Journal of

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Introduction: Along with advances occurring in cardiopulmonary bypass (CPB) including improved anesthesia intubation and early surgical interventions, it has become possible to correct life-threatening congenital heart anatomic abnormalities in patients. Our study aimed to collect information from one of the most important centers of pediatric cardiac surgery in Iran since there is limited information in this field in our country.

Methods: In this study, the results of  pediatric cardiac surgery and mortality were studied for two years. A retrospective, descriptive study was carried out on 789 patients   within two years. 401cases were excluded as they had congenital heart disease complexity. The patients were referred from other pediatric cardiac medical centers. Information related to kinds of surgical operation and deaths were collected from patients' documents and questionnaire. The data were analyzed by descriptive statistical tests in SPSS version-16.

Results: The age range of patients varied from three days to 18 years. The male/female ratio was 53.4%. CPB was not applied for 21.1% of patients. The frequency of operations included PDA (Patent Ductus Arteriosous) [D&L] (%8.5), total correction for TF (Tetralogy of Fallot) (%16.7), systemic to pulmonary shunt (%26.5), ASD (Atrial Septal Defect) and VSD (Ventricular Septal Defect) closure (%5.4) and (%28), respectively as well as coarctationrepaire (%14.6). Total mortality rate was 8.6%. Analysis of the operated population showed that outcome of patients has improved in recent years.

Conclusions: Compared with other studies, the results of our investigation showed that children's congenital heart surgery is on the rise.

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Introduction: Congenital cardiac abnormalities are among the problems that affect the quality of life of children and parents, especially the mothers. Meanwhile, mothers are severely stressed and harmed due to their emotions and feelings, so that the suffering can reduce their quality of life. This study aimed to compare the quality of life of mothers of healthy children and mothers of children with congenital heart disease after cardiac surgery.
Methods: This is a cross-sectional descriptive-analytic study where 100 mothers of healthy children and 60 mothers of children with congenital heart disease referring to Vali-e-Asr Hospital in 2016 and 2017 were incorporated. The mothers' quality of life was assessed using the World Health Organization Quality of Life Scale (WHOQOL-BREF). The collected data was analyzed in SPSS software (version 22) using independent t-test, Mann-Whitney, Kruskal-Wallis and Chi-square. The significance level for all tests was considered less than 0.05.
Results: According to the findings, the mean age of mothers with healthy children was 31.91 ± 8.57 years and that of mothers of children with congenital heart disease was 32.48 ± 6.46 years. The mean score of the quality of life of mothers with healthy children was 71.25 ± 16.43 and that of the second group was 66.51 ± 15.46. The mean scores of social relationships, mental health, and physical health in mothers with healthy children were significantly higher than those of mothers after their child's surgery. However, there was no significant difference between the two groups as for the mean score of overall quality of life and environmental health.
Conclusions: According to the results, it was found that the quality of life of the mothers with unhealthy children is lower than that of mothers with healthy children. Therefore, proper training and timely counseling are recommended as potential measures to somewhat improve their quality of life.

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Introduction: Congenital heart abnormalities are the most common type of congenital conditions in children. Annually,about 1 to 2 percent of children are born with these diseases. As chronic diseases, congenital conditions of children usuallycause limitations in the quality of their lives, especially if they are in need of frequent and complex surgical procedures. Regarding the role of parents, especially mothers, in the care of these children, their quality of life is of particular importance. This study, therefore,aimed to evaluate and compare the life quality of mothers with healthy children and mothers with children with a congenital heart disease in need of surgery.
Methods: This research is a cross-sectional descriptive-analytical study, in which 100 mothers of healthy childrenand 60 mothers ofchildren with severe congenital heart disease – who were referred to Birjand Valiasr Hospital in 2017 –were entered into the study. The life quality of the mothers was assessed using the Life Quality Questionnaire of World Health Organization (WHOQOL-BREF). Data were analyzed by SPSS software (version 22). P-values less than 0.05 were considered significant.
Results: According to the findings of this study, the age means of the mothers with healthy children and that of mothers of children with congenital heart diseasewere 31.91±8.57 and 32.48±6.46 years, respectively. The mean score of life quality of motherswith healthy children was 71.25±16.43; it was 41.25±19.27 for mothers ofchildren with congenital heart diseases in need of surgery. Life quality and all its sub-scales (social relationships, mental health, environmental health and physical health) were significantly higher in mothers with healthy children than in mothers ofchildren with severe congenital heart diseases in need of surgery before operation(P <0.001).
Conclusions: It was found that the life quality of mothers with children with severe congenital heart disease was significantly different from that ofmothers with healthy children. Therefore, in order to improve their quality of life, appropriate planning in different areas should be performed.

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• Introduction: Congenital heart disease (CHD) occurs in about 8 cases per 1,000 live births and is responsible for 30% of all neonatal deaths. The reportedly high prevalence of CHD underscores the necessity of the implementation of local evaluations and screening programs in order to plan for appropriate interventions. Regarding this, the present study was performed to determine the prevalence of CHD and its associated risk factors among the newborns with respiratory distress and cyanosis admitted to Vali-e-Asr Hospital in Birjand, Iran, in 2016.
• Methods: This cross-sectional analytical study was conducted on the records of 199 neonates admitted to the Neonatal Unit of Vali-e-Asr Hospital due to respiratory distress and cyanosis in 2016. The data were collected using a predesigned form covering such information as gender, type of delivery, and gestational diabetes. Data analysis was performed in SPSS software (version 22) using the Chi-squared test. P-value less than 5% was considered statistically significant.
• Results: Out of the 199 neonates enrolled in this study, 168 (84.4%) cases suffered from CHD. Patent ductus arteriosus (PDA) was the most common anomaly among the neonates (n=85, 50.5%), followed by atrial septal defect (n=41, 24.4%), ventricular septal defect (n=36, 21.4%), transposition of great arteries (n=4, 2.2%), and other complex heart anomalies (e.g., shone complex; n=2, 1.1%), respectively. The results revealed that the presence of CHD, especially PDA, in neonates was associated with their parental interfamily marriage (P=0.024).
• Conclusions: The high prevalence and mortality of CHD necessitate the control of premarital and preconception potential risk factors (e.g., inter-family marriage) and preparation for the implementation of effective interventions for neonates with respiratory distress and cyanosis.

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• Lipomas are the most common benign tumors of connective tissue which can be found in different parts of the human body. In rare cases, their size may be so giant which could be an indication for surgery. Here, we present a case of congenital multiple asymmetrical giant lipomas of the back in a young female. She presented with a chief complaint of physical discomfort due to the giant size and weight of the lipomas. Apart from that, she was in total health. She was born with 5 small lipomas in the back and flank region which had become giant lipomas with estimated weigh around 16 kg in total by the age of 24. The patient underwent seven operations to remove or debulk the mass over a 24-year period since the recurrence of the growths was inevitable.
• Considering the size of the mass and differential diagnosis, such as liposarcoma, and since no risk factor or underlying etiology was detected for the patient`s condition, surgical excision was the preferred technique for this case.

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• Introduction: One way of monitoring angiographic organ is the clinical examination that is dependent on the accuracy of the person performing it; Therefore, human error may be high and require frequent examination is at least 24 hours after angiography. Meanwhile, checking for arterial or intravenous blood gases due to the small size of the vein and the arteries in children is difficult, unlike adults is difficult and requires skill and mastery in the field of venipuncture. Therefore, it is advisable to use simpler and less costly methods such as pulse oximeters to monitor organs that are more accurate and comfortable for the staff and patient. Therefore, this study was conducted to compare post angiography pulse oximetry and physical exam in cardiac congenital of pediatric patients.
• Methods: This study was conducted with 45 patients with congenital heart disease undergoing diagnostic or therapeutic angiography. They were selected by convenience sampling method from among patients referring to the angiography department of Vali-e-Asr Hospital in Birjand, Iran, in 2016. Trained personnel performed the clinical examinations, including temperature, colour, and pulse check (dorsalis pedis and tibialis posterior) for both the angiographic and control organs. Pulse oximetry was also performed concurrently using the Massimo pulse oximeter for both organs and continued for up to 6 hours after angiography. Demographics form and patients' clinical records were used to collect data. The obtained data were analyzed in SPSS software (V: 23) using the Kappa test.
• Results: The agreement between the pulse oximetry of the angiographic and control organs was mild only at one, two, and six hours after angiography, and was moderate three hours after angiography (kappa=0.656 after three hours).
• Conclusions: Although there was no clinical agreement between pulse oximetry and clinical examination, according to our findings pulse oximetry is more accurate, and given the results in the first one to two hours after angiography evaluation of organ is more sensitivity.

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• Introduction: Lacrimal drainage system disorders are among the most common ocular disorders faced by physicians. The prevalence of congenital nasolacrimal duct obstruction (CNLDO) is different in various studies, and the role of such factors as maternal and neonatal demographic characteristics have not been completely investigated in CNLDO. The present study aimed to assess the prevalence of CNLDO and its related factors in newborns in Sabzevar.
• Methods: This cross-sectional study was conducted on 541 newborns in Shahidan Mobini hospital, Sabzevar, Iran from January to February 2015 after obtaining informed consent from their parents. Data were collected by interviewing mothers, filling out checklists, and physical examination of the newborns. Data analysis was carried out in Stata software using a logistic regression test at a 95% confidence interval.
• Results: The prevalence of CNLDO was 22.6%. Purulent ocular discharge was the most common symptom (16.82%). There was a significant relationship between the neonate’s head circumference and the incidence of CNLDO (P= 0.01); nonetheless, CNLDO in the newborns showed no significant association with weight, height, and Apgar score (P>0.05).
• Conclusion: Considering the significant prevalence of CNLDO in preterm and term neonates, as well as its variable prevalence rates reported in different studies and the absence of overlapping of various risk factors of such disorder, it is recommended to carry out more thorough studies from birth to 1 year of age.

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• Introduction: Heart valve disease in pediatricsis an increasing global concern, especially in developing countries. This study aims to determine the clinical outcomes of pulmonary valve replacement (PVR) surgery in pediatrics.
• Methods: The authors retrospectively identified all pediatrics undergoing PVR surgery at Imam Reza hospital (Mashhad, Iran) between 2000 and 2020. Their medical records were reviewed for demographic characteristics, clinical data (intra-operative and postoperative), and follow-up results. Echocardiography and electrocardiography were performedonall patients before the surgery and periodically after the surgery.
• Results: Among 50 pediatrics undergoing surgical PVR, 38 (76%) were female and others were male (14%), with a mean age of 10.39±5.31 years. The leading cause of PVR was the Tetralogy of Fallot. There was a significant relationship between age at the time of surgery and the size of the pulmonary valve(P=0.02).There were also statistically significant differences between QTc intervals before and after surgery (P=0.001). Further more, there was a significant correlation between QTc intervals before and after surgery and the ageof pediatrics at the time of surgery (P=0.01, r=-0.6).There was also a statistically significant relationship between the ICU stay (P=0.01) and the weaning time of the mechanical ventilation (P=0.03).
• Conclusion: It is recommended that this procedure ispostponed as much as possible to decrease postoperative life-threatening events in pediatrics.In addition, if this surgery is conducted with good surgical and nursing management, it can be safe with low complications among other cardiac valve procedures.

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• The Tetralogy of Fallot is the most common cyanotic congenital heart disease that is often associated with chromosomal aberration had a high prevalence rate of all congenital heart diseases. The association of the Tetralogy of Fallot (ToF) with other congenital cardiac defects, especially with hypertrophic cardiomyopathy, is infrequent. While, unlike the tetralogy of Fallot, hypertrophic cardiomyopathy is accompanied by specific gene loci.
• We report this scarce association in a 6-month male infant, cyanotic since birth and known with ToF, in her first hospital admission for treatment of recurring cyanotic crises. After the preliminary evaluations and the unsuccessful stenting of the patent ductus arteriousus (PDA) under an angiographic manner, he was prepared to undergo cardiac surgery to establish a modified Blalock-Taussig Shunt procedure. The patient was discharged in good condition and suggested that his parents follow up on his cardiac function after it. On follow-up at the age of three, the child had no cyanotic crises in this period.

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• Introduction: Congenital heart defects (CHDs) are diagnosed in ≈8 to 10 per 1,000 live births in the United States. This study aimed to determine the outcomes in children with congenital heart disease.
• Methods: This cross-sectional descriptive-analytical study was conducted using the census method on all patients with a CHD who referred to the pediatric heart clinic in Valiasr Hospital, Birjand, Iran, and underwent heart operation during the desired period. The information contained in the patients' files was used to collect the required data. The rest of the information was gathered by calling the patients' parents. The collected data were analyzed in the SPSS-19 software using the Chi-square test and Fisher's exact test. The significant level was considered at the p-value of ≤ 0.05.
• Results: The results of the statistical analysis indicated that the type of intervention (i.e., operation, intrusion) had no statistically significant relationship with demographic indicators of gender, age by month, chromosomal abnormalities, the type of heart disease, and the development of children under-study; however, it showed a statistically significant relationship with the type of initial complaint. The rate of operation was significantly higher in the subjects, especially in those who had an initial complaint of murmurs. Based on the results, the age of children had a statistically significant relationship with the need for pacemakers and the amount of pleural effusion; these two outcomes increased significantly with the child's aging.
• Conclusion: A large number of demographic and clinical factors were effective in children's postoperative outcomes. Consequently, by conducting further studies at a wider level and controlling variables it is possible to compare the findings, achieve more favorable results, and improve clinical indicators.