The present study was performed to evaluate the clinical symptoms and cardiovascular complications in patients with typical and atypical Kawasaki disease (KD). This retrospective study was conducted on the medical records of 176 patients with KD for three years. The study population was divided into two groups of typical and atypical based on the KD clinical criteria. The two groups were compared in terms of demographic data, clinical symptoms, cardiac lesions, and laboratory markers. Based on the diagnostic criteria, 105 (60%) and 71 (40%) patients were diagnosed with typical and atypical KD, respectively. The mean age of the typical patients (38.16 months) was higher than that of the atypical group (24.03 months) at the time of diagnosis (p < 0.05). The results revealed no significant difference between the two groups regarding the seasonal distribution of KD onset (p = 0.422). However, the most common season for the diagnosis of the disease was spring, followed by winter. There was no significant difference between the two groups in terms of fever duration (p = 0.39). Furthermore, vomiting was more common in the atypical patients than in the typical group (p = 0.017). In terms of the cardiac lesions, ectasia (p = 0.005) and lack of tapering of the distal coronary vessels (p = 0.015) were more frequently detected in the atypical group than in the typical group. Considering the laboratory findings, thrombocytosis (p = 0.010) and anemia (p = 0.048) were more common in the atypical group, compared to those in the typical group. On the other hand, the typical group had a higher serum alanine aminotransferase level (adjusted for age) (p = 0.012) and Hyponatremia (serum sodium concentration ≤130 mmol/L) (p = 0.034). Based on the findings of the current study, the fever duration from onset to diagnosis was slightly more in atypical KD patients than in the typical group, but not statistically significant, possibly due to more timely diagnosis of atypical KD. There was no difference in coronary aneurysm between the two groups at the time of diagnosis. The atypical group had a higher frequency of coronary ectasia and lack of tapering, indicating cardiac involvement. Consequently, these conditions should be given more attention in the atypical patients. Furthermore, the higher frequency of anemia and thrombocytosis in the atypical patients can be useful for diagnosis of this kind of KD.

Nowadays transcatheter device closure of ventricular septal defects (VSDs) is an attractive and feasible alternative to surgical closure of congenital VSDs. Isolated congenital VSDs constitute the most common form of congenital heart disease (CHD) in infants and children and account for 20-30% of all types of cardiac malformations. Most of the VSDs are located in the membranous portion of the ventricular septum (perimembranous VSDs). There are also less common types of VSDs located in the muscular portion (muscular VSDs), below the pulmonary valve (subpulmonary or supracristal VSDs), and near the junction of the tricuspid and mitral valves (inlet type VSDs). Indications for closure of VSDs include a hemodynamically significant left to right shunt and prevention of long-term complications, including pulmonary hypertension, progressive ventricular dilatation, aortic insufficiency, double-chambered right ventricle, and endocarditis. In this chapter, we review the technical details for achieving a successful procedure, as well as some tips and tricks on using off-label devices during transcatheter approach in VSD closure.

IntroductionHarvesting of the greater saphenous vein is almost an inevitable part of coronary artery bypass grafting (CABG) operations, and it is done by two main techniques, i.e., conventional or open vein harvesting (OVH) and the minimally-invasive endoscopic vein harvesting (EVH). This study aimed to compare these two techniques in off-pump CABG procedures with respect to clinical and pathological outcomes.MethodsThis cohort study was conducted on CABG candidates during a one-year period from October 2013 through September 2014 in the Department of Cardiac Surgery at Mashhad University of Medical Sciences. Eighty-seven patients voluntarily underwent EVH, and another 86 patients matched for age, gender, and other cardiovascular risk factors were selected for OVH. They were followed up for six weeks, and the main outcome measures were infections of the wound, pain, duration of hospital stay, and the costs of hospitalization. Paired sample t-test, independent t-test, or their non-parametric equivalents and the chi-squared test were used by SPSS version 17.0 for data analysis.ResultsThe mean duration of time for vein harvesting was shorter in the EVH group (p < 0.001), and the pain score was lower (p = 0.04). No infections occurred at the site of the wound. The length of hospital stay was not significantly different for the two groups (OVH versus EVH: 8.5 ± 3.3 versus 8.4 ± 3.2 days; p-value: 0.08). Hospitalization costs were significantly higher in the EVH group (OVH versus EVH: 5.8 ± 4.7 versus 7.3 ± 2.0 million Tomman; p-value: 0.008), yet no difference was diagnosed with respect to endothelial damage in the vein grafts harvested by the EVH and OVH techniques.ConclusionEVH is considered as a minimally invasive and safe vein harvesting technique in our Center, and it can reduce the harvesting time and post-operative pain. In addition, its efficiency was similar to that of OVH.

Inherited cardiomyopathies are a prevalent cause of heart failure and sudden cardiac death. Both hypertrophic (HCM) and dilated cardiomyopathy (DCM) are genetically heterogeneous and typically present with an autosomal dominant mode of transmission. Whole exome sequencing and autozygosity mapping was carried out in eight un-related probands from consanguineous Middle Eastern families presenting with HCM/DCM followed by bioinformatic and co-segregation analysis to predict the potential pathogenicity of candidate variants. We identified homozygous missense variants in TNNI3K, DSP, and RBCK1 linked with a dilated phenotype, in NRAP linked with a mixed phenotype of dilated/hypertrophic, and in KLHL24 linked with a mixed phenotype of dilated/hypertrophic and non-compaction features. Co-segregation analysis in family members confirmed autosomal recessive inheritance presenting in early childhood/early adulthood. Our findings add to the mutational spectrum of recessive cardiomyopathies, supporting inclusion of KLHL24, NRAP and RBCK1 as disease-causing genes. We also provide evidence for novel (recessive) modes of inheritance of a well-established gene TNNI3K and expand our knowledge of the clinical heterogeneity of cardiomyopathies. A greater understanding of the genetic causes of recessive cardiomyopathies has major implications for diagnosis and screening, particularly in underrepresented populations, such as those of the Middle East.

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Can intracoronary stem cell injection permanently improve cardiac function after myocardial infarction?'. Altogether 314 papers were found using the reported search, of which five represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. We conclude that stem cells may have controversial effects on cardiac function in long-term follow-up of more than two years as they improved the left ventricular ejection fraction and end systolic volume index just in two studies in which none of them utilized cardiac magnetic resonance imaging (MRI), as the most reliable method, to quantify cardiac function. However, all remaining three trials which reported negative results used cardiac MRI for assessment of cardiac indexes which may be more precise and accurate than echocardiographic assessments. So the reliability of the positive trials is lower than negative resulted trials in terms of cardiac function assessment method. Stem cell therapy almost always offered short-term benefits over the best medical treatment, but the long-term benefits are still a matter of debate.

Background: Congenital heart defect (CHD) is the most common type of congenital disorder, especially in the Asian population. However, there are no comprehensive studies assessing the prevalence of this disease in the Iranian population.Objective: This study aimed to determine the frequency of cardiac abnormalities with a focus on CHD diagnosed by fetal echocardiography in pregnant women. Methods: This cross-sectional study utilized the medical records of 635 pregnant women who referred to Imam Reza Hospital in Mashhad, Iran, for fetal echocardiography from 2012 to 2017. All medical records of mothers who referred to a pediatric cardiologist at the CHD center have been assessed in this study. The American Heart Association guideline was used to detect the risk of CHD. Data were analyzed in SPSS version 16. Results: The mean age of the pregnant women was 29.6±5.13 years and their mean gestational age was 23.5±5.9 weeks. The incidence of fetal cardiac abnormalities was highest in those aged between 26 to 31 years. Fetal cardiac abnormalities were reported in 33.22% of the women. Moreover, the frequency of intra-cardiac echogenic focus was estimated at 25.6% and the frequency values of the complex CHD and ventricular septal defect (VSD) were obtained at 21.76% and 8.05%, respectively. Conclusion: According to the obtained results from this study, fetal cardiac abnormalities were found in more than one-third of pregnant women. In addition, the intracardiac echogenic focus was the most common detected cardiac abnormality. The most prevalent detected CHDs were complex CHD and VSD. This indicates a likelihood that the CHD is more common among Asian populations.

Background:We studied the patient journey in surgical wards in order to find an effective and efficient way of scheduling in surgical wards. Methods:We applied Root cause analysis (RCA) model within three months in a referral hospital. After understanding root causes of the events occurred through a focus discussion group, required interventions were proposed according to literatures, experiences, and preference of the stakeholders. Possible interventions were also analyzed based on its ability to reduce contributing factors in the events and the belief of process-owner that if interventions can be implemented. Results:The results were provided for five main steps: 1) the most important root cause was "not prioritizing patients and pre-scheduling the number of surgical procedures in the days before". 2) Constraints indicated that workforce weren't allocated proportionally to the number of surgical operations in varying shift lengths, increased numbers of on-calls physicians increased related costs, the admission of patients in VIP wards have been getting a high priority, and surgeon compensation based on fee for service method was challenging. 3) The current situation of allocating three rooms on average for each physician can be changed depending on numbers of surgeries. 4) Proposed interventions are establishing a computer registration system, reforming payment methods, setting up an electronic waiting list, development of scheduling guidelines, and Applying MIP model. Conclusions:Implementing of scheduling reforms requires a comprehensive action plan system and predefined functional indicators. These should be achieved with considering comments of all clinical and technical groups to ensure the feasibility of an operating room schedule. Key messages: Scheduling in the surgical wards has a critical effect on surgical procedures. An electronic based registry software system is required at least in our country. This system should prepare in the form of dashboard management tool with predefined criteria.  Development of such this system needs cooperation of both clinical and IT staff.Physicians are the most important key stakeholders to participate. Acknowledgments: The authors are grateful to the Chief Executive and to Surgery Department personnel of the Teaching Hospital, Iran, for their active collaboration and for sponsoring the present research, and to Ms. Azary and Ms. Hsseiny for contributing to the development of this Study. BACKGROUND: hospitals are always looking for cost reduction solutions, improving financial assets, andincreasingpatient's satisfaction and quality (1). A ward in a hospital, which needs to be paid attention specifically is the surgical ward since these facilities are the largest cost and income centers of a hospital and impact on the performance of the entire hospital(2). In addition, management of surgery department is so difficult not only for various preferences of M. Vejdani et al. J Fundam Appl Sci. 2017, 9(1S), 647-661 649 stakeholders, but also for insufficient resources in de...

Background: Pulmonary hypertension (PH) is a serious problem with high mortality in children, so early diagnosis of this condition is critical. Echocardiography is a non-invasive hemodynamic assessment tool, which facilitates serial follow-ups for hypertensive patients. Objectives: The aim of this study was to evaluate the importance of echocardiographic parameters in children with PH and their correlation with cardiac catheterization parameters. Methods: This cross-sectional study was performed on the records of 20 children with primary PH referred to the pediatric Cardi-