Functionally unicuspid aortic valve in an adult: Case report and literature review.

Aortic stenosis is one of the most prevalent cardiac valvular diseases throughout the world and has a significant impact on quality of life. While there are several etiologies, we will be discussing the case of a male in his mid-thirties of southeast Asian descent with a bicuspid aortic valve which was found to be functionally unicuspid and complicated by aortic dilation. Following a comprehensive review of literature, it appears this subset of aortic stenosis is not commonly encountered. In addition to presenting this fascinating case, we will review the epidemiology, classification and management of aortic stenosis. Furthermore, we will examine the latest evidence-based literature on bicuspid aortic valve and unicuspid aortic valve and discuss interventions and diagnostic tools that may improve clinical prognosis.

Ocular and orbital manifestations of granulomatosis with polyangiitis: a systematic review of published cases.

Objective: Granulomatosis with polyangiitis (GPA) is an autoimmune disorder characterized by necrotizing granulomatous inflammation of small and medium-sized vessels. This systematic review aimed to highlight the most common ophthalmic manifestations and to uncover their associations with antineutrophil cytoplasmic antibody (ANCA) positivity and the presence of granulomas. Methods: A literature search of PubMed, Web of Science, and Scopus electronic databases was performed from journal inception to March 21, 2021, for case reports and a series of ophthalmic GPAs. Cytoplasmic-ANCA (c-ANCA), perinuclear-ANCA (p-ANCA), and granulomas were analyzed against many ophthalmic signs and symptoms. 306 patients with GPA were retrospectively studied. Results: Granulomas were present in 47.7% of our sample, c-ANCA in 59.2%, and p-ANCA in 10.8%. Scleritis was significantly associated with higher odds for c-ANCA positivity. Eye discharge, episcleritis, proptosis, and central nervous system (CNS) involvement were each significantly associated with lower odds for c-ANCA positivity. Orbital mass was significantly associated with lower odds for p-ANCA positivity. CNS involvement was significantly associated with higher odds for p-ANCA positivity (OR:3.08, 95% CI:1.02, 9.36, p=0.047) and orbital mass was significantly associated with lower odds for p-ANCA positivity. Conclusions: We recommend that clinicians should consider ocular or orbital GPA in patients presenting with non-specific eye complaints, such as vision impairment, orbital mass, or proptosis, and obtain further assessments to determine the possible presence of granuloma, c-ANCA, or p-ANCA. Abbreviations: GPA = Granulomatosis with Polyangiitis, ANCA = antineutrophil cytoplasmic antibody, c-ANCA = cytoplasmic-ANCA, p-ANCA = perinuclear-ANCA, CNS = central nervous system, AAVs = ANCA-associated vasculitides, SD = standard deviation, GU = genitourinary, ENT = ear nose and throat, OR = odds ratio, CI = confidence interval.

Efficacy and Safety of Mavacamten in the Treatment of Hypertrophic Cardiomyopathy: A Systematic Review.

BACKGROUND: Current pharmacological options for hypertrophic cardiomyopathy (HCM) are not disease-specific; while it treats symptoms, mavacamten targets the underlying pathology. We aim to assess the efficacy and safety of mavacamten, a first-in-class cardiac myosin inhibitor, in symptomatic obstructive HCM. METHODS: This systematic review of the literature followed the PRISMA guidelines. Title/abstract and topics were searched using the following term: "mavacamten". The electronic research literature databases included the Cochrane Library, MedLine, and clinicaltrials.gov from July to August 2022. Primary efficacy endpoint was to assess clinical response at the end of treatment compared with baseline, defined as, at least one New York Heart Association (NYHA) class reduction. Two secondary endpoints from baseline were determined. The first was defined as improvement in mixed venous oxygen pressure (pVO2). The second was defined as reduction of the post-exercise left ventricular outflow tract (LVOT) gradient. RESULTS: We included in our analyses data from four studies that met our review eligibility criteria. There were three randomised placebo-controlled clinical trials and one non-randomised open-label clinical trial. All four studies showed a reduction in NYHA class from mavacamten use. Three out of four studies demonstrated >1 NYHA functional class improvement ranging from 34% to 80%, while only one study showed a smaller percentage of patients remaining at class 3. Three out of four studies measured pVO2 as an outcome, and all three studies noticed an increase in peak oxygen consumption after mavacamten treatment. Additionally, three out of four studies measured post-exercise LVOT gradient reduction as an outcome and all three found significant reduction in the post-exercise LVOT gradient after treatment. The most commonly observed adverse side effects were atrial fibrillation and decreased left ventricular ejection fraction, but all participants recovered without long-term sequelae and only one patient dropped out of the trial. CONCLUSIONS: Mavacamten has a greater efficacy than placebo in the treatment of HCM. It also showed promising tolerability and efficacy profiles in the treatment of HCM in adults. The three endpoints used in the evaluation of studies were reduction in NYHA class, increase in pVO2, and post-exercise LVOT gradient reduction. Mavacamten showed greater reduction in NYHA, larger effects on increase of pVO2, and significant reduction of the LVOT gradient. Mavacamten was also found to be well tolerated, like the placebo. The side effect profile was limited for the majority of individuals taking mavacamten. In the future, authors recommended dose-optimisation studies, and studies that evaluate mavacamten both in comparison to, and in conjunction with other current treatments.

A Case of Synchronous Primary Tumors of the Left Ovary and Uterus.

BACKGROUND Synchronous tumors occur when 2 separate primary tumors are diagnosed within 6 months. They can originate from the same site or different locations. For example, synchronous primary tumors of uterine and ovarian origin are a common type. Diagnosis can be challenging, however is critical to determine whether a patient has multiple primary tumors or a single tumor with metastasis to guide effective treatment. Compared with endometrial cancer that has spread to the ovary, synchronous primary tumors of the uterus and ovaries typically require less aggressive treatment. CASE REPORT A 45-year-old woman with nonspecific symptoms of headache and confusion had imaging studies that revealed a neoplasm in her brain, which was likely causing her symptoms. The masses were metastatic lesions, and the primary cancer was determined to be synchronous endometrial ovarian cancer (SEOC). She underwent bilateral frontal craniotomy for tumor resection and diagnostic tests. She had an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. She was stable during hospitalization but lost to follow-up after discharge. CONCLUSIONS Regular gynecologic examinations, including bimanual palpation of the ovaries during cervical cancer screenings, are essential for detecting cancer early and improving chances of recovery. This case also highlights the indolent growth and high risk of metastasis associated with SEOC. Although this type of cancer is rare, patients with it can be at increased risk of developing metastatic lesions in other parts of their bodies. To manage synchronous tumors effectively, a multidisciplinary approach and close collaboration between medical professionals are necessary to ensure best patient outcomes.

A case report of carbamazepine-induced oropharyngeal dysphagia in a patient with primary progressive multiple sclerosis.

Seventy-year-old male with primary progressive multiple sclerosis that had a severe episode of oropharyngeal dysphagia following initiation of carbamazepine. He was being treated for trigeminal neuralgia. Four days after discontinuation of carbamazepine resulted in a complete resolution of the patient's dysphagia, and he returned to baseline.

Can Moxidectin Be an Anthelmintic Alternative for Trichuris trichiura and Strongyloides stercoralis: A Systematic Review.

Strongyloides stercoralis and Trichuris trichiura parasitic infections are two of the many neglected tropical diseases. These parasitic infections are of considerable public health relevance, particularly in resource-limited countries. Moxidectin, a well-established drug in veterinary medicine, is now a Food and Drug Administration (FDA) approved medication for human onchocerciasis. For the past five years, this medication has been under clinical trials to evaluate its efficacy and safetiness in other helminthic infections. Moxidectin might complement the already existing treatment and control of soil-transmitted helminthiasis (STH). Therefore, we systematically reviewed existing human interventional studies to evaluate the efficacy and safety of this medication when administered alone or in combination with other antiparasitic medications in order to achieve a cure.