Cardiac rhabdomyoma: an uncommon culprit in sudden infant death.

Sudden death in infants remains a common and poorly understood cause of childhood mortality in the USA. Pediatric cardiac tumors, although rare, may underlie some cases of unexplained sudden infant death. Autopsy is a crucial diagnostic step in these cases, as both gross and microscopic examination of the heart may uncover occult cardiac tumors. Rhabdomyomas are the most common cardiac tumors in childhood and may result in arrythmia and sudden death. We present a case of sudden death in a healthy 5-month-old infant which initially appeared "SIDS-like" until thorough histological analysis revealed an underlying cardiac rhabdomyoma. The case is of particular importance in that the gross examination of the heart was considered completely normal, and the tumor only involved certain portions of the heart microscopically. Had a single random section of myocardium been the only heart section examined microscopically, the diagnosis might have been missed. This case emphasizes the importance of thorough microscopic examination in infant cases, especially in cases where the heart appears grossly normal.

Primary intracardiac germ cell tumor.

We present the echocardiography images in a 6 year old girl who presented with a history of scalp swelling after trivial trauma which was subsequently diagnosed as metastases from primary intracardiac germ cell tumour.

STEMI or not STEMI? A multimodality imaging approach to a challenging intracardiac mass with a tricky presentation.

Cardiac tumors, due to the various clinical scenarios and their histological subtypes, are still challenging for clinicians. They are differentiated into primary and secondary. The latest are more common and are usually lung and breast cancers, melanomas, and lymphoma metastasis. We present a case of a 73-year-old woman, with a history of breast cancer 10 years earlier, admitted to Cath lab for an elevation of the ST-segment of the electrocardiogram, myocardial infarction. Echocardiogram showed a curious abnormality in the myocardial wall. Thanks to a multimodality imaging strategy, including contrast-enhanced echocardiography and cardiac magnetic resonance, characterization of the underlying pathology was clear and, thus, the appropriate management and therapy.

This is the case of a cardiac metastatic tumor, whose certain diagnosis can only be made by myocardial biopsy ­ an invasive sample of heart tissue ­ unfortunately not available in our case. Alternatively, we puzzled data from two second-level imaging techniques: contrast-enhanced cardiac ultrasound and cardiac magnetic resonance, which allowed us to better evaluate the nature of this cardiac mass. The former gave information about its blood supply, the latter gave information about tissue characterization. In this paper, we show how a complete integration of data from clinical and a stepwise multimodality imaging-based approach may allow a diagnosis in a complex clinical case.

The Spectrum of ECG Abnormalities in a Large Cohort of Cardiac Masses.

BACKGROUND: Cardiac masses represent a heterogeneous clinical scenario. Potential electrocardiographic red flags of malignancy remain to be investigated. OBJECTIVES: To describe the spectrum of electrocardiographic abnormalities in a large cohort of cardiac masses and to evaluate potential red flags suggestive of malignancy. METHODS: Observational cohort study of 322 consecutive patients with a cardiac mass and available ECG at Bologna University Hospital. All masses were diagnosed by histological examination or, in the case of cardiac thrombi, by radiological resolution after proper anticoagulant therapy. Multivariable regression analysis was used to assess potential predictors of malignancy among electrocardiographic 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=0.014), bradyarrhythmias (p=0.009), ischemic-like repolarization abnormalities (ST-segment deviation, both depression and elevation, and negative T-wave; p<0.001), low voltages (p=0.001) and right axial deviation (0.025) 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 time of 20.7 months). CONCLUSION: ECG is frequently abnormal in case of malignant cardiac tumors. Some specific electrocardiographic changes are strongly suggestive for malignancy and type of infiltration.

Myxoma Involving the Pulmonary Valve - A Case Report of an Atypical Cardiac Tumor, Treated Using an Unusual Approach.

Background: Atypical myxoma has been reported in various locations in the heart, however, myxoma involving the pulmonary valve is rare. Here we present a case of pulmonic valve myxoma which was resected via a percutaneous approach. Case Report: A 66-year-old female with known metastatic adenocarcinoma of the lung, and chronic obstructive pulmonary disease presented with acute onset shortness of breath for two days. The patient experienced respiratory arrest en-route to the hospital and required intubation. Computed tomography angiography (CTA) of the chest revealed a new 1.4 × 1.6 cm intracardiac mass along the pulmonary valve. Further evaluation with cardiac magnetic resonance imaging revealed it to be a large vascular tumor on the ventricular side of the pulmonary valve, attached with a narrow stalk. Due to high surgical risk, the patient underwent transesophageal echocardiographic guided percutaneous removal of the mass. Pathology confirmed the mass to be a myxoma. Conclusion: Atypical myxoma should be considered in the differential diagnosis of valvular masses. Percutaneous resection of valvular masses may be feasible in high-risk surgical patients. LEARNING POINTS: Pulmonary valve myxoma is a rare condition and the literature on the characteristics and treatment options for pulmonary valve myxoma is limited.Our patient was treated with a minimally invasive treatment approach: removal of a tumor with intra operative transesophageal echocardiographic guidance using AngioVac and Flow Triever catheters.Percutaneous resection of valvular masses may be feasible in high surgical risk patients.

A case of left atrial intimal sarcoma with rhabdomyosarcoma differentiation: a case report and literature review.

Primary cardiac malignancies are rare, with cardiac sarcomas being the main type. Among these, intimal sarcomas are the most common. However, they tend to occur in the great vessels and are rare in the heart, with only a few isolated cases reported. We report a challenging case of a patient with left atrial intimal sarcoma with rhabdomyosarcoma differentiation. The patient was admitted after a physical examination detected left heart occupancy, and initial imaging suspected a left atrial thrombus. The patient then underwent extracorporeal circulation-assisted open cardiac surgery with resection of an atrial mass. The postoperative pathological findings were suggestive of an arterial intimal sarcoma, which included areas of rhabdomyosarcoma differentiation within the tumor tissue. Unfortunately, the patient's tumor recurred 4 months later, and she died due to treatment failure. This case highlights the rarity and risk of misdiagnosis of cardiac intimal sarcoma. Additionally, we aim to improve the understanding of intimal sarcoma through a review of immunohistochemistry and gene amplification techniques.

Congenital Blood Cyst of a Child: A case report and review of literature.

Blood-filled cysts of the heart valves are frequently reported at postpartum autopsies of infants. They are seen as round nodules mostly in the paediatric age group in infants less than 2 months of age and disappear spontaneously within 6 months of life. We report a unique case of an 11-month-old girl who presented at a tertiary healthcare hospital in 2022 with a blood-filled cyst on the posterior leaflet of the pulmonary valve that was successfully treated. This case report highlights the characteristics and course of a paediatric patient with blood-filled cysts. Further studies are yet needed to better understand the diagnostic approaches to blood-filled cysts as well as treatment modalities to fill the gap in clinical settings.

Cardiac solitary fibrous tumor - an extremely rare but potentially fatal diagnosis.

The background of this review is a description of the case of a 28-year-old man with an extremely rare cardiac solitary fibrous tumor (SFT). Although this tumor was removed surgically and in the 6-month follow-up examination no relapse was noted, recurrence was observed and confirmed in the magnetic resonance imaging 4 months later. SFT prevalence, symptoms and signs, treatment options and prognosis are reviewed.

Rheumatic heart disease of the mitral valve alongside the papillary fibroelastoma of the aortic valve: A case report.

Key Clinical Message: Besides thromboses, it's crucial to also consider the rare possibility of tumors like papillary fibroelastomas when evaluating worsening cardiopulmonary symptoms in patients with severe rheumatic mitral stenosis and atrial fibrillation. Abstract: Cardiac papillary fibroelastoma is a rare and benign endocardial tumor typically found on the aortic valve. The simultaneous occurrence of rheumatic heart disease affecting the mitral valve and papillary fibroelastoma on the aortic valve is infrequent, with limited documented instances. This unique case can enhance our understanding of the clinical presentation, diagnostic approaches, management options, and implications for patient outcomes in these two conditions. We present the case of a 47-year-old woman who was admitted to the hospital due to worsening dyspnea and fatigue, during which time she discovered an aortic valve papillary fibroelastoma. Further investigations revealed two thrombi in her left atrium and left atrial appendage, along with significant rheumatic mitral valve stenosis. The patient underwent thrombectomy, mitral valve replacement, and valve-sparing aortic valve tumor resection. Following surgery, the patient's recovery was unremarkable. This case report emphasizes the need for a comprehensive evaluation in patients with rheumatic mitral stenosis, considering all possible etiologies. While thrombi are typical in mitral stenosis and atrial fibrillation, the rare presence of tumors like papillary fibroelastomas should be recognized, underscoring the importance of further assessment when suspicion arises. Importantly, individuals with asymptomatic cardiac papillary fibroelastomas should undergo surgical treatment to minimize the potential risk of tumoral embolization.

Epidemiology of Cardiac Myxoma in the Kingdom of Bahrain.

BACKGROUND: Cardiac myxomas (CM) are the most prevalent type of primary cardiac tumour. The majority of primary cardiac tumours, including CM, are found to be benign. In the context of this study, the objective was to investigate and analyse the experience of CM over a period of 10 years, specifically in Bahrain. By examining this particular subset of cardiac tumours, valuable insights can be gained regarding their prevalence, clinical presentation, diagnostic methods, treatment approaches, and outcomes in the Bahraini population. METHODS: We retrospectively evaluated the medical records of 20 patients who presented with CM at the Mohammed bin Khalifa bin Salman Al Khalifa Specialist Cardiac Centre in the Kingdom of Bahrain from January 2010 to January 2021. All patients had transthoracic echocardiography to establish a preoperative diagnosis. All of the patients underwent an operation using the median sternotomy, and a histopathology examination confirmed the final diagnosis. RESULTS: The mean age at the time of presentation was 57 (± 18.1) years, ranging from 17 to 80 years, and 55% (12 patients) were female. Dyspnea (n=8, 40%) and peripheral embolism (n=4, 20%), which include cerebrovascular accidents and acute monocular vision loss, were the most frequently observed symptoms. The largest diameter of the myxoma was 5.1 cm (±1.7). The left atrium was the predominant location for myxoma formation (n=16, 80%), with the majority of the myxomas attached to the atrial septum. CONCLUSION: CM poses a significant risk of cardiac and systemic complications. Early detection and timely gross-complete resection result in excellent early and long-term outcomes.

Anesthetic management of a patient with incidental left atrial myxoma for proximal femur nailing: A case report.

Cardiac myxomas are rare tumors with risks of cardiac outflow obstruction and embolic events. Surgical excision of the tumor at the earliest is the definitive treatment. We report the successful anesthetic management of a 65-year-old female patient with incidental left atrial myxoma for right proximal femur nailing. The patient was asymptomatic with no significant cardiac history. Since fracture reduction cannot be deferred for a prolonged period, the case was taken up under general anesthesia with invasive blood pressure monitoring.

Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study.

Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

Aortic valve fibroelastoma presenting with myocardial infarction with non-obstructive coronary arteries (MINOCA): A case report and review of the literature.

Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors more often involving the left-sided valves and related with threatening embolic complications. We report the case of a 35-year-old woman presenting with relapsing-remitting chest pain and elevated cardiac troponins. After a negative coronary angiography, an integrated imaging assessment based on echocardiography and cardiac magnetic resonance showed a pedunculated mass on the aortic valve causing an intermittent obstructive engagement of the right coronary ostium. A tailored surgical treatment was performed and the histopathological examination of the specimen revealed mesenchymal tissue with the characteristics of CPF.

Mechanical thrombectomy for the treatment of large vessel occlusion due to cancer-related cerebral embolism: A systematic review.

BACKGROUND: Cancer-related cerebral embolism due to direct tumor embolization results in a rare acute ischemic stroke with large vessel occlusion (LVO). Despite the established status of mechanical thrombectomy (MT) in LVO management, its effectiveness and safety remains inadequately explored in this specific patient group. METHODS: We conducted a systematic review following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, using the Nested Knowledge AutoLit software, encompassing databases like Embase, PubMed, Scopus, and Web of Science, from their inception up to 9 May 2023. RESULTS: In the review of 35 studies encompassing 37 cases, mean patient age was 52 years, and 30% were female. Cardiac myxoma (29.7%), cardiac papillary fibroelastoma (16.2%), and squamous cell carcinoma of the lung (8.1%) were the most frequent underlying cancers. The left middle cerebral artery was the most commonly affected occlusion site (24.3%). Of the patients, 67.5% underwent MT alone, while 32.5% received MT combined with intravenous thrombolysis. Successful reperfusion (thrombolysis in cerebral infarction (TICI) 2b-3) was achieved in 89.1% of cases, with 59.4% reaching TICI 3. Functional independence was observed in 29.7% of patients. CONCLUSION: While limitations exist, this comprehensive study highlights the potential benefits of MT in a patient group historically excluded from major trials, warranting further investigation.

The mass that should not be there: Multi-modality imaging shapes diagnostic reasoning.

A rare case of pulmonary artery fibroelastoma that demonstrates the importance of multimodality imaging and serial scans in reducing diagnostic uncertainty.

Isolated primary cardiac angiosarcoma.

Cardiac angiosarcoma (CAS) is the most prevalent malignant primary cardiac tumor in adults, often affecting young males. We present a case of this rare entity in a young female, highlighting the multidisciplinary team's role and multimodality imaging in the diagnosis and management.

Clinical presentation, diagnostic evaluation, and management of undifferentiated/unclassified cardiac sarcoma: A case report and literature review.

This case report details a challenging instance of undifferentiated/unclassified cardiac sarcoma in a 28-year-old female, presenting with diverse symptoms like muscle weakness, shortness of breath, and hemoptysis. Diagnostic hurdles led to an initial misdiagnosis of granulomatosis with polyangiitis before discovering a sizable left atrial mass, ultimately diagnosed as high-grade undifferentiated/unclassified sarcoma. Despite initial surgical intervention, the patient's condition worsened, underscoring the complexities in managing such cases involving cardiac sarcomas. This case emphasizes the diagnostic complexities associated with primary cardiac sarcomas, particularly the challenges in achieving accurate diagnoses and formulating effective treatment strategies.

Competency based curriculum for cardiovascular magnetic resonance: A position statement of the Society for Cardiovascular Magnetic Resonance.

This position statement guides cardiovascular magnetic resonance (CMR) imaging program directors and learners on the key competencies required for Level II and III CMR practitioners, whether trainees come from a radiology or cardiology background. This document is built upon existing curricula and was created and vetted by an international panel of cardiologists and radiologists on behalf of the Society for Cardiovascular Magnetic Resonance (SCMR).