Project description:BackgroundDuring paediatric cardiac Cine-MRI, data acquired during cycles of different lengths must be combined. Most of the time, Feinstein's model is used to project multiple cardiac cycles of variable lengths into a mean cycle.ObjectiveTo assess the effect of Feinstein projection on temporal resolution of Cine-MRI.Methods1/The temporal errors during Feinstein's projection were computed in 306 cardiac cycles fully characterized by tissue Doppler imaging with 6-phase analysis (from a population of 7 children and young adults). 2/The effects of these temporal errors on tissue velocities were assessed by simulating typical tissue phase mapping acquisitions and reconstructions. 3/Myocardial velocities curves, extracted from high-resolution phase-contrast cine images, were compared for the 6 volunteers with lowest and highest heart rate variability, within a population of 36 young adults.Results1/The mean of temporal misalignments was 30 ms over the cardiac cycle but reached 60 ms during early diastole. 2/During phase contrast MRI simulation, early diastole velocity peaks were diminished by 6.1 cm/s leading to virtual disappearance of isovolumic relaxation peaks. 3/The smoothing and erasing of isovolumic relaxation peaks was confirmed on tissue phase mapping velocity curves, between subjects with low and high heart rate variability (p = 0.05).ConclusionsFeinstein cardiac model creates temporal misalignments that impair high temporal resolution phase contrast cine imaging when beat-to-beat heart rate is changing.
Project description:We describe the case of an antenatally diagnosed massive cardiac tumor in a fetus requiring cardiorespiratory support immediately following birth. We further discuss the successful management of this case and highlight the importance of a multidisciplinary team in managing such complicated cases. (Level of Difficulty: Advanced.).
Project description:Cardiac rhabdomyomas are a possible early manifestation of the Tuberous Sclerosis Complex (TSC). They often regress spontaneously but may grow and cause cardiac dysfunction, threatening the child's life. Treatment with rapalogs can stop the growth of these cardiac tumors and even make them shrink. Here, we present the case of a successful treatment of a cardiac rhabdomyoma in a fetus with TSC by administering sirolimus to the mother. The child's father carries a TSC2 mutation and the family already had a child with TSC. After we confirmed the TSC diagnosis and growth of the tumor with impending heart failure, we started treatment at 27 weeks of gestation. Subsequently, the rhabdomyoma shrank and the ventricular function improved. The mother tolerated the treatment very well. Delivery was induced at 39 weeks and 1 day of gestation and proceeded without complications. The length, weight, and head circumference of the newborn were normal for the gestational age. Rapalog treatment was continued with everolimus. Metoprolol and vigabatrin were added because of ventricular preexcitation and epileptic discharges in the EEG, respectively. We provide the follow-up data on the child's development in her first two years of life and discuss the efficacy and safety of this treatment.
Project description:Cardiac rhabdomyomas are described as sporadic as well as associated with tuberous sclerosis. Association with LVOT obstruction with anorectal malformation or imperforate anus has not been reported so far in published literature. In this case report we describe one such case.
Project description:The long-term prognosis of a fetus with cardiac rhabdomyoma (CR) depends on the correlation with tuberous sclerosis complex (TSC). In recent years, the numerous variations of uncertain significance (VUS) of TSC genes produced by high-throughput sequencing have made counseling challenging, studies until now have tended to side-step the tricky topics. Here, we integrated detailed parental phenotype, echocardiography, neuro MRI, and genetic information to conduct a comprehensive evaluation of 61 CR fetuses. As a result, multiple CRs and cerebral lesions appeared in 90 and 80%, respectively of fetuses with pathogenic (P)/likely pathogenic (LP) TSC1/TSC2 variations. Overall, 85.7% of the live-born infants with P/LP presented with TSC-associated signs. While, 85.7% of VUS without nervous findings had good prognoses. Genetic evidence and cerebral MRI findings are the most sensitive index to assess long-term prognosis, which complement and confirm each other for a TSC diagnosis. In total, 68.9% of fetuses with CR could benefit from this multidisciplinary approach, which turned out to be potentially clinically actionable with precise clinical/genetic diagnosis or had a foreseeable outcome. Our practice provides a practical and feasible solution for perinatal management and prognostic guidance for fetuses with CR.
Project description:The brightness of aurorae in Earth's polar region often beats with periods ranging from sub-second to a few tens of a second. Past observations showed that the beat of the aurora is composed of a superposition of two independent periodicities that co-exist hierarchically. However, the origin of such multiple time-scale beats in aurora remains poorly understood due to a lack of measurements with sufficiently high temporal resolution. By coordinating experiments using ultrafast auroral imagers deployed in the Arctic with the newly-launched magnetospheric satellite Arase, we succeeded in identifying an excellent agreement between the beats in aurorae and intensity modulations of natural electromagnetic waves in space called "chorus". In particular, sub-second scintillations of aurorae are precisely controlled by fine-scale chirping rhythms in chorus. The observation of this striking correlation demonstrates that resonant interaction between energetic electrons and chorus waves in magnetospheres orchestrates the complex behavior of aurora on Earth and other magnetized planets.
Project description:Heart failure (HF) is a leading cause of morbidity and mortality. Studies in animal models and patients with HF revealed a prominent role for CD4+ T cell immune responses in the pathogenesis of HF and highlighted an active crosstalk between cardiac fibroblasts and IFNγ producing CD4+ T cells that results in profibrotic myofibroblast transformation. Whether cardiac fibroblasts concomitantly modulate pathogenic cardiac CD4+ T cell immune responses is unknown. Here we show report that murine cardiac fibroblasts express major histocompatibility complex type II (MHCII) in two different experimental models of cardiac inflammation. We demonstrate that cardiac fibroblasts take up and process antigens for presentation to CD4+ T cells via MHCII induced by IFNγ. Conditional deletion of MhcII in cardiac fibroblasts ameliorates cardiac remodelling and dysfunction induced by cardiac pressure overload. Collectively, we demonstrate that cardiac fibroblasts function as antigen presenting cells (APCs) and contribute to cardiac fibrosis and dysfunction through IFNγ induced MHCII.
Project description:Rhabdomyoma is a well characterised entity in a neonate. Herein, we report a massive biventricular rhabdomyoma in a neonate presenting with cyanosis and congestive heart failure which was confirmed on autopsy. The report is for documentation of an unusually large tumour.
Project description:Primary tumors of the heart are rare where nearly half of the benign cardiac masses are myxomas. Clinical features of myxoma are determined by their location, size, and mobility. Most patients present with one or more of the triad of embolism, intracardiac obstruction, and constitutional symptoms. Herein, we present the case of a 60-year-old female with a history of genital prolapse who had new onset worsening dyspnea two days after an elective total abdominal hysterectomy, bilateral salpingo-oopherectomy, and vaginal repair. She was initially thought to have a pulmonary embolism so had a computed tomography scan that revealed a cardiac mass, which was diagnosed to be a myxoma. Although rare, atrial myxomas can present in any patient population. This case report is educational as it highlights the atypical presentation of an atrial myxoma. To facilitate appropriate management, high degree of suspicion should be complemented with a comprehensive physical examination and set of investigations. <Learning objective: Cardiac myxomas are benign tumours that usually arise in the atria. Myxomas are rare, but can be present in any patient population. If symptoms are present, surgical resection of myxoma is indicated.>.
Project description:Dermatomyositis (DM), a myopathy associated with inflammation and muscle weakness, has historically been difficult to diagnose. Recently, nuclear matrix protein (NXP-2) antibodies have been described as a myositis-specific antibody that may aid in the diagnostic evaluation. We present the case of a 21-year-old, previously healthy, African American male with DM. He presented to our outpatient clinic with periorbital swelling and a rash, for which he was started on prednisone by an ophthalmologist. Towards the end of the prednisone taper, he began to experience muscle weakness, a worsening rash, and dysphagia to solids with a resultant loss of 60 pounds within a month. He was transferred to a tertiary care hospital where he was further evaluated and ultimately diagnosed with dermatomyositis, supported by skin and muscle biopsies, and was found to be positive for NXP-2. He was given intravenous immunoglobulin (IVIG) and high-dose steroids with improvement.