مقالات رزومه دکتر اکبر مولایی
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Introduction
Overally the anomaly of the coronary arteries are rare. Its incidence according to angiographic, autopsy and echocardiographic findings, are about 1%, 0.29% and less than 0.1% respectively. Usually these are the incidental finding during angiography without clinical presentation. These anomalies may be accompanied by myocardial ischemia, arrhythmia, syncope and sudden death.
Case PresentationWe present a 14-year-old Iranian boy who experienced chest pain and shortness of breath after engaging in physical activity. The right coronary artery (RCA) had an intramural and inter-arterial course and was proven to have originated from the left sinus of Valsalva by computed tomographic coronary angiography (CTCA). The RCA began adjunct to the ostium of the left main coronary artery and coursed intramural at first and then continued between the aorta and pulmonary artery. The patient underwent corrective surgery. The RCA was cut from origin and reanastomosd to the right coronary sinus after preparing the sinus. The patient recovered well and he was discharged with the ability to perform sports activity. One year Follow up of the patient was uneventful.
ConclusionsEven though single ostium coronary artery abnormalities are uncommon, they have become more frequently identified in recent years using non-invasive techniques such transthoracic echocardiography, which is then followed by CT angiography or conventional angiography. These patients are susceptible to sudden death, so attention to symptoms of the patients and comprehensive approach to them can be lifesaving.
Keywords: Right Coronary Artery, Abnormal Origin, Intramural, Inter-arterial} -
زمینه و هدف
سندرم QT طولانی (LQTS) نوعی اختلال مادرزادی یا اکتسابی در مرحله رپولاریزاسیون است که باعث طولانی شدن فاصله QT در ECG سطحی می شود. طبق تعریف در سندرم QT طولانی فاصله QT اصلاح شده بیشتر از ms440 در مردان و ms 460 در زنان می باشد. اشکال اکتسابی QT طولانی، ناشی از اختلالات متابولیک و داروهای مختلف هستند. سندرم QT طولانی (LQTS) همراه با حملات سنکوپ و مرگ ناگهانی مشخص می شود. جرول و لانژ- نیلسن در ابتدا LQTS را در همراهی با کری مادرزادی حسی- عصبی شرح دادند. ازآنجا که تعداد افراد دچار کری مادرزادی در جامعه کم نیستند و با توجه به اینکه با شناسایی این بیماران با انجام نوار قلب و اقدامات ساده، می توان از مرگ ناگهانی این افراد به میزان قابل توجهی جلوگیری کرد، هدف از این مطالعه تعیین میزان شیوع سندرم QT طولانی در کودکان با کری مادرزادی حسی- عصبی، در بیمارانی است که تحت عمل جراحی کاشت حلزون قرار گرفته اند.
روش کاردر این مطالعه که یک مطالعه مشاهده ای مقطعی تک مرکزی بود، تمام کودکان مبتلا به کری مادرزادی که تحت عمل جراحی کاشت حلزون قرار گرفته بودند وارد مطالعه شدند. نوار قلبی بیماران و وجود QT طولانی پس از محاسبه QT با استفاده از فرمول بازت و همراهی آن با سنکوب و مرگ ناگهانی مورد بررسی قرار گرفتند و در نهایت با استفاده از کرایتریای شوارتز کودکانی که مبتلا به سندرم QT طولانی بودند تشخیص داده شده و همراهی آن با کری مادرزادی ارزیابی گردید.
یافته هااز 357 بیمار مورد مطالعه، 204 مورد (57/1 %) مذکر و 153 مورد (42/9%) مونث بودند. میانگین سن فعلی بیماران مورد مطالعه برابر2/93 ± 7/25 سال با میانه 7 سال بود. میانگین فاصله QT در بیماران مورد مطالعه برابر 26/89 ±291/01 میلی ثانیه با میانه 280 میلی ثانیه بود. میانگین فاصله QTc در بیماران مورد مطالعه برابر25/74± 400/52 میلی ثانیه با میانه 404 میلی ثانیه بود. فراوانی Long QTc در بیماران مورد مطالعه برابر 17 مورد (4/8%) و میزان شیوع سندرم QT طولانی 5 مورد (1/4%) بود.
نتیجه گیریمیزان شیوع سندرم QT طولانی در کری عصبی در کودکان مراجعه کننده جهت کاشت حلزون در شهر تبریز در سال 1398 برابر با 1/4 درصد بود.
کلید واژگان: سندرم QT طولانی, کری مادرزادی, کودکان, ایران}Background & objectivesLong QT syndrome (LQTS), congenital or acquired disorder, is characterized by a prolonged QT interval associated with syncope attacks and sudden death. Jervell and Lange-Nielsen first described LQTS in conjunction with sensory-neural congenital deafness. In long QT syndrome, the modified QT interval is greater than 440 ms and 460 ms in men and in women respectively. Acquired forms of prolonged QT are the consequence of metabolic disorders and various medications.Since the number of people with congenital deafness in the society is not low and studies in Iran about this syndrome, especially in children are limited, therefore identification of these patients by performing an ECG and simple measures, may be useful in reducing sudden death among these patients. The aim of this study was to determine the prevalence of prolonged QT syndrome in children with congenital sensory-neural deafness which undergoing cochlear implant surgery.
MethodsIn this study, a single-center cross-sectional observational study, all children with congenital deafness which undergoing cochlear plantation at northwest of Iran were enrolled. Patients' heart rate and QT interval were calculated using Bazett's formula. The association of long QTc with syncope and sudden death, evaluated by using Schwartz's criteria in children with long QT syndrome.
ResultsOf the 357 studied patients, 204 (57.1%) were male and 153 (42.9%) were female. The mean±SD of the current age of the patients was 7.15±2.93 years with a mean of 7 years. The mean±SD of QT interval in the studied patients was 291.01±26.89 ms with a mean of 280 ms. the mean±SD of the QTc interval in the studied patients was 400.52±25.74 ms with a median of 404 ms. Frequency of Long QTc in the studied patients was 17 cases (4.8%) and long QT syndrome was 5 cases (1.4 %).
ConclusionThe prevalence of prolonged QT syndrome in patients with congenital deafness in northwest of Iran in 2019 was 4.8%, and 2 cases had syncope and 3 cases had sudden death.
Keywords: Long QT Syndrome, Congenital Deafness, Children, Iran} -
Objectives
Ventricular septal defect (VSD) is the most common congenital heart disease (CHD) (40%). Traditionally, VSDs have been repaired surgically; however, recently VSD closure by device has been developed. This study aimed to compare these two methods in terms of effectiveness, outcomes, and complications.
Materials and MethodsThis study was conducted on VSD patients who were treated by either surgery or trans catheter technique in Madani Heart Center from March 2011 to March 2017. The required data regarding effectiveness, outcomes, and complications were recorded based on the patient’s documents. The extracted data were analyzed by SPSS version 18.0.
ResultsA total of 93 patients were enrolled (43 patients in transcatheter [A] and 50 patients in open-heart surgery [B] group) in this study. The gender distribution was approximately the same. The mean age of group A was 10.55 years while it was 8.8 years for group B. The duration of ICU and hospital stay, total hospitalization cost, and frequency of blood products transfusion were significantly lower in group A than in group B. However, postoperative pacemaker insertion, arrhythmias, thromboembolism, and mortality rates did not show any significant difference between the two groups. The success rates in group A and B were 97.7% and 96%, respectively.
ConclusionsThe results of our study demonstrated that open-heart surgery, due to its nature, required longer hospital and ICU stay, higher cost, and higher blood transfusion rate than transcatheter therapy, but it was associated with similar complications and success rates. Therefore, device closure in appropriately selected patients is recommended as an alternative to the surgical approach.
Keywords: Ventricular septal defect, Open-heart surgery, Transcatheter, Outcomes} -
BackgroundThe modified Fontan operation is one of the essential surgical techniques in children with congenital heart diseases. Thus, we sought to identify the characteristics as well as the early and late outcomes of patients who have undergone Fontan operation during more than 10 years.MethodsThe medical records of 52 patients who underwent modified Fontan operation from March 21th, 2006 to March 20th, 2018, at Tabriz Children's Hospital including the baseline, clinical and surgical data were extracted and reviewed. Patients were invited for follow up visits and clinical examination and echocardiographic evaluations were conducted.FindingsThe most prevalent heart anomaly was tricuspid valve disorders (28 cases, 51.9%). The majority of patients (42 cases, 80.2 %) underwent intracardiac lateral tunnel Fontan. The most prevalent complications were prolonged pleural effusion and neurologic events (each one in 10 cases, 19.2%). The in-hospital mortality rate was 15.3%. The patients were followed for a median of 7 years. The survival of those successfully followed was 75 percent. The most common valvular disorder was mitral valve regurgitation (MR), which was present in 84% of cases. The group of patients with single ventricle had a significantly higher mortality rate than the other groups (P = 0.018).ConclusionSurvival rate of patients undergoing a Fontan completion is acceptable. However, patients remain at risk for morbid events. Moreover, patients with Fontan circulation should be strictly followed to investigate the development of MR and to timely repair in severe cases to prevent further deterioration. Of note, due to some limitations of our study, further multi-centric studies are required to confirm our findings.Keywords: complication, extracardiac Fontan, Fontan operation, intracardiac lateral tunnel Fontan, Outcome}
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Multiple interatrial defects, termed fenestrated ASDs that require closure are not uncommon. The problem arises when a centrally located defect or a patent foramen oval (PFO) is associated with another peripherally located defect. In cases like this, all attempts at crossing the true defect might totally fail or might be difficult because the wire or the catheter crosses the central defect repeatedly despite the use of a sizing balloon. In order to overcome such an issue, we introduce a new technique by which not only the procedure and the fluoroscopy time will be reduced, but also it ceases the mistakes about the number of defects, their size and location.
Keywords: Multiple ASDs, Peripheral ASD, Transcatheter Closure} -
ObjectiveThe balloon angioplasty is a controversial procedure for the treatment of coarctation of the aorta (COA). This study determines the results of balloon angioplasty of native COA in infants.Materials And MethodsTwenty-five subjects had undergone balloon angioplasty under drug-induced sedation using the retrograde technique through the femoral artery. In all the cases a Tyshak Mini balloon had been used. The patients had all been followed by thoracic echocardiography. Data were analyzed with SPSS 16.ResultsTwenty-five patients under 2 years of age with native COA, had undergone balloon angioplasty.The median age and weight of the subjects were 55 (12-700) days and 4860 ± 192 g respectively. Mean stenotic site diameter was 2.31± 0.58 mm before procedure and 5.41 ± 1.09 mm after procedure (PConclusionBased on the results of this study and reviewed studies, balloon angioplasty might be an alternative procedure in infants with native COA. However timely diagnosis and improvement in angioplasty techniques are necessary to improve the outcomeKeywords: Angioplasty, Aortic Coarctation, Balloon, Infant}
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