ABSTRACT
Background: Patients with cardiac tumors may present challenges for surgical resection due to poor clinical condition. Echocardiography-guided transapical radiofrequency ablation for cardiac tumors (TARFACT) potentially offers a less invasive palliative therapy option. Objectives: This study aimed to evaluate the safety and efficacy of TARFACT. Methods: Five patients with cardiac tumors (mucinous liposarcoma, myocardial hypertrophy with inflammatory cell infiltration mass, fibrous tissue tumor hyperplasia, myocardial clear cell sarcoma, and cardiac rhabdomyoma) were included. All patients underwent TARFACT and were assessed with electrocardiogram, echocardiographic imaging, biochemical analysis, and pathological confirmation. Results: The median follow-up for all patients was 9 (range 4-12) months. Three surviving patients were alive at their last follow-up (9, 12, and 12 months, respectively), whereas 2 patients with late-stage tumors survived 6 months and 13 months after TARFACT, respectively. After TARFACT, all patients showed significant reductions in tumor size: the mean length decreased from 6.7 ± 2.0 cm to 4.7 ± 1.8 cm (P = 0.007); and the mean width decreased from 5.0 ± 2.1 cm to 2.5 ± 0.7 cm (P = 0.041). NYHA functional class also improved: median (IQR) decreased from 3.0 (1.5) to 2.0 (1.0) (P = 0.038), Peak E-wave on echocardiography showed a mean increase from 64.4 ± 15.7 cm/s to 76.6 ± 18.6 cm/s (P = 0.008), and NT-pro BNP levels had a median (IQR) reduction from 115.7 (252.1) pg/mL to 55.0 (121.6) pg/mL (P = 0.043). Conclusions: TARFACT is a novel palliative treatment option for cardiac tumors, reducing accessible tumors and improving clinical symptoms in a preliminary group of patients. (Cardiac Tumors Interventional [Radio Frequency/Laser Ablation] Therapy [CTIH]; NCT02815553).
ABSTRACT
Cardiac tumors continue to be one of the most uncommon primary tumors. Secondary cardiac tumors are rare and occur in difficult instances. A multidisciplinary team is required for diagnosis and management. Within this case study, we present a 24-year-old female patient who experienced dyspnea and was diagnosed as having metastatic heart and lung choriocarcinoma that was effectively managed with heart surgery and chemotherapy.
ABSTRACT
Intraventricular cardiac hemangiomas are very rare tumors that account for 5-10% of all cardiac tumors. We report a case of an asymptomatic 21-year-old young male who was incidentally detected with ventricular mass on echocardiography followed by magnetic resonance imaging. Surgical resection was done for confirmation and to avoid potential complications. Histopathology and immunohistochemical studies confirmed the diagnosis of a hemangioma of the ventricle. Subsequent regular follow-up has revealed no complications or recurrence, underscoring the favorable long-term prognosis. This case report highlights the importance of diagnostic evaluation even in asymptomatic individuals, allowing for early recognition and effective management of this rare cardiac entity.
ABSTRACT
BACKGROUND: Primary cardiac tumors are uncommon, with approximately 70-80% classified as benign. Myxomas constitute roughly half of all benign cardiac tumors, while cardiac hydatid cysts are exceptionally rare. Shortness of breath is a prominent symptom associated with these conditions. Echocardiography serves as the primary diagnostic tool, enabling early detection. The preferred course of action involves the surgical excision of the mass. CASE PRESENTATION: Our first case, a 51-year-old female presented to the cardiology outpatient clinic with complaints of shortness of breath and palpitations persisting for 3 months. Physical examination and tests were within normal limits. Echocardiography revealed a right atrial myxoma measuring 65 * 35 mm. Despite not affecting valve and ventricular functions, the mass was surgically resected. Our second case, a 55-year-old male admitted to the cardiology clinic with recurrent fever, shortness of breath, and chest pain. Physical examination and tests were normal. Echocardiography revealed an echogenic mass with a septate appearance adhering to the free wall of the right atrium. The patient, diagnosed with a hydatid cyst, underwent surgical resection. CONCLUSIONS: We came across two different cases of RA masses which happened to be myxoma and hydatid cysts, and we managed accordingly. The right atrial myxomas typically present with nonspecific shortness of breath unless they cause valve obstruction. In cases where clinical findings suggest infection in the right atrial masses, it is essential to consider hydatid cyst as a potential diagnosis alongside the initial consideration of vegetation.
ABSTRACT
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.
ABSTRACT
BACKGROUND: Cardiac Magnetic Resonance (CMR) parametric mapping is underexplored in cardiac tumors. OBJECTIVES: To evaluate the contribution of mapping sequences on the characterization of pediatric tumors. METHODS: All pediatric patients referred for cardiac tumors at Bambino Gesù Children's Hospital from June 2017 to November 2023, who underwent CMR with mapping sequences, were included. The diagnosis of tumor type was performed according to signal characteristics on different sequences. Mass parametric mapping for each subtype and interobserver variability was assessed. RESULTS: Sixteen patients were enrolled. The mean age at CMR was 7 ± 5 years. "Traditional" mass-type assessment diagnosed hemangioma (Group A) in 3 patients (19%), fibroma (Group B) in 4 patients (25%), rhabdomyoma (Group C) in 6 patients (37%), and lipoma (Group D) in 3 patients (19%). The ANOVA analysis revealed significant differences in mass native T1 and mass extracellular volume (ECV) values among the four subgroups (p<0.001 for both comparisons). The mean native T1 and ECV values were respectively 1465 ± 158 msec and 54 ± 4% for Group A, 860 ± 118 msec and 93 ± 4% for Group B, 1007 ± 57 msec and 23 ± 5% for Group C, and 215 ± 13 msec and 0 ± 0% for Group D. CONCLUSIONS: Mass mapping analysis is feasible and reproducible in children. ECV values provide the most accurate differentiation. Mass ECV consistently resembles normal myocardium in rhabdomyoma, is extremely high (approaching 100%) in fibroma, equals to zero in lipoma, and matches blood pool ECV (1-Hct) in hemangioma.
ABSTRACT
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.
ABSTRACT
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.
Subject(s)
Echocardiography , Electrocardiography , Heart Neoplasms , Magnetic Resonance Imaging, Cine , Multimodal Imaging , ST Elevation Myocardial Infarction , Humans , Female , Aged , Multimodal Imaging/methods , Heart Neoplasms/diagnosis , Heart Neoplasms/secondary , ST Elevation Myocardial Infarction/diagnosis , Echocardiography/methods , Diagnosis, Differential , Magnetic Resonance Imaging, Cine/methods , Breast NeoplasmsABSTRACT
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.
Subject(s)
Cysts , Female , Humans , Infant , Cysts/congenital , Pulmonary Valve/abnormalitiesABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
Subject(s)
Aortic Valve , Cardiac Papillary Fibroelastoma , Heart Neoplasms , Myocardial Infarction , Humans , Female , Adult , Aortic Valve/surgery , Aortic Valve/pathology , Aortic Valve/diagnostic imaging , Heart Neoplasms/pathology , Heart Neoplasms/complications , Heart Neoplasms/surgery , Heart Neoplasms/diagnostic imaging , Cardiac Papillary Fibroelastoma/surgery , Cardiac Papillary Fibroelastoma/pathology , Cardiac Papillary Fibroelastoma/complications , Myocardial Infarction/etiology , Myocardial Infarction/pathology , Treatment Outcome , Coronary Angiography , Coronary Vessels/pathology , Coronary Vessels/diagnostic imaging , Coronary Vessels/surgery , Biopsy , Fibroma/pathology , Fibroma/surgery , Fibroma/complicationsABSTRACT
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.
ABSTRACT
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.