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Browsing by Author "Kurmann, Reto D."
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Item Case Report: A Rare Case of Right-Sided Papillary Fibroelastoma in a 1-Year-Old With Congenital Heart Disease(Frontiers Media, 2021-01-27) Ahmad, Ali; El-Am, Edward A.; Kurmann, Reto D.; Hagler, Donald J.; Bois, Melanie C.; Maleszewski, Joseph J.; Klarich, Kyle W.; Medicine, School of MedicineIntroduction: Cardiac papillary fibroelastomas (PFEs) are the most common primary benign cardiac tumors, although they are somewhat unusual in children and typically seen on the left-sided cardiac valves. Case summary: A 10-week-old patient was found to have a partial atrioventricular canal defect, with associated tricuspid and mitral regurgitation. He was medically managed until 1 year of age, when surgical correction was done. During the procedure, a PFE was found incidentally on the TV. Conclusion: This is one of the youngest patients to be reported with PFE, thus adding to the literature of these unusual cases in children.Item Case Report: A Tale of a Cardiac Mass: Looks Like a Papillary Fibroelastoma, Acts Like a Non-bacterial Thromboendocarditis(Frontiers Media, 2021-11-12) Ahmad, Ali; Arghami, Arman; El-Am, Edward A.; Foley, Thomas A.; Kurmann, Reto D.; Klarich, Kyle W.; Medicine, School of MedicineIntroduction: Benign cardiac tumors and tumor like conditions are a heterogeneous collection of mass lesions that vary widely in their characteristics, such as presentation, size, and location. In some instances, these tumors are found incidentally, and therefore a broad differential diagnosis should be considered. Case: An elderly male with significant unintentional weight loss and a high risk for cancer presented with an incidental valvular cardiac mass. The mass was thought to be a non-bacterial thromboendocarditis on initial clinical evaluation. After multiple imaging modalities, the mass was suspected to be a papillary fibroelastoma (PFE), which was resected due to high stroke risk and multiple previous chronic infarcts on brain MRI. Conclusion: This case highlights the need for a comprehensive cardiac evaluation of a valvular tumor to discern the etiology and rule out other underlying pathophysiological processes that may require alternative interventions to cardiac surgery.Item Increased Risk of Valvular Heart Disease in Systemic Sclerosis: An Underrecognized Cardiac Complication(Journal of Rheumatology, 2021) Kurmann, Reto D.; El-Am, Edward A.; Radwan, Yasser A.; Sandhu, Avneek S.; Crowson, Cynthia S.; Matteson, Eric L.; Warrington, Kenneth J.; Mankad, Rekha; Makol, Ashima; Medicine, School of MedicineObjective: Cardiac involvement is a poor prognostic marker in systemic sclerosis (SSc). While diastolic dysfunction, myocardial fibrosis, and arrhythmias are traditionally considered features of primary cardiac involvement in SSc, the incidence of valvular heart disease (VHD) is not well reported. Our objective was to examine the prevalence of VHD at the time of SSc diagnosis and incidence of VHD during follow-up compared to non-SSc subjects. Methods: Medical records of patients with suspicion of SSc were reviewed to identify incident cases. SSc subjects were matched 1:2 by age and sex to non-SSc subjects. Results: The study included 78 incident SSc cases and 156 non-SSc comparators (56 yrs [± 15.7], 91% female). A nearly 4-fold increase in the prevalence of moderate/severe VHD prior to SSc diagnosis compared to non-SSc subjects (6% vs 0%; P = 0.004) was identified. During follow-up, 18 SSc and 12 non-SSc patients developed moderate/severe VHD. The cumulative incidence of VHD at 10 years after SSc incidence/index was 17.9% (95% CI 10.7-29.9) in patients with SSc compared with 2.3% (95% CI 0.7-7.0) in non-SSc subjects (HR 4.23, 95% CI 2.03-8.83). Coronary artery disease was the only significant risk factor for VHD. Conclusion: Patients with SSc have a 4-fold increase in the prevalence of moderate/severe VHD at diagnosis compared to non-SSc patients. They also have a 4-fold increased risk of developing moderate/severe VHD after diagnosis of SSc. Aortic stenosis and mitral regurgitation have a much higher prevalence in patients with SSc, besides secondary tricuspid regurgitation. Underlying mechanisms for this association require further elucidation.Item Systemic Sclerosis Portends Increased Risk of Conduction and Rhythm Abnormalities at Diagnosis and During Disease Course: A US Population-Based Cohort(Sage, 2021) Radwan, Yasser A.; Kurmann, Reto D.; Sandhu, Avneek S.; El-Am, Edward A.; Crowson, Cynthia S.; Matteson, Eric L.; Osborn, Thomas G.; Warrington, Kenneth J.; Mankad, Rekha; Makol, Ashima; Medicine, School of MedicineObjectives: To study the incidence, risk factors and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis (SSc) vs. non-SSc comparators. Methods: An incident cohort of patients with SSc (1980-2016) from Olmsted County, MN was compared to age- and sex-matched non-SSc subjects (1:2). Electrocardiograms (ECGs), Holter ECGs, and need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: 78 incident SSc cases and 156 comparators were identified (mean age 56 y, 91% female). The prevalence of any conduction disorder prior to SSc diagnosis compared to non-SSc subjects was 15% vs. 7% (p=0.06), and any rhythm disorder was 18% vs. 13% (p=0.33). During a median follow-up of 10.5 years in patients with SSc and 13.0 years in non-SSc comparators, conduction disorders developed in 25 patients with SSc with cumulative incidence of 20.5% (95% CI: 12.4-34.1%) vs. 28 non-SSc patients with cumulative incidence of 10.4% (95% CI: 6.2-17.4%) (HR: 2.57; 95% CI: 1.48-4.45), while rhythm disorders developed in 27 patients with SSc with cumulative incidence of 27.3% (95% CI: 17.9-41.6%) vs. 43 non-SSc patients with cumulative incidence of 18.0% (95% CI: 12.3-26.4%) (HR: 1.62; 95% CI: 1.00-2.64). Age, pulmonary hypertension and smoking were identified as risk factors. Conclusion: Patients with SSc have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to non-SSc patients. These findings warrant increased vigilance and screening for ECG abnormalities in SSc patients with pulmonary hypertension.