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1.  Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy 
Annals of Pediatric Cardiology  2014;7(3):217-220.
Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited.
PMCID: PMC4189242  PMID: 25298700
Goldenhar syndrome; right circumflex aortic arch-embryology; vascular ring-twin-coarctation
2.  Management of Hypertension in Children with Cardiovascular Disease and Heart Failure 
Although primary chronic hypertension (HTN) is increasingly common in adolescence, secondary forms of HTN are more common among children. Primary HTN is associated with being overweight and/or a positive family history of HTN. Carotid intima-media thickness, a known risk factor for atherosclerosis is frequent in both adults and children with HTN and other associated cardiovascular (CV) risk factors including obesity, dyslipidemia, diabetes and chronic kidney disease. Left ventricular (LV) hypertrophy is also a common finding in children and adolescents with newly diagnosed HTN. Children with certain medical conditions such as congenital heart disease and Kawasaki disease can develop premature atherosclerosis heart disease that may lead to coronary heart disease and heart failure. Life-style interventions are recommended for all children with HTN, with pharmacologic therapy added for symptomatic children based on the presence of co-morbidities. As an example, beta-blockers, angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blocker and/or calcium channel blockers would be best for children with CV risk factors such as diabetes or renal disease, whereas an ACE inhibitor in combination with a beta-blocker and diuretics including spironolactone are recommended for patients with heart failure and reduced LV ejection fraction. This report will summarize new developments in the management of pediatric HTN complicated with CV disease and heart failure and will address the appropriate antihypertensive therapy that could potentially reduce the future burden of adult CV disease.
PMCID: PMC3990921  PMID: 24791185
Atherosclerosis; cardiovascular disease; heart failure; hypertension
3.  A Novel Method for Quantification of Left Ventricular Noncompaction Using Two-Dimensional Echocardiography in Children 
Iranian Journal of Pediatrics  2013;23(5):519-524.
Although there are several echocardiographic criteria, there is not yet a general consensus about the diagnosis of left ventricular noncompaction. The current criteria are mostly based on the areas with maximal noncompaction in the heart. The echocardiographer may miss this maximal point leading to a misdiagnosis. Accordingly, we suggested a new method to measure the percentage of myocardial noncompaction using two-dimensional echocardiography.
In this study, the new method was examined on 4 noncompaction and 26 dilated cardiomyopathies, and 25 normal subjects. The percentage of noncompaction was measured at 3 levels (apical, papillary muscle and mitral valve) and averaged.
The mean percentages of myocardial noncompaction were 3.59±2.27, 8.86±5.52 and 34.7±26.1 in the control, dilated cardiomyopathy and noncompaction groups, respectively. A value of 17% or greater could distinguish left ventricular noncompaction from dilated cardiomyopathy with 92% specificity and 100% sensitivity and from normal subjects with 100% specificity and sensitivity. This percentage had a statistically significant association with noncompacted to compacted myocardial thickness ratio (P<0.001).
This method showed good correlations with the existing echocardiographic and magnetic resonance criteria. However, it is not dependent on finding the area of maximal involvement. Being comparable to magnetic resonance imaging in accuracy, it is easier to perform and more available.
PMCID: PMC4006499  PMID: 24800010
Left Ventricular Noncompaction; Cardiomyopathy; Echocardiography; Children
5.  The validity of Iran’s national university entrance examination (Konkoor) for predicting medical students’ academic performance 
BMC Medical Education  2012;12:60.
In Iran, admission to medical school is based solely on the results of the highly competitive, nationwide Konkoor examination. This paper examines the predictive validity of Konkoor scores, alone and in combination with high school grade point averages (hsGPAs), for the academic performance of public medical school students in Iran.
This study followed the cohort of 2003 matriculants at public medical schools in Iran from entrance through internship. The predictor variables were Konkoor total and subsection scores and hsGPAs. The outcome variables were (1) Comprehensive Basic Sciences Exam (CBSE) scores; (2) Comprehensive Pre-Internship Exam (CPIE) scores; and (3) medical school grade point averages (msGPAs) for the courses taken before internship. Pearson correlation and regression analyses were used to assess the relationships between the selection criteria and academic performance.
There were 2126 matriculants (1374 women and 752 men) in 2003. Among the outcome variables, the CBSE had the strongest association with the Konkoor total score (r = 0.473), followed by msGPA (r = 0.339) and the CPIE (r = 0.326). While adding hsGPAs to the Konkoor total score almost doubled the power to predict msGPAs (R2 = 0.225), it did not have a substantial effect on CBSE or CPIE prediction.
The Konkoor alone, and even in combination with hsGPA, is a relatively poor predictor of medical students’ academic performance, and its predictive validity declines over the academic years of medical school. Care should be taken to develop comprehensive admissions criteria, covering both cognitive and non-cognitive factors, to identify the best applicants to become "good doctors" in the future. The findings of this study can be helpful for policy makers in the medical education field.
PMCID: PMC3461424  PMID: 22840211

Results 1-5 (5)