Our 10-Year Experience with Atrial Myxomas: Is Concurrent Valve Intervention Really Warranted?

INTRODUCTION: Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted. METHODS: A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients. RESULTS: A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement. CONCLUSION: Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.

Resultados Perinatais e Seguimento em Longo Prazo de Tumores Cardíacos Fetais: Estudo de Coorte Histórica de 30 Anos / Perinatal Results and Long-Term Follow-Up of Fetal Cardiac Tumors: A 30-Year Historical Cohort Study

Resumo Fundamento: Seguimento de coorte retrospectiva de 30 anos que se aproxima da história natural dos tumores cardíacos diagnosticados no feto uma vez que nenhum caso foi submetido à interrupção da gestação. Objetivo: Avaliar a morbidade e mortalidade perinatal e em longo prazo em fetos com diagnóstico de tumor cardíaco. Como objetivo secundário avaliar os fatores que influenciaram os resultados perinatais e pós-natais. Método: Estudo de coorte retrospectiva envolvendo 74 gestantes com diagnóstico ecocardiográfico fetal de tumor cardíaco acompanhadas em dois serviços de referência no período de maio de 1991 a novembro de 2021. Foi realizada análise descritiva dos dados por meio de frequências absolutas (n) e relativas (%), mediana e intervalos interquartis. Para avaliar a associação entre as características ecocardiográficas e as manifestações clínicas com os resultados perinatais e pós-natais, foi aplicado o teste exato de Fisher. O cálculo da sobrevida global foi realizado pelo método de Kaplan-Meier e a comparação de curvas pelo teste de log-rank. O tempo de seguimento, calculado em meses, foi definido a partir da data de alta do hospital à data do status atual (vivo/censura ou óbito). O nível de significância considerado foi de 5% (p<0,05). Resultados: o rabdomioma é o tipo mais frequente (85%) de tumor cardíaco; apresenta alta morbidade (79,3%) e mortalidade geral de 17,4%; a presença de hidropisia fetal preditiva de óbito. Conclusão: A presença de hidropisia fetal teve impacto na mortalidade, sendo fator importante para aconselhamento e estabelecimento de prognóstico. A maioria dos óbitos ocorrem antes da alta hospitalar.

Abstract Background: This was a 30-year retrospective cohort study that approximates closely to the natural history of cardiac tumors diagnosed in the fetus, since there was no case of pregnancy interruption Objective: To assess morbidity and mortality in the perinatal period and at long term in fetuses diagnosed with cardiac tumor. Our secondary objective was to assess the evaluating factors of perinatal and postnatal results. Methods: This was a retrospective cohort study with 74 pregnant women with an echocardiographic diagnosis of fetal cardiac tumor at two referral centers between May 1991 and November 2021. A descriptive analysis was performed, and data were expressed as absolute (n) and relative (%) frequencies, median and interquartile range. Fisher's exact test was used to evaluate the association of echocardiographic characteristics and clinical manifestations with perinatal and postnatal results. Global survival was calculated using the Kaplan-Meier method and the curves were compared by the log-rank test. The time of follow-up, calculated in months, corresponded to the time elapsed from hospital discharge to current status (survived/ censoring or death). The level of significance was set at 5% (p<0.05). Results: Rhabdomyoma is the most common type of cardiac tumor (85%), with a high morbidity (79.3%) and overall mortality of 17.4%. The presence of fetal hydrops was a predictor of death. Conclusion: The presence of fetal hydrops had an impact on mortality, and hence is an important factor in counselling and determining the prognosis. Most deaths occurred before hospital discharge.

Transaortic removal of a large primary sarcoma from the left ventricle assisted by strategic partial resection and endoscopic guidance: a case report.

BACKGROUND: Surgical resection remains the mainstay of treatment for cardiac sarcoma, a rare but lethal disease. Achieving complete removal of a large-sized left ventricular sarcoma remains a challenge even with various surgical approaches that have been employed. CASE PRESENTATION: We present a case of a 74-year-old woman with shortness of breath who underwent surgical removal of a primary cardiac sarcoma, measuring 6 × 3.5 × 3 cm, attached to the septum of the left ventricle and caused sub-aortic valve obstruction. Transaortic approach was chosen and the access to this entire huge mass was enabled by using interim partial resection which created a space for further dissection and subsequent deeper endoscopic views. The further dissection was finally able to be advanced on the apex, and the residual mass was completely resected with gross tumor-free margins. CONCLUSION: Interim partial resection and endoscopic guidance can highly facilitate the transaortic removal of even large left ventricular sarcomas.

Our 10-Year Experience with Atrial Myxomas: Is Concurrent Valve Intervention Really Warranted?

ABSTRACT Introduction: Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted. Methods: A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients. Results: A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement. Conclusion: Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.

Serie de pacientes operados de masas y quistes cardíacos en un hospital general de España: Una experiencia de 20 años / Series of patients operated for masses and cysts in the heart in a Spanish general hospital: A 20-year experience

Fundamento. Las masas y quistes cardíacos son entidades bien conocidas, cuya reducida prevalencia y sintomatología inespe-cífica dificultan su diagnóstico. El objetivo del estudio fue ca-racterizar el cuadro de los pacientes afectos en nuestro medio para orientar futuros diagnósticos.Metodología. Estudio descriptivo de los pacientes intervenidos de tumores y quistes cardíacos entre 2002 y 2022 mediante la búsqueda en el registro del Servicio de Cardiología y Cirugía Cardíaca de la Clínica Universidad de Navarra (Pamplona, Es-paña). Se recogieron variables sociodemográficas, clínicas, his-tológicas y quirúrgicas.Resultados. Se identificaron 13 pacientes, la mayoría (76,92%) mujeres, con media de edad 63,08 años (DE: 15,17). El 92,31% de los pacientes tenían al menos un factor de riesgo cardiovascular, siendo los más prevalentes un IMC ≥25 kg/m2 y la hipertensión arterial (61,54% y 53,85%, respectivamente). El tipo de masa car-díaca más frecuente fue el mixoma (69,23%). El 46,15% de masas cardiacas fueron hallazgos incidentales; el síntoma más frecuen-te fue la disnea (53,85%) y el 30,77% de los pacientes se encontra-ban asintomáticos. . La prueba de imagen más empleada para en el diagnóstico fue la ecocardiografía transtorácica Doppler color (69,23%). La concordancia entre los diámetros medios precirugía y postcirugía resultó muy alta (CCI = 0,807, IC95%: 0,450-0,943).Conclusiones. Se describieron los cuadros de los pacientes, apor-tando información poco descrita en la literatura, como los facto-res de riesgo cardiovascular más frecuentes en estas entidades. Se describieron un caso de leiomiosarcoma cardíaco y un caso de sarcoma intimal del tronco pulmonar, dos tipos de tumores extremadamente raros de los que existen pocos casos descritos (AU)

Background. Masses and cysts in the heart are well-known entities, but their low prevalence and non-specific symptoms makes the diagnosis difficult. We aimed to characterize the fea-tures of these entities in our environment.Methods. We carried out a search of patients who underwent surgery for tumors and cysts in the heart between 2002 and 2022 in the registry of the Department of Cardiology and Car-diac Surgery of Clínica Universidad de Navarra (Pamplona, Spain). Sociodemographic, clinical, histological, and surgical variables were collected.Results. We identified 13 patients; mean age was 63.08 ± 15.17 years, 76.92% were female and 92.31% had at least one car-diovascular risk factor, e.g., BMI ≥ 25 kg/m2 and high blood pressure (61.54% and 53.85%, respectively). The most com-mon type of cardiac tumors were myxomas (69.23%). Around half (46.15%) were incidental; the most frequent symptom was dyspnea (53.85%); 30.77% of the patients were asymptomatic. The most commonly used imaging technique for the diagno-sis was transthoracic Doppler echocardiography (69.23%). The agreement between the mean diameters before and after sur-gery was very high (ICC = 0.807, 95%CI: 0.450-0.943).Conclusions. We describe the features of masses and cysts in the heart (77% female patients) and provide information scarcely available in the literature, e.g., the most frequent car-diovascular risk factors for this population. A case of cardiac leiomyosarcoma and a case of intimal sarcoma of the pulmo-nary trunk are described, two extremely rare tumors for which there are few described cases (AU)

A monster into the heart: an unusual presentation of cardiac leiomyosarcoma.

BACKGROUND: Malignant primary cardiac tumors are infrequent and can lead to an unfavorable prognosis if not identified and treated promptly. Early detection and prompt treatment of malignant primary cardiac tumors are crucial for a better prognosis. This article presents a case of primary cardiac leiomyosarcoma and reviews the literature on this topic. CASE PRESENTATION: Female patient that developed recurrent pericardial effusion and hemodynamic instability caused by a cardiac tumor, later identified as leiomyosarcoma. Multidisciplinary treatment was administered to the patient. CONCLUSIONS: The initial approach to this type of pathology should include multimodality imaging to establish a prompt diagnosis leading to complete standard treatment, to minimize risks to the patient's heart function which may include resection with complete margins of the neoplasm, otherwise the prognosis may be poor.

Cardiovascular Magnetic Resonance Versus Histopathologic Study for Diagnosis of Benign and Malignant Cardiac Tumours: A Systematic Review and Meta-Analysis.

BACKGROUND: The gold standard for diagnosis of cardiac tumours is histopathological examination. Cardiovascular magnetic resonance (CMR) is a valuable non-invasive, radiation-free tool for identifying and characterizing cardiac tumours. Our aim is to understand CMR diagnosis of cardiac tumours by distinguishing benign vs. malignant tumours compared to the gold standard. METHODS: A systematic search was performed in the PubMed, Web of Science, and Scopus databases up to December 2022, and the results were reviewed by 2 independent investigators. Studies reporting CMR diagnosis were included in a meta-analysis, and pooled measures were obtained. The risk of bias was assessed using the Quality Assessment Tools from the National Institutes of Health. RESULTS: A total of 2,321 results was obtained; 10 studies were eligible, including one identified by citation search. Eight studies were included in the meta-analysis, which presented a pooled sensitivity of 93% and specificity of 94%, a diagnostic odds ratio of 185, and an area under the curve of 0.98 for CMR diagnosis of benign vs. malignant tumours. Additionally, 4 studies evaluated whether CMR diagnosis of cardiac tumours matched specific histopathological subtypes, with 73.6% achieving the correct diagnosis. CONCLUSIONS: To the best of our knowledge, this is the first published systematic review on CMR diagnosis of cardiac tumours. Compared to histopathological results, the ability to discriminate benign from malignant tumours was good but not outstanding. However, significant heterogeneity may have had an impact on our findings.

Management of primary cardiac leiomyosarcoma.

Background: Primary cardiac cancer is a rare event with various clinical presentations and often causes unexpected symptoms or sudden death. Case reports with this diagnosis are scarce. Case presentation: We present an unusual manifestation of leiomyosarcoma of the left atrium in a female patient, 33 years old. Presenting difficulty to walk, dyspnoea at rest, skin pallor, cough with hemoptoics and syncope. A transthoracic echocardiogram showed cavitary enlargement of the left atrium, moderate to significant mitral stenosis with an adherent mass in the anterior leaflet, left ventricular systolic function preserved at rest, and mild aortic and tricuspid insufficiency. The procedure was complete resection of the tumour or negative microscopic margins (R0 resection), 25 sessions of radiotherapy, 5 cycles of adjuvant chemotherapy using gemcitabine (900 mg/m2 on days 1 and 8) and docetaxel (75 mg/m2 on day 8), with a resolution of the clinical picture. After 5 years of follow-up, the patient had no metastases or recurrence of the initial tumour. Conclusion: The nonspecific symptoms presented in the reported case demonstrate that the cardiac tumour can mimic other cardiac disorders, such as coronary artery disease or pericarditis, rarely representing the first manifestation of a previously unknown malignancy.

[Right intraatrial lipoma. A case report]. / Lipoma intrauricular derecho. Reporte de un caso.

We present the case of a 56-year-old male patient with a history of atrial flutter for six months; he was admitted to the hospital for presenting a mass of 8 cm in maximum diameter in the right atrium, which prolapsed through the tricuspid valve into the right ventricle. Emergency surgery was scheduled, performing exeresis of the tumor and tricuspid annuloplasty. The Pathological anatomy determined that the removed mass corresponded to a cardiac lipoma.

Left Ventricular Papillary Fibroelastoma.

INTRODUCTION: Cardiac papillary fibroelastoma is a rare benign primary cardiac tumor typically found on the valvular structures of the heart. Nonvalvular papillary fibroelastomas are exceedingly rare. We report the case of a 66-year-old male who presented with cerebellar infarctions and was found to have a mobile mass attached to the endocardial surface of the anteroseptal wall of the left ventricle. Cardiac magnetic resonance imaging demonstrated late gadolinium enhancement. Surgical excision of the cardiac mass was performed via the transaortic approach without intra-operative complications. Histopathologic examination confirmed the diagnosis of a papillary fibroelastoma. Some aspects related to the etiology, diagnosis and management of this entity are discussed.

Inflammatory myofibroblastic tumor: A rare entity with a complex diagnosis.

Inflammatory myofibroblastic tumor is a neoplasm with uncertain behavior. We describe a case in a 66-year-old female who underwent resection of a left atrial tumor suspected to be a cardiac myxoma which was subsequently diagnosed as an inflammatory myofibroblastic tumor. After three years' follow-up the patient underwent a second operation to remove tumoral occurrence in the right atrium, diagnosed as an intimal sarcoma. It cannot be confirmed whether the tumoral recurrence with a different diagnosis (intimal sarcoma) was a progression from the primary tumor or the metachronous appearance of a spontaneous sarcoma.

Common benign primary pediatric cardiac tumors: a primer for radiologists.

Cardiac tumors are neoplasms arising from or located in the heart or the pericardium. Although rare, primary cardiac tumors in children require an accurate and timely diagnosis. Most pediatric primary cardiac tumors are benign (around 90%). Echocardiography is the first imaging modality used due to its availability, noninvasiveness, inexpensiveness, and absence of ionizing radiation. Computed tomography (CT) and magnetic resonance imaging (MRI) offer better soft tissue visualization as well as better visualization of extracardiac structures. A great advantage of MRI is the possibility of measuring cardiac function and blood flow, which can be important for obstructing cardiac tumors. In this article, we will offer a brief review of clinical, echocardiographic, CT, and MRI features of cardiac rhabdomyomas, fibromas, teratomas, and lipomas providing their differential diagnosis.

Diagnóstico Errôneo de Angiossarcoma Cardíaco na Era COVID-19 / Misdiagnosis of a Cardiac Angiosarcoma During the COVID-19 Pandemic

Resumo Os últimos meses de 2019 foram marcados pelo surgimento de uma nova pandemia, denominada "COVID-19". Desde então, essa infecção e suas complicações têm sido a prioridade de profissionais de saúde, com muitos sintomas atribuídos às suas apresentações precoces e tardias. Até o momento, outras doenças, mesmo em situações fatais, têm sido negligenciadas ou diagnosticadas incorretamente devido à atribuição dos sintomas do paciente à presença da infecção por COVID-19. Apresentamos aqui um caso de angiossarcoma cardíaco, em um menino que, cerca de 2 meses antes, havia sido infectado com COVID-19. Dado o histórico de infecção, a abordagem inicial foi o manejo da miopericardite pós-COVID-19. No entanto, o quadro do paciente piorou, exigindo reavaliação por multimodalidades com maior precisão. Por fim, o paciente foi diagnosticado com um tumor cardíaco. Este artigo procura enfatizar a importância da atenção a outras doenças e condições fatais na era COVID-19, com ênfase em evitar diagnósticos incorretos de outras doenças.

Abstract The final months of 2019 saw the emergence of a new pandemic termed "COVID-19". Since then, this infection and its complications have been the priority of healthcare providers, with many symptoms attributed to its early and late presentations. Thus far, other diseases, even fatal situations, have been overlooked or misdiagnosed due to the attribution of patient symptoms to the presence of COVID-19 infection. We herein present a case of cardiac angiosarcoma in a young boy who had previously become infected with COVID-19 about two months earlier. Given the history of infection, the initial approach was post-COVID-19 myopericarditis management. However, the patient's condition worsened, necessitating reevaluation via multimodalities with higher precision. Ultimately, the patient was diagnosed with a cardiac tumor. This article seeks to underscore the significance of taking heed of other diseases and fatal conditions during the COVID-19 pandemic with an emphasis on avoiding misdiagnosing other diseases.

Clinically atypical abdominal manifestation of the lipoma localized in the right atrium: "Invagination hypothesis" revisited.

Cardiac lipomas are the second most common cardiac tumors. They are usually asymptomatic and diagnosed as incidental findings. We describe a 71-year-old patient with a tumor in the right atrium. In echocardiography and MRI scan, the diagnosis of a cardiac lipoma was suspected. Moreover, MRI demonstrated continuity of pericardial fat and the tumor in the right atrium by infolding of the atrial wall and epicardial adipose tissue in the space between the atrial walls, which might be a hint for the Waterstone groove hypothesis. An operative resection was performed which confirmed the suspected diagnosis.

The diagnostic accuracy of contrast echocardiography in patients with suspected cardiac masses: A preliminary multicenter, cross-sectional study.

Background: To evaluate the diagnostic accuracy of contrast echocardiography (CE) in patients with suspected cardiac masses. Methods: A multicenter, prospective study involving 108 consecutive patients with suspected cardiac masses based on transthoracic echocardiography performed between November 2019 and December 2020 was carried out. CE examinations were performed in all patients. The echocardiographic diagnosis was established according to the qualitative (echogenicity, boundary, morphology of the base, mass perfusion, pericardial effusion, and motility) and quantitative (area of the masses and peak intensity ratio of the masses and adjacent myocardium A1/A2) evaluations. Results: Final confirmed diagnoses were as follows: no cardiac mass (n = 3), pseudomass (n = 3), thrombus (n = 36), benign tumor (n = 30), and malignant tumor (n = 36). ROC analysis revealed the optimal A1/A2 with cutoff value of 0.295 for a cardiac tumor from a thrombus, with AUC, sensitivity, specificity, PPV, and NPV of 0.958 (95% confidence interval (CI): 0.899-0.988), 100, 91.7, 95.7, and 100%, respectively. CE was able to distinguish malignant from benign tumors with an AUC of 0.953 (95% CI: 0.870-0.990). Multivariate logistic regression analysis revealed that tumor area, base, and A1/A2 were associated with the risk of malignant tumor (OR = 1.003, 95% CI: 1.00003-1.005; OR = 22.64, 95% CI: 1.30-395.21; OR = 165.39, 95% CI: 4.68-5,850.94, respectively). When using A1/A2 > 1.28 as the only diagnostic criterion to identify the malignant tumor, AUC, sensitivity, specificity, PPV, and NPV were 0.886 (95% CI: 0.784-0.951), 80.6, 96.7, 96.7, and 80.7%, respectively. Conclusion: CE has the potential to accurately differentiate cardiac masses by combining qualitative and quantitative analyses. However, more studies with a large sample size should be conducted to further confirm these findings. Clinical trial registration: http://www.chictr.org.cn/, identifier: ChiCTR1900026809.