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Herein we present a case of a neonate with congenital left ventricular diverticulum (LVD), a rare anomaly, with an unusual course and unexpected findings. The neonate was born at 35 weeks in Namazi Hospital (Shiraz, Iran) and presented with a pulsatile umbilical mass immediately after birth. Based on multiple imaging modalities, the presence of a connection between the left ventricular apex and the umbilicus was confirmed. Percutaneous closure of LVD was unsuccessful. The patient's clinical course deteriorated after developing sepsis and multiorgan failure. The patient passed away before any corrective surgery could be performed. Unexpected findings in post-mortem evaluation were severe hepatic macrovesicular steatosis (suggestive of metabolic liver disease) and regulatory factor X6 (RFX6) heterozygous missense mutation in whole-exome sequencing. [ABSTRACT FROM AUTHOR]

Background: Kawasaki disease (KD) is the most frequent cause of coronary artery aneurysm (CAA) in children. This study tried to evaluate the accuracy of different KD scores developed for prediction of CAA, in an Iranian population. Methods: This is a cross-sectional retrospective investigation on pediatric patients with a diagnosis of KD. Clinical manifestations, laboratory, and echocardiographic data were recorded. Five Kawasaki scores, including Kobayashi, Egami, Sano, Nakano, and Harada, were assessed and analyzed in relation to CAA and intravenous immunoglobulin (IVIG) resistance. Results: During five years, we recruited 121 cases of KD under 13 years of age. The rates of CAA and IVIG resistance were 16.5%, and 13.2% respectively. The IVIG resistance group was significantly younger than responder patients. All five scores had low sensitivity in predicting CAA or IVIG resistant cases; the highest sensitivity pertained to the Harada score with 50% sensitivity and 59% specificity (the area under the curve: 0.545, with a 95% confidence interval: 0.423 to 0.667) in predicting CAA, which is lower than the usual acceptable criteria for a screening test. The specificity of all other scores were more than 85% in predicting CAA or IVIG resistance. Gender, fever before therapy and laboratory data showed no significant difference between the groups. Conclusion: The Kobayashi, Egami, Sano, Nakano and Harada scores have limited usefulness in the Iranian population to predict high risk patients for coronary artery involvement or IVIG resistance; in our study, age under one year was a risk factor for IVIG resistance. [ABSTRACT FROM AUTHOR]

Background: The effect of balloon angioplasty in treatment of coarctation of the aorta (COA) in pediatric patients is very important. Objectives: This study aimed to assess the efficacy and safety of balloon angioplasty for coarctation of the aorta (CoA) and its effects on heart function in children above 3 months of age. Patients and Methods: In this retrospective study, we reviewed the immediate outcomes of 100 consecutive pediatric patients above 3 months of age with native CoA who were treated by balloon angioplasty at a tertiary pediatric heart center from June 2002 to August 2012. The patients were followed by echocardiography. Statistical analyses were performed by descriptive statistics using the SPSS statistical software, version 20 and the significance level was set at 0.05. Results: The patients' ages ranged from 4 months to 15 years, with the mean of 51.56 ± 42.22 months. Additionally, their body weight ranged from 4 to 63 kg (mean: 15.44 ± 10.62 kg) at the time of CoA repair. Technical success of balloon dilation was achieved in 91/95 patients (95.7%). Besides, systolic gradient significantly reduced from 48.29 ± 21.62 mmHg (range 7 - 82 mmHg) to 13.21 ± 9.96 mmHg (range 0 - 34 mmHg) (P < 0.0001). The mean follow-up period was 2.21 ± 0.94 years. Recoarctation and cardiac dysfunction occurred only in 4 patients. Z-scores of the Left Ventricular (LV) end diastolic and systolic dimensions were more than 2 standard deviations in 15 (17.1%) and 22 (25%) patients, respectively. In addition, there were 16 children (18.8%) with abnormal E/A ratios and 6 ones (6.8%) with E/Ea > 15. Conclusions: Percutaneous balloon angioplasty was a safe and effective treatment option for native CoA in the children above 3 months old. However, impairment of LV diastolic function should be mentioned as an important issue in long-term follow-up. [ABSTRACT FROM AUTHOR]

Due to the significant differences between the physiology and pathology of adults and neonates, clinical guidelines for adults are not directly applicable to children. This study was performed to evaluate the effects of high- and low- dose captopril on the neonates with large left-to-right shunts. Methods: The study was conducted on 20 neonates with congenital heart disease, left-to-right shunt, and cardiac failure. Based on the Ross scoring system, the neonates were clinically evaluated by measuring renin, aldosterone, and B-type natriuretic peptide, and performing echocardiography. For each neonate, the treatment of heart failure started with digoxin and frusemide, and reevaluation was conducted 3 days after the treatment. Afterwards, the neonates were randomly divided into 2 groups; low- (0.03mg/kg) and high-doses (0.5mg/kg) of captopril were administered, and the reevaluation was carried out, after one week of therapy. Results: The study revealed higher reduction of the Ross score in the high-dose group; however, the change was not statistically significant (P=0.56). B-type natriuretic peptide and aldosterone reduced further in the high-dose group; again the changes were not statistically significant (P=0.4). Moreover, the treatment with captopril increased the pulmonary blood flow (QP), and pulmonary-to-systemic blood flow (QP/QS) in both groups; though the changes were not significant. Conclusion: According to the present study, although high-dose captopril can decrease B-type natriuretic peptide and the neonates' clinical symptoms, the resultant changes are not statistically significant. Therefore, clinical decision making should follow a case-by-case basis for each neonate, in order to select the effective dose of captopril. [ABSTRACT FROM AUTHOR]