The Top Most-Cited and Influential Published Articles in Atrial Fibrillation from 1900 to 2019.

Citation classics are widely being implemented in the field of medical and scientific research assessment. The frequency of citation of a particular article is used to gauge its contribution and impact on the clinical world of practice and research. A thorough search of the literature showed a lack of bibliometric analysis on atrial fibrillation (AF). Thereby the main purpose of this study is to pinpoint the trend of the top 100 cited articles on AF. In June 2019, 2 databases, Scopus and Web of Science, were used to acquire the articles published on AF, which belonged to various genres including medicine, medical and interventional cardiology, electrophysiology, and thoracic surgery. The data were thoroughly reviewed and analyzed by 2 reviewers with regards to the number of citations for each article, publications per area, document type, first author name, country of origin, institute of origin, and year of publication. Approval of the Institutional Board Review was not required as we used publically available data retrospectively. The number of citations in the top 100 articles ranged from 622 to 6,641 times with an average citation of 1041.3 per article. The most significant number of articles was published in the year 2004 which ranged up to 11 in total. All the articles are published in 21 English language journals. In these 100 articles, the most were from the United States (n = 63) followed by Canada (n = 24), and other countries (n = 17). The top 5 institutions include McMaster University (n = 9), Institut de Cardiologie de Montreal (n = 8), Population Health Research Institute (n = 8), Harvard Medical School (n = 7), and Mayo Clinic (n = 7). In conclusion, authors seeking to publish a highly referenced article on AF will be determined by source journal, the language of publication, geographic origin, methodology, or research outcome.

Laurence-Moon-Bardet-Biedl Syndrome with Coexisting Abdominal Distension and Positive Fluid Thrill: A Rare Manifestation Reported in Karachi, Pakistan.

Laurence-Moon-Bardet-Biedl syndrome (LMBBS) is a rare autosomal recessive (AR) disorder. It is characterized predominantly by hypogonadism, polydactyly, retinitis pigmentosa, obesity, and mental retardation. Herein, we present a classic case of LMBBS with generalized body edema, abdominal distension, and positive fluid thrill in a 32-year-old male. LMBBS patients are friendly with a happy predisposition, proper management, and regular examinations should be done in order to maintain healthy organ function and to avoid an early death. Renal failure is the most common cause of mortality in LMBBS patients.

A Challenging Diagnosis of Systemic Lupus Erythematosus with Status Epilepticus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder of unknown pathogenesis. In SLE, the body's immune system mistakenly attacks healthy tissues and organs thereby involving multiple body systems including joints, skin, blood, brain, heart, and lungs. SLE has a wide variation in the symptoms, hence making the diagnosis more challenging at the time of initial presentation. Sometimes, the patient presents with Status epilepticus (SE) without prior history of epilepsy, as SE is common at the beginning in the course of SLE. In this report, there is a case showing correlation of seizures with SLE, without prior history of epilepsy. A 43-year-old female presented in the emergency department of the hospital with SE. Her previous medical and family history for epilepsy was unremarkable. The patient had high titers for positive anti-nuclear antibody (ANA), while other autoimmune workup was negative. A complete evaluation of the symptoms and investigations revealed that she met the criteria of American College of Rheumatology (ACR) for the diagnosis of SLE. Hence, physicians should be diligent with regards to the variations in the initial presentation and complications of SLE. With the advancement in treatment modalities of SLE, it can be managed successfully, if diagnosed early.

A Case Report of Seronegative Limbic Encephalitis.

Most patients with autoimmune encephalitis do not present with well-described symptoms. Demographic data and information regarding co-morbidities could help in diagnosing the underlying disorder, but a definitive diagnosis is made by the result of autoimmune antibodies. Limbic encephalitis (LE), a variant of autoimmune encephalitis, is the inflammation of the limbic system of the brain. The disorder presents with the rapid development of confusion, working memory impairment, mood changes, and often seizures. LE could have paraneoplastic or non-paraneoplastic etiology. We present the case of a 15-year-old girl with seronegative LE, who presented with cognitive decline and seizures. This condition is rare, and therefore poses a great challenge in diagnosis at an early stage.

Neuropsychiatric Manifestation of Addison's Disease: A Rare Case Report.

Addison's disease (AD), also known as primary adrenal insufficiency, is a rare autoimmune disorder affecting males and females equally. The most common cause of AD is autoimmune adrenalitis and other causes include metastatic cancers, tuberculosis and acquired immunodeficiency syndrome. AD presents with a wide variety of signs and symptoms and thus, making a diagnosis challenging. The common symptoms of this disease include weakness and fatigability, orthostatic hypotension, nausea, vomiting, diarrhea, anorexia and weight loss. Addison's disease often presented with other autoimmune disorders, such as autoimmune polyglandular syndrome. We herein report a case of a patient who presented in a hospital emergency department, with Addisonian crisis and predominant neuropsychiatric manifestation. On review of the patient's history, combined with biochemical testing, a diagnosis of Addison's disease was established. This type of presentation is relatively uncommon.

Midterm outcomes of right ventricular outflow tract reconstruction using the Freestyle xenograft.

OBJECTIVE: Various options exist for right ventricular outflow tract (RVOT) reconstruction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined. DESIGN: We performed a retrospective review of all non-Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by surgery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression. RESULTS: Between January 2002 and December 2015, there were 182 patients identified. Sixteen patients were lost to follow-up and 3 patients died, unrelated to cardiac surgery. Of the remaining 163 patients, the median age was 12.2 years (interquartile range 6.4-16.4), median weight was 39.0 kg (interquartile range 19.9-59.3), and the median body surface area was 1.23 m2 (interquartile range 0.79-1.64). Ninety-three (57%) patients had tetralogy of Fallot. The median follow-up was 5.4 years (interquartile range 2.9-8 years). There were no operative or cardiac-related deaths. Thirty-eight patients (23%) required reintervention. The rate of freedom from reintervention was 93.2% (95% CI 86.7%-96.6%) at 5 years and 48.4% (95% CI 34.9%-60.6%) at 10 years. Age < 10 years, weight < 39 kg, and body surface area <1.2 m2 at the time of valve placement, as well as valve size ≤25 mm were significantly associated with need for earlier reintervention. CONCLUSIONS: The Freestyle root in the RVOT is associated with excellent survival and low midterm need for reintervention. Its longevity is comparable to published data on homografts and other bioprosthetic valves.