done 1 day before and 1 week and 1 month after surgery. The effects of demographic variables, types of anomalies, and cardiopulmonary bypass factors on strain were evaluated. Results: The study population comprised 13 female and 12 male patients at a mean age of 9.4 ± 9.8 years. Compared to the preoperative data, repeated measurements of strain in 9 segments of the ventricles showed a significant reduction 1 week after surgery, followed by a significant augmentation 1 month postoperatively (p value = 0.001 for all 9 segments). The reduction in strain at the middle segment of the left ventricular free wall was significant in the cyanotic patients (p value = 0.037). The increase in strain at the middle segment of the septum and the right ventricular basal and middle segments was significant (p value = 0.021, p value = 0.015, and p value = 0.021, respectively) in the patients with a shorter pump time. Conclusion: Our patients experienced an early decline in myocardial function after cardiac surgery, but their myocardium recovered its contractility gradually.

Treatment of femoral artery thrombosis with streptokinase and heparin after cardiac catheterization.

BACKGROUND: Thrombosis is the most common complication during cardiac catheterization via femoral artery access. Alongside heparinization, fibrinolytic therapy is recommended if there are signs of ischemia in the lower extremity. OBJECTIVES: Given the paucity of data in the existing literature on streptokinase (SK) therapy in pediatrics, we designed this study to assess the efficacy of SK in pediatric patients with diagnosed femoral artery thrombosis following cardiac catheterization. PATIENTS AND METHODS: The study population initially consisted of 1788 pediatric patients who underwent cardiac catheterization via the femoral artery access. Diminished or absent pulses in the lower extremity were detected in 123 patients, 45 of whom (2.5% of 1788) required treatment and were therefore considered for the next stage of study. Treatment was comprised of post-procedural intravenous heparin, either 50 U/kg/Q4h or 10 - 20 U/kg/h continuously. After heparinization for 24 hours, if the pulse of the affected extremity was not palpable, heparin therapy was continued (heparin-treated group, n = 28), and if the symptoms of femoral artery ischemia were persistent, heparin was discontinued and intravenous SK with a loading dose of 2000 U/kg over 20 - 30 minutes was commenced (SK-treated group, n =17). RESULTS: In the presence of pulselessness in the lower extremity, a maintenance dose of SK (1000 U/kg/h, during 1 - 24 hours) was intravenously administered. Regarding the return of the pulses post-therapeutically, normal and weak/absent pulses were detected in seven (25.2%) and 21 (74.8%) of the 28 patients, respectively, in the heparin-treated group (P value < 0.001), whereas normal and weak/absent pulses were detected in 15 (88.2%) and two (11.8%) of the 17 patients, respectively, in the SK-treated group (P value < 0.001). CONCLUSIONS: Our findings demonstrated a high success rate and a low complication rate for systemic SK therapy in femoral artery thrombosis after catheterization.

Nitroblue tetrazolium test in patients with beta-thalassemia major.

OBJECTIVE: To assess the neutrophil function in thalassemia major (TM) patients and compare it with the control group, and to recognize its relevant factors. METHODS: This was a retrospective cohort study, which was carried out from October 2007 to February 2008 in the Thalassemia Research Center in Boo Ali Sina Hospital in Sari, Mazandaran, north of Iran. The study population consisted of TM patients in Boo Ali Sina Teaching Hospital. The method of sampling in the case group was systematic, and it was target based in the control group. The sample size determined was based on previous studies. Thalassemia major was diagnosed based on hemoglobin electrophoresis (case group). The control group was their brothers and sisters, who had +/-5 years of age difference, and were of the same gender as the patients. Data collection was based on interview, investigating demographic characteristics, and also obtaining medical information from the medical records of the patients. The neutrophil function was assessed by performing nitroblue tetrazolium (NBT) reduction test. The test was carried out on both groups, and the data were analyzed by software using SPSS version 13.0. RESULTS: In this study, 39 patients and 39 healthy controls were compared. The average age of the patients was 21.6 +/- 5.3 years, and it was 22.4 +/- 5.1 years in healthy controls (p=0.7). There was a significant correlation between the tests' results, and the patients' age (p=0.008). The rate of impaired NBT results in the patients was 36%, while it was 10% in controls, which were significantly different. The neutrophil activity based on NBT test was 89.9 +/- 11.6% in the case group, and 93.7 +/- 2.51% in the control group, (p=0.025). CONCLUSION: This study indicates that neutrophil activity in thalassemic patients was significantly lower, compared to the normal control group, especially in young patients. Based on the results, evaluation of neutrophil function, and pyogenic infections in TM patients seems necessary.