Comparing Clinical and Hemodynamic Parameters between the Two Aortic Valve Replacement in Patients with Severe Aortic Stenosis.

Context: Various techniques have been proposed for suturing following prosthetic aortic valve implantation, but each has its potential side effects such as patient-prosthesis mismatch (PPM) and paravalvular leak (PVL). Aims: In the present study, we aimed to compare the postoperative sequels of aortic valve replacement (AVR) following the use of two suturing techniques including from the inside of the aorta as the common and classic suturing techniques as compared to new approach including from the outside of the aorta. Settings and Design: This cross-sectional study was performed on patients suffering from symptomatic severe aortic valve disease and candidates for AVR in one of three referral hospitals for cardiovascular disease patients in Shiraz from 2019-2021. Subjects and Methods: The patients underwent one of the two considered surgical techniques for AVR including traditional approach (n = 60) or the tested approach (suturing from the outside of the aorta) (n = 30). Statistical Analysis Used: For statistical analysis, results were presented as mean ± standard deviation for quantitative variables and were summarized by frequency (percentage) for categorical variables. Results: PVL and PPM were revealed in none of the participants undergoing the tested approach, while the pointed complications were found in 41.7% and 25.0% of patients underwent traditional approach, respectively, indicating a significant difference. There was no evidence of abnormal dimensionless valve index (DVI) in the patients undergoing sutures from outside the aorta, whereas the rate of DVI abnormality in the patients undergoing traditional procedures was shown to be 18.3%. The mean aortic valve gradient was significantly higher in the group scheduling for the traditional surgical method. Conclusions: Applying new suturing method as suturing from the outside of the aorta, due to the selection of appropriate valve size, the risk for adverse consequences including PPM, PVL, or DVI can be significantly reduced.

Clinical features of patients with Yin Yang 1 deficiency causing Gabriele-de Vries syndrome: A new case and review of the literature.

BACKGROUND: Gabriele-de Vries syndrome is a rare autosomal dominant genetic disease caused by de novo pathogenic variants in YY1. In this study, we report a 10-year-old boy with a de novo novel pathogenic variant in YY1, the first Iranian patient with Gabriele-de Vries Syndrome. METHODS: The novel de novo pathogenic variant detected in this study (NM_003403:c.690delA, p.Glu231Ilefs*25) was identified by whole-exome sequencing and confirmed by Sanger sequencing. RESULTS: The proband presented with delayed motor and speech development, ataxia, abnormal gait, autistic behavior, brain atrophy, and severe learning disability. Finally, we provide a case-based review of the clinical features associated with Gabriele-de Vries Syndrome. Thus far, merely 13 Gabriele-de Vries Syndrome patients have been reported in the literature. CONCLUSION: The investigations for a suspected case of Gabriele-de Vries Syndrome must involve molecular diagnosis of the disease and its underlying genetic defect because the clinical investigations are generally variable and nonspecific.

Uncovering Cardiac Involvement in Childhood Diabetes: Is it Time to Move Toward Speckle Tracking Echocardiography in Childhood Diabetes Management?

CONTEXT: One of the most common endocrine disorders in children is diabetes which is the leading cause of premature cardiovascular disease in adulthood. AIMS: This study is aimed to investigate the extend of cardiac involvement in diabetic children by speckle tracking echocardiography (STE) in comparison to two-dimensional (2D) echocardiography and routine laboratory data. SETTINGS AND DESIGN: A cross-sectional study conducted on patients under 18 years of age who deal with type one diabetes mellitus for more than 5 years. SUBJECTS AND METHODS: To compare the STE results, we included the STE data of 25 normal age-matched children. All patients underwent laboratory analysis for lipid profile, blood sugar, and 2D echocardiography plus STE. STATISTICAL ANALYSIS USED: Two-sample independent t-test, Chi-square test, logistic regression test, Spearman's rank correlation coefficient, and the Pearson correlation coefficient. RESULTS: From March 2018 to 2019, we included 53 patients, mean age 15.8 ± 0.39 years and 52.8% female, and 25 nondiabetic control in this study. STE revealed global longitudinal strain (LS) -18.4 versus - 24.2 for patient (44 valid cases) versus control group, respectively, with significant statistical difference. Diabetic patients had lower LS in all segments compared to the control group. CONCLUSIONS: STE has very high sensitivity to detect cardiac involvement far earlier than 2D echocardiography. None of the routine biomarkers or demographic features can predict cardiac involvement based on segmental abnormalities of STE. Active investigation to clear the remote impact of STE abnormalities and its practical role in childhood diabetes management is highly recommended.

How accurate is frozen section pathology compared to permanent pathology in detecting involved margins and lymph nodes in breast cancer?

BACKGROUND: Frozen section (FS) pathology has multiple limitations, and different institutions report variable experiences with the use of FS for diagnosis of tumor involvement. We aimed to compare the FS accuracy with that of permanent pathology (gold standard) regarding marginal involvement and lymph node status using data from the largest breast cancer registry in Iran. METHODS: In this retrospective study, women who had both FS and permanent pathology reports were included. The two pathology reports were cross compared with regard to the involvement of tumor margins and sentinel lymph nodes. RESULTS: Overall, 2786 patients entered the study. Mean age of patients was 48.96±11.44 years. A total of 1742 margins were analyzed. Accordingly, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of FS pathology for detection of involvement of involved margins were 78.49%, 97.63%, 65.1%, and 98.7%, respectively. The accuracy and area under the curve (AUC) for FS pathology were 96.61% and 0.73 (95% CI: 0.64-0.831), respectively. A total of 1702 sentinel lymph node biopsies were assessed. Sensitivity, specificity, PPV, and NPV, of FS pathology for detection of lymph node involvement, were 87.1%, 98%, 95.5%, and 93.3%, respectively. Accuracy and AUC of FS for diagnosis of involved lymph nodes were 94.1% and 0.926 (95% CI: 0.909-0.942), respectively. CONCLUSION: Frozen pathology is a suitable method for identifying involved sentinel lymph nodes in patients with breast cancer, but this method has a less than optimum efficacy for detecting and confirming marginal involvement.

Aortopathic effect of androgenic anabolic steroids.

BACKGROUND: Anabolic androgens have been reported to be associated with cardiovascular complications. One study revealed that increase in vascular stiffness in bodybuilders is associated with anabolic androgens and improvement in vascular function may occur following anabolic androgens discontinuation. The aim of this study was to investigate any possible relation between aortic elastic properties and anabolic androgens. METHODS: Study population was divided into 3 groups: Group-1 [n = 35] consisted of bodybuilders who denied any current or previous use of anabolic androgens. Group-2 [n = 18] was bodybuilders with regular using of anabolic androgens for at least 2 year prior to the start of our study. Group-3 was 13 healthy age-matched sedentary men as a control group. Cardiac echocardiography was performed in the bodybuilders and controls and indexes of aortic function were calculated. RESULTS: Aortic stiffness was approximately twofold higher in anabolic androgens user bodybuilders compared with drug-free bodybuilders [P < 0.001]. CONCLUSION: The present study demonstrates that chronic anabolic androgens use clearly produces significant decrease in the elastic properties of aorta.