Cardiac Myxomas in Carney Complex: Single Institution Multidecade Experience.

BACKGROUND: We present our surgical experience with cardiac myxomas in the setting of Carney complex (CNC). METHODS: We searched our institutional data explorers to identify patients diagnosed with CNC. We gathered clinical, surgical, and recurrence data from electronic medical records. In total, 38 patients with CNC were documented from 1970 through 2023. RESULTS: Cardiac myxomas developed in 24 patients (63.1%) in the setting of CNC. The median age of onset for cardiac myxoma occurrence was 39.0 years (interquartile range [IQR], 25.0-56.0 years). Most patients were females (62.5%), and all underwent surgery. A total of 42 myxomas (52.7%) were extracted from the left atrium, 12 (15.0%) from the right ventricle, 11 (13.7%) from the right atrium, and 6 (7.5%) from the left ventricle. Among the 24 myxoma patients, 13 (54.1%) experienced at least 1 myxoma recurrence. The median time for the first myxoma recurrence was 7.5 years (IQR, 3.8-10.0 years). There were 27 recurrences (52.9%) from the same chamber, 11 (29.4%) from different chambers, and the localizations in 9 (17.6%) were undocumented. The freedom from tumor recurrence was 100% (95% CI, 100%-100%), 66.7% (95% CI, 44.7%-99.5%), and 16.7% (95% CI, 4.7%-59.1%) at 1, 5, and 10 years, respectively. The long-term survival was 100% at 10 and 15 years. CONCLUSIONS: Cardiac myxomas developed in nearly two-thirds of CNC patients (63.1%) in this study, and more than half (54.1%) experienced recurring instances. Consistent monitoring through echocardiograms is essential for detecting asymptomatic first-time occurrences or recurrences. Surgical removal remains the key treatment method for managing cardiac myxomas associated with CNC.

Echocardiographic findings in cancer-associated non-bacterial thrombotic endocarditis: clinical series of 111 patients from a single institution.

AIMS: Echocardiographic assessment of cancer-associated non-bacterial thrombotic endocarditis (Ca-NBTE) is limited to case reports and small clinical series. The study aimed to identify heart valve abnormalities and its relation to embolic complications and cancer types. METHODS AND RESULTS: Manual review of echocardiographic images and medical records of Mayo Clinic patients (31 March 2002-30 June 2022) was performed. Ca-NBTE in 111 patients (mean age 63.2 ± 9.7 years, 66.7% female) predominantly affected mitral valves (MV) (69), 56 aortic (AV), 8 tricuspid (TV), and rarely pulmonic (PV) (1). In 18 patients, 2 valves were involved, 3 and 4 valve involvement in only a single patient each. Embolic complications were prevalent (n = 102, 91.9%). Ca-NBTE affected MV more frequently on the upstream (atrial) (90% vs. 49.3%) and TV downstream (ventricular) side (75% vs. 37.5%). NBTE size (cm) varied significantly among valves, with TV hosting the largest masses (0.63-2.40 × 0.39-1.77), compared with MV [(0.11-1.81 × 0.11-1.62), (length P = 0.001; width P = 0.03)] and AV [(0.20-2.70 × 0.11-1.51), (length P = 0.001; width P = 0.056)]; MV masses were borderline longer in systemic compared with cerebral emboli (P = 0.057). Majority of MV (79.6%) and AV (69.6%) had thickened leaflets. NBTE lesions commonly affected closing margins (73.9% MV, 85.7% AV, and 62.5% of TV) but rarely commissures of MV (8.7%), yet fairly frequently of AV (41.1%). Five patients had severe regurgitation of MV and 5 AV. CONCLUSION: Ca-NBTE manifests mainly as thrombotic mobile masses attached to thickened MV and AV, with distinct variations in size based on valve type. Embolic destination but not cancer type is associated with NBTE mass size and location.

A Case Series of Minimally Invasive Robotic-Assisted Resection of Cardiac Papillary Fibroelastoma: The Mayo Clinic Experience.

Papillary fibroelastomas (PFEs) are small, slowly growing benign cardiac tumors with clinically significant risk of embolization. Surgical excision is the definitive treatment of symptomatic PFE and is conventionally performed through a median sternotomy. In this study, we report a series of 12 patients, who underwent robotic-assisted PFE removal at the Mayo Clinic. PFE involved the mitral valve, left atrium, and tricuspid valve. No major complications occurred after the procedure, and most patients were discharged 4 days after the surgery. On follow-up, 1 patient demonstrated pericarditis.

Cancer-associated non-bacterial thrombotic endocarditis-Clinical series from a single institution.

Premortem clinical presentation of cancer-associated non-bacterial thrombotic endocarditis (Ca-NBTE), therapy, and the clinal course is limited to case reports and small clinical series. An electronic search of Mayo Clinic records (03/31/2002-06/30/2022) with a subsequent manual review was performed to identify adult patients with echocardiographically detected NBTE and active malignancy, excluding those with infectious endocarditis or lupus anticoagulant/antiphospholipid antibodies. In this retrospective cohort study, we analyzed 115 Ca-NBTE patients (mean age 63.2 ± 9.7 years, 66.1% female) involving 71 (61.7%) mitral, 58 (50.4%) aortic, 8 (6.9%) tricuspid, and 1 (0.9%) pulmonary valve. The most common cancer was lung (n = 45 cases (39.1%), followed by pancreatic (n = 19, 16.5%), gynecological (17, 14.8%), gastrointestinal (n = 10, 8.7%), and 10 (8.7%) with hematologic malignancy; 6 patients had two active cancers. Embolic complications at presentation were frequent: 94 (81.7%) brain, 11 splenic, 10 renal, 6 coronary, and 4 to the extremities. Of 104 anticoagulated patients, 60 received low molecular weight heparin, 17 unfractionated heparin, 16 apixaban, 8 warfarin, and 3 rivaroxaban. There were 18 arterial thromboembolisms; the Kaplan-Meier estimates of the incidence at 2 years were consistent with a rate of 15.9% [95% Confidence Interval (CI) 9.9-23.3], including 14 strokes (12.4%, 95%CI, 7.1-19.2), and 8 other arterial emboli (10.5%, 95%CI, 4.7-18.9); there were 10 venous thromboembolisms (8.9%, 95%CI, 4.5-15.0). Fourteen major bleedings occurred (12.8%, 95%CI, 7.3-19.9) and 94 patients died during follow-up (77.9%, 95%CI, 71.1-85.8). Ca-NBTE predominantly affected women with lung adenocarcinoma or digestive tract cancers and manifested by stroke with high mortality and frequent embolic and bleeding complications during anticoagulation therapy.

Case Report: A myxoma with a far reach.

A 73-year-old woman presented to the emergency department with a syncopal episode and a history of dizzy spells. A transthoracic echocardiogram demonstrated a large left atrial mass extending into the right upper pulmonary veins. Subsequently, cardiac magnetic resonance imaging and coronary computed tomography angiography with three-dimensional reconstruction and printing of the heart and mass were performed, which demonstrated a high index of suspicion for an atypical left atrial myxoma. The mass was excised robotically, and the pathology report confirmed a diagnosis of myxoma.

Oral Health Clearance Outcomes for Cardiovascular Surgery.

Objective: To determine the risk of morbidity and mortality in patients receiving dental extractions before planned cardiovascular surgery (CVS) and examine factors that may affect the chance of oral health clearance. Patients and Methods: A retrospective medical record review was performed of patients who underwent dental screening before CVS from January 1, 2015, to December 31, 2021, at a major medical institution. A total of 496 patients met the inclusion criteria and were divided into 2 groups. Group 1 patients were cleared to advance to planned CVS (n=390). Group 2 patients were not cleared for surgery and subsequently underwent dental extractions before planned CVS (n=106). Results: Six patients (5.7%) experienced postoperative complications after dental extraction that resulted in an emergency room visit. No deaths occurred after dental extraction before CVS. However, 4 patients died within 30 days of CVS, 3 from Group 1 (0.77%) and 1 from Group 2 (0.94%). Dental extraction before planned CVS showed a borderline significant association with death based on unadjusted (P=.06) and age-adjusted analysis (P=.05). Patients who reported seeing a dentist routinely had a significantly higher chance of oral health clearance (P <.001). No differences were noted between the 2 groups with regard to age, sex, or 30-day hospital readmission rate. Conclusion: Patients who had dental extractions completed before planned CVS may be at an increased risk of mortality. Further studies are needed to examine this relationship. Emphasis should be on prioritization of routine dental visits before planned CVS.

Resection of a Solitary Right Ventricular Metastasis in Oligorecurrent Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC), constituting the predominant manifestation of liver cancer, stands as a formidable medical challenge. The prognosis subsequent to surgical intervention, particularly for individuals presenting with a solitary tumor, relies heavily on the degree of invasiveness. The decision-making process surrounding therapeutic modalities in such cases assumes paramount importance. This case report illuminates a rather unusual clinical scenario. Here, we encounter a patient who, following a disease-free interval, manifested an atypical presentation of HCC, specifically, a solitary cardiac metastasis. The temporal interval of remission adds an additional layer of complexity to the case. Through a multidisciplinary planning process, the decision was made for surgical removal of the metastatic tumor.

Case report: New is not always better: treatment of non-bacterial thrombotic endocarditis.

An elderly female with metastatic adenocarcinoma of the lung and atrial fibrillation presented with multiple embolic strokes while on anticoagulation with Apixaban. After further investigation, a TEE showed lesions of non-bacterial thrombotic endocarditis on the mitral valve. A decision to switch the patient to LMWH for anticoagulation was then made and a follow-up TEE showed resolution of the NBTE. In this abstract, we show that heparin should remain as the anticoagulation agent of choice in the setting of NBTE associated with malignancy.

Trends in the Prevalence of Infiltrative Cardiomyopathy Among Patients With in-Hospital Cardiac Arrest.

Sarcoidosis, amyloidosis, hemochromatosis and scleroderma are the most forms of infiltrative/nonischemic cardiomyopathy (NICM) associated with sudden cardiac death. In patients who undergo in-hospital cardiac arrest, a high index of suspicion is required to rule out NICM as an underlying contributor. We aimed to analyze the prevalence of NICM among patients with in-hospital cardiac arrest and identify factors associated with increased mortality. We analyzed data from the National Inpatient Sample, and identified patients who were hospitalized across 10 years from 2010 to 2019 with a diagnosis of cardiac arrest and NICM. The total number of patients with in-hospital cardiac arrest was 19,34,260. The total number with NICM was 14,803 (0.77%). Mean age was 63 years. Overall prevalence of NICM across the years ranged between 0.75% to 0.9%, with a significant temporal increase (P < 0.01). Incidence of in-hospital mortality ranged between 61% to 76% for females and 30% to 38% for males. The following comorbidities were more prevalent in patients with NICM than those without: heart failure, chronic obstructive pulmonary disease (COPD), chronic kidney disease, anemia, malignancy, coagulopathy, ventricular tachycardia, acute kidney injury and stroke. The following factors were independent predictors of in-hospital mortality-age, female gender, Hispanic race, history of COPD and presence of malignancy (P = 0.042). The prevalence of infiltrative cardiomyopathy in patients with in-hospital cardiac arrest is increasing. Females, older patients and Hispanic population are at an increased risk of mortality. Sex and race-based disparities in the prevalence of NICM in patients with in-hospital cardiac arrest is an area of further research.

Clinical Impact of Cardiac Fibromas.

Cardiac fibromas are rare primary tumors that can cause significant morbidity and mortality. There has not been a large clinical case review since 1994. This study provides an updated analysis of clinical impact, thereby enhancing understanding, increasing awareness, and revealing important factors in the diagnosis and management of cardiac fibromas. A retrospective case series was conducted at a tertiary care institution by reviewing radiology, surgical and pathology archives (1964 to 2020). Cases were included if cardiac fibroma was diagnosed through imaging or pathology. Demographics, symptomatology, electrophysiologic data, radiographic findings, pathology, interventions, and outcomes were examined. A total of 26 patients with cardiac fibromas were identified, including 12 women. The median age was 20.5 years (0 days to 72 years). Symptoms included palpitations (commonly due to ventricular tachycardia, 31%), syncope (15%), angina (15%), heart failure (12%), emboli (4%), and murmur (27%). One patient had Gorlin syndrome. A total of 22 patients were diagnosed through imaging, 15 of whom were biopsy-confirmed. A total of 9 patients were initially observed. A total of 2 eventually had surgery, 1 was lost to follow-up, 3 were asymptomatic, 1 had heart failure and atrial fibrillation, and 1 had atrial fibrillation and tachy-brady syndrome, requiring ablation and pacemaker placement. A total of 19 underwent resection. A total of 4 required complex operations, 1 required a second resection, and 1 operative death occurred. In conclusion, cardiac fibromas primarily affect the pediatric population; however, this study demonstrates a significant prevalence in adults. Ventricular tachycardia is common, and multimodality imaging is diagnostically sensitive. Resection is largely successful in symptomatic patients. Surveillance may be appropriate for asymptomatic patients.

Operative management of cardiac papillary fibroelastomas.

OBJECTIVE: Papillary fibroelastomas are associated with an increased risk of embolic strokes. Excision of papillary fibroelastomas may be the primary indication for surgery (primary) or performed during other cardiac operations (secondary). The present study summarizes our experience with primary and secondary fibroelastoma surgery. METHODS: We analyzed the medical records of patients who underwent surgical excision of papillary fibroelastoma between January 1998 and February 2020. Patient characteristics, indications for operation, tumor size and location, and operative and long-term outcomes were evaluated. RESULTS: Among the 294 patients (median age: 66 years, 62% female), papillary fibroelastoma was the primary indication for surgery in 136 patients (46%), and 51% of patients had a history of stroke or transient ischemic attack. When papillary fibroelastoma was a secondary indication for surgery (158 patients, 54%), the lesion was identified preoperatively in 39%. Papillary fibroelastomas were located most commonly on the aortic valve and least commonly in the right side of the heart. For valvular papillary fibroelastoma resected from a normal valve, valve shave was sufficient in 96% (196/205). Operative mortality was low in both groups (primary, 0% vs secondary, 2.5%, P = .13), and early neurologic events occurred in 1.3%. Recurrence rate was 15.8% at 10 years. The estimated survival for patients with primary papillary fibroelastoma at 10 years was 78.4%, whereas for secondary papillary fibroelastoma removal it was 53.6% (log rank, P = .003). CONCLUSIONS: Resection of papillary fibroelastomas can be performed safely, with preservation of the native valve, and with low rates of neurologic events. Operative and long-term outcomes after fibroelastoma resection are excellent.

Recurrence of Pathologically Proven Papillary Fibroelastoma.

BACKGROUND: Papillary fibroelastoma (PFE) is the most common primary benign cardiac tumor. Because PFEs have the potential to embolize, they often are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. We aimed to determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. METHODS: We retrospectively identified all patients from a single center with pathologically proven PFE, treated from January 1995 through December 2018. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. We compared echocardiographic images obtained intraoperatively (after excision) and at dismissal with those of the most recent examination to assess the possibility of PFE recurrence. RESULTS: We included 98 patients in the study. The mean duration of follow-up was 5.4 (SD 3.7) years (range, 1 to 17); the median duration of follow-up was 4.3 years (interquartile range, 1.9 to 7.7). Twelve patients (12.2%) had echocardiographically supported PFE recurrence. Three patients had the recurrent lesion surgically reexcised, and pathologic analysis showed that two were recurrent PFEs and one was a Lambl excrescence. Initial clinical presentation of stroke or transient ischemic attack was more common for the recurrence group (for the first PFE) than for the nonrecurrence group (83% vs 26%; P < .001). CONCLUSIONS: Contrary to findings from previous studies, PFEs do recur after surgical excision. These findings emphasize the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses.

Clinical and echocardiographic characteristics of patients with pathology proven right-sided papillary fibroelastomas.

BACKGROUND: Papillary fibroelastoma (PFE) are one of the most common primary cardiac neoplasms. They are most commonly found on cardiac valves especially on the left side of the heart and have been associated with an increased risk of embolization. To date, no large studies looked at the clinical significance of right-sided PFE (R-PFE). Therefore, we sought to better characterize patients with R-PFE and its clinical sequelae. METHODS: We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with R-PFE were analyzed. Medical records and echocardiograms were reviewed for clinical and PFE characteristics. RESULTS: Twenty-nine patients with R-PFE (mean age 70 ± 11 years; 62% women) were included in the analysis. PFEs were located on the right atrial (RA) wall in 31%, tricuspid valve in 34%, right ventricular (RV) wall in 7%, RV outflow tract in 4%, and pulmonary valve in 10% of patients. The remaining patients (14%) had multifocal PFEs. Around 38% of patients had cancer diagnosed before PFE diagnosis, and 34% had associated congenital heart disease. Seventeen (59%) patients had angina or dyspnea at the time of the presentation, and 3 (10%) had embolic symptoms. One patient with PFE located on the RA with associated patent foramen ovale had a stroke at time of presentation. Two patients, one with PFE on the RA wall and another patient with PFE at the tricuspid valve, had pulmonary embolism at the time of presentation. Median maximal length for PFE varied by location, ranging from a media of 8 mm to 16 mm. Of the 12 patients with follow up echocardiogram 1 year after PFE removal; 3/12 (25%) had documented PFE recurrence. CONCLUSION: R-PFE can be present in patients thromboembolic events when there is a right-to-left shunt. They can be a rare cause of pulmonary embolism, and at least in those that had follow-up echocardiograms, had a significant recurrence rate.

Case Report: A Tale of a Cardiac Mass: Looks Like a Papillary Fibroelastoma, Acts Like a Non-bacterial Thromboendocarditis.

Introduction: 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.

Case Report: A Rare Case of Right-Sided Papillary Fibroelastoma in a 1-Year-Old With Congenital Heart Disease.

Introduction: 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.