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We report an antenatal presentation of a huge pericardial mature teratoma that was referred as congenital pulmonary airway malformation (CPAM) in the late third trimester of pregnancy. Initial ultrasound evaluation revealed a huge predominantly cystic lesion with mixed echogenicity in the left hemithorax. A provisional diagnosis of pleural tumor was considered in view of previous scans at 20‒28 weeks being normal and associated pleural effusion. Magnetic resonance imaging of the fetus reported the lesion to be CPAM which was supported by postnatal computed tomographic imaging done on day 2 of life. However, intraoperatively, the lesion was found to be of pericardial origin which on subsequent histopathological examination was confirmed to be mature teratoma. We recommend considering potential differential diagnosis other than CPAM, especially when the lesion is found for the first time in the late third trimester. [ABSTRACT FROM AUTHOR]

Right ventricular outflow tract obstruction (RVOTO) is a cause of hemodynamic instability that can lead to right ventricular dysfunction. Cardiac tumors located in the right ventricle or surrounding structures can cause RVOTO. Herein, we present a rare case of a 21-year-old male with palpitations due to RVOTO caused by a cardiac multicomponent mesenchymal tumor. The tumor was localized in the right ventricular outflow tract, resulting in right side heart enlargement, tricuspid regurgitation, and RVOTO. Hence, tumor resection was performed. The patient was in a stable condition and discharged home on the 6th post-operative day. However, histopathological examination of the tumor specimen suggested a three-component mesenchymal tumor containing mucinous components, formed blood vessels, and fibrous tissue, which is like an atypical capillary hemangioma. After seven years of follow-up, the patient had no right heart enlargement, tricuspid regurgitation, and tumor recurrence. We believe surgical treatment is effective, and this case will provide a reference for clinicians to treat and evaluate the prognosis of similar three-component mesenchymal cardiac tumor cases in the future.

BackgroundCardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors most commonly found on left-sided cardiac valves. Right atrial CPFs are extremely rare, accounting for only 2% of all CPFs. Median sternotomy is a typical approach for surgical excision of CPFs in most cases. Herein, we report an extremely rare case of multifocal CPFs involving the right atrium and aortic valve that were surgically excised via minimally invasive right anterolateral thoracotomy.Case SummaryA 59-year-old Chinese man was admitted because of an incidental finding of a right atrial mass on transthoracic echocardiography during a routine check-up. The mass was initially diagnosed as a myxoma, and the patient was scheduled for minimally invasive excision via right anterolateral thoracotomy. An additional mass on the non-coronary cusp of the aortic valve was identified using intraoperative transesophageal echocardiography. The patient still underwent complete tumor excision via right anterolateral thoracotomy. Both neoplasms were pathologically diagnosed as CPFs.ConclusionsThis case highlights the need for a comprehensive cardiac evaluation of cardiac tumors because CPFs can manifest as multifocal lesions. Moreover, minimally invasive surgery is highly feasible as the CPF can be easily excised, and the valve can usually be preserved.

We analyzed 54 cases of cardiac myxoma (n = 40) and non-myxoma (n = 14) diagnosed at a single community-based tertiary care center over 20 years. The data were retrospectively collected for patients between the period January 2000 and September 2020 from the hospital database. We described patient characteristics and clinical features of cardiac myxoma. In patients with diagnosis of cardiac myxoma, the median age was 64 years (range 41–78), with 58% females. Cardiac myxoma patients presented in a variety of ways, as dyspnea (42%), palpitations (20%), and chest pain (15%). Transesophageal echocardiogram was performed in 82.5% of patients. Chest computed tomography (CT) was performed in 32.5%, while cardiac magnetic resonance imaging (CMRI) was performed in 10%. Ischemic evaluation was performed in the majority of patients, primarily having cardiac catheterization. All cardiac myxomas were a single mass and the most common location was the left atrium (n = 34, 85%), followed by the right atrium (n = 6, 15%). 33 (83%) of them were larger than 2 cm. We differentiated cardiac myxoma from non-myxoma mass, which was most commonly a thrombus by histopathology. More patients with cardiac myxoma underwent surgical resection and required hospital and ICU stay than non-myxoma patients. No patients in either group experienced inpatient mortality or a mass recurrence with a median follow-up period of 2 years.

Abstract Background The primitive neuroectodermal tumors (PNETs) are a family of highly malignant tumors with a multidirectional differential potential. The tumors are characterized by aggressive small round tumor cells that originate from the spinal cord of the central and sympathetic nervous systems. Cases involving the pericardium are extremely rare. Herein, we present a case of peripheral primitive neuroectodermal tumor (pPNET) that originated in the pericardium. Case presentation A 23-year-old woman presented with cough and progressive dyspnea for 1 month, followed by eyelid and facial edema for 10 days, without any apparent cause. Significantly elevated tumor markers were detected in her blood. A cardiac ultrasound revealed a 74 mm × 61 mm spherical mass that was attached to the left pericardium, as well as massive pericardial effusion. Positron emission tomography-CT (PET-CT) showed focal hypermetabolism in the left pericardium. Via histopathology and immunohistochemistry, the spherical mass was identified as PNETS. The patient was successfully treated with a combination of surgical resection via thoracotomy and postoperative chemotherapy, and she was disease-free for 7 years at follow-up. Unfortunately, at 7 years after the treatment, the patient’s pPNET recurred. Positron emission tomography-MRI (PET-MRI) and 64-slice coronary CTA revealed that the aorta and multiple coronary arteries were involved. Subsequently, the patient refused a heart transplant and voluntarily left the hospital. Conclusions This paper reports on a rare and recurrent case of PNET in the parietal pericardium. With respect to the different biologic characteristics and prognoses of pPNETs (compared to other known pericardium tumors), it is essential to consider this entity as a differential diagnosis in pericardium tumors.

Abstract Background Primary cardiac tumors are rare, and approximately 90% of them are benign. Myxoma is the most common type of these tumors occurring in the left atrium in 75–85% of cases. The tumor can cause the left atrio-ventricular valve obstruction and embolization phenomenon. Case presentation We reported a case of 54-year-old man with complaints of dyspenea and amnesia. In our patient, transthoracic echocardiography revealed a mass of 28*63 mm attached to the upper intra-atrial septum, which was prolapsing through the mitral valve into the left ventricle during diastole, being indicative of the left atrial myxoma. On examination, he was alert and conversant, and no pathological abnormality was observed in the examination of cardiovascular, gastrointestinal, respiratory, hepatic, renal and nervous systems. After myxoma diagnosis, the tumor was removed under cardiac surgery and discharged under good conditions. In the telephone follow-up after discharge, the patient recovered and did not report the disease and surgery complications. Conclusions Patients with cardiac myxoma are usually asymptomatic, but they may have manifestations related to the embolism phenomenon or intracardiac obstruction. Therefore, myxoma may represent an emergency. Surgery should be performed as soon as possible. If surgery is delayed, the patient may suffer from serious and irreversible complications, such as stroke and cardiac arrest.

Cardiac lipoma is a rare tumor and usually presents as lipomatous hypertrophy of the septum. Cardiac mass in the RV usually occurs due to thrombus from venous thromboembolism or cardiac tumor or vegetation. We present a case of cardiac lipoma in an elderly female who presented with dyspnea on exertion and aspergilloma in the lung, with echocardiogram revealing a cardiac mass. Endomyocardial biopsy identified the mass as lipoma. Multimodality approach may help in identifying the correct diagnosis in case of cardiac tumors. Cardiac tumors should be kept as a differential diagnosis in an undiagnosed right ventricular mass not responding to anticoagulation.

• Cardiac myxoma are the most common primary tumor of the heart and myxomas represent the most common ones. • In rare cases, areas of glandular differentiation have been demonstrated in typical myxomas. • In the presence of glandular features attention must be taken to exclude a misdiagnosis of cardiac metastases of adenocarcinoma. 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. [ABSTRACT FROM AUTHOR]

Primary cardiac tumors constitute around 10% of all tumors of the heart. Atrial myxomas are the most common tumors of the heart, constituting around 50%–70% of all tumors. Most of them arise from the left atrium but multiple-site involvement is seen in 5%. Biatrial myxomas are rare and present in around 2.5% of patients. Myxomas can have varied symptoms. We present a case of biatrial myxoma that underwent successful surgical resection.

Intracardiac masses are unusual findings in infants, and most of them are benign. Nevertheless, they may be associated with a significant degree of hemodynamic instability and/or arrhythmias. Malignant tumors of the heart rarely occur in children. Rhabdoid tumors are aggressive tumors with a dismal prognosis even when diagnosed early. Although rhabdomyomas are common cardiac tumors in infants, they are mostly benign. The most common sites of involvement are the kidneys and central nervous system, but soft tissues, lungs, and ovaries may also be affected. The diagnosis can be challenging, particularly in sites where they do not usually occur. In the present paper, we report the case of a 2-year-old boy diagnosed with cardiac rhabdoid tumor highlighting the importance of molecular studies and recent genetic discoveries with the purpose of improving the management of such cases. The aim of this educational case report and literature review is to raise awareness of cardiac masses in children and to point out diagnostic hints toward a cardiac tumor on various imaging modalities. Given the rarity of all tumors involving the heart and the lack of symptom specificity, a high degree of suspicion is needed to arrive at the correct diagnosis. [ABSTRACT FROM AUTHOR]

Background: Considering the few reported cardiac masses, PET/CT in the imaging workup of cardiac masses is not well established. This retrospective study analyzed the role of 18F-FDG PET/CT imaging in cardiac/pericardial masses. Methods and results: Fifty-nine patients with newly diagnosed cardiac/pericardial masses who underwent PET/CT and transthoracic echocardiography (TTE) were recruited. Echocardiographic and PET/CT characteristics were evaluated for predictive value in differentiating malignant and non-malignant lesions using histologic confirmation as the gold standard. The McNemar test was used to test the differences in sensitivity between PET/CT and TTE. 18F-FDG PET/CT had higher sensitivity in determining the malignancy of cardiac/pericardial masses compared to TTE (sensitivity, 96.6% vs 72.4%, P =.039). However, when pericardial masses were excluded from the analysis, the difference in sensitivity between the two was not statistically significant (sensitivity, 95.6% vs 78.3%, P =.219). 18F-FDG PET/CT identified two malignant pericardial masses missed on TTE, changed the diagnostic orientation of TTE in 15 patients, and found seven patients with extracardiac lesions in 29 malignant patients. Conclusions: PET/CT was an effective additional image modality in patients with suspected malignant cardiac mass for further confirmation and to screen for potential metastasis. [ABSTRACT FROM AUTHOR]

Technical details for complex cardiac tumor resection are sparse. We describe the operative technique of modified autotransplantation for resection of a complex pericardial synovial sarcoma in a 63‐year‐old, Caucasian female. Surgical exposure demonstrated tumor origin at the superior cavoatrial junction and invasion of the aorta, main pulmonary artery, superior pulmonary veins, and left atrial roof. Full macroscopic surgical resection was achieved. The patient received adjuvant radiation for microscopic positive margins and remains alive and with no tumor progression at one year postoperatively. We conclude that modified autotransplantation is a challenging but effective surgical technique when performed with careful patient selection and availability of skilled, cardiothoracic surgeons at a cardiac center of excellence. [ABSTRACT FROM AUTHOR]

Abstract The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site ( https://www.scmr.org ) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Abstract Background Primary cardiac schwannoma in the left atrium and schwannomatosis are rare diseases. Case presentation We report the case of a 46-year-old asymptomatic man who had tumor resection for parapharyngeal schwannoma at another institute 1 year ago. He was presented to our hospital for further evaluation of an abnormal cardiac shadow that was found incidentally. Computed tomography and transesophageal echocardiography revealed a cardiac tumor originating from the posterior wall of the left atrium, an atrial septal defect, and two other mediastinal tumors. The cardiac mass was completely excised with normal margins of the surrounding atrial wall. The post-resection defect and atrial septal defect were repaired using bovine pericardium. Pathological findings were compatible with benign schwannoma, and a diagnosis of schwannomatosis was made based on his medical history. Conclusion Primary cardiac schwannoma is an exceedingly rare tumor, and the incidence in schwannomatosis has not been reported in the literature.

Cardiac tumors are rare, and their treatment differs interindividually regarding the histopathological proprieties and the stage of disease. Authors present a case of symptomatic cardiac melanoma metastasis that expressed an ERBB2 (HER2) gene amplification in a course of the disease that has not yet been reported. The frail patient with a history of pulmonary and renal carcinoma, was admitted to the hospital due to a symptomatic left atrial tumor mass. The patient underwent a tumor-resecting cardiac surgery. At first mistaken for myxoma on echocardiography, the histopathological examination of the tumor revealed a melanoma of acral or mucosal origin. The melanoma metastasis was negative for common genetic mutations in BRAF, NRAS or KIT genes, and for the presence of NTRK genes fusions, but carried ERBB2 (HER2) gene amplification. The absence of standard gene mutations rendered it unresponsive to treatment with BRAF and MEK inhibitors. This molecular finding is rare in melanomas and represented a therapeutic target for off-label systemic treatment with drugs, primarily aimed at ERBB2 positive breast, gastric, and gastroesophageal junction cancers. A rare finding like this justifies molecular genetic analysis of unusual tumor specimen and guarantees optimal treatment for uncommon types of cardiac metastatic tumors.

This report describes the surgical treatment of giant right ventricular fibroma in a newborn. Cardiac uhrasonography and CT showed a large mass in the right ventricle wall, which narrowed the right ventricular inflow tract. The newborn patient gradually developed symptoms such as shortness of breath, oliguria, and pericardial effusion. We performed tumor excision, but due to severe damage to the right ventricular wall and right heart failure, the patient relied on cardiopulmonary bypass. Then, we immediately restored the opening of the ductus arteriosus, enlarged the foramen ovale, and used various vasoactive drugs to ensure the smooth resuscitation of the patient. This is a kind of operation for the youngest patients. The perioperative treatment experience indicated the feasibility of excision of giant right ventricular fibroma for newborn patients.

Abstract Background Venous obstruction in dogs caused by large intracardiac masses can result in severe morbidity with few safe treatments. Hypothesis/Objectives Retrospective study to report results after transatrial stent placement in dogs with naturally occurring cardiac masses causing venous obstruction. Animals Three client‐owned dogs diagnosed with large cardiac masses. Methods Retrospective study of patients that received transatrial stents extending from the caudal vena cava, across the right atrium, and into the cranial vena cava (CrVC). Procedures, complications, and outcomes were recorded based upon medical records, referring veterinarians, and client communications. Results Two dogs had similar clinical signs suggestive of congestive hepatopathy including marked ascites and lethargy. One dog had clinical signs of CrVC syndrome including head and neck swelling with pitting edema and pleural effusion. After stent placement, venous pressure gradients were decreased and repeat angiography confirmed that vascular patency was reestablished. Resolution of clinical signs was marked in all 3 dogs with only mild complications including tachyarrhythmias and hypertension in 1 dog during the perioperative period. Two dogs that required additional transatrial stent placement for reobstruction 6 and 14 months later improved after the second stent implantation. Survival times poststenting for the dogs were 3, 21, and 37 months, with cause of death related to the cardiac tumor in all dogs. Conclusions and Clinical Importance Endovascular transatrial stenting may provide a long‐term palliative treatment option for dogs with clinical signs attributable to tumor‐induced venous obstruction when more traditional treatments are declined or not indicated.

Background: Cardiac tumors in infants and children are rare. The most common cardiac tumor is rhabdomyoma, which may be associated with tuberous sclerosis. However, not all cardiac rhabdomyomas are pathognomonic for tuberous sclerosis, and not all congenital cardiac tumors are rhabdomyomas. During the prenatal period, early cardiac tumor detection provides important information about fetal wellbeing, delivery planning, and necessary postnatal care.Case Presentation: We report a 36-year-old African American pregnant women. At 32 weeks 5 days gestational age, the male fetus had a fetal echocardiogram due to fetal arrhythmia. The fetal echocardiogram showed two small echogenic, RV apex and septal masses, suspicious of rhabdomyomas. After a routine pregnancy and a normal spontaneous vaginal delivery (39 weeks 1 day), the male baby was admitted to the neonatal intensive care unit for further monitoring and postnatal evaluation.Conclusions: Rhabdomyomas are extremely rare and unique tumors. These tumors are very dangerous, but they usually regress after birth. During the prenatal period, early cardiac tumor detection provides important information about fetal wellbeing, delivery planning, and necessary postnatal care. We present this case to share our findings with our pediatric colleagues. Although a rarely reported case, we hope this cardiac rhabdomyoma case report and literature review can increase cardiac tumor awareness. [ABSTRACT FROM AUTHOR]

Background: Synovial sarcomas are tumors typically located in the extremities and characterized by a t(X;18)(p11.2;q11.2) chromosomal translocation. With only around 100 cases reported in the literature, cardiac synovial sarcomas are extremely rare.Case Presentation: We describe a case of a 59-year-old male who presented to his primary care physician with chest pain, palpitations, and dyspnea and was diagnosed with atrial flutter. Following atrial ablation, a transthoracic echocardiogram incidentally revealed a 5.5 × 5.0 cm heterogeneous mass. Further workup found a heterogeneous mass with mild fluorodeoxyglucose uptake that was abutting the left atrium, left ventricle, and left pulmonary veins. The tumor was resected and confirmed to be a monophasic synovial sarcoma with a SS18-SSX gene fusion. Four months post-operative, the patient had recovered well from surgery. He is currently undergoing concurrent radiation and chemotherapy.Conclusions: Due to the rarity of this tumor, guidelines on diagnosis and treatment come only from case reports. Our case describes a primary cardiac synovial sarcoma arising from the left atrium in the atrioventricular groove in which diagnosis of atrial flutter preceded detection of the mass. [ABSTRACT FROM AUTHOR]

Background: Most cardiac myxomas occur in the atria. Myxomas arising from the heart valves are rare, and there are only a few reports of myxomas arising from the pulmonary valve. Complete resection and prevention of embolization at the time of the first surgery are important to prevent the recurrence of myxomas. Case presentation: An 82-year-old female was scheduled to undergo surgery for a fracture of the right femoral neck. The preoperative echocardiography showed a mass in the right ventricular outflow tract. The mass was 36 × 30 mm in size and entered into the pulmonary artery during systole. Cardiac synchronous computed tomography showed a stalked bifurcated mass near the pulmonary valve, which was suspected to be a myxoma. Surgical findings showed a lumen-occupying tumor when the main pulmonary artery was incised. Since the tumor was a single mass with a stalk on the pulmonary valve (right and left pulmonary valve cusps), tumor resection and pulmonary valve replacement (bioprosthetic valve) were performed. A right prosthetic femoral head insertion was performed on postoperative day 36, and the patient was transferred to the hospital on postoperative day 44. However, 1 year later, the patient developed a large myxoma (recurrence) that completely occluded the right pulmonary artery and died of right heart failure. Conclusions: We report the case of a patient with a very rare myxoma arising from the pulmonary valve, which was treated with tumor resection and pulmonary valve replacement surgery; however, the patient developed another myxoma 12 months later and this tumor was larger than the primary tumor. The surgical margins were indistinct, and there was a high possibility of residual tumor in the pulmonary artery wall; hence, an extended resection should have been considered. The recurrence of myxoma, in this case, suggests that it is important to completely resect the primary tumor during the first surgery and to prevent intraoperative embolization. [ABSTRACT FROM AUTHOR]

We report the case of a 65‐year‐old male patient who presented with chest pain and was found to have a mediastinal paraganglioma between the left atrium and main pulmonary artery. This is the first reported case of a mediastinal paraganglioma resection utilization transection of the main pulmonary artery. [ABSTRACT FROM AUTHOR]

Lipomatous Hypertrophy of the Interatrial Septum (LHIS) is an unusual condition usually found as an incidental finding on echocardiography. It can create a mass-like bulge typically sparing the fossa ovalis. We describe a 73-year-old overweight Cypriot woman, who visited our clinic in November 2018 and was complaining of dyspnea on exertion, chest tightness, and two episodes of paroxysmal atrial fibrillation for the last eight months. A big sessile mass was detected in the interatrial septum measuring 3.7x4.7 cm during transoesophageal echocardiography. The patient underwent resection of the mass and pericardial patch reconstruction of the defect with significant improvement of her clinical status during the follow-up period. The histopathological analysis suggested LHIS. In patients with persistent cardiac symptoms, a prompt resection is a useful option, although surgery is generally considered unnecessary in this clinical setting. Preprint of this article is available on: https://www.authorea. com/users/380290/articles/496351-a-patient-with-chronic- dyspnea-and-episodes-of-paroxysmal-atrial-fibrillation-in-the- presence-of-a-right-atrial-mass. [ABSTRACT FROM AUTHOR]

Cardiac angiofibroma is a very rare diagnosis when a patient develops an intracardiac mass. It is a primary benign cardiac tumor with a scarcity of information in the literature. This case report illustrates a 26‐year‐old man with a complaint of chronic chest tightness who was first diagnosed with right ventricle tumor by echocardiography then underwent cardiac magnetic resonance imaging (CMR) which confirmed the presence of a highly‐vascular tumor with radiologically benign behavior. Then his tumor was excised, his postoperative course was uncomplicated and he was well within almost 2 months after discharge. Ultimately the histopathologic findings demonstrated vascular and stromal tissue in favor of angiofibroma and excluded the other diagnoses with immunohistochemical and trichrome staining. Angiofibroma is a benign, highly vascular tumor, mostly discovered in the nasopharynx. When it is found in the heart, CMR and pathology are pivotal to rule in its diagnosis. It is isointense in T1 weighted and hyperintense in T2 weighted sequences with intense enhancement following contrast injection. Its pathology contains an admixture of vasculatures with CD31 positive immunoreactivity for endothelial cells and fibrotic tissue with bluish coloration in trichrome staining. Eventually, its treatment includes merely surgical excision given its benign nature. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. • Right-sided PFE can have various serious clinical presentations. • Right-sided PFE can be a cause of systemic emboli if associated with a shunt. • Surgical removal provides an excellent management approach. [ABSTRACT FROM AUTHOR]

This systematic review sought to investigate the current evidence regarding surgical management of primary cardiac tumors in children. Twenty-four studies were deemed eligible, reporting on 713 pediatric patients. Cumulative 30-day mortality rate was 5.5% and 7.5% after surgery. It was revealed a statistically lower mortality rate in the group of patients below 1 year of age, justifying a more aggressive surgical approach for primary cardiac tumors, except for rhabdomyoma that can regress spontaneously. [ABSTRACT FROM AUTHOR]

Cardiac tumours are some of the rarest primary tumours, while cardiac metastasis are more common yet still relatively rare. Seventy five percent of primary cardiac tumours are benign tumours. Cardiac tumours present with a range of obstructive, embolic, arrhythmic or systemic symptoms, and in many cases may present asymptomatically. The clinical presentation depends largely on the size and location of the mass. With advances in cardiac imagining and the introduction of cardiopulmonary bypass, the diagnosis and surgical treatment of these rare tumours has improved the prognosis and outlook for benign and malignant tumours. Management depends on tumour histology, size and location as well as the clinical presentation. Conservative management is reserved for small, benign tumours that can undergo regular echocardiographic follow-up. Symptomatic benign tumours are treated with surgical resection and the results are excellent. Malignant primary cardiac tumours have a poor prognosis with high rates of relapse and a median survival of 10–24 months. [ABSTRACT FROM AUTHOR]

Abstract Cardiac magnetic resonance in cardiac mass: Cardiac magnetic resonance (CMR) should be considered in the evaluation of patients with cardiac mass. Especially, when the diagnosis is not certain, CMR could provide paramount information that could be helpful for the decision on cardiac surgery.

Abstract Atrial myxomas are the most prevalent primary cardiac tumors. The clinical presentation is variable and often poses a diagnostic challenge. Here we describe the case of a 52-year-old woman who presented with troponin-positive chest pain, exertional dizziness, and dyspnea as a consequence of a massive left atrial myxoma, which was successfully treated with surgical resection.

Abstract Background Cardiac tumors are rare, and their diagnosis is challenging. The level of evidence for the management of cardiac tumors came from case reports or small case series. We aim to present our multicenter experience in the diagnosis and management of cardiac tumors. Results We performed a prospective study, including 82 cases of cardiac tumors encountered at three centers in 5 years. The mean age was 62 ± 5.8 years old (range 2 months–74 years), and 91.4% were females constituted. The median EuroSCORE II was 4.27%. All cases underwent radical excision of the tumor. Five cases underwent additional mitral valve replacement and one case underwent coronary bypass grafting. Pathological diagnoses were left atrial myxomas (n = 65, 79.3%), right atrial myxomas (n = 5, 6.1%), bi-atrial myxomas (n = 2, 2.4%), right ventricular outflow tract myxoma (n = 1, 1.2%), myxosarcoma (n = 1, 1.2%), thymoma (n = 1, 1.2%), recurrent hemangioendothelioma (n = 1, 1.2%), round cell tumor (n = 1, 1.2%), cardiac lymphoma (n = 1, 1.2%), rhabdomyomas (n = 2, 2.4%), and renal cell carcinomas (n = 2, 2.4%). Two patients (2.4%) had re-exploration for bleeding. One patient (1.2%) with right atrial thymoma experienced a severe vasoplegia with unstable hemodynamics. Two patients had operative mortality (2.4%), one with recurrent cardiac hemangioendothelioma. During 1-year follow-up, one patient (1.2%) died because of metastatic myosarcoma. Recurrence was reported in 2 patients (2.4%). Conclusions The clinical scenario of cardiac tumors depends mainly on tumor location and size rather than the histopathology. Surgical resection is feasible in benign tumors while very challenging in malignant tumors.

Background. The most common cardiac tumor in children is rhabdomyoma. Its frequency is 58.3% in the early neonatal period. Rhabdomyoma is associate with tuberous sclerosis pretty often (50-86% of all cases) and it be its diagnostic marker.Clinical case description. This article presents case study of the newborn from the mother with burdened obstetric and gynecological anamnesis, type 1 diabetes, nicotine addiction. Multiple mass lesions were revealed in the child’s heart on the 31 st week of gestation. On examination: systolic murmur over the heart region, fibrous nodules on the skin all over the body up to 1-5 mm in diameter, indurated on palpation. Diagnosed echocardiographically: multiple attached, round masses were revealed in the cavity of left ventricle of 7.9×4.1 mm in size, in outflow track of left ventricle (near the aortic root) — 9.3×4.8 mm, in the cavity of right ventricle — 4.3×3.2 mm, in the cavity of right auricle — 5.1×5.0 mm. Pathologic activity of cardiac conduction system was presented only by single supra-ventricular and ventricular extrasystoles due to the results of 24-hour monitoring.Conclusion. Children with cardiac rhabdomyoma diagnosed in prenatal period are threatened by tuberous sclerosis, thus, complex dynamic observation and molecular genetic testing is needed.

Abstract Background A cardiac lipoma is a rare primary cardiac tumor. They are usually asymptomatic and carry a good prognosis. Cardiac Magnetic Resonance Imaging (CMR) is the confirmatory investigation of choice. Case presentation We present a case of left ventricular lipoma in an asymptomatic patient, which was successfully treated with surgical resection. Conclusion Cardiac lipomas are rare and are usually benign. There is no guideline on the management of cardiac lipomas and treatment is individualized.

As the majority of cardiac tumors are benign, malignant tumors may be easily missed. The case presented here was a female with complaints of dyspnea who was diagnosed with a left atrial mass of regular shape. After surgical excision with a pre-diagnosis of hemangioendothelioma in the frozen-section, the operation finished with mitral valve replacement. The pathological diagnosis from the immunohistochemical examination was of low-grade fibromyxoid-sarcoma. At three months postoperatively, the patient was re-hospitalized with dyspnea. Echocardiography detected a giant mass in the left atrium, 45 x 24 mm in size. The hemodynamic collapse resulted in mortality. The idea that cardiac tumors are more frequently benign causes malignant masses to be overlooked. With the perspective of an internal medicine specialist, the characteristics of each cardiac tumor should be evaluated with both clinical and imaging methods before the cardiac surgery.

A 79-year-old female patient who presented with a cardiac mass detected by conventional echocardiography was ultimately diagnosed with a malignant tumor by myocardial contrast echocardiography. A positron emission tomography/computed tomography examination showed tumors in the right atrium consistent with the findings of the contrast-enhanced ultrasound. Finally, the patient was confirmed by pathology to have cardiac lymphoma. Because no lesions were found elsewhere in the body, primary cardiac lymphoma was diagnosed by combining multi-modal imaging examination and pathological examination. Although conventional echocardiography may identify a cardiac mass, it is difficult to identify whether they are malignant or not. Myocardial contrast echocardiography helps to identify the location, shape, and size of the mass, its relationship with the surrounding tissue, and evaluate its blood supply. Thus, this imaging modality is of great value for identifying the likely etiology of a cardiac mass. Multi-modal imaging is complementary to echocardiography for determining the location of cardiac masses, invasion of surround structures, extra cardiac spread, and determination of whether a mass is likely benign or malignant. Multi-modality imaging provides an important basis for clinical treatment and decision-making.

A 69-year-old patient was admitted to the Department of Cardiology due to an accidental finding on a chest X-ray, enlarged heart outline, accompanied by worsening of heart failure to NYHA II with LVEF, about 30%. In the X-ray description, an enlargement of the left atrium silhouette with local calcifications. The patient underwent TTE, confirming the presence of a pedunculated tumor of the left atrium attached to the ceiling measuring 3.5x3.5x2.2 cm. Due to the ischemic heart disease manifestation patient uderwent coronarography confirming the presence of single-vessel coronary artery disease with changes in the middle segment of the LAD. Patient was treated with CABG LIMA-LAD surgery and removeal the left atrial tumor. Post operation tissue material prooved the preseance of left atrium myxoma. The postoperative course was uneventful, the patient was discharged home.

Background: Carney complex is a rare syndrome characterized by skin pigmentation, endocrine disorders, and myxomas. It is particularly notorious for its tendency to exhibit aggressive cardiac myxomas. Herein, we present a case of a juvenile female patient diagnosed with Carney complex who underwent a right lateral mini-thoracotomy., Case Presentation: A 13-year-old girl presented with sudden-onset left hemiplegia and dysarthria. Magnetic resonance imaging revealed multiple areas of restricted diffusion. Echocardiography identified a tumor in the left atrium, suspected to be related to Carney complex based on her medical history and physical examination findings. Surgery was performed via right lateral mini-thoracotomy, which minimized the risk of embolism and ensured a cosmetically favorable outcome. The left atrial wall defect was repaired with autologous pericardium. At 3 years postoperatively, follow-up echocardiography indicated no tumor recurrence and normal cardiac function., Conclusions: Ongoing follow-ups are essential due to the aggressive nature of the Carney complex and its high recurrence rates. Right lateral mini-thoracotomy offers the advantage of avoiding re-sternotomy and minimizing adhesion dissection, making it the optimal choice for this case., (© 2024. The Author(s).)

Papillary fibroelastoma of aortic valve is a rare benign tumor that can present with symptoms of obstruction or embolization and can be asymptomatic. The main stay of diagnosis is echocardiography. The size of the tumor affects the sensitivity of transthoracic echocardiography which can miss small size tumors. The treatment is surgical resection. Here, we report a case of papillary fibroelastoma of aortic valve that was detected intraoperatively by transesophageal echocardiography and was missed by transthoracic echocardiography in a patient undergoing coronary artery surgery. The tumor was resected successfully with preservation of aortic valve with no complication. This case highlights importance of intraoperative transesophageal echocardiography in making the diagnosis., Competing Interests: None declared., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Background: Papillary fibroelastoma (PFE) is an uncommon benign cardiac tumor, typically arising from valvular structures. Nonvalvular PFEs are exceedingly rare and may present with diverse symptoms., Case Presentation: We present a case of a 46-year-old woman with positional vertigo and exertional dyspnea. Transthoracic echocardiography revealed a mobile oval-shaped mass in the left ventricle. Cardiac magnetic resonance imaging suggested a large papillary fibroelastoma. The tumor was successfully resected, and histopathological examination confirmed the diagnosis., Conclusion: This case highlights the importance of thorough imaging and surgical intervention in managing large, nonvalvular PFEs, which, although rare, can lead to significant complications., Competing Interests: Conflict of interest: The authors declare no conflict of interest., (© 2024 Saudi Heart Association.)

Cardiac hemangiomas are rare and often misdiagnosed due to their nonspecific clinical presentations. We report a case of a 70-year-old man presenting with chills and cold sweats, initially suspected of having infective endocarditis based on echocardiographic findings of a mobile mass on the mitral valve. Laboratory results showed leukocytosis and elevated C-reactive protein, but blood cultures were negative. Transesophageal echocardiography later revealed a well-defined mass with characteristics suggestive of a tumor. Surgical excision confirmed the diagnosis of hemangioma. Postoperative recovery was uneventful, with no mitral regurgitation. This case highlights the importance of considering cardiac tumors in the differential diagnosis of intracardiac masses.

Background: Cardiac neoplasms may cause life-threatening symptoms associated with cerebral infarction, ventricular arrhythmias, and heart failure. Emergency surgery or preoperative treatment may be required for these patients. However, no study has reported the surgical outcomes in cases involving cardiac neoplasms with life-threatening complications. The current study investigated the mid- to long-term outcomes of surgery in patients with cardiac neoplasms in life-threatening conditions. Methods : This study retrospectively analyzed 36 consecutive patients who underwent resection for cardiac neoplasms with life-threatening cardiovascular, respiratory, and cerebral nervous system complications from January 2000 to December 2022. Their mean age at surgery was 54.9 years. In terms of fatal events, one patient who experienced a ventricular tachycardia storm caused by a left ventricular neoplasm was placed under deep sedation and managed with a ventilator preoperatively. Seven patients who presented with limb motor paralysis and visual defects had cerebral infarction. Two of the seven patients with cerebral infarction received cerebrovascular treatment before cardiac surgery. Results : During the follow-up period, cerebral- and cardiovascular-related deaths were not recorded. All postoperative cerebral and cardiovascular complications were new-onset cerebral infarction (n = 2) (with symptoms that improved during the long term). The mean follow-up period was 6.2 years. The 5- and 10-year survival rates of all patients were 89.8% and 78.7%, respectively. There were no significant differences in postoperative prognosis between patients with preoperative cerebral infarctions and those without. Conclusions : The long-term surgical outcome of patients with life-threatening symptomatic cardiac neoplasm was good. Thus, preoperative complications did not affect prognosis.

Introduction: Cardiac hemangioma is one of the rarest tumors, with only a few cases described. Unlike other cardiac tumors, its symptoms are nonspecific, making misdiagnosis easy. Cardiac hemangioma can present with various clinical manifestations, including valve disorder, arrhythmia, pericardial effusion, and embolism. Echocardiography is the most direct examination, and surgical resection the simplest and most effective treatment., Patients and Methods: We present a new case of lobulated cardiac hemangioma causing tricuspid regurgitation and discuss the clinical features, diagnosis, and treatment of this rare tumor., Results: After surgical resection and tricuspid valve replacement, the patient recovered well., Conclusion: For cardiac hemangiomas involving the tricuspid valve, tumor resection combined with valve surgery is an effective treatment option., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

We present two cases of cardiac metastases adjacent to the right ventricle in a 55-year-old male and a 61-year-old female, both treated with magnetic resonance (MR)-guided adaptive stereotactic radiation therapy (SBRT). The prescribed regimen was 30Gy delivered in 3 fractions using a 1.5 Tesla magnetic resonance linear accelerator (MR-linac). Patients exhibited favorable tolerance to the treatment, with no observed acute toxicity., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Chen, Weng, Sobremonte, Lee, Hughes, Mohammedsaid, Zhao, Wang, Zhang, Niedzielski, Shete, Court, Liao, Lee and Yang.)

Paragangliomas are rare extra-adrenal neuroendocrine tumors originating from chromaffin tissue that present a diagnostic and therapeutic challenge due to their diverse clinical manifestations and low incidence. While these tumors often manifest as catecholamine-secreting functional tumors, their clinical presentation can vary, leading to delayed diagnosis and challenging management. This study presents the case of a 22-year-old patient with cardiac paraganglioma who initially presented with angina-like symptoms, highlighting the importance of considering this rare condition in young individuals with nonspecific complaints. Diagnostic imaging, including transthoracic echocardiography, CT angiography, and MRI, played a crucial role in identifying the tumor's location and vascularization. Surgical excision, including pulmonary artery graft and CABG, was the primary management approach, which was accompanied by intraoperative complications that later led to CCU admission, followed by postoperative complications, ultimately leading to the patient's death. This case highlights the significance of early recognition and management of complications following a surgical approach to treat paragangliomas.

Background: The presence of cardiac myxoma in the right atrium (RA) is rare. There is limited knowledge regarding the clinical symptoms and diagnosis of RA myxoma. This systematic review aimed to provide a summary of the clinical and laboratory characteristics, electrocardiogram (ECG) findings, and outcome previous cases with RA myxoma., Methods: A comprehensive search was conducted in PubMed, Web of Science, and Scopus to identify relevant studies. Inclusion criteria were case reports and case series written in English that provided sufficient data on the manifestation of RA myxoma. Descriptive statistics were used for quantitative analysis., Results: The search identified 619 patients from 480 eligible studies. The patient's mean age was 45.7 ± 17.6 years, and 55.4% of cases were female. The most common clinical manifestations of RA myxoma were cardiac, systemic, and neurologic manifestations which reported in 77.0%, 34.8%, and 21.1% of cases, respectively. Besides, 11.7% of RA myxoma were asymptomatic. ECG findings revealed normal in 39.4% reported cases. The ECG abnormalities included tall or peaked P-wave, RA and LA enlargement (19.2%), abnormal T-wave (14.0%), sinus tachycardia (11.8%), and incomplete or complete RBBB (11.2%). Echocardiography remained the diagnostic method in a majority of the cases. The mortality rate of RA myxoma was low (9.2%) during the follow-up., Conclusions: This systematic review provides a comprehensive summary of the clinical and laboratory manifestations and outcomes of RA myxoma, contributing to the existing knowledge on this rare cardiac tumor., (© 2024. The Author(s).)

Despite the low incidence of primary cardiac tumors, recently at our institution, we have experienced two very rare tumors in the span of just a few months. Hibernomas are rare tumors of brown adipose tissue origin that share the benign clinical features of a lipoma, but on imaging mimic the more aggressive sarcoma. Here we present two separate cases of otherwise healthy patients who were found incidentally to have these asymptomatic tumors., Competing Interests: The authors have no disclosures to make., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)