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.

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.

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.

A Malignant Lymphoma Growing Inside a Cardiac Mixoma: A Case Report

Abstract Introduction: Lymphomas arising from cardiac myxomas represent a particularly rare pathology, with only few cases reported in the literature. Case presentation: We report a complete excision of a malignant lymphoma arising from a cardiac myxoma in a 44-year-old female patient. The myxoma presented like a floating mass within the left atrium with a maximum diameter of 3.5 cm. The clinical post-operative period was uneventful and the patient was dismissed on the 6th post-operative day. Conclusion: This case reinforces the concept of radical excision of cardiac neoplasms.

Fast growth rate of a right atrial myxoma

ABSTRACT Primary cardiac tumors are rare, with an incidence between 0.0017 and 0.19%, and are asymptomatic in up to 72% of cases. Approximately 75% of tumors are benign, and nearly 50% of these are myxomas. Concerning location, 75% of myxomas are in the left atrium, 15 to 20% in the right atrium, and more rarely in the ventricles. The finding of cardiac myxomas usually implies immediate surgical excision to prevent embolic events and sudden cardiac death. Reports with documented growth rate are rare, and the actual growth rate remains a controversial issue. We report the rapid growth rate of a right atrial myxoma in an oligosymptomatic 69-year-old patient, with negative previous echocardiographic history in the last two years, who refused surgery upon diagnosis, enabling monitoring of myxoma growth.

Clinicopathological and magnetic resonance imaging characteristics analysis of cardiac tumors in children / 肿瘤研究与临床

Objective:To investigate clinicopathological and magnetic resonance imaging (MRI) characteristics of pediatric cardiac tumors.Methods:The clinical, pathological and MRI data of 7 patients with pediatric cardiac tumors confirmed by pathological examination in Children's Hospital of Chongqing Medical University from February 2012 to December 2016 were retrospectively analyzed.Results:There were 3 males and 4 females with first diagnosis age ranging from 1 month to 3 years. As for clinical presentation, most cases were featured with cardiac murmur and enlarged cardiac boundary; only 1 case had acute cerebral infarction, and 1 case did not show any abnormal performance. Pathological findings showed that 6 cases of benign tumors (including 2 cases of fibroma, 1 case of rhabdomyoma, 1 case of myxoma, 1 case of lipoma and 1 case of hemangioma), 1 case of malignant tumor (primitive neuroectodermal tumor of pericardium). MRI results showed that the signal intensity of malignant tumor was higher than that of normal myocardium in each sequence; significant differences were found in benign tumors; first-pass perfusion, cardiac cine image and late gadolinium enhancement were the most obvious.Conclusions:The clinical presentations of pediatric cardiac neoplasms are atypical. Each tumor type has pathognomonic pathological features. MRI has great advantages in the diagnosis and differential diagnosis of cardiac tumors especially for benign tumors.

Efficacy and safety of catheter ablation in patients with new-onset atrial arrhythmia after surgical excision of left atrial myxoma / 中华心血管病杂志

Objective: To evaluate the safety and efficacy of catheter ablation in patients with new-onset atrial arrhythmia after surgical excision of left atrial myxoma. Methods: Nine patients with new onset atrial arrhythmia and a prior history of left atrial myxoma, who received surgical myxoma excision and catheter ablation between September 2014 and November 2019, were included in the present study. Baseline characteristics, procedural parameters during catheter ablation, severe perioperative adverse events, recurrence rate of arrhythmia and clinical prognosis were analyzed. Kaplan Meier survival analysis was used to define the maintenance rate of sinus rhythm after catheter ablation in this patient cohort. Results: Nine patients were included. The average age was (55.8 ± 9.1) years old (3 male), there were 3 patients (3/9) with paroxysmal atrial fibrillation (PAF) and 6 patients (6/9) with atrial flutter or atrial tachycardia (AFL or AT). Ablation was successful in all patients, there were no perioperative complications such as stroke, pericardial effusion, cardiac tamponade, vascular complications or massive hemorrhage. During a mean follow-up time of 40.0 (27.5, 55.5) months, sinus rhythm was maintained in six patients (6/9) after the initial catheter ablation. The overall sinus rhythm maintenance rate was 2/3. In addition, 1 out of the 3 AF patients (1/3) developed recurrence of AF at 3 month after ablation, and 2 out of the 6 AFL or AT patients (2/6) developed late recurrence of AF or AFL (19 months and 29 months after ablation), two out of three patients with recurrent AFs or AFL received repeated catheter ablation and one patient remained sinus rhythm post repeat ablation. Meanwhile, there was no recurrence of atrial myxoma, no death, stroke, acute myocardial infarction and other events during the entire follow-up period. Conclusions: Catheter ablation is a safe and feasible therapeutic option for patients with new-onset atrial arrhythmia after surgical excision of left atrial myxoma.

Metástasis cardíaca en paciente con cáncer de colon: Informe de un caso / Cardiac metastasis in colon cancer: A case report

RESUMEN Las masas cardíacas constituyen un reto diagnóstico y terapéutico. Muchas veces se descubren casualmente y la evaluación confirma, finalmente, la presencia de un tumor cardíaco. Estos son neoplasias originadas en cualquier capa del corazón y se dividen en primarios y secundarios. Los primarios tienen una incidencia en autopsias de 0,001 - 0,03%, que contrasta con la frecuencia 20-40 veces mayor de los secundarios. Se presenta un paciente de 28 años de edad diagnosticado 1 año antes de adenocarcinoma de colon transverso infiltrante, en quien hace 3 meses se constató metástasis a cadena ganglionar intraabdominal y se realizó tratamiento quirúrgico más quimioterapia. Posteriormente comenzó con taquicardia y acudió a su centro de salud donde se le realizó un ecocardiograma transtorácico que constató una imagen de aspecto tumoral en ventrículo derecho. Por tomografía se demostró una masa tumoral compleja inoperable y el paciente falleció en su hogar, bajo cuidados paliativos, un mes y medio después del egreso.

ABSTRACT Cardiac masses are a diagnostic and therapeutic challenge. They are often found incidentally and assessment eventually confirms the presence of a heart tumor. They are neoplasms that originate in any layer of the heart and are divided into primary and secondary. The primary ones have a 0.001 - 0.03% incidence in autopsies, contrasting with the 20-40 times higher frequency of the secondary ones. We present the case of a 28-year-old patient diagnosed one year before with infiltrating transverse colon adenocarcinoma in whom intra-abdominal lymph node chain metastases were confirmed three months ago, receiving surgical treatment and chemotherapy. Later, he began with tachycardia and presented to his health care center where a transthoracic echocardiogram was performed, which showed a tumor-like image in the right ventricle. The CT-scan showed an unresectable complex tumor mass and the patient died at home, under palliative care, a month or so after discharge.

Fibroelastoma papilar mitral como causa de ictus en paciente joven / Mitral papillary fibroelastoma as a cause of stroke in a young patient

RESUMEN El fibroelastoma papilar es un tumor poco frecuente, con una prevalencia estimada entre el 1 y el 7,9% de todos los tumores cardíacos primarios, los que -a su vez- presentan una muy baja incidencia (entre 0,001 y 0,28%), pues la gran mayoría de ellos (casi un 90%) son benignos. Se presenta el caso de un hombre de 41 años de edad que debutó con un accidente cerebrovascular. En el estudio ecocardiográfico se halló un tumor cardíaco en la valva anterior mitral sugerente de fibroelastoma papilar. Se decidió la intervención quirúrgica ante la posibilidad de complicaciones embólicas derivadas, con recurrencia de episodios isquémicos cerebrovasculares. Se realizó resección triangular de dicha tumoración y anuloplastia mitral con buen resultado. El estudio anatomopatológico confirmó el diagnóstico.

ABSTRACT Papillary fibroelastoma is an infrequent tumor, with a prevalence estimated between 1 and 7.9% of all primary cardiac tumors, which -at the same time- have a very low incidence (between 0.001 and 0.28%), because the great majority of them (almost a 90%) are benign. Here is presented the case of a 41-year-old man who had a stroke. A cardiac tumor in the anterior mitral valve suggestive of papillary fibroelastoma was found in the echocardiographic study. The surgical intervention was decided because of the possibility of resulting embolic complications with recurrence of cerebrovascular ischemic events. A triangular resection of the tumor and mitral annuloplasty were performed with good results. The pathological study confirmed the diagnosis.

The current management of cardiac tumours: a comprehensive literature review

Abstract Objective: To understand the current evidence and guidelines behind the appropriate management of cardiac tumours. Methods: A comprehensive electronic literature search has been performed in major databases - PubMed, Embase, Scopus, Ovid, and Google Scholar. All articles that discussed all different forms of cardiac tumours, their clinical presentation, diagnosis, and management methods have been critically appraised in this narrative review. Results: All relevant studies have been summarized in appropriate sections within our review. Cardiac tumours are rare but can be catastrophic and life-threatening if not identified and managed on timely manner. Utilization of all the available imaging methods can be of equivocal importance, relevant to each cardiac tumour. Surgical excision is the ultimate treatment method, however histopathological results can guide the adjunct treatment. Conclusion: Early detection of cardiac tumours has significant effect on planning the method of intervention. Technological advancements and increased availability of imaging modalities have enabled earlier and more accurate detection of these tumours. Novel medical therapies, recommendations for screening, and operative techniques have all contributed to overall improving knowledge of these tumours and ultimately patient outcomes.

Primary biatrial cardiac Rhabdomyosarcoma

Abstract Primary malignant neoplasms of the heart are rare. Cardiac rhabdomyosarcoma is the second most common primary sarcoma. We report a rare case of a 49-year-old woman with a huge biatrial cardiac rhabdomyosarcoma treated by performing surgical resection followed by salvage chemotherapy for local recurrence. Cardiac sarcoma that occupy both atria are extremely rare. Although the prognosis of cardiac rhabdomyosarcoma is dismal, surgical resection should be recommended as a first line therapy to clarify the diagnosis and to relieve symptoms associated with the tumor.

Tumores cardíacos primarios / Primary cardiac tumors. Experience in 72 cases

Background: Primary cardiac tumors have a low incidence, and their presentation form and clinical course are not well known. Aim: To describe the characteristics, treatment, follow up and survival of patients with primary cardiac tumors (PCT). Material and Methods: Review of all surgical procedures for PCT performed between 1984 and 2019 in a regional general hospital. The information was obtained from surgical protocols, surgical and pathology databases, medical records and registries of the Chilean National Identification Service. The clinical features of patients, immediate and long term outcomes are described. Results: Seventy-two surgical procedures for PCT were reviewed. Patients' age was 55 ± 15 years and 60% were women. The most common histological types were myxoma in 49 patients (68%), papillary fibroelastoma in 13 (18%) and sarcoma in 6 (8.3%). Forty-nine (68.1%) were symptomatic and all tumors were found on echocardiography. Fifty-one (71%) were in the left atrium, 10 (14%) in the aortic valve and eight (11%) in the right atrium. The surgical procedures were tumor resection in 48 patients (67%) and resection and repair with patch in 23 (32%). Mean postoperative stay was 6.6 ± 4.4 days, eight patients (11%) had complications and no patient died in the immediate postoperative period. Long term survival was higher in patients with benign PCT as compared with those with malignant tumors. Conclusions: Most PCTs in this group of patients were benign neoplasms and the most common tumors were myxomas. The main diagnostic method is echocardiography and the prognosis of surgical treatment is excellent when the tumors are benign. (Rev Med Chile 2020; 148: 327-335)

Mixoma cardíaco: experiencia de 28 años en resección quirúrgica / Cardiac myxomas. Analysis of 78 cases

Background: The incidence rates of cardiac tumors are low. Aim: To report the clinical presentation of cardiac myxomas and long-term evolution after resection. Material and Methods: Review of a database of surgical patients undergoing surgical resection of a cardiac myxoma in a public hospital between 1990 and 2018. Results: Seventy-eight patients aged 53 ± 15 years (65% females) were included. The most frequent comorbidities were arterial hypertension (40.5%), hypothyroidism (15%) and diabetes mellitus (12%). The main presenting symptoms were dyspnea (33%), neurological deficit secondary to embolism (30%) and acute pulmonary edema (5%). The most common location was the left atrium, in 87%. During surgery, cardiopulmonary bypass and aortic cross-clamp times were 50.2 ± 19.6 and 33.4 ± 15.2 min, respectively. One patient died due to severe neurological involvement. Follow-up was completed in seventy-seven patients, with a mean echocardiographic follow-up time of 10.4 ± 7.7 years. Thirty-four patients were followed for more than 10 years. Six patients (7.7%) died during the follow-up and in six patients (7%) a recurrence was identified. Conclusions: Cardiac myxoma usually has nonspecific symptoms. Surgical excision offers excellent short and long-term results. Complications and recurrence rates are low in non-hereditary myxomas but obligates to perform echocardiographic follow-up for early diagnosis of recurrence.