Application of Three-Dimensional Printing Technology in the Perioperative Management of Cardiac Tumours: A Review and Analysis.

Background: Multimodal imaging plays a crucial role in evaluating suspected cardiac tumours. In recent years, three-dimensional (3D) printing technology has continued to advance such that image-based 3D-printed models have been incorporated into the auxiliary diagnosis and treatment of cardiac tumour diseases. The purpose of this review is to analyze the existing literature on the application of 3D printing in cardiac tumour surgery to examine the current status of the application of this technology. Methods: By searching PubMed, Cochrane, Scopus and Google Scholar, as well as other resource databases, a completed review of the available literature was performed. Effect sizes from published studies were investigated, and results are presented concerning the use of 3D surgical planning in the management of cardiac tumours. Results: According to the reviewed literature, our study comes to the point that 3D printing is a valuable technique for planning surgery for cardiac tumours. As shown in the review report, Mucinous and sarcomatous tumours are the most commonly used tumours for 3D printing, magnetic resonance imaging (MRI) and computed tomography (CT) are the most commonly used technologies for preparing 3D printing models, the main printing technology is stereolithography, and the most used 3D modeling software is Mimics. The printing time and cost required for 3D printing are affected by factors such as the size of the type, complexity, the printed material and the 3D printing technology used. The reported research shows that 3D printing can understand the anatomy of complex tumour cases, virtual surgical simulation, as well as facilitate doctor-patient communication and clinical teaching. Conclusions: These results show that the development of 3D printing technology has brought more accurate and safe perioperative treatment options for patients with cardiac tumours. Therefore, 3D printing technology is expected to become a routine clinical diagnosis and treatment tool for cardiac tumours.

Genetics of Cardiac Tumours: A Narrative Review.

Cardiac tumours can occur in association with genetic syndromes. Rhabdomyomas have been reported in association with tuberous sclerosis, myxomas with Carney's complex, cardiac fibromas with Gorlin syndrome, and paragangliomas with multiple endocrine neoplasm syndrome. The presentation and prognosis of cardiac tumours associated with genetic syndromes differ compared with sporadic cases. Knowledge about the associated syndromes' genetic features and extracardiac manifestations is essential for the diagnosis, prognosis, and management of cardiac neoplasms. Moreover, identifying genetic mutations in benign and malignant cardiac tumours is needed to personalise management and improve treatment outcomes. Thus, this review discusses the genetic abnormalities associated with cardiac tumours, the current genetic screening recommendations, and the effect of those genetic mutations on the outcomes.

Cardiovascular magnetic resonance imaging in suspected cardiac tumour: a multicentre outcomes study.

AIMS: Cardiovascular magnetic resonance (CMR) imaging is a key diagnostic tool for the evaluation of patients with suspected cardiac tumours. Patient management is guided by the CMR diagnosis, including no further testing if a mass is excluded or if only a pseudomass is found. However, there are no outcomes studies validating this approach. METHODS AND RESULTS: In this multicentre study of patients undergoing clinical CMR for suspected cardiac tumour, CMR diagnoses were assigned as no mass, pseudomass, thrombus, benign tumour, or malignant tumour. A final diagnosis was determined after follow-up using all available data. The primary endpoint was all-cause mortality. Among 903 patients, the CMR diagnosis was no mass in 25%, pseudomass in 16%, thrombus in 16%, benign tumour in 17%, and malignant tumour in 23%. Over a median of 4.9 years, 376 patients died. Compared with the final diagnosis, the CMR diagnosis was accurate in 98.4% of patients. Patients with CMR diagnoses of pseudomass and benign tumour had similar mortality to those with no mass, whereas those with malignant tumour [hazard ratio (HR) 3.31 (2.40-4.57)] and thrombus [HR 1.46 (1.00-2.11)] had greater mortality. The CMR diagnosis provided incremental prognostic value over clinical factors including left ventricular ejection fraction, coronary artery disease, and history of extracardiac malignancy (P < 0.001). CONCLUSION: In patients with suspected cardiac tumour, CMR has high diagnostic accuracy. Patients with CMR diagnoses of no mass, pseudomass, and benign tumour have similar long-term mortality. The CMR diagnosis is a powerful independent predictor of mortality incremental to clinical risk factors.

Ventricular tachycardia as the presenting feature in two patients with cardiac lipoma and cardiac fibroma.

We hereby present two patients with benign cardiac tumours presenting as ventricular tachycardia (VT). Most such tumours have a favorable prognosis, unless complicated by arrhythmias. Intracavitary tumours are easily diagnosed by echocardiography. Intramural tumours as in our patients may be missed at times by echocardiography. Multimodality imaging helped confirm the diagnosis and etiology, since biopsy was not safe. Surgical removal was not feasible due to extensive infiltration. The patients are so far doing well on medical therapy.

Differential diagnosis of heart tumours.

Cardiac tumours represent a wide spectrum of neoplastic and non-neoplastic masses. Plenty of them, especially primary cardiac neoplasias, are rare diseases. Last revision of WHO classification introduced several changes in their histopathological assessment. Furthermore, an increasing amount of knowledge in molecular characteristics of the tumours bolstered discussion about the classification of primary cardiac sarcomas and primary intimal sarcoma of the heart became a hot topic of last years. This work aims at individual neoplastic and non-neoplastic cardiac tumours with focus at their characteristic histopathological features and main differential diagnoses.

Large intra-cardiac fibroma identified on cardiac MRI - a case report and review of literature.

Primary cardiac tumours are rare. Cardiac fibroma is a benign tumour of the heart. It is fairly common among children and adolescents and is rarely encountered in adults. We present the case of a thirty-eight year old lady who presented with shortness of breath and was found to have a very large intra-cardiac mass that had cardiac magnetic resonance (CMR) features consistent with cardiac fibroma. The patient was referred for tumour resection, however could not survive the surgery.

New-onset cardiac rhabdomyoma beyond infancy in a patient with tuberous sclerosis complex.

Cardiac rhabdomyomas in patients with tuberous sclerosis complex are usually detected antenatally or during infancy, with subsequent stabilisation or spontaneous regression. Development of a new cardiac rhabdomyoma beyond infancy is very rare. We report a male child who needed resection of a large rhabdomyoma in neonatal life, and then developed a new-onset rhabdomyoma at 2 years of age in a different location, needing another resection. Routine surveillance for cardiac rhabdomyomas in asymptomatic patients with tuberous sclerosis is essential.

A Rare Case of Undifferentiated Pleomorphic Cardiac Sarcoma.

We present a case of a patient with a four-month history of gradual-onset dyspnea and generalized body weakness. During the clinical evaluation, a mass was found in the left atrium. Coronary angiography was performed and showed normal coronary arteries. We proceeded with a complete surgical excision of the tumoral mass, and histopathology confirmed it as undifferentiated cardiac sarcoma. Six months after the surgical intervention and adjuvant chemotherapy, the patient is in complete remission, with no evidence of a recurrence of the malignant pathology. Cardiac sarcoma is a rare clinical finding and a diagnostic and therapeutic challenge due to its numerous non-specific clinical presentations.

Recurrent Pericardial Effusion Secondary to Cardiac Angiosarcoma.

Cardiac angiosarcoma is a malignant cardiac tumour. We present the case of a young patient in his mid-30s with recurrent pericardial effusion. He had flu-like symptoms a month earlier and had shortness of breath, lethargy, and tightness in his throat for the past ten days. Echocardiography demonstrated global pericardial effusion > 4 cm with tamponade features, and the patient was blue-lighted to our hospital. He underwent emergency pericardiocentesis, and > 1 litre of pericardial fluid was drained. Computed tomography of the chest, abdomen, and pelvis revealed small-volume ascites and moderate right-sided pleural effusion, with associated lobar collapse. The patient presented to the hospital with global pericardial effusion requiring emergency pericardiocentesis three weeks later and underwent cardiac magnetic resonance imaging demonstrating global pericardial effusion and a 48 × 26 mm pericardial space mass adjacent to the right atrium. He underwent surgical resection of the tumour, followed by chemotherapy, and tolerated the treatment well. The patient is currently under follow-up.

Case report-right atrial mass: a very rare presentation of endometrial cancer metastasis.

Background: We report a case of a 47-year-old woman with right atrial metastasis of endometrioid adenocarcinoma, which is an uncommon clinical presentation for patients with endometrial cancer (EC). The principal aim of this case is to demonstrate the possibility of distant metastasis, something rarely encountered among this group of patients. Case summary: Our patient, diagnosed with EC and receiving chemotherapy and radiotherapy after surgery, was found to have enhanced 18-fluorodeoxyglucose uptake inside the right atrium on the repeat positron emission tomography-computed tomography scan at the ninth month after initial diagnosis. Following trans-oesophageal echocardiography, cardiac magnetic resonance imaging showed a hyper-vascular mass with right atrial lateral wall involvement likely to be malignant in nature. A right atrial tumour was successfully removed by cardiovascular surgeons, and a pericardial patch was placed at the site of the excised atrium. The pathological examination showed EC metastasis. Following surgery, systemic treatment was planned for recurrent EC. The patient had an uneventful recovery after the surgery. Discussion: Endometrial cancer is the most common gynaecologic malignancy and the fourth most common cancer in women. The lymphatic pathway is the main metastatic behaviour of EC; however, haematogenous metastases are not uncommon, especially in patients with higher stages of the disease. Our patient did not show any signs and symptoms of cardiac involvement. Nevertheless, clinicians should be alert for symptoms of cardiac involvement like new-onset murmur, embolism, or dyspnoea. Having known the behavioural pattern of the primary tumour, timely utilization of diagnostic imaging methods in accordance with clinical suspicions in patients with rapidly growing tumours can be lifesaving.

Iatrogenic aortic valve fenestration rupture following coronary angiography: a case report.

Background: Aortic valve fenestration (AVF) is a relatively common anatomical variation, while its rupture is a rare cause of aortic regurgitation (AR), especially following coronary angiography. This case report highlights the importance of echocardiographic evaluation and multidisciplinary discussion for the differential diagnosis of fibroelastoma or endocarditis. Case summary: A 66-year-old man presented with acute shortness of breath following coronary angiography for an inferior ST-elevation myocardial infarction. Echocardiography revealed severe aortic regurgitation associated with a filamentous mobile structure on the non-coronary cusp. A multidisciplinary discussion was conducted to determine the cause of the AR, leading to the patient undergoing aortic valve replacement. A gross inspection of the valve confirmed AVF rupture. Conclusion: Iatrogenic AVF rupture following coronary angiography is an exceptionally rare complication. Thorough echocardiographic evaluation plays a significant role in differentiating AVF rupture from other valve pathologies, such as fibroelastoma, Lambl's excrescence, or endocarditis. Echocardiographic features, including a very thin, linear, and flail-like mobile lesion, the paracommissural location, frequent involvement of the non-coronary cusp, and an eccentric jet of the AR, are suggestive of AVF rupture. While medical history and clinical examination contribute to the diagnostic process, definitive diagnosis relies on surgery and anatomopathology.

Diagnostic and Therapeutic Challenges Associated With Left Ventricular Tumors.

Cardiac tumors are uncommon. Sometimes it is challenging to differentiate non-invasively between different kinds of cardiac tumors and thrombi, which is critical to dictate the subsequent treatment. In addition, not all high-risk cardiac tumors are amenable to surgical resection posing a therapeutic challenge. We report a case of cardiac papillary fibroelastoma in the left ventricular cavity with a 10-year follow-up, with no embolic complications.

Multiple Right Ventricular Haemangiomas.

Right ventricular haemangiomas are rare benign tumours, usually solitary and commonly located in the right heart. We report a 49-year-old female who presented with four masses in the right ventricle, three arising from the right ventricular free wall and one arising from the anterior leaflet of the tricuspid valve. She subsequently underwent total excision of the tumours and an anteroinferior commissuroplasty for severe tricuspid regurgitation complicating the excision. Histology confirmed cavernous haemangioma. Solitary haemangioma of the right ventricle has been reported severally in the literature but, to the best of our knowledge, this is the first report of multiple right ventricular haemangiomas.

Myxofibrosarcoma or myxoma: malignant transformation or misdiagnosis.

Inadequate excision, seeding, multicentricity and malignancy are often ascribed for recurrence of atrial myxomas. Differentiation between benign myxomas and malignant myxofibrosarcomas can be challenging, and a high index of clinical suspicion and vimentin staining on immunohistochemistry is needed to confirm the diagnosis. Early recurrence is common in these patients and completeness of excision is the key. Prognosis despite successful excision is poor. We report a benign atrial myxoma in a young female patient which upon recurrence turned out to be a myxofibrosarcoma.

Swinging calcified amorphous tumour in the ascending aorta.

A 70-year-old woman with heart failure and end-stage renal disease on dialysis was found to have a 2.9 cm × 0.9 cm swinging mass attached to the posterior wall of the ascending aorta, 3 cm above the aortic valve. Due to the risk of embolization, she underwent an aortotomy and mass excision. The mass had extensive calcifications with degenerative changes and no evidence of malignancy. This represents an exceedingly rare location for a calcified amorphous tumour. Our review adds to the literature establishing the proximal aorta as a characterized location for a calcified amorphous tumour and provides a treatment approach to prevent embolization.

Cardiac myxoma: single tertiary centre experience.

BACKGROUND: Although cardiac myxoma (CM) are rare and benign, they can cause life-threatening complications, such as hemodynamic disturbances or embolization. Surgical excision of the tumour is the treatment of choice. The aim of the study was to evaluate the epidemiological characteristics, clinical presentation, imaging findings, and outcomes of surgical treatment of patients with CM treated in the largest tertiary care centre in Slovenia. PATIENTS AND METHODS: We retrospectively analysed the medical records of all patients referred to our institution between January 2005 and December 2020 and identified 39 consecutive adult patients with pathologically confirmed CM. RESULTS: The average annual incidence of CM in the study was 3 per 2 million population per year. Patients were more often female (n = 25, 64%). The mean age at diagnosis was 63.1 ± 13.6 years. Dyspnoea was the most common presenting symptom (31%). CM was an incidental finding in 11 patients (28%). Seven patients presented with thromboembolic event (18%). Transthoracic echocardiography (TTE) was performed in all patients, however additional imaging was required in 22 patients (56%). All patients in our series were successfully treated surgically without in-hospital mortality. During the follow-up period (6 months to 16 years) three patients (8%) died, and all deaths were unrelated to CM. There was no recurrence of CM during the follow-up. CONCLUSIONS: Our single-centre study confirms that CM is rare cardiac tumour with diverse clinical presentation. Our data shows data that CM might be more prevalent than considered before. Surgical resection of the tumour is safe with excellent short- and long-term outcomes.

Manifestation and management of intravenous leiomyomatosis: A systematic review of the literature.

Intravenous leiomyomatosis (IVL) is characterized by the presence of vascular extension and invasion of benign smooth muscle lesions in a worm-like manner from uterine fibroids into the systemic vasculature system. Surgery with complete tumour resection remains the main treatment approach, however both treated and untreated of this disease is associated with high morbidity and mortality. The aim of this systematic review is to highlight the systemic manifestations and surgical management of IVL.