Clinical presentation and neurovascular manifestations of cardiac myxomas and papillary fibroelastomas: a retrospective single-institution cohort study
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ABSTRACT:
SUBMITTER: Mathavan A
PROVIDER: S-EPMC10503431 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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Project description: Not available
| S-EPMC6058221 | biostudies-literature
Project description:BackgroundCardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors most commonly found on left-sided cardiac valves. Right atrial CPFs are extremely rare, accounting for only 2% of all CPFs. Median sternotomy is a typical approach for surgical excision of CPFs in most cases. Herein, we report an extremely rare case of multifocal CPFs involving the right atrium and aortic valve that were surgically excised via minimally invasive right anterolateral thoracotomy.Case summaryA 59-year-old Chinese man was admitted because of an incidental finding of a right atrial mass on transthoracic echocardiography during a routine check-up. The mass was initially diagnosed as a myxoma, and the patient was scheduled for minimally invasive excision via right anterolateral thoracotomy. An additional mass on the non-coronary cusp of the aortic valve was identified using intraoperative transesophageal echocardiography. The patient still underwent complete tumor excision via right anterolateral thoracotomy. Both neoplasms were pathologically diagnosed as CPFs.ConclusionsThis case highlights the need for a comprehensive cardiac evaluation of cardiac tumors because CPFs can manifest as multifocal lesions. Moreover, minimally invasive surgery is highly feasible as the CPF can be easily excised, and the valve can usually be preserved.
| S-EPMC9382589 | biostudies-literature
Project description: Not available
| S-EPMC6098176 | biostudies-literature
Project description:Papillary fibroelastomas (PFEs) are the most common benign cardiac tumors, typically found on valvular surfaces but less frequently on extravalvular structures. Despite their benign nature, PFEs are associated with serious embolic events. This study analyzes 13 cases of pathology-proven PFEs in the left atrial appendage (LAA) and pulmonary vein ridge. Data on patient demographics, clinical presentations, imaging, and histopathology were reviewed. The median patient age was 73 years, with slight male predominance. Tumor sizes ranged from 2 mm to 20 mm. Twelve PFEs were detected via transesophageal echocardiography, and neurologic events were observed in 4 patients. This case series emphasizes the importance of maintaining a broad differential diagnosis for LAA masses because PFEs can easily be mistaken for thrombi.
| S-EPMC11911848 | biostudies-literature
Project description:AimsCardiac myxomas usually develop in the atria and consist of an acid-mucopolysaccharide-rich myxoid matrix with polygonal stromal cells scattered throughout. These human benign tumours are a valuable research model because of the rarity of cardiac tumours, their clinical presentation and uncertain origin. Here, we assessed whether multipotent cardiac stem/progenitor cells (CSCs) give rise to atrial myxoma tissue.Methods and resultsTwenty-three myxomas were collected and analysed for the presence of multipotent CSCs. We detected myxoma cells positive for c-kit (c-kitpos) but very rare Isl-1 positive cells. Most of the c-kitpos cells were blood lineage-committed CD45pos/CD31pos cells. However, c-kitpos/CD45neg/CD31neg cardiac myxoma cells expressed stemness and cardiac progenitor cell transcription factors. Approximately ≤10% of the c-kitpos/CD45neg/CD31neg myxoma cells also expressed calretinin, a characteristic of myxoma stromal cells. In vitro, the c-kitpos/CD45neg/CD31neg myxoma cells secrete chondroitin-6-sulfate and hyaluronic acid, which are the main components of gelatinous myxoma matrix in vivo. In vitro, c-kitpos/CD45neg/CD31neg myxoma cells have stem cell properties being clonogenic, self-renewing, and sphere forming while exhibiting an abortive cardiac differentiation potential. Myxoma-derived CSCs possess a mRNA and microRNA transcriptome overall similar to normal myocardium-derived c-kitpos/CD45neg/CD31negCSCs , yet showing a relatively small and relevant fraction of dysregulated mRNA/miRNAs (miR-126-3p and miR-335-5p, in particular). Importantly, myxoma-derived CSCs but not normal myocardium-derived CSCs, seed human myxoma tumours in xenograft's in immunodeficient NOD/SCID mice.ConclusionMyxoma-derived c-kitpos/CD45neg/CD31neg CSCs fulfill the criteria expected of atrial myxoma-initiating stem cells. The transcriptome of these cells indicates that they belong to or are derived from the same lineage as the atrial multipotent c-kitpos/CD45neg/CD31neg CSCs. Taken together the data presented here suggest that human myxomas could be the first-described CSC-related human heart disease.
| S-EPMC7735815 | biostudies-literature
Project description:The extent of surgery among patients with T2 papillary thyroid carcinoma (PTC) remains controversial. Thus, we herein aimed to evaluate the risk factors for recurrence, particularly based on the extent of surgery, among patients with T2 PTC at a single tertiary institution. We assessed 251 patients who underwent thyroid surgery for T2 PTC from January 2009 to December 2014 at Seoul St. Mary's Hospital (Seoul, Korea). The mean follow-up duration was 100.7 months. Eleven (4.4%) patients had recurrence. The recurrence rates did not significantly differ in terms of the extent of surgery (p = 0.868). Patients with a high lymph node ratio (LNR) had a significantly higher recurrence rate than those with a low LNR (p < 0.001). According to a recurrence pattern analysis, five of six patients in the lobectomy group had recurrence in the ipsilateral lateral compartment. A multivariate analysis revealed that a high LNR was a significant risk factor for recurrence (hazard ratio: 11.025, p = 0.002). Our results suggest that patients without clinical evidence of any lymph node metastases and those with limited lesions in the thyroid gland can undergo lobectomy and LNR can serve as an independent risk factor for predicting recurrence in T2 PTC.
| S-EPMC9360027 | biostudies-literature
Project description:Papillary fibroelastoma (PFE) is a well-known primary cardiac tumor, but multiple PFEs are rare. We report an interesting case with multiple PFEs that were clearly demonstrated and evaluated with real time three-dimensional (RT3D) transesophageal echocardiography (TEE). A 77-year-old woman was referred to our institution with a diagnosis of osteoarthritis of the hip. Transthoracic echocardiography showed an abnormal structure on the aortic valve. Although two-dimensional TEE revealed typical characteristics of multiple PFE, RT3D TEE clearly demonstrated their number and location on the right and non-coronary cusp of the aortic valve. These results were subsequently confirmed by surgery and pathological findings. RT3D TEE is an exceptionally useful tool for pre-surgical evaluation of PFE. <Learning objective: PFE is a well-known primary cardiac tumor, but multiple PFEs are rare. We report an interesting case with multiple PFEs that was clearly demonstrated and evaluated with RT3D TEE. RT3D TEE is an exceptionally useful tool for pre-surgical evaluation of PFE.>.
| S-EPMC6281484 | biostudies-literature
Project description:Myotonic dystrophy type 1 (DM1) is the most prevalent inherited neuromuscular dystrophy in adults. It is a multisystem disease with cardiac manifestations. Whilst these are well-defined in adults, there are scarce published data in the pediatric population. This study aimed to investigate the yield and progression of cardiac disease in pediatric DM1 patients, focusing on congenital DM1 (cDM1).MethodsA retrospective observational study of all pediatric DM1 patients referred to our center (December 2000-November 2020) was conducted. Patients were classified into DM1 forms according to age of symptom onset and disease severity. Patients underwent clinical and cardiac evaluation with 12-lead ECG, transthoracic echocardiography and 24-h ECG Holter monitoring.Results67 DM1 pediatric patients were included: 56 (83.6%) cDM1 and 11 (16.4%) non-cDM1. Median follow-up time of cDM1 patients was 8.0 [3.25-11.0] years. 49 (87.5%) cDM1 patients had baseline 12-lead ECG and 44 (78.6%) had a follow-up 12-lead-ECG, with a median follow-up time from diagnosis to baseline ECG of 2.8 [1.0-8.5] years and to follow-up ECG of 10.9 [5.7-14.2] years. Overall, 43 (87.8%) presented ECG abnormalities, most commonly in the form of asymptomatic conduction disease (n = 23, 46.9%), of which 21 (42.9%) had first degree atrioventricular block (1st AVB). There was an increase of prevalence from baseline to follow-up ECG in low QRS voltage (16.7%), poor R wave progression (13.9%), abnormal repolarisation (11.9%) and 1st AVB (7.6%). one patient (1.8%) underwent pacemaker implantation for syncope in the context of progressive conduction disease. No patients developed left ventricular systolic dysfunction. 4 (7.1%) cDM1 patients died during follow up, including three who died suddenly with no clear cause of death.ConclusionsThis study is the first to analyse the prevalence and progression of ECG abnormalities in cDM1 pediatric patients. The high prevalence of abnormal findings, progressive changes and number of potentially associated events (1 pacemaker implantation and 3 unexplained sudden deaths) stresses the importance of systematic and continued cardiac evaluation of these patients.
| S-EPMC9218560 | biostudies-literature
Project description:Background and purposeTakayasu arteritis is a rare, large-vessel vasculitis that presents with symptoms related to end-organ ischemia. While the extracranial neurovascular manifestations of Takayasu arteritis are well-established, little is known regarding the intracranial manifestations. In this study, we characterize the intracranial and cervical neurovascular radiologic findings in patients with Takayasu arteritis.Materials and methodsPatients with Takayasu arteritis who presented to our institution between 2001 and 2016 with intracranial and/or cervical vascular imaging were included in this study. Images were evaluated for the presence of vascular abnormalities, including intracranial or extracranial stenosis, vessel-wall thickening, dissection, subclavian steal, aneurysms, infarcts, and hemorrhages. Descriptive analyses are reported.ResultsSeventy-nine patients with Takayasu arteritis met the criteria for inclusion in this study. The most common presenting neurologic symptoms were headache (32.9%) and dizziness (15.2%). Intracranial and extracranial vascular imaging was performed in 84.8% and 89.9% of patients, respectively. Among patients with intracranial vascular imaging, 3 (3.9%) had intracranial aneurysms, 3 (3.9%) had acute large-vessel occlusion, 6 (7.6%) had intracranial vasculitis, and 1 (1.3%) had reversible cerebrovascular constriction syndrome. Among patients with cervical vascular imaging, 42 (53.1%) had some degree of narrowing of the common carotid artery and 18 (22.8%) had narrowing of the ICAs. Seventeen patients (23.6%) had subclavian steal.ConclusionsIntracranial vascular abnormalities in patients with Takayasu arteritis presenting with neurologic symptoms are not rare, with cerebral vasculitis seen in 7.8% of patients, and stroke secondary to large-vessel occlusion, in 3.9% of patients. Cervical vascular manifestations of Takayasu arteritis were present in most patients in our study.
| S-EPMC7960250 | biostudies-literature
Project description:•The coumadin ridge is a very unusual location for a cardiac PFE.•Although benign, large, mobile cardiac PFEs can pose a significant embolic risk.•Multimodality CV imaging can differentiate cardiac PFE from other cardiac tumors.
| S-EPMC6058251 | biostudies-literature