Heart Myxomas Research Articles

Chronic thromboembolic pulmonary hypertension in a patient with myxoma of the heart and thrombophilia of mixed genesis

Objective. To describe the clinical case of a young patient with chronic thromboembolic pulmonary hypertension (CTEPH) with heart myxoma and thrombophilia of mixed origin, who underwent surgical treatment.Materials and methods. Patient Z., 24 years old, was admitted to the cardiology department with cough with sputum production that occurs during physical activity, including streaks of blood, shortness of breath with slight physical exertion, legs and feets edema, weakness and dizziness. The examination was carried out: electrocardiography; multislice computed tomography pulmonary angiography; echocardiography (EchoCG); transesophageal EchoCG; magnetic resonance imaging of the heart with contrast; screening for coagulopathy.Results. Based on clinical data, medical history, laboratory, instrumental diagnostic, we have put the diagnosis: Myxoma of the right atrium. Patent foramen ovale. Thrombophilia of mixed origin: hereditary – heterozygous mutation of the prothrombin gene (factor 2), acquired – hyperhomocysteinemia. Postthrombotic deep vein disease of the left lower limb. Recurrent pulmonary embolism (last relapse dated October 19, 2022). CTEPH functional class III (World Health Organization), moderate risk. Congestive heart failure with reduced right ventricular ejection fraction, functional class III (by the New York Heart Association). Relative tricuspid valve regurgitation grade 3. The patient underwent surgery: thromboendarterectomy from the right pulmonary artery under conditions of circulatory arrest and deep hypothermia, removal of a right atrium myxoma under conditions of artificial circulation and blood cold cardioplegia: tricuspid valve plasty according to De Vega. After surgery patient’s condition was significantly improved.Conclusion. The features of diagnosis and treatment of a patient suffering from CTEPH and cardiac myxoma are outlined. Surgery: Pulmonary thrombectomy and cardiac myxoma removal may improve the patient’s condition with CTEPH and left atrial myxoma.

Regularities of structural organisation of the heart myxomas in patients with Karney complex

Myxomas of the heart are one of the most common primary heart tumours, which most often develop in the left atrium, are characterised by a benign nature and no relapses after surgical removal of the tumour. Karney complex is an autosomal dominant disease characterised by numerous tumours, in particular, myxomas of the heart. Insufficient coverage of this pathology in the literature leads to an erroneous diagnosis, progression of the disease and inadequate treatment. The purpose of the study was to determine the morphological features of heart myxomas in patients with Karney complex. Using light microscopy, a morphological examination of the heart myxomas was performed, which were removed during 7 operations. Histological sections were made from the operating material, which were stained with hematoxylin and eosin, according to Van Gieson, fuchselin, and Masson's Trichrome Stain in the Zerbino-Lukasevich modification. A comparison of the group of heart myxomas of patients with the Karney complex (16 tumours) with a group with myxomas of the heart that occur sporadically (278 tumours) was made to identify morphological features. It was established that myxomas of the heart are multiple, they were detected with the same frequency in the right and left chambers of the heart. It was generalised that in myxomas of the heart, signs of both alteration and high proliferative activity of endotheliocytes and fibrous connective tissue cells are noted. Secondary myxomas of the heart in patients with the Karney complex have the same structural organisation as primary tumours, but most often they are not relapses, more often they are multiple and do not have a predominant localisation in the left atrium. The analysis established an identical cellular composition of both groups of heart myxomas, which suggests the same source of tumour growth, but the presence of more complex cell formations in sporadic myxomas and the predominance of single cells in Karney heart myxomas indicate the existence of differences in their morphogenesis

Infected cardiac myxomas: state of the problem and a cardiac surgery case

The article presents a case of a truly infected heart myxoma in a 25-year-old patient. His main complaint during hospitalization was a daily increase in body temperature for 8 months up to 39 °C. Resection of the tumor was performed. During the surgery, a tumor typical of cardiac myxoma detected. After the operation, the body temperature rose to 37.8 °C for 7 days, which later returned to normal. A feature of the microstructure of this cardiac myxoma was a large number of inflammatory foci in the form of leukocyte infiltrates, often with foci of necrosis and destruction in the center, characteristic of abscesses. In the areas of inflammation there were basophilic clusters of microorganisms, which might be morphologically classified as Toxoplasma gondii. According to the classification of S.G. Revankar et al., the described histological findings allow in this case to diagnose «definite infected cardiac myxoma». The described clinical case is a first confirmed case of myxoma infection in our practice. The characteristic clinical features of the disease are supplemented by the results of histopathological examination, which revealed the causes of the temperature reaction.

Clinical Characteristics and Surgical Outcomes of Right Heart Myxoma and Its Comparison with Left Heart Myxoma: A Single-Center Retrospective Study.

Cardiac myxomas are commonly located in the left atrium but rarely affect the right side of the heart. We retrospectively analyzed 28 patients receiving surgical treatment for right heart myxomas at our center and aimed to summarize the clinical features and surgical outcomes of right heart myxomas. Between May 2001 and June 2022, 244 patients with sporadic cardiac myxomas underwent complete surgical resection. Twenty-eight patients (28/244, 11.48%) were right heart myxomas. Among the 28 right heart myxoma cases, 25 underwent median sternotomy and 3 underwent robotic or total thoracoscopic procedures. The clinical features, operative information, and follow-up data of right heart myxoma were comprehensively reviewed, and clinical characteristics between right heart myxoma and left heart myxoma were also compared. A significant difference was noted in sex between right heart myxoma and left heart myxoma (P <.05). Right heart myxoma had a higher asymptomatic rate (17.86% vs. 3.70%, P =.007) and a lower embolization rate (3.57% vs. 30.09%, P =.003) than left heart myxoma. The most common attachment site of right heart myxoma is the atrial septum. The mean operative duration and cardiopulmonary bypass time of right heart myxoma resection were 207.71 ± 53.40 minutes and 63.86 ± 29.73 minutes, respectively, with an in-hospital mortality rate of 3.57%. During the follow-up, 2 patients died of noncardiac causes. The overall 1-, 2-, and 5-year actuarial survival rates after right heart myxoma resection were 95.8%, 90.8%, and 84.7%, respectively. As a rare cardiac tumor, the clinical characteristics of right heart myxoma are different from typical left heart myxoma in some aspects, such as sex, asymptomatic rate, and embolization rate. Prompt surgical resection of right heart myxoma gives excellent early and midterm results.

Heart Myxoma of the Left Atrium Complicated by Takotsubo

Objective: Myxomas are the most common primary cardiac tumor. It is estimated that more than 75% of myxomas originate in the left atrium either at the mitral annulus or the fossa ovalis border of the interatrial septum; 20% arise from the right atrium, while 5% stem from both atria and the ventricle. Methods: 56 year-old woman hospitalized in our cardio department for progressively worsening exertional dyspnea NYHA class III with no significant cardiovascular risk factor; Clinical examination blood pressure 130/70 mmHg with pulse rate of 71bpm, a grade 3/6 blowing holosystolic murmur at the apex along. Electrocardiogram: Atrial Fibrillation with heart rate of 70 bpm. Results: The patient had good ventricular function, LVEF 60%; moderate mitral eccentric regurgitation secondary to restriction of the small valve; pulmonary arterial systolic pressure was 30±5 mmHg. We noted the presence of a tissue mass occupying almost the whole of the left auricle. The patient was operated on with a complete resection of the myxoma on the second day after surgery. Ultrasound monitoring showed left ventricular dysfunction with basal hyperkinesia associated with apical aknesia; with LVEF estimated at 25%, with hemodynamic instability with placement of ECMO for 6 days. Coronary angiography was performed, which became normal. Therefore, takotsubo was retained as diagnosis. Unfortunately, the cardiac MRI was not available. Evolution was marked by the recovery of the LVEF after 10 days postoperative. Anatomy pathology confirms typical appearance related to myxoma. Conclusions: Takotsubo heart disease is a rare postoperative complication in patients operated on for myxoma of the left atrium.

Порушення функції клапанів при міксомах серця: механізм пошкодження і методи корекції

The aim – to analyze the frequency and nature of lesions of the valve apparatus, to evaluate the results of surgical correction of the affected valves in heart myxomas. Materials and methods. In one-centre study, during period from 01.01.1969 to 01.10.2020, 962 patients with morphologically verified primary heart tumors were observed. Myxomas of the heart were found in 856 (89.0 %) patients. The pathology of the valve apparatus in combination with cardiac myxomas was noted in 70 (8.1 %) patients. The volume and nature of valve damage differed and depended on size, consistency and localization of myxoma, the degree of its mobility, and the presence of areas of pronounced fibrosis and calcification on the tumor. Results. Surgical correction of lesions of valve structures was performed in 65 patients. In 5 patients, it was possible to remove tumor tissue from the valve apparatus, followed by prevention of disease recurrence without damaging valve and subvalvular structures. Surgical techniques included both plastic manipulation and valve replacement. The features of the long-term postoperative period in such patients include progressive valve dysfunction manifested by the progression of insufficiency due to further deformation of the valve apparatus. Conclusions. Alongside with myxoma removal, it is necessary to conduct a thorough examination of the valve apparatus and correction of valve damage by various methods, including plastic surgery and valve replacement, depending on the nature and severity of the damage. Key words: cardiac tumors, myxoma, valve structures, surgical treatment.

Heart myxoma under the mask of interstitial lung lesion: a difficult case from practice

The aim of the investigation was to describe a clinical case of the development of interstitial lung lesions in a 57-year-old man with a large left atrial myxoma, which had a reverse development after myxomectomy.Materials and methods. Patient V., 57 years old, was hospitalized in the therapeutic department of the City Clinical Hospital No. 1 named after N. I. Pirogov in connection with bilateral polysegmental pneumonia. A month before hospitalization, he underwent an outpatient examination for progressive dyspnea. Myxoma of the heart was diagnosed. Due to the progression of respiratory failure and the appearance of fever, he was hospitalized. Amidst the multistage antibiotic therapy, there was a torpid course of lung pathology with syndromes of bilateral dissemination and “ground glass”, bilateral lymphadenopathy of the mediastinum, high pulmonary hypertension, and systemic inflammatory reaction syndrome. Thromboembolism of the branches of the pulmonary artery, tuberculosis, sepsis, infective endocarditis, neoplastic processes of pulmonary and other localization were excluded.Results. The clinical picture corresponded to interstitial lung lesions within the framework of paraneoplastic syndrome in heart myxoma. It was decided to urgently carry out myxomectomy.Conclusion. The clinical case demonstrates the development of a rare variant of paraneoplastic syndrome in left atrial myxoma, which was suspected during the patient’s treatment for bilateral polysegmental pneumonia. The progression of the pulmonary lesion was explained by active interstitial inflammation and was supported by the immunological activity of the heart tumor.The performed myxomectomy, despite the persisting syndrome of systemic inflammatory reaction and infiltration of the lung tissue, led in 2 weeks to complete resolution of interstitial lung lesions and pulmonary hypertension, which confirmed the causal relationship between myxoma of the heart and involvement in the pathological process of the lungs.

Sudden death by massive systemic embolism from cardiac myxoma. Role of the clinical autopsy and review of literature

Cardiac myxoma is a rare benign neoplasm of the heart. Historically myxomas were incidental findings during autopsies, however improved imaging techniques made these diagnosis possible in living patients, making the surgical treatment of these neoplasms achievable.Cardiac myxomas may occur both sporadically and in a familial context, often in the clinico-pathological picture of the Carney complex. While familial myxomas occur in the context of well-known genetic mutations, the molecular etiology of sporadically occurring myxomas is still not completely clear.We must note however that many of the patients affected by myxomas are asymptomatic; when symptoms are present they are often nonspecific and hard to decipher, especially when referring to sporadically occurring heart myxomas.In this paper we describe a case of sudden death from the massive embolization of a left atrial cardiac myxoma. We also reviewed all the cases in the literature of sudden death from heart myxoma embolism.An accurate epidemiology of heart myxomas would be the key to outline the best treatment practices and the etiology of sporadic myxomas, nevertheless this target could only be pursued with a deep revaluation of the clinical autopsy as a fundamental diagnostic tool.

MR-TOMOGRAPHY OF THE HEART IN A PATIENT WITH LEFT ATRIUM MYXOMA BEFORE SURGICAL TREATMENT

Among the large number of benign tumors of the heart myxoma most frequently diagnosed neoplasm intracavitary (occurs in 50%). The clinical picture of a myxoma is extremely various. Often it is diagnosed as a casual find. Usually the diagnosis is established according to an echocardiography. Magnetic resonance imaging is used for inspection of patients with suspicion on myxoma in cases when information of echocardiography is not enough and before surgical treatment. We present clinical follow-up 58 years old patients with left atrial myxoma, which before surgical treatment has executed a heart MRI.

The Questions of Tactics of Surgical Treatment of Primary Heart Tumors

&#x0D; Introduction. In the structure of cardiac neoplasms, myxoma accounts for 80–90% of benign heart tumors. The frequency of diagnosis of primary heart tumors (PHT), of which more than 80% are morphologically benign tumors, ranges from 0.09% to 1.9% of the total number of hospitalized patients.&#x0D; The aim of the study is to identify important issues of diagnosis and tactics of the surgical treatment of primary heart tumors.&#x0D; Material and methods. At the In Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine for the period from January 1, 1969 to January 1, 2019, 962 patients operated due to primary cardiac tumors. The myxomas of heart (MH) founded in 856 (89.0%) patients, of which in 752 (87.8%) cases – MH of left atrium (LA). The right atrial myxomas (RA) were diagnosed in 77 (9.0%) cases, MH in the left (LV) and in the right (RV) ventricles each in 8 (0.9%) cases, respectively. Multicentric tumor growth with the damage of two or three chambers of the heart was found in 11 (1.3%) patients. The age of patients with MH was from 3 to 78 years old (the middle age 47.5 ± 3.4), of which 621 (72.6%) were between 31 and 60 years old. Non-myxomic benign tumors were observed in 37 (3.9%) cases, malignant tumors – in 67 (7.0%) cases.&#x0D; Results and discussion. The III and IV functional classes according to the NYHA classification included 311 (36.3%) and 73 (8.5%) patients, respectively, which in these groups often required urgent surgical treatment. Hospital mortality over the past 19 years was 0% in the surgical treatment of MH, namely, 493 operations performed without lethal outcomes.&#x0D; Conclusions. The accepted tactics of emergency diagnosis and surgical intervention ensures the effectiveness of treatment with MH, as evidenced by the data of long-term results: 574 (78.3%) patients assigned to the NYHA functional class I, and 108 (14.8%) patients to NYHA functional class II. Survival rate up to 20 years was 79.8%.

Heart myxoma develops oncogenic and metastatic phenotype

Heart myxomas have been frequently considered as benign lesions associated with Carney's complex. However, after surgical removal, myxomas re-emerge causing dysfunctional heart. To identify whether cardiac myxomas may develop a metastatic phenotype as occurs in malignant cancers, a profile of several proteins involved in malignancy such as oncogenes (c-MYC, K-RAS and H-RAS), cancer-associated metabolic transcriptional factors (HIF-1α, p53 and PPAR-γ) and epithelial-mesenchymal transition proteins (fibronectin, vimentin, β-catenin, SNAIL and MMP-9) were evaluated in seven samples from a cohort of patients with atrial and ventricular myxomas. The analysis was also performed in: (1) cardiac tissue surrounding the area where myxoma was removed; (2) non-cancer heart tissue (NCHT); and (3) malignant triple negative breast cancer biopsies for comparative purposes. Statistical analysis applying univariate (Kruskal-Wallis and Dunn's tests) and multivariate analyses (PCA, principal component analysis) revealed that heart myxomas (7-15 times) and myxoma surrounding tissue (22-99 times) vs. NCHT showed high content of c-MYC, p53, vimentin, and HIF-1α, indicating that both myxoma and its surrounding area express oncogenes and malignancy-related proteins as occurs in triple negative breast cancer. Based on ROC (receiver operating characteristics) statistical analysis, c-MYC, HIF-1α, p53, and vimentin may be considered potential biomarkers for malignancy detection in myxoma.

The case of diagnosis of the left and right atria giant myxomas

A rare case of the simultaneous left and right atria giant myxomas is reported in the article. A short review of epidemiologic, morphologic and genetic features of these benign tumors is submitted in the issue. The clinical manifestations of the disease are often presented with nonspecific symptoms, so myxoma are often late diagnosed. Timely detection of the heart myxomas allows a correct choice of surgical tactics and contributes to patients’ recovery.

The Tactics of Safe Removal of Heart Myxomas; The Experience of 796 Operations

Objective: In the structure of cardiac new formation, myxoma constitute 80-90% of benign heart tumors. The aim of the study was to determine the features of diagnosis and to search the tactics of optimal and the safe surgical treatment of myxoma of the heart. Material and methods: In N.M. Amosov Institute of Cardio-Vascular surgery of the Academy of Medical Sciences of Ukraine for period from 1.01.1969 to 1.01.2017 889 patients with primary heart tumors had surgical treatment. The myxomas of heart (MH) was founded in 796 (89,5%) cases, from them in 696 (87,4%) cases – MH of left atrium (LA). The other benign heart tumours were observed in 33 (3,7%) cases, malignant heart tumours – in 60 (6,8%) cases. Results: Hospital mortality in the surgical treatment of MН was 4.9% (39 cases). Conclusions: Over the past 16 years, 433 operations have been performed without lethal results, due to the peculiarities of the tactics of emergency diagnosis and surgical treatment, and radicality of removal of heart tumors.

Recurrence of cardiac myxoma. Clinical and morphological aspects

Aim: to conduct a one-center retrospective research of heart myxoma relapses in a large group of patients. Materials and methods. Since 1992 to 2016 115 surgical removal of sporadic cardiac myxoma was performed (44 male, 71 female).Results. Two (1.7%) patients had recurrence of sporadic myxoma of the left atrium. One patient had the tumor returned due to inadequate removal of the tumor. Myxoma was removed without excising the place of fi xation to the interatrial septum. The second patient possibly had recurrence of myxoma due to the conservation of a small tumor fragment in another part of the interatrial septum that was not diagnosed during the removal of the primary tumor. Conclusion. Recurrence of sporadic myxoma of the left atrium is rare. The reasons for the recurrence may be different but usually it is due to non-radical removal of the tumor. The results of our research show that even removal of the myxoma together with the interatrial septum does not fully guarantee the prevention of recurrence. The recurrence of sporadic cardiac myxoma is possibly associated with minor formations that are not diagnosed during surgery. We cannot exclude the possibility of forming myxoma denovo also.

Left atrial myxoma: A rare case report

Primary cardiac tumors are rare and myxomas of heart alone account for 80% of benign tumors. Extensive sampling of specimen is important not only to study diverse histologic feature of myxoma cells but also to rule out primary malignant tumor with myxomatous differentiation. Resected margin was free from tumor in the present case, thus reducing the chances of recurrence.

Morphological characteristic of surgically removed cardiac myxomas: 25-YEAR EXPERIENCE IN V.I. SHUMAKOV FEDERAL RESEARCH CENTER OF TRANSPLANTOLOGY AND ARTIFICIAL ORGANS

Analysis of intracardiac space-occupying lesions, surgically removed at V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs over 25 years (in the period from 1992 until 2016) was carried out. During that period 134 patients underwent surgery. Preoperative clinical diagnosis before surgery was heart myxoma. From 134 patients, only in 115 (85.8%) patients the removed intracardiac lesions were myxomas: left atrium – 103 (89.7%) patients, right atrium – 10 (8.7%), right ventricle – 2 (1.8%) and mitral valve – 1 (0.8%) patient. 9 patients had thrombi in the heart, 10 patients had not myxomas, but other benign and malignant heart tumors. This article presents macroscopic, histolological and ultrastructural characteristic of cardiac myxomas. Recurrence of the left atrium myxoma occurred in 2 patients. They were re-operated in four and six years after the first removal of myxoma. 2 patients died during hospital period. One patient with atherosclerosis of the coronary arteries as a concurrent disease died of embolization of the stent coronary artery with myxoma particles with the development of acute heart failure. Death of the 2nd patient was not directly related to the surgery, and was due to the acute heart failure as well in connection with the presence of concurrent disease, coronary heart disease.

Management of a Heart Myxoma Through a Right Thoracotomy in a Patient With a Previous Sternotomy

Myxoma is the most common benign cardiac tumor, presenting most frequently in the left atrium. Median sternotomy has been traditionally used to provide access to the heart and the mediastinal structures in cardiac operations. Recently, right thoracotomy and minithoracotomy have been used as an alternative. We present a case of a patient with left atrial myxoma, who had been subjected to coronary artery bypass graft 2 years ago. The previous full median sternotomy urged the need for another approach. A right lateral thoracotomy with one lung ventilation was chosen, offering excellent access to the heart and mediastinum. The patient was set under cardiopulmonary bypass and cardioplegia and the mass was excised through a left atrial incision. The perioperative course was free of complications. We suggest that right thoracotomy is a safe and efficient way of treating cardiac tumors in selected patients, when median sternotomy is considered complicated, not possible or contraindicated. J Med Cases. 2016;7(2):52-53 doi: http://dx.doi.org/10.14740/jmc2405w

Management of a Heart Myxoma Through a Right Thoracotomy in a Patient With a Previous Sternotomy

Management of a Heart Myxoma Through a Right Thoracotomy in a Patient With a Previous Sternotomy

Thirteen years follow-up of heart myxoma operated patients: what is the appropriate surgical technique?

Cardiac myxoma is a benign neoplasm that represents the most prevalent primary tumor of the heart. If not treated with the right surgical technique recurrence occurs. Aim of our study is to present our surgical approach and the histology of the tumors resected. All patients, except for one, underwent extracorporeal circulation and mild hypothermia, right atrial or both atrial incision and excision of the fossa ovalis, followed by prosthetic patch suturing. All specimens were submitted for microscopic evaluation (haematoxylin-eosin). We contacted personally each patient and asked them to complete a standardized questionnaire, concerning their peri-operative characteristics. Six cases were "active" myxomas, 3 were "mildly active" and 3 were "inactive". "Normal differentiation" was seen in 6, "medium" in 1 and "poor" in 5 cases. In our series there were no recurrences recorded during the follow-up period. The ideal approach, according to our experience is right atrial or both atrial incision as described by Shumacker and King, with excision of the fossa ovalis and the surrounding tissues and closure with a pericardial patch. Such a technique provides an excellent long-term survival in these patients.

The complex of myxomas, spotty skin pigmentation and endocrine overactivity (Carney complex): imaging findings with clinical and pathological correlation

The complex of myxomas, spotty skin pigmentation and endocrine overactivity, or Carney complex (CNC), is a familial multiple endocrine neoplasia and lentiginosis syndrome. CNC is inherited in an autosomal dominant manner and is genetically heterogeneous. Its features overlap those of McCune-Albright syndrome and other multiple endocrine neoplasia (MEN) syndromes. Spotty skin pigmentation is the major clinical manifestation of the syndrome, followed by multicentric heart myxomas, which occur at a young age and are the lethal component of the disease. Myxomas may also occur on the skin (eyelid, external ear canal and nipple) and the breast. Breast myxomas, when present, are multiple and bilateral among female CNC patients, an entity which is also described as “breast-myxomatosis” and is a characteristic feature of the syndrome. Affected CNC patients often have tumours of two or more endocrine glands, including primary pigmented nodular adrenocortical disease (PPNAD), an adrenocorticotropin hormone (ACTH)-independent cause of Cushing’s syndrome, growth hormone (GH)-secreting and prolactin (PRL)-secreting pituitary adenomas, thyroid adenomas or carcinomas, testicular neoplasms (large-cell calcifying Sertoli cell tumours [LCCSCT]) and ovarian lesions (cysts and cancinomas). Additional infrequent but characteristic manifestations of CNC are psammomatous melanotic schwannomas (PMS), breast ductal adenomas (DAs) with tubular features, and osteochondromyxomas or “Carney bone tumour”. Teaching Points • Almost 60 % of the known CNC kindreds have a germline inactivating mutations in the PRKAR1A gene. • Spotty skin pigmentation is the major clinical manifestation of CNC, followed by heart myxomas. • Indicative imaging signs of PPNAD are contour abnormality and hypodense spots within the gland. • Two breast tumours may present in CNC: myxoid fibroadenomas (breast myxomatosis) and ductal adenomas. • Additional findings of CNC are psammomatous melanotic schwannomas (PMSs) and osteochondromyxomas.