Isfahan Twins Registry (ITR): An Invaluable Platform for Epidemiological and Epigenetic Studies: Design and Methodology of ITR.

Twin studies are one of the main tools for studying the interaction between genes and the environment in the development of complex diseases such as cancers, cardiovascular diseases and diabetes. The Isfahan Twin Registry (ITR) was launched in Isfahan in 2017 as a pilot study to establish a nationwide twin registry in Iran and aims to obtain comprehensive information about complex diseases and their risk factors from twins and multiples living in Isfahan. ITR will continue to recruit twins and multiples until all twins residing in Isfahan are registered in the registry. Twins are identified from welfare agencies, public health homes, maternity hospitals, Persian Twins Association and the local media. Demographic information, twin similarities, lifestyle, family history of diseases and past medical history are collected using validated questionnaires. Anthropometric measurements and blood pressure are measured by health professionals. Hematology panel, fasting blood sugar, total cholesterol, low-density lipoprotein, high-density lipoprotein, aspartate aminotransferase, alanine aminotransferase and quantitative C-reactive protein are measured by an automated analyzer. Extra samples are obtained for future studies. For twins aged under 6 years, parents complete the questionnaires for their children and a brief questionnaire for themselves. Currently, 998 persons (395 pairs and 67 multiples) are registered in the ITR and have provided their data. Results of preliminary data analysis are discussed in this article. We plan to carry out longitudinal assessments. ITR can play an important role in future epigenetic, biomarkers and omics studies using the biobank materials.

Essential hypertension in children, a growing worldwide problem.

Hypertension is one of the most common diseases worldwide. For many decades, it was considered as a problem related to adult population; however, its incidence in children has also been increased in recent years. Although secondary causes of hypertension are more common in children, few studies have been published focusing on the growing epidemic rate of essential hypertension in children and adolescents. Considering the importance of essential hypertension and its cardiovascular consequences, we review briefly its epidemiology and risk factors in children.

Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation.

Better postoperative management of patients who have undergone single ventricle (SV) Fontan procedure could potentially reduce long-term complications and improve the quality of life for patients. The present study determined the effect of tadalafil on myocardial and endothelial function and exercise performance after modified Fontan operation. Patients who had undergone SV modified Fontan operation were enrolled in this clinical trial. The demographic characteristics of the patients were recorded. Before administration of tadalafil and after the trial, ventricular function (MPI, EF, FS, E/A, VTI), exercise performance, and endothelial function were evaluated for sonographic and biochemical markers (FMD, IMT, ICAM, VCAM, NO) using echocardiography, exercise testing, vascular ultrasonography, and biochemical measurements, respectively. A single dose of tadalafil of 1 mg/kg was administered daily for 6 weeks, and the functional class of the patients before and after tadalafil was determined. A total of 15 patients completed this clinical trial. Tadalafil was shown to have a significant effect on myocardial function, exercise performance, and improvement in NYHA functional class (p < 0.05) of study population. It had no significant effect on the biochemical variables and endothelial function except for IMT (p > 0.05), which decreased significantly after tadalafil administration (p < 0.05). The findings indicate that tadalafil is a safe, well-tolerated agent for the use after modified Fontan operation to improve myocardial function and exercise performance and possibly reduce long-term morbidity and mortality of patients. More conclusive results could be obtained from further study with a larger sample size and long-term follow-up.

Effect of long-term Vitamin C intake on vascular endothelial function in diabetic children and adolescents: A pilot study.

BACKGROUND: This study attempted to determine the effects of long-term use of Vitamin C on vascular endothelial function. MATERIALS AND METHODS: During a pilot clinical trial study conducted at Imam Hussein Hospital (Isfahan) in 2014-2015, a total of forty diabetic patients were selected and then assigned randomly into two twenty-subject groups receiving Vitamin C and placebo tablets. The patients were treated with Vitamin C or placebo for 6 months. All patients were examined through echocardiography in terms of cardiac function before and after treatment. To evaluate the endothelial function (flow-mediated dilatation [FMD], intima-media thickness), they underwent arterial Doppler. Moreover, the chemical indices of vascular function were tested through intercellular adhesion molecule and vascular cell adhesion molecule (VCAM). Finally, the results were compared between the two groups. RESULTS: Based on the results, the mean left ventricular mass significantly reduced after the intervention in the group treated with Vitamin C (from 76.35 ± 25.6-68.62 ± 22.66; P = 0.015) while there was no significant difference observed in the control group (from 67.58 ± 25.38-71.63 ± 26.84; P = 0.19) but no statistically difference between the two groups-based repeated measures ANOVA test (P = 0.6). In addition, the mean of VCAM changes was significantly difference between the two groups (P < 0.001). CONCLUSION: Long-term use of Vitamin C in diabetic patients can improve certain echocardiographic parameters such as ejection fraction, fractional shortening, and FMD, which in turn enhances vascular endothelial function.

An isolated left common carotid artery from the main pulmonary artery in a neonate with aortic valve atresia.

A 20-day-old girl was referred to our clinic for systolic murmur, cyanosis, and dyspnoea with feeding. Echocardiography revealed an atretic aortic valve. CT angiography scan revealed that the left common carotid artery originated from the distal main pulmonary artery. The plan was patent ductus artriosus stenting and bilateral pulmonary artery banding and then follow-up for any possible future intervention.

Transcatheter occlusion of patent ductus arteriosus: success rate and complications 5 years experience: single center in isfahan.

BACKGROUND AND AIM: Percutaneous occlusion of patent ductus arteriosus (PDA) has become increasingly attractive with the evolution of devices and techniques. This study aimed to report the total experience of a novel arterial occlusion device (Duct Occlude pfm) and Amplatzer. METHODS: A descriptive study which selected non-randomized pediatric patients with patent ductus arteriosus (PDA) was performed between May 2007 to February 2012 on 99 children aged 8 months to 16 years who underwent attempted closure of PDA. Transcatheter occlusion was attempted in all the cases of coil through a 4-5 F and for Amplatzer 6-7 F delivery catheter. FINDINGS: Devices were successfully deployed in 98% of patients. Amplatzer was used for thirty four patients (34%) due to medium to large PDA and coil was selected for sixty five patients (66%) due to small to medium size PDA.One day after the procedure, complete occlusion was accomplished in 42 (69%) out of 61 patients in whom the detachable coil device had been used on color-flow Doppler echocardiography. Embolization of a coil occurred on 1 occasion. CONCLUSION: METHODS of detachable coil and Amplatzer type occlusion system compares favorably with other methods of transcatheter PDA occlusion.

Percutaneous balloon valvuloplasty inpatients with pulmonary valve stenosis: a single center experiment.

BACKGROUND: Pulmonary valve stenosis (PVS) is one of the most important acyanotic congenital heart diseases in children. Over the last years, balloon pulmonary valvuloplasty (BPV) has become the treatment of choice for the relief of moderate to severe valvular pulmonic stenosis in all age groups. The aim of current study was to report our regional survey on the short term outcomes of BPV among patients with PVS treated in Isfahan Hearth center. METHODS: Thirtyseven patients with moderate and severe PS based on echocardiography, aged between 0.6 and 16 years, underwent catheterization and angiography. Balloon pulmonary valvuloplasty was performed for thirty four patients. Right ventricle and pulmonary artery pressure and pressure gradient was measured before and after valvuloplasty. RESULTS: The pulmonary valvuloplasty was successful in 32 (85%) of 34 attempts. Immediately after dilation, right ventricle and pulmonary artery pressure and pressure gradient decreased from 73.0 +/- 48.0 to 38.4 +/- 23.1 mmHg, from 35.2 +/- 22.1 to 20.7 +/- 9.7 mmHg and from 79.0 +/- 32.1 to 33.4 +/- 19.9 mmHg, respectively (p < 0.001).There was no major complication. Two patients with significant subvalvar PS referred to surgeon for infundibular resection. CONCLUSION: BPV was an effective procedure to treat PVS in patients with moderate to severe stenosis. It effectively reduced right ventricle-pulmonary artery systolic pressure gradient with low complication.

The association of dietary macronutrient quality indices with depression and anxiety symptoms and quality of life in Iranian adults: The LipoKAP study.

BACKGROUND: Macronutrients' quality may impact differently on mental health and quality of life (QOL). This study aimed to investigate the potential relationship between the carbohydrate quality index (CQI), fat quality index (FQI), protein quality index (PQI), the affective mental symptoms and QOL among Iranian adults. METHODS: The LipoKAP is a cross-sectional study, conducted with 2456 adults in Iran. A validated food frequency questionnaire was used to evaluate usual dietary intakes. A validated Iranian version of the Hospital Anxiety and Depression Scale was used to assess the severity of anxiety and depression. QOL was assessed by EQ-5D. RESULT: In the fully adjusted model, participants in the highest tertile of CQI had lower QOL than those in the lowest tertile (OR = 1.35; 95 % CI: 1.06, 1.73). Individuals in the top tertile of FQI (OR = 0.71; 95 % CI: 0.55, 0.91) and PQI (OR = 0.78; 95 % CI: 0.60; 1.01) were less likely to report lower QOL than those in the bottom tertile. An inverse association was found between PQI and depressive symptoms (OR = 0.72, 95 % CI: 0.55, 0.95), but not for CQI and FQI. LIMITATIONS: The cross-sectional design of the study and the use of a memory-based dietary tool may limit the generalizability of our findings. CONCLUSION: Higher PQI was associated with lower risk of depressive symptoms and having a low-quality life. Although CQI and FQI were not related to depressive and anxiety symptoms, higher values of FQI were associated with better QOL, while CQI showed an inverse association.

Surgical repair of tetralogy of Fallot using autologous right atrial appendages: short- to mid-term results.

OBJECTIVES: The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS: The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS: The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS: The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

Factor analysis of markers of inflammation and oxidation and echocardiographic findings in children with a positive family history of premature coronary heart disease.

The objective of this study was to determine the predictive factor of a combination of traditional and new risk factors for coronary heart diseases (CHDs) as well as the echocardiographic findings in children of parents suffering premature myocardial infarction in comparison with controls. Overall, 239 adolescents aged 12 to 18 years including 112 children of parents with premature CHD (<55 years) and 127 age- and sex-matched controls without such a family history. In addition to measuring anthropometric indexes and blood pressure, serum fasting blood sugar, lipid profile, C-reactive protein, Lpa, and oxidized LDL as well as carotid-intima media thickness and left ventricular mass were determined. Factor analysis showed that clusters of inflammatory factors and markers of oxidation as well as carotid-intima media thickness and left ventricular mass were correlated with each other and were associated with a positive parental history of premature CHD in youths. These findings complement the functional and structural changes in arteries of adults with a familial predisposition to CHD and underscore the importance of using a high-risk approach for primordial/primary prevention of CHD from early life and of considering the screening of children and siblings in the management of patients with premature CHD.

CASCADE screening and registry of familial hypercholesterolemia in Iran: Rationale and design.

BACKGROUND: Familial hypercholesterolemia (FH) is one of the most common genetic disorders, which leads to premature coronary artery disease (CAD). It has been suggested that heterozygous FH affects around 1:250 to 1:500 in the general population or even more than this, and homozygous FH affects 1:1000000 of the population. If patients with FH are not diagnosed and treated early in life, many of them will develop premature CAD event. As most of the patients with FH are undiagnosed, it is recommended that the general population be screened for high risks of the events since early treatments can reduce the risk of premature CADs. The clinical diagnostic criteria for FH consist of increased plasma low-density lipoprotein cholesterol (LDL-C), clinical features and family history of CAD. However, deoxyribonucleic acid (DNA)-based detection of FH mutation has high diagnostic values. As there was no screening for FH in Iran up until now, we have started screening and registering patients with FH using the CASCADE method. METHODS: We detected FH subjects in the general population by screening laboratories according to their high LDL-C levels (more than 190 mg/dl or 150 mg/dl if receiving treatments), while our second approach was hospital-based in which one screens hospitalized patients with premature CAD events. RESULTS: We intended to screen families of indexed patients to provide standard care and therapy in order to optimize their LDL-C. CONCLUSION: This article provides detailed information on the rationale and design of this screening and registry in Iran.

Dental Caries and Gingival Evaluation in Children with Congenital Heart Disease.

BACKGROUND: Dental health is one of the most important health burdens of children health. The association between dental health and endocarditis has been already demonstrated, but there is controversy about different frequency of dental caries, periodontitis, and saliva microorganism in comparison to healthy population and children with congenital heart diseases (CHDs). In this study, we evaluated these differences. METHODS: Seventy-six healthy children and 68 CHD patients were enrolled in the present case-control study. Dental decay, periodontitis, oral microorganisms, serum calcium, phosphorus, and frequency of carbohydrate and protein consumption of all participants were evaluated by standards method. RESULTS: CHD patients experienced more periodontitis, but the difference was not significant (0.12 vs. 0.09, P = 0.2). In healthy children, the mean saliva colony counts of Streptococcus mutans were more significant (50639 ± 3324 vs. 35285 ± 27226, P = 0.03), which was diminished by adjusting the carbohydrate consumption. The mean colony count of Lactobacilli in children with CHD was nonsignificant higher than healthy children (P = 0.3). CONCLUSIONS: Pediatric patients with CHD experience insignificantly higher dental decay, periodontitis, and saliva Lactobacilli colony counts. The frequency of decayed tooth and gingival diseases in healthy children is high, and hence, more dental care attention in our health system is needed for healthy children.

Comparison of cost-effectiveness and postoperative outcome of device closure and open surgery closure techniques for treatment of patent ductus arteriosus.

BACKGROUND: Various devices have been recently employed for percutaneous closure of the patent ductus arteriosus (PDA). Although the high effectiveness of device closure techniques has been clearly determined, a few studies have focused on the cost-effectiveness and also postoperative complications of these procedures in comparison with open surgery. The present study aimed to evaluate the clinical outcome and cost-effectiveness of PDA occlusion by Amplatzer and coil device in comparisong with open surgery. METHODS: In this cross-sectional study, a randomized sample of 201 patients aged 1 month to 16 years (105 patients with device closure and 96 patients with surgical closure) was selected. The ratio of total pulmonary blood flow to total systemic blood flow, the Qp/Qs ratio, was measured using a pulmonary artery catheter. The cost analysis included direct medical care costs associated with device implantation and open surgery, as well as professional fees. All costs were calculated in Iranian Rials and then converted to US dollars. RESULTS: There was no statistical difference in mean Qp/Qs ratio before the procedure between the device closure group and the open surgery group (2.1 ± 0.7 versus 1.7 ± 0.6, P = 0.090). The mean measured costs were overall higher in the device closure group than in open closure group (948.87 ± 548.76 US$ versus 743.70 ± 696.91 US$, P < 0.001). This difference remained significant after adjustment for age and gender (Standardized Beta = 0.160, P = 0.031). PDA closure with the Amplatzer ductal occluder (1053.05 ± 525.73 US$) or with Nit-Occlud coils (PFM) (912.73 ± 565.94 US$, P < 0.001) was more expensive than that via open surgery. However, the Cook detachable spring coils device closure (605.65 ± 194.62 US$, P = 0.650) had a non-significant cost difference with open surgery. No event was observed in the device closure group regarding in-hospital mortality or morbidity; however, in another group, 2 in-hospital deaths occurred, two patients experienced pneumonia and seizure, and one suffered electrolyte abnormalities including hyponatremia and hypocalcemia. CONCLUSION: Although open surgery seems to be less expensive than device closure technique, because of lower mortality and morbidity, the latter group is more preferable.

Possible correlation among echocardiographic measures, serum brain natriuretic peptide, and angiotensin II levels in hypertensive kidney transplanted children.

OBJECTIVES: Ambulatory blood pressure monitoring is the standard for determining patients at risk of hypertension. Left ventricular hypertrophy is common in kidney transplant recipients. We evaluated the correlation between blood pressure measures achieved by ambulatory blood pressure monitoring and conventional (office) methods. MATERIALS AND METHODS: This cross-sectional study was done from December 2009 to October 2010 at Alzahra Hospital in Isfahan, Iran. Sixty five participants, 35 kidney transplant recipients under 20 years old, and 30 control subjects of the same age were recruited. Five kidney recipients did not complete the study and were excluded. Blood pressure was measured by ambulatory blood pressure monitoring and conventional methods. Echocardiographic study was done for kidney transplant recipients. Serum brain natriuretic peptide and angiotensin II levels were determined in case and control groups. RESULTS: Office-recorded systolic and/or diastolic hypertension was observed in 43.4% and 55.3% of patients. According to ambulatory blood pressure monitoring, 86% of kidney transplant recipients had systolic BP load. Left ventricular hypertrophy (defined according to the left ventricular mass index [left ventricular mass index/height]) was seen in 53.3% of the patients. The existence of left ventricular hypertrophy revealed a positive correlation with ambulatory blood pressure monitoring systolic and diastolic night blood pressure and systolic nondipper. Left ventricular mass index showed a positive correlation with brain natriuretic peptide level. Furthermore, the existence of left ventricular hypertrophy was positively correlated with angiotensin II level. CONCLUSIONS: Only ambulatory blood pressure monitoring systolic and diastolic blood pressures (nondippers) were positively correlated with left ventricular hypertrophy and higher left ventricular mass index. Serum levels of brain natriuretic peptide and angiotensin II had a positive relation with left ventricular hypertrophy. Measuring brain natriuretic peptide and angiotensin II in the clinical setting screens patients at risk of left ventricular hypertrophy.