Cardiac Manifestation of Rosai-Dorfman Disease: A Case Report and a Systematic Review.

BACKGROUND: Rosai-Dorfman disease is a rare condition that typically presents as a nodal disease. Cardiac involvement is extremely uncommon, occurring in 0.1-0.2% of cases, which has hindered our understanding. We report a case of Rosai-Dorfman disease (RDD) related cardiac manifestation in a patient without nodal involvement. Further, we conduct a comprehensive review of the literature to consolidate data on how patients with cardiac manifestations of RDD are typically managed and treated. METHODS: A systematic review of PubMed, Web of Science, and Embase was conducted to identify cases of RDD with cardiac involvement. Out of 464 studies identified, 42 publications encompassing 43 patients met the criteria and were incorporated in this review. We gathered data on patient demographics, as well as their management and treatment approaches. Additionally, we share our own experience with a patient who presented with a cardiac mass related to RDD. RESULTS: Out of the 43 patients, only 20.9% (n = 9) had a documented history of RDD prior to cardiac manifestations. Nodal involvement was reported in 32.6% (n = 14), while extranodal extracardiac involvement was reported in 46.5% (n = 20). Upon presentation, the most prevalent symptoms were dyspnea (48.8%, n = 21), chest discomfort (41.9%, n = 18), and lower extremity edema (16.3%, n = 7). Cardiac manifestations were most frequently found in the right atrium (41.9%, n = 18) and pericardium (18.6%, n = 8). Treatment encompassed systemic medical therapy (34.9%, n = 15) and cardiac surgery (39.5%, n = 17). The median follow-up period was 12 months (with a range of 1 to 36), and 8 patients (18.6%) experienced mortality. Our patient, who had a cardiac mass in the left atrium, underwent resection and has remained symptom-free without any recurrence for the past 5 years. CONCLUSION: The frequency of cardiac related-RDD manifestations may be greater than initially perceived. These results underscore the significance of identifying RDD and its cardiac-related presentations, facilitating timely diagnosis and treatment for affected individuals.

Aortic Valve Repair Using HAART 300 Geometric Annuloplasty Ring: A Review and Echocardiographic Case Series.

Aortic valve repair (AVr) aims to preserve the native aortic leaflets and restore normal valve function. In doing so, AVr is a more technically challenging approach than traditional aortic valve replacement. Some of the complexity of repair techniques can be attributed to the unique structure of the functional aortic annulus (FAA), which, unlike the well-defined mitral annulus, is comprised of virtual and functional components. Though stabilizing the ventriculo-aortic junction (VAJ), a component of the FAA, is considered beneficial for patients with chronic aortic insufficiency (AI), the ideal AVr technique remains a subject of much debate. The existing AVr techniques do not completely stabilize the VAJ which may increase susceptibility to recurrent AI due to VAJ dilation. An emerging new technique showing promise for the treatment of both isolated and complex AI is AVr using HAART 300TM geometric annuloplasty ring (GAR). The GAR is implanted below the valve leaflets in the left ventricular outflow tract (LVOT), providing stability and creating a neo-annulus. As with other AVr subtypes, this procedure has a learning curve. There are unique surgical and echocardiographic aspects of AVr with GAR, including the appearance of the LVOT, the aortic valve leaflets, and their motion which cardiac anesthesiologists and echocardiographers must be familiar with. In this work, using an eight-patient echocardiographic case series, we provide an overview of this novel AVr technique, including some unique aspects of device sizing, patient selection, expected post-repair echocardiographic features, and a review of outcomes data.

Robotic approach to a subcarinal functional paraganglioma.

INTRO: Functional mediastinal paragangliomas arise from extra-adrenal tissues and are rare. These cases create challenges related to diagnosis, peri-operative management, and surgical management. We present a case that demonstrates a planned robot-assisted thoracoscopic resection of a mediastinal paraganglioma that ultimately required a trans-sternal resection of the tumor off the left atrium. CASE REPORT: Our patient is a 42-year-old male with a prolonged history of refractory hypertension, palpitations, headaches, and diaphoresis, which led to the discovery of a subcarinal functional mediastinal paraganglioma. The patient was brought to the operating room for a right robotic-assisted thoracoscopic subcarinal dissection with attempted resection of the mass. Subsequently, the patient's paraganglioma was successfully resected off the left atrium using a trans-sternal approach, cardiopulmonary bypass, and cardioplegic arrest. He was successfully transitioned to minimal anti-hypertensive medication post-operatively. DISCUSSION: Pheochromocytomas are neural-crest derived tumors that typically arise from the adrenal medulla. Rarely, paragangliomas arise in the thoracic cavity, at an approximate incidence of 2%. Our sequential approach offered the potential for a minimally invasive resection, and though initially unsuccessful, safely elucidated the feasibility of resection using cardiopulmonary bypass after confirming no invasion of the airway, esophagus, or other mediastinal structures.

Coronary Revascularization in High-Risk Stable Patients With Significant Comorbidities: Challenges in Decision-Making.

PURPOSE OF REVIEW: There is a growing cohort of complex high-risk patients with stable ischemic heart disease (SIHD) who present for coronary artery bypass graft (CABG) surgery and percutaneous coronary intervention (PCI). These patients are older, have complex coronary disease, and a substantial comorbidity burden including frailty. The procedural risks and outcomes of CABG and PCI in these patients are more difficult to assess based on the available literature, which has generally studied a younger population with a lower comorbidity burden. RECENT FINDINGS: There have been initiatives to recalibrate and expand risk models derived from procedural registries to inform the care of complex higher-risk patients, including patients "turned down" for CABG. There is greater recognition of the need for improved assessment of risk, quality, and benefits of coronary revascularization in higher-risk SIHD patients with a substantial comorbidity burden. Clinicians and patients should be aware that there are significant evidence gaps regarding revascularization in complex high-risk patients. The limitations of procedural-derived risk scores should be understood when presenting treatment options. Future randomized controlled trials and expanded registries are greatly desired and should be achievable. Meanwhile, a multidisciplinary heart team approach should be employed for proper decision-making.

Circulating MIR148A associates with sensitivity to adiponectin levels in human metabolic surgery for weight loss

Objective: We sought to discover secreted biomarkers to monitor the recovery of physiological adiponectin levels with metabolic surgery, focusing on epigenetic changes that might predict adiponectin function. Design: We conducted a prospective observational study of patients undergoing metabolic surgery by Roux-en-Y Gastric Bypass (RYGB) for weight loss in a single center (IRB GHS # 1207-27). Methods: All patients (n = 33; 27 females; 6 males) signed informed consent. Metabolites, adiponectin and MIR148A were measured in fasting plasma. We followed MIQE for transcript profiles. Results: Patients lost on average 47 ± 12% excess BMI (%EBMI) after 12 weeks. Adiponectin pre, post or delta (post minus pre) did not correlate with %EBMIL. A decrease in adiponectin following weight loss surgery was observed in a subset of patients, chi-square test of independence rejects the null hypotheses that the liver DNA methyltransferase 1 (DNMT1) and delta adiponectin are independent (chi-square statistics χ2 = 6.9205, P = 0.00852, n = 33), as well as MIR148A and delta adiponectin are independent (chi-square statistics χ2 = 9.6823, P = 0.00186, n = 33). The presence of plasma MIR148A allows identification of patients that appear to be adiponectin insensitive at baseline. Conclusion: We combined the presence of plasma MIR148A, the concentration of total adiponectin and the expression of DNA methyltransferase 1 (DNMT1) in liver biopsy tissue to identify patients with non-physiological adiponectin. Weight loss and physical activity interventions complemented with the new method presented here could serve to monitor the physiological levels of adiponectin, thought to be important for long-term weight loss maintenance.

Developing a new hybrid revascularization program: a road map for hospital managers and physician leaders.

Hybrid coronary revascularization, which involves minimally invasive direct coronary artery bypass surgery using the left internal mammary artery to left anterior descending and percutaneous coronary intervention using drug-eluting stents for the remaining diseased coronary vessels, is an innovative approach to decrease the morbidity of conventional surgery. Little information is available to guide hospital managers and physician leaders in implementing a hybrid revascularization program. In this article, we describe the people-process-technology issues that managers and leaders are likely to encounter as they develop a hybrid revascularization program in their practice.