Valve-Sparing Excision of Multiple Papillary Fibroelastomas in the Aortic Valve and Left Ventricular Outflow Tract / 日本心臓血管外科学会雑誌

Primary cardiac fibroelastoma is a relatively rare tumor and is often detected incidentally by echocardiography. We report a case of multiple fibroelastomas that were found incidentally by follow-up echocardiography for hypertrophic cardiomyopathy and were treated with valve-sparing excision. The patient was a 71-year-old man, in whom a 10-mm tumor on the ventricular septum below the right coronary cusp and 3-mm tumors on the left ventricular side of the left and right coronary cusps were detected. Although he had no symptoms, because the tumors were mobile, surgery was performed for preventing embolization and making a definitive diagnosis. The tumor on the ventricular septum was excised together with the surrounding endocardium and part of the myocardium. The tumors on the valve leaflets were excised with the aortic valve cusps spared. Histopathologically, all the tumors were fibroelastomas. The postoperative course was uneventful.

Giant fibroma of left ventricular apex with T wave inversion: 2 cases report and literature review / 中国综合临床

Objective:To investigate the clinical features, diagnostic methods and treatments of left ventricular apical fibroma.Methods:The clinical manifestations, ECG, imaging features and treatment plans of 2 patients with giant fibroma of left ventricular apex diagnosed in September 2020 and May 2022 were analyzed retrospectively, and the related literature was reviewed.Results:Both patients had slight chest distress and discomfort after activities. The ECG showed T-wave inversion of different degrees, which were misdiagnosed as “myocarditis” and “coronary heart disease” respectively. The cardiac magnetic resonance imaging and echocardiography showed left ventricular apical mass. Coronary artery stenosis was not found in coronary angiography. One patient required conservative treatment, and there was no significant change in clinical symptoms and tumor size in the follow-up for half a year; Another patient underwent cardiac mass removal, and the pathological examination after operation confirmed that it was cardiac fibroma, and there was no recurrence in the follow-up 2 years.Conclusion:Fibroma of left ventricular apex is a rare cardiac tumor, which is easy to be missed and misdiagnosed, and is one of the rare causes of T-wave inversion. Cardiac magnetic resonance imaging, CT and echocardiography are commonly used imaging methods to diagnose cardiac fibroma, and surgical resection is an effective treatment.

Ethical and Clinical Dilemma from an Incidental Cardiac Lipoma in a Young and Healthy Patient

Incidental cardiac tumors are rare and mostly detected on autopsy as patients largely remain asymptomatic. However, diagnosis of an incidental cardiac mass on unrelated workup can pose significant ethical and clinical challenge to the care team. Surgical resection has been the most successful intervention for most primary cardiac tumors; which involves cardiopulmonary bypass?assisted major surgery and is not risk free. Cardiac lipoma is the second most common primary cardiac benign tumor. We report a case of a young otherwise healthy patient who had a cardiac lipoma on computerized tomography scan that was done to rule out kidney stone.

Sarcoma de la vena pulmonar / Sarcoma of the pulmonary vein

Resumen Los sarcomas son neoplasias primarias, cuya ubicación es rara en grandes vasos y excepcional en las venas pulmonares. Los dos tipos más frecuentes en las venas pulmonares son el leiomiosarcoma y el fibrosarcoma, con una distribución alrededor de la cuarta a la quinta décadas de vida, un pronóstico de 23 meses y 60% de metástasis al momento del diagnóstico. La presentación clínica es inespecífica ya que simula situaciones como embolia pulmonar, falla cardiaca descompensada y masa en la aurícula izquierda. Para su diagnóstico se cuenta con diferentes herramientas, como la ecocardiografía, la tomografía computarizada, la angiografía coronaria, la resonancia magnética y la tomografía por emisión de positrones (PET TC). El tratamiento incluye resección quirúrgica radical, manejo adyuvante con quimioterapia y radioterapia, e incluso, en casos seleccionados, trasplante de corazón. Se presenta el caso de una paciente con diagnóstico inicial de embolia pulmonar, con un episodio de edema pulmonar secundario a masa en la aurícula izquierda y extensión de un sarcoma de vena pulmonar derecha, con desenlace fatal. Se aporta a la literatura con el caso y la revisión de tema.

Abstract Sarcomas are primary neoplasms, whose location is rare in large vessels and in the pulmonary veins is exceptional. The two most frequent types in the pulmonary vein are leiomyosarcoma and fibrosarcoma, distribution around 4 and 5 decades of life, with a prognosis of 23 months and 60% metastasis at the time of diagnosis. The clinical presentation is nonspecific simulating situations such as pulmonary embolism, decompensated heart failure and mass in the left atrium. Different tools are available for its diagnosis, like echocardiography, computed tomography, coronary angiography, magnetic resonance imaging and PET CT. Treatment includes radical surgical resection, adjuvant therapy with chemotherapy, and radiation therapy, even heart transplantation in selected cases. It is presented the case of a patient with an initial diagnosis of pulmonary embolism, with an episode of pulmonary edema secondary to a mass in the left atrium, extension of a sarcoma of the right pulmonary vein, with a fatal outcome. We contributed to the literature with the case and review of theme.

Síndrome de Li-Fraumeni Presentación metacrónica de sarcoma de tejidos blandos, sarcoma cardiaco y cáncer gástrico / Li-Fraumeni Syndrome Metachronous presentation of soft-tissue sarcoma, heart sarcoma and gastric cancer

Resumen El síndrome de Li-Fraumeni (SLF) es un trastorno autosómico dominante hereditario con predisposición al cáncer, está asociado con anomalías en el gen de la proteína tumoral p53 (TP53), que se manifiesta por una amplia gama de neoplasias malignas que aparecen a una edad temprana. Se expone al caso de un adulto joven en quien hicimos este diagnóstico, y se describen las perspectivas terapéuticas en investigación. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2198).

Abstract Li-Fraumeni syndrome (LFS) is a hereditary autosomal dominant disorder with a predisposition to cancer. It is associated with abnormalities of the tumor protein p53 (TP53) gene, manifesting with a broad range of malignant neoplasms which appear at an early age. We discuss the case of a young adult in whom we did this diagnosis, and we describe the therapeutic perspectives being researched. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2198).

A Case of Non-Bacterial Thrombotic Endocarditis with Atypical Massive Vegitation / 日本心臓血管外科学会雑誌

The patient was a 68-year-old woman. She was diagnosed with uterine cancer after experiencing irregular genital bleeding. Contrast-enhanced computed tomography showed a 30 mm left ventricular mass and splenic infarction, and head MRI showed multiple cerebral infarctions. The patient was suffering from systemic embolism caused by the cardiac mass, and we decided to perform cardiac mass removal prior to uterine cancer treatment. A yellowish-white thrombus-like mass attached to the mitral valve, subvalvular tissue, and left ventricular endocardium was removed by a trans-septal approach under cardiopulmonary bypass. Pathological examination revealed that the mass was a fibrin-based thrombus with almost no inflammatory findings, we diagnosised non-bacterial thrombotic endocarditis (NBTE). Postoperatively, the patient developed Takotsubo cardiomyopathy, and treatment for uterine cancer, was delayed. Hypercoagulability was not controlled well, and she developed recurrence of left ventricle vegitation, acute arterial occlusion of the lower extremities and inferior vena cava thrombosis, making active intervention for uterine cancer difficult. The patient was treated palliatively and died on POD 36. NBTE tends to be characterized by multiple small growths, but giant vegetation may also occur as in this case. Unless the primary disease causing the hypercoagulability is treated, recurrence of NBTE is possible, and prompt treatment of the primary disease is required.

Surgical experience of pediatric cardiac fibromas / 中华胸心血管外科杂志

Objective:To summarize single-center experience and short-term outcomes of surgical treatment of pediatric cardiac fibromas.Methods:There was a retrospective study of 10 patients who underwent surgical treatment of cardiac fibromas between January 2018 and October 2021. Fibromas were located in the left ventricle in 7 cases, and in the right ventricle in 3 cases. Mean tumor diameter was(5.6±2.0) cm.Results:Median age at surgery was 3.1 years old(5 months-9 years old). Nine patients received complete resection, and the other one received partial resection. One patient with giant left ventricular fibroma required extracorporeal membrane oxygenation support for ventricular fibrillation after weaning from cardiopulmonary bypass, and was successfully weaned on the third postoperative day. There was no early mortality. Median follow-up time was 6 months(1 month-3 years). No tumor recurrence nor ventricular arrhythmia was documented during the follow-up period.Conclusion:Pediatric cardiac fibromas can be usually treated by complete resection, with relief of cardiac dysfunction and ventricular arrythmia after surgery, and with satisfactory results.

A Case of Multiple Cardiac Papillary Fibroelastoma in the Aortic Valve / 日本心臓血管外科学会雑誌

We report a case of multiple papillary fibroelastoma (PFE) on the aortic valve. A healthy woman in her 60 s was referred to a nearby doctor with the chief complaint of palpitation and was admitted to our hospital for detailed examination. A mobile tumor was found by transthoracic echo, and she was introduced to us. We undertook excision surgery for her. Initially, it was thought to be a single tumor, and a pedunculated tumor adhering to the central aortic side of the left coronary cusp with a length of 7 mm was removed. After closing the aorta, we recognized a remaining mobile tumor by transesophageal echo. We decided to perform cardiac arrest again, and we recognized one tumor with 6 mm long string-like mobile mass on the right and non-coronary commissure of cardiac side, and a thorn-like mass of about 1.5 mm on the non-coronary cusp of the cardiac side. We removed both tumors from the aortic valve using shaving resection and preserved the aortic valve. All of these were found to be PFE pathologically as if they were three tumors that followed the developmental stage of PFE. After surgery, PFE recurrence is extremely rare. In the case of a pedunculated tumor, it is possible to remove the tumor and preserve the valve. However, as in this case, if both sides of the valve were not carefully inspected, it can be overlooked. In addition, small tumor resection could avoid the risk of new cerebral infarction and myocardial infarction due to recurrence and could avoid reoperation.

Clinical characteristics and surgical treatment of cardiac tumors in 28 patients / 中国胸心血管外科临床杂志

@#Objective    To analyze the clinical characteristics and surgical treatment results of cardiac tumors. Methods    Clinical data of 28 patients with cardiac tumors, who underwent cardiac surgery in our hospital from 2013 to 2019 were retrospectively reviewed. There were 12 males and 16 females. The median age was 11.5 years, ranging 1 month to 69 years; and the median weight was 39.0 (4.8-100.0) kg. All patients underwent tumor resection under cardio-pulmonary bypass with general anesthesia, and postoperative pathological analysis was performed. Results    There were 27 patients with primary cardiac tumors and 1 patient with secondary cardiac tumor. Among the primary cardiac tumors, there were 16 myxomas, 5 rhabdomyomas, 3 inflammatory myofibroblastomas, 2 fibroids and 2 hemangiomas. The secondary cardiac tumor was chondrosarcoma. A total of 28 cardiac tumors occurred in 29 locations, including 14 in the left atrium, 3 in the right atrium, 1 in the left ventricle, 7 in the right ventricle, 2 in the mitral and 2 in the tricuspid. Cerebral embolism occurred in 6 patients, peripheral vascular embolism in 3 patients, and syncope in 3 patients. There was no death during the perioperative period and in the long term follow-up. Complete resection was performed in 25 patients and partial resection was performed in 3 patients. During the follow-up, 2 patients with myxoma received reoperation for recurrence. Among the 3 patients with partial resection, residual tumor tissue disappeared in 2 patients, and in the other patient, there was no change in the size of the residual tumor tissue during the follow-up compared to that at discharge. Conclusion    Cardiac surgery is an effective method for patients with primary benign cardiac tumors. However, whether the cardiac tumor tissue can be completely removed depends on the anatomical location of the tumor during the operation. If there are systemic embolism events and syncope, the possibility of cardiac tumors should be considered.

Mixoma auricular derecho en la infancia / Right atrium myxoma in chilhood

Los tumores cardíacos primarios son poco frecuentes y representan solo el 5% de todos los tumores cardíacos. El 75% de estos tumores son benignos, y el mixoma es el tumor benigno más común en el adulto (50%). La mayoría de los mixomas cardíacos se localizan en la aurícula izquierda, solo el 18-20% se localizan en la aurícula derecha, y es aún más infrecuente la aparición de un tumor bilobulado, los signos y síntomas con los que se presentan son inespecíficos y varían de acuerdo a su comportamiento. Presentamos un caso con estas características en una paciente escolar de 9 años de edad que ingresó al Hospital del Niño Manuel Ascencio Villarroel

Primary cardiac tumors are rare and represent only 5% of all cardiac tumors. 75% of these tumors are benign, and myxoma is the most common benign tumor in adults (50%). Most cardiac myxomas are located in the left atrium, only 18-20% are located in the right atrium, and the appearance of a bilobed tumor is even more infrequent, the signs and symptoms with which they occur are nonspecific and they vary according to their behavior. We present a case with these characteristics in a 9-year-old school patient who was admitted to Manuel Ascencio Villarroel Children's Hospital.

Cardiac Rhabdomyoma Causing Progressive Dynamic Severe Right Ventricular Outflow Tract Obstruction in an Infant

Multiple cardiac masses were incidentally detected in a neonateon twelve day of life. Failure to thrive, feeding difficulty andsevere dynamic right ventricular outflow tract obstructiondeveloped at 7 months of age. Surgical resection of intracardiacmasses relieved symptoms and histological studies confirmedrhabdomyoma. Progressive increase in the size ofrhabdomyoma during infancy is an uncommon presentation andsurgery can be life-saving.

Masas cardíacas, utilidad de la ecocardiografía transesofágica durante el intraperioperatorio / Intracardiac tumors: utility of transesophageal echocardiography during the periodic intraoperative

We present our experience in cardiac tumor resection surgery in adult patients: 30 subjects with sternotomy approach with later diagnosis of myxomas (12), fibroelastomas (7), sarcomas (4), cardiac methastasis of a breast cancer and cardiac invasion of renal tumor (6), and 3 with videothroacoscopic approach with diagnosis of atrial myxoma (2) and intraventricular sarcoma (1). We highlight the usefulness of TEE as an essential monitor in this subtype of cardiac surgery in allowing location confirmation and completion of resection. Likewise as anesthesiologists and active participants of the surgical team, we were able to document absence of residual heart defects, lesions or perforations or dysfunction of heart valves. Evaluation of preexisting anatomy and function and post Cardiopulmonary Bypass ventricular function and circulation were important in early diagnosis of complications.

Presentamos nuestra experiencia en resección de tumores cardíacos en pacientes adultos: 30 casos con resecciones tumorales por esternotomía: 12 mixomas, 7 fibroeslastomas, 4 sarcomas, 6 tumores renales con invasión cardíaca y 1 metástasis cardíaca de cáncer de mama. En 3 pacientes las resecciones tumorales fueron por videotoracoscopía (2 mixomas y un sarcoma intraventricular). La cirugía por video supone un nuevo desafío para el anestesiólogo. Destacamos la utilidad de la ecocardiografía transesofágica como monitor, hoy imprescindible en cirugía cardíaca. Éste nos permitió confirmar la localización del tumor y su completa resección. Así mismo, como anestesiólogos y formando parte activa en el equipo quirúrgico, pudimos documentar la ausencia de defectos residuales, perforaciones o incompetencia de las válvulas cardíacas; evaluar la función ventricular y la volemia postcirculación extracorpórea, obtener información no conocida previamente y realizar así un diagnóstico precoz de complicaciones.

Cardiac Tumor Resection through Right Mini-thoracotomy / 日本心臓血管外科学会雑誌

We herein report a case of cardiac tumor resection through a right mini-thoracotomy. A 48-year-old man exhibited no symptoms. A mass was detected incidentally in the right atrium on computed tomography. We performed resection under cardiopulmonary bypass through a right mini-thoracotomy. Histopathological examination confirmed that this tumor was a lipoma. The patient's postoperative recovery was uneventful. He was discharged on postoperative day 6. As cardiac tumor resection through right mini-thoracotomy is minimally invasive, this approach may be useful for surgery in cases of benign cardiac tumors.

Demographic and Clinical Features of Left Atrial Tumors in South Indian Population: A Case Series

Myxomas are the most common type of primary cardiac tumor. They cause a variety of clinical manifestations depending on size and anatomical location. Sometimes, manifestations are atypical challenging differential diagnosis and the therapeutic approach. Left atrial myxomas are commonly missed clinically and often lead to grave consequences. We present here a series of 6 cases of left atrial myxomas with demographic and clinical characterization of the patients that were managed successfully.

Feasibility study of one-stage surgery of intracardiac leiomyomatosis through an abdominal approach / 中国实用外科杂志

OBJECTIVE: To investigate the feasibility of abdominal surgical resection of intracardiac leiomyomatosis.METHODS: The clinical data of 4 cases of intracardiac leiomyomatosis performed in zhongshan Hospital,Fudan University from December 2015 to August 2017 were analyzed retrospectively. RESULTS: Four patients underwent onestage surgery through an abdominal approach,without death. The operative time was 185-420(315.3 ± 86.4) min.Extracorporeal circulation time was 22-175(71.8±60.4)min and blood loss was 600-3000(1475.0±941.7)mL. Acute renal failure occurred in one of the patients after operation,the rest had no surgical complications. After 8-26 months of follow-up,there was no sign of recurrence and metastasis. CONCLUSION: One-stage surgery of intracardiac leiomyomatosis through an abdominal approach is feasible. But,how to determine the indications of surgery remains to be further explored and studied.

Tumorectomy to Avoid Sudden Death by Pulmonary Embolism in a Patient with a Cardiac Tumor Originating from the Esophagus / 日本心臓血管外科学会雑誌

Tumor metastasis to the endocardium is rare. The patient was 58-year-old man who was given a diagnosis of a metastatic tumor to the right atrium and right ventricle. The tumor originated from the esophagus, and the patient was at risk of sudden death caused by acute pulmonary embolism. We performed tumorectomy to reduce the risk of sudden death. The postoperative course was satisfactory, and the tumorectomy was followed by chemotherapy. The 5-year survival rate in such cases has been reported to be only approximately 11%. However, resection of tumor mass may be useful for improving postoperative QOL and reducing the risk of sudden death.

A Case of Left Atrial Villous-Type Myxoma Mimicking Papillary Fibroelastoma / 日本心臓血管外科学会雑誌

A female in her 60 s with a mobile cardiac tumor in the left atrium was referred to our hospital. The tumor was diagnosed as myxoma by echocardiography, CT scan, and MRI. Under cardiac arrest using cardiopulmonary bypass, we resected the tumor through the superior trans-septal approach. We resected two tumors (tumor 1 and 2) with adequate rims of the interatrial septum to avoid recurrence. Although the tumor had a broad base and extended to near the mitral annulus, mitral valve repair was not necessary. Tumor 1 was gelatinous and seemed to be a myxoma ; tumor 2 had a lot of papillary structures attached to the solid mass and presented with the figure of a sea anemone when it was put in saline, which is typical for papillary fibroelastomas. The defect of the interatrial septum was closed with the autologous pericardium. The postoperative course was uneventful. Unexpectedly, both tumor 1 and 2 were diagnosed as myxomas on pathological examination. Tumor 2 (papillary lesion) included a few tumor cells with positive staining of calretinin typical for myxoma and was diagnosed as a “villous-type myxoma”. Because it is difficult to distinguish myxoma from papillary fibroelastoma by pre- and intraoperative findings, it would be recommended to resect tumors with an adequate margin to prevent recurrence.

Multiple Papillary Fibroelastomas Involving All Four Heart Valves / 日本心臓血管外科学会雑誌

Papillary fibroelastoma (PFE) is a rare primary cardiac tumor that usually involves an aortic or mitral heart valve. We encountered a case of a 32-year-old woman, who presented with syncope and was found to have multiple PFEs involving all four heart valves during surgery. The echocardiography was performed and showed two mobile masses near the tricuspid and mitral valves. Moreover, the enhanced computed tomography (CT) showed thickened aortic cusps, which may indicate the possibility of heart tumor. Intraoperatively, we first found multiple tumors at each cusp of the aortic valve, ranging in size from 5 to 10 mm which were excised without injury of aortic cusps themselves. These showed a sea anemone-like appearance and were suspected to represent PFE. We then observed the tricuspid and mitral valves, and both valves showed tumors of similar appearance in each cusp. Furthermore, we found a tumor at the pulmonary valve, even though there had been no evidence of its presence on echocardiography or CT. We confirmed that these masses were PFEs by histological study after the operation. We should keep in mind that PFE can develop in multiple valves. To the best of our knowledge, this is the first description of multiple PFEs involving all four heart valves.

Sirolimus therapy for fetal cardiac rhabdomyoma in a pregnant woman with tuberous sclerosis

Rhabdomyoma is the most common fetal cardiac tumor, and its development is related to tuberous sclerosis. Fetal cardiac rhabdomyomas often spontaneously regress in utero or after birth, but large tumors can cause hemodynamic obstruction. Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, has been used as an immunosuppressant after organ transplantation. The mTOR inhibitors are well-known to have anti-tumor activity, and they have been used for the treatment of patients with tuberous sclerosis. In the current case, fetal cardiac rhabdomyoma was completely resolved in utero during oral sirolimus treatment in the mother with tuberous sclerosis. This case shows that oral sirolimus therapy in pregnancy may be a treatment for multiple or large fetal cardiac rhabdomyomas.

Determination of residual mass in left ventricle by intraoperative transesophageal echocardiography after a giant and floating left atrial myxoma resection / Determinação da massa residual em ventrículo esquerdo por ecocardiografia transesofágica intraoperatória após ressecção de mixoma gigante e flutuante em átrio esquerdo

Abstract Atrial myxoma is a benign tumor of the heart that occurs primarily in the left atrium. Floating or large left atrial myxomas frequently cause functional mitral stenosis, may also affect mitral valve structure and flow, and lead to mitral regurgitation. Systemic embolization occurs in around 30% of cases either from tumor fragmentation or complete tumor detachment hence it should be removed as soon as it is detected. Intraoperative transesophageal echocardiography has a vital importance in the surgery. After resection of myxoma, intraoperative transesophageal echocardiography must be performed to rule out residual mass. The case here reported is of a 48-year old female, who presented with giant and floating left atrial myxoma. Residue mass was detected with intraoperative transesophageal echocardiography in the left ventricle after the resection of myxoma. Subsequently, the residue mass was successfully removed. Complete resection must be required to prevent possible complications such as recurrence, embolization in atrial myxomas. Transesophageal echocardiography performed intraoperatively is vital importance to confirm that the myxoma is completely resected.

Resumo Mixoma atrial é um tumor benigno do coração que ocorre principalmente no átrio esquerdo. Os mixomas flutuantes ou grandes em átrio esquerdo com frequência causam estenose mitral funcional, podendo também afetar a estrutura e o fluxo da válvula mitral e levar à insuficiência mitral. A embolização sistêmica ocorre em cerca de 30% dos casos, quer pela fragmentação do tumor ou pelo desprendimento total do tumor; portanto, o tumor deve ser removido assim que detectado. A ecocardiografia transesofágica intraoperatória tem uma importância vital na cirurgia. Após a ressecção do mixoma, a ecocardiografia transesofágica intraoperatória deve ser feita para excluir a massa residual. O caso aqui relatado é o de uma paciente de 48 anos que apresentou um mixoma de átrio esquerdo gigante e flutuante. A massa residual foi detectada com ecocardiografia transesofágica intraoperatória no ventrículo esquerdo após a ressecção do mixoma. Posteriormente, a massa residual foi removida com sucesso. A ressecção completa é necessária para evitar possíveis complicações, como recorrência e embolização em mixomas atriais. A ecocardiografia transesofágica realizada no intraoperatório é de vital importância para confirmar a ressecção completa do mixoma.