Long-Term Efficacy Analysis of Surgical Resection of 70 Primary Right Heart Tumors.

INTRODUCTION: The aim of the study was to investigate the clinical characteristics, surgical treatment, and long-term efficacy of primary right heart tumors. METHODS: This study is retrospective analysis of the clinical data of 70 patients with primary right heart tumors admitted to our department between 1980 and 2022 (observation group) and 70 patients with left heart tumors during the same period (control group). The surgical treatment was performed under cardiopulmonary bypass after differential diagnosis by echocardiography, cardiac CTA, and PET-CT before the surgery. The perioperative characteristics, recurrence rate, and long-term survival rates of right heart tumor versus left heart tumor were compared. RESULTS: The most common pathological types of right heart tumors were myxoma (60%), lipoma (8.57%), and papillary elastofibroma (7.14%). During the perioperative period, there were 1 case of systemic embolism in the observation group, compared with 6 in the control group (p = 0.026), 13 cases of malignant tumor in the observation group versus 1 in the control group (p = 0.01). During the follow-up period, there were 15 cases of tumor recurrence and 17 cases of death in the observation group versus 4 (p = 0.002) and 7 in the control group (p = 0.006), comparatively. CONCLUSION: Compared with left heart tumors, primary right heart tumors had a higher incidence of malignant tumors and a lower risk of systemic embolism during perioperative period. During the follow-up period, primary right heart tumors had a higher rate of tumor recurrence and a lower long-term survival rate.

Myxoma with rich blood supply in the left atrium.

Cardiac myxoma is the most common primary benign cardiac tumors, mostly found in the left atrium. It was previously reported that the main component of myxoma was myxoid stroma riched in acid-mucopolysaccharide, the blood vessels in which were sparsely distributed, being characterized as hypovascular tumor by contrast echocardiography (CE) and computed tomography angiography (CTA). There are few reports of myxoma with rich blood supply and we report one in the left atrium.

A case of malignant melanoma of the small intestine with cardiac metastasis.

Malignant melanoma (MM) is notorious for its high metastatic potential, with cardiac metastasis being particularly severe as it involves cardiac structures and can lead to significant cardiac functional issues. While there is no standardized treatment approach, early detection and intervention can improve prognosis.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Cardiac Rhabdomyomas Presenting with Critical Cardiac Obstruction in Neonates and Infants: Treatment Strategies and Outcome, A Single-Center Experience.

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC. We present the outcomes of neonates and an infant who received treatment for symptomatic rhabdomyomas at a tertiary cardiology center. Medical records were reviewed to obtain clinical, demographic, and outcome data. Six patients received interventions for symptomatic rhabdomyomas, median age at presentation was 1 day old (range from 1 to 121 days old), and 67% of the patients had a pathogenic mutation in TSC gene. One patient underwent surgical resection of solitary tumor at right ventricular outflow tract (RVOT) successfully. In the four patients with left ventricular outflow tract (LVOT) obstruction, two patients received combined therapy of surgical debulking of LVOT tumor, Stage I palliation procedure, and mTORi and two patients received mTORi therapy. One patient with RVOT obstruction underwent ductal stenting and received synergistic mTORi. Four of the five patients had good response to mTORi demonstrated by the rapid regression of rhabdomyoma size. 83% of patients are still alive at their latest follow-up, at two to eight years of age. One patient died on day 17 post-LVOT tumor resection and Hybrid stage one due to failure of hemostasis, in the background of familial factor VII deficiency. Treatment of symptomatic rhabdomyoma requires individualized treatment strategy based on the underlying pathophysiology, with involvement of multidisciplinary teams. mTORi is effective and safe in inducing rapid regression of rhabdomyomas. A standardized mTORi prescription and monitoring guide will ensure medication safety in neonates and infants with symptomatic cardiac rhabdomyoma. Although the majority of tumors responded to mTORi, some prove to be resistant. Further studies are warranted, ideally involving multiple international centers with a larger number of patients.

An extranodal Richter's syndrome presenting with cardiac diffuse large B-cell lymphoma: a case report.

BACKGROUND: Richter's syndrome (RS) defines the transformation of chronic lymphocytic leukemia into high-grade lymphoma, which usually involves lymph nodes and bone marrow. Extranodal involvement of the heart is an extremely rare condition. Patients with heart involvement tended to have a low response to chemotherapy and relative poor prognosis. The transformation process of RS is often insidious and nonspecific making it challenging to diagnose. CASE PRESENTATION: A 64-year-old woman wih a history of chronic lymphocytic leukemia (CLL) presented with intermittent chest pain and was diagnosed with non-ST-elevation myocardial infarction (NSTEMI). However, the contrast enhanced echocardiography revealed a large irregular mass, measuring about 75.4 mm × 37.5 mm, located on the lateral and posterior wall of the right ventricle. Biopsy of the cardiac mass and the results revealed diffuse large B-cell lymphoma. CONCLUSIONS: We present a case of a 64-year-old woman with aggressive diffuse large B-cell lymphoma involving the heart. This case could provide some insights in the diagnosis of cardiac lymphoma.

Unusual Location of a Papillary Fibroelastoma Causing a Multi-territorial Stroke.

A 56-year-old male presented with a multi-territorial stroke without traditional cerebrovascular risk factors. A transesophageal echocardiogram revealed an intracardiac lesion attached to the lateral wall of the left atria, consistent with an atrial myxoma. Surgical excision of the lesion was performed and revealed that lesion was in fact a papillary fibroelastoma with thrombus attached, which demonstrates a novel mechanism by which intracardiac masses can cause cerebral events.

Hot-snare assisted percutaneous mechanical aspiration of a calcified amorphous tumor.

A 24-year-old female with history of an atrial septal defect post-patch closure (bovine pericardium) presented 4 years postoperative with an incidentally identified mass originating from the septal patch .

Arrhythmogenic Left Ventricular Mass as the Initial Presentation of Cardiac Sarcoidosis.

Cardiac tumors of the left ventricle are rare, and cardiac magnetic resonance is the preferred imaging tool for evaluation given superior tissue characterization. We present a case of a patient with arrhythmia and left ventricular mass that was ultimately diagnosed with cardiac sarcoidosis, reminding us that tissue is the issue.

Unusual presentation and delayed diagnosis of cardiac angiosarcoma.

BACKGROUND: Primary cardiac angiosarcomas are very rare and present aggressively with high rates of metastasis. Given the poor prognosis, particularly once disease has spread, early diagnosis and multidisciplinary treatment is essential. CASE PRESENTATION: We present the case of a 46-year-old male who presented with chest pain, intermittent fevers, and dyspnea. Workup with computed tomography scan and transesophageal echocardiography demonstrated a right atrial pseudoaneurysm. Given the concern for rupture, the patient was taken to the operating room, where resection of the pseudoaneurysm and repair using a bovine pericardial patch was performed. Histopathology report initially demonstrated perivascular lymphocyte infiltrate. Six weeks later, the patient represented with chest pain and new word finding difficulty. Workup revealed multiple solid lung, pericardial, brain, and bone nodules. Eventual biopsy of a cardiophrenic nodule demonstrated angiosarcoma, and rereview of the original pathology slides confirmed the diagnosis of primary cardiac angiosarcoma. CONCLUSIONS: Primary cardiac angiosarcomas are often misdiagnosed given the rarity of these tumors, but early diagnosis and initiation of treatment is essential. The unique presentation of our case demonstrates that clinical suspicion for cardiac angiosarcoma should be maintained for spontaneous pseudoaneurysm originating from the right atrium.

Complete resection of a giant intrapericardial cardiac synovial sarcoma.

Synovial sarcoma of the heart is a rare tumor. Herein we would like to report a case of giant intrapericardial cardiac synovial sarcoma that originated from the right ventricle and grew outward near the diaphragm. After making adequate preoperative preparation, we performed the surgery as quickly as possible and resected the tumor completely. Based on the identification of the translocation on chromosome 18 rearrangement, the tumor can be diagnosed as a primary cardiac synovial sarcoma. Through this study, we aim to afford more information about cardiac synovial sarcomas as well as a reference for similar cases.

A case of cardiac undifferentiated pleomorphic sarcoma in late pregnancy: A case report.

Cardiac tumors are uncommon and most of them are benign. Although cases of malignant cardiac tumors are rare, it is still necessary to improve awareness in both clinical and pathological diagnosis. Since cardiac tumors often have a high degree of malignancy, it is vital to determine what form of intervention can increase recurrence-free survival and overall survival. In this paper, we report on a 42-year-old woman in the third trimester of pregnancy who had a cardiac undifferentiated pleomorphic sarcoma. According to her medical history, the patient had never had a cardiac tumor or any other disease. She was treated surgically and a left atrial mass was removed immediately after cesarean section. No other treatments were applied after the surgery, and, unfortunately, the tumor reoccurred 6 months later. We reviewed some literature and found one case in which the patient was treated using radiotherapy and survived for another 2 years after the third tumor recurrence. This suggests that neoadjuvant therapy effectively improves the survival rates of such patients.

Calcified amorphous tumor attached to the membranous septum: a rare clinical entity.

We report a rare case of a pedunculated calcified amorphous tumor (CAT) of the left ventricle attached by a stalk to the membranous septum in a 74-year-old woman who presented with a cerebrovascular accident. We believe this is the first report of a CAT attached to the membranous septum.

Association between gut microbiota and malignant cardiac tumors: A two-sample Mendelian randomization study.

BACKGROUND: Recent studies provide compelling evidence linking the gut microbiota to most cancers. Nevertheless, further research is required to establish a definitive causal relationship between the gut microbiota and malignant cardiac tumors. METHODS: The genome-wide association studies (GWAS) data on the human gut Microbiota, included in the IEU Open GWAS project, was initially collected by the MiBioGen consortium. It encompasses 14,306 individuals and comprises a total of 5,665,279 SNPs. Similarly, the GWAS data on malignant cardiac tumors, also sourced from the IEU Open GWAS project, was initially stored in the finnGen database, including 16,380,303 SNPs observed within a cohort of 174,108 individuals within the European population. Utilizing a two-sample Mendelian randomization (MR) methodology, we examined whether there exists a causal association between the gut microbiota and cardiac tumors. Additionally, to bolster the credibility and robustness of the identified causal relationships, we conducted an extensive array of sensitivity analyses, encompassing Cochran's Q test, MR-PRESSO tests, MR-Egger interpret test, directionality test and leave-one-out analysis. RESULTS: Our analysis unveiled seven distinct causal associations between genetic susceptibility in the gut microbiota and the incidence of malignant cardiac tumors. Among these, the Family Rikenellaceae, genus Eubacterium brachy group, and genus Ruminococcaceae UCG009 exhibited an elevated risk of cardiac tumors, while the phylum Verrucomicrobia, genus Lactobacillus, genus Ruminiclostridium5, and an unknown genus id.1868 were genetically linked to a reduced risk of cardiac tumors. The causal relationship between these two bacteria, belonging to the phylum Verrucomicrobia (OR = 0.178, 95% CI: 0.052-0.614, p = 0.006) and the genus Ruminococcaceae UCG009 (OR = 3.071, 95% CI: 1.236-7.627, p = 0.016), and cardiac tumors was further validated through sensitivity analyses, reinforcing the robustness and reliability of the observed associations. CONCLUSION: Our MR analysis confirms that the phylum Verrucomicrobia displays significant protection against cardiac tumor, and the genus Ruminococcaceae UCG009 leads to an increasing risk of cardiac tumor.

First report of a successful surgical management of left atrial myxoma coexisting with pulmonary squamous cell carcinoma and thymic cyst.

BACKGROUND: Primary cardiac tumors, while rare, present significant clinical challenges due to their diverse pathology and presentation. Lung cancer frequently metastasizes to the heart; however, cases involving primary cardiac tumors of different origins alongside primary lung cancer are exceedingly rare in the literature. CASE PRESENTATION: We report the case of a 53-year-old female who presented with hemoptysis and was subsequently diagnosed with a left atrial myxoma, pulmonary squamous cell carcinoma, and a thymic cyst. This coexistence of multiple non-homologous tumors in a single patient is exceedingly rare. CONCLUSION: This case underscores the complexity of diagnosing and managing patients with multiple distinct tumors. The simultaneous occurrence of a primary cardiac myxoma, pulmonary squamous cell carcinoma, and thymic cyst is unprecedented, providing valuable insights for future clinical practice.

Biatrial myxoma in a young lady.

A healthy 28-year-old woman, presenting with a chronic cough for approximately 6 months, was referred for echocardiography. The images revealed the presence of two masses in each atrium without an inter-atrial septal defect. No additional abnormalities were detected during the clinical examinations. Subsequently, the patient underwent a successful surgical procedure for the removal of the cardiac masses.

Spectrum of electrocardiographic abnormalities in a large cohort of cardiac masses.

BACKGROUND: Cardiac masses represent a heterogeneous clinical scenario. Potential electrocardiographic (ECG) red flags of malignancy remain to be investigated. OBJECTIVES: The purpose of this study was to describe the spectrum of ECG abnormalities in a large cohort of cardiac masses and to evaluate potential red flags suggestive of malignancy. METHODS: This was an observational cohort study of 322 consecutive patients with a cardiac mass and available ECG at Bologna University Hospital. All masses were diagnosed by histologic examination or, in the case of cardiac thrombi, by radiologic resolution after proper anticoagulant therapy. Multivariable regression analysis was used to assess potential predictors of malignancy among ECG abnormalities. All-cause mortality at follow-up was evaluated. RESULTS: Of 322 patients, 98 (30.4%) had malignant tumors. Compared with patients with benign masses, those with malignant tumors exhibited a higher heart rate, right-axis deviation, greater depolarization, repolarization abnormalities, and bradyarrhythmia at presentation. Regarding specific ECG features, a higher heart rate on admission (P = .014), bradyarrhythmias (P = .009), ischemic-like repolarization abnormalities (ST-segment deviation, both depression and elevation, and negative T-wave; P <.001), low voltages (P = .001), and right-axis deviation (P = .025) were identified as independent predictors of malignancy. Considering these specific ECG alterations, a malignancy-oriented ECG was associated with higher mortality at follow-up (median 20.7 months). CONCLUSION: ECG frequently is abnormal in cases of malignant cardiac tumors. Some specific ECG changes are strongly suggestive for malignancy and type of infiltration.

Glandular cardiac myxoma with intraepithelial ductal neoplasia features: Report of the first case.

Cardiac myxoma are the most common primary tumor of the heart in adults. In approximately 2-5%, glandular differentiation occurs within these tumors. In the presence of glandular features attention must be taken to exclude and prevent a misdiagnosis of cardiac metastases of adenocarcinoma. Nevertheless, the localization in the left atrium, the solitary disposition of the cardiac mass, the histological features and the immunohistochemistry performed, argued against the possibility of a metastatic nature of the tumor. We report the case of an 80-year-old woman, with a prior medical history of breast cancer, that underwent surgery for a cardiac myxoma that histologically showed glandular features. Herein, we highlight the importance of a careful diagnosis of this entity, as it can be easily confused for a metastasis, especially in patients with a history of malignancy.

A rare inflammatory myofibroblastic tumor appearing both inside and outside the heart.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is an uncommon cardiac tumor that primarily affects infants, children, and young adults. While complete surgical resection generally leads to a favorable prognosis, accurate diagnostic tests remain limited. CASE PRESENTATION: We describe the case of a 26-year-old female who had a dual tumor inside and outside the heart and was misdiagnosed by echocardiography and MRI. We also review 71 cases of cardiac IMTs from the literature regarding their epidemiology, clinical presentation, and outcome. CONCLUSION: Early detection of this rare disorder is essential for optimal surgical management.

Clinical characteristics and surgical outcomes of cardiac myxoma: A meta-analysis of worldwide experience.

Cardiac myxoma is the most common primary cardiac tumor. However, existing literature mainly consists of single-center experiences with limited subjects. This systematic review aimed to provide data on clinical characteristics and surgical outcomes of cardiac myxoma. We performed a thorough literature search on May 23, 2023 on PubMed, ProQuest, ScienceDirect, Scopus, and Web of Science. The inclusion criteria were English full-text, observational studies, and included >20 subjects. From the search, 112 studies with a total of 8150 patients were included in the analysis. The mean age was 51 years (95 % confidence interval [95%CI] = 49.1-52.3), and the majority were females (64.3 % [95 % CI = 62.8-65.8 %]). The most common clinical manifestation was cardiovascular symptoms. Echocardiography can diagnose almost all cases (98.1 % [95 % CI = 95.8-99.6 %]). Cardiac myxoma was mostly prevalent in left atrium (85.3 % [95%CI = 83.3-87 %]) and predominantly with pedunculated morphology (75.6 % [95%CI = 64.1-84.3 %]). Post-tumor excision outcomes were excellent, with an early mortality of 1.27 % (95 % CI = 0.8-1.8 %), late mortality rate of 4.7 (95 % CI = 2.5-7.4) per 1000 person-years, and recurrence rate at 0.5 (95 % CI = 0.0-1.1) per 1000 person-years. Tumor excision is warranted in a timely manner once the cardiac myxoma diagnosis is established.