Right ventricular haemangioma as a rare cause of chest pain: a case report

Background Cardiac haemangioma is a rare primary cardiac tumour. Most patients with cardiac haemangioma have no typical symptoms, and some may present with non-specific manifestations, such as shortness of breath, heart palpitations, or cardiac insufficiency, making it difficult to distinguish cardiac haemangioma from other diseases. We report a case of cardiac haemangioma that present with chest pain. This haemangioma was finally completely excised to relieve the patient’s symptoms and a avoid poor prognosis. Case summary A 14-year-old boy presented with an intermittent and progressive non-exertional chest pain for 2 weeks. Echocardiography showed a space-occupying mass at the right ventricular apex, which was later confirmed by computed tomography angiography and magnetic resonance imaging (MRI). The mass was successfully resected, and postoperative pathology confirmed a cardiac cavernous haemangioma. The patient had an uneventful postoperative recovery at the 8-month follow-up. Discussion Cardiac haemangioma is a benign tumour with no typical clinical manifestations, and very few patients may present with chest pain. Preoperative echocardiography, computed tomography, and MRI are helpful for diagnosis, and surgery can relieve symptoms and may improve the prognosis of patients with cardiac haemangioma.

A Case Report of primary cardiac intimal sarcoma presenting with atrial fibrillation and a Left Atrial Mass

Background Intimal sarcoma is an exceedingly rare type of primary cardiac tumour. It is characterized by poorly differentiated spindle-shaped cells that can mimic smooth muscle and is strongly associated with MDM2 genetic amplification. Owing to its rarity and nondistinctive histological features, diagnosis remains a significant challenge. Case Summary In this case report, we describe a case of primary cardiac intimal sarcoma in a 37-year-old woman who presented with atrial fibrillation and a left atrial mass. Despite having a histological sample from an excised left atrial mass, the diagnosis was not made until she presented with back pain secondary to metastatic disease to the spine. Discussion Primary cardiac intimal sarcoma is an extremely rare diagnosis. The mainstay management of intimal cardiac sarcoma is aggressive surgical resection. Unfortunately, the prognosis of cardiac sarcomas remains very poor, with a mean survival between three months to one year. This case of cardiac intimal sarcoma highlights the difficulty in establishing a diagnosis, particularly given the unusual presentation of atrial fibrillation.

Papillary fibroelastoma of posterior mitral leaflet, confused as vegetation or calcification

Cardiac papillary fibroelastoma (CPF) is the most common valvular tumour of the heart and the second most common primary cardiac tumour. Fewer than 50 cases being reported in the literature [1]. As the advancement in 3D echocardiography, its diagnosis in living patient is become easy. The CPF could be found on valve leaflets, chordae tendineae, and both ventricles. We present an interesting case of the CPF originating from the posterior leaflet of the mitral valve confusing with vegetation or annular calcification. The patient underwent successful surgical removal of the PFE

Prevalence of congenital cardiac tumours in 25,000 neonates

Background Primary cardiac tumours in children are rare, with an estimated prevalence of 0.014% in foetuses, whereas in the paediatric population, a 100-fold difference in autopsy prevalence has been reported (0.0017% to 0.28%). In neonates, rhabdomyoma is the most common primary cardiac tumour, followed by teratoma and fibroma, all of which are benign. Cardiac rhabdomyomas are associated to tuberous sclerosis in >50% of cases. The prevalence of cardiac tumours in neonates has not previously been assessed in a large population-based cohort. Purpose To determine the prevalence of congenital cardiac tumours by systematic echocardiography in a large, population-based, cohort of neonates. Methods Systematic transthoracic echocardiography (TTE), including standard sub-xiphoid, apical, left parasternal, and suprasternal views, was performed in neonates included in a population study. Cardiac tumours were defined as solid, echogenic masses, and description included location, size, and numbers (one/multiple). Information regarding subsequent diagnosis of tuberous sclerosis was collected from review of medical charts. Results A total of 25,537 neonates (age 12±9 days, 51.6% male) were enrolled in the study and underwent TTE. Cardiac tumours were detected in 7 neonates (4 males), corresponding to a prevalence of 0.3 [0.1; 0.6] per 1,000 live-births. Characteristics including sex, gestational age at birth, birth length and weight, APGAR 5-score, age and weight at TTE, and maternal age and pre-pregnancy BMI, did not significantly differ between the neonates with (n=7) and without (n=25,530) cardiac tumours (all p>0.05). In 6 out of 7 neonates, the cardiac tumours were located in the ventricles (86%), with multiple tumours seen in five (83%) (Fig. 1A+B), and with size ranging from 2.0x1.9mm to 10.0x5.1mm. In the seventh neonate, two cardiac tumours were detected in close proximity to the aortic and pulmonary valves in the ascending aortic and main pulmonary artery wall (Fig. 1C), with sizes 5.1x4.7mm and 3.7x3.2mm, respectively. All seven neonates had otherwise structurally normal hearts and no flow obstruction. Three neonates with multiple cardiac tumours in the ventricles were subsequently diagnosed with tuberous sclerosis. Conclusions Systematic TTE in a population-based cohort revealed a prevalence of congenital cardiac tumours of 0.3 per 1,000 live-births. The majority of the tumours were located in the ventricles and most often multiple cardiac tumours were encountered. Detection of congenital cardiac tumours in the ventricles was associated with tuberous sclerosis in half the cases. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Danish Heart Association, Danish Children's Heart Foundation

P847 Echocardiographic imaging in primary cardiac tumour-leiomyosarcoma

Funding Acknowledgements N.A Primary cardiac tumours are rare entities with an incidence rate of 0.0017% to 0.019% of which 25% are malignant. Leiomyosarcomas, a form of primary cardiac sarcoma, accounts for 8-9% of all the cardiac sarcomas (1). Primary cardiac sarcomas typically present late with either quite advanced local cardiac symptoms or non-specific symptoms arising from metastasis (2). We herein report a rare presentation of leiomyosarcoma in a sixty-seven year old female Caucasian female, originally suspected following transthoracic echocardiogram. A large mass filling the Left Atrium(L.A) with a degree obstruction to the Mitral Valve (M.V) was firstly noted on transthoracic echocardiogram. This mass had a broad base, appeared to be originating from the pulmonary veins and was not encapsulated. A small circumferential pericardial effusion was noted. Overall biventricular function was preserved and there was no evidence of pulmonary hypertension. A contrast-enhanced computed tomography scan of the chest, abdomen and pelvis was performed for staging which in addition to known cardiac mass revealed widespread bony metastases. A core biopsy of one of the lumbar paravertebral metastatic deposit was carried out to establish the diagnosis. We hope to add to the pool of increasing overall understanding of primary cardiac sarcomas and share our fascinating echocardiogram findings. In addition, we believe this case supports our recommendation for general practitioners in the United Kingdom to have open access to requesting echocardiograms in order to identify such aggressive cardiac malignancies at an earlier stage, with the hope to improve prognosis. Abstract P847 Figure. Depicting LA mass occupying the whole of

Resection of a large primary left ventricle tumour by cardiac autotransplantation in a 2-month-old infant: a case report

Background Surgery is the fundamental method for the treatment of primary cardiac tumours. However, due to the inaccessibility of anatomy and the proximity of important structures, it is very difficult to completely resect tumours of the left atrium or left ventricle without damaging the normal tissues. Cardiac autotransplantation for the resection of cardiac tumours is carried out by taking out the heart from the body, resecting cardiac tumours, and then transplanting the heart back into the body. Case summary This article presents a successful case of cardiac autotransplantation for the complete resection of primary cardiac tumour in a 2-month-old infant and shares the noteworthy experience. Discussion Tumours located in the left atrium and left ventricle are difficult to be exposed because of their deep posterior location and proximity to important anatomical structures such as mitral valve and chordae tendineae. How to resect the tumours completely without damaging the normal tissues is a great challenge. This case proves that cardiac autotransplantation is a good solution for tumours that are difficult to be resected completely by orthotopic cardiac transplantation.

Rare Association between Giant Right Ventricular Myxoma and Right Coronary Artery Tumour Blush with Complicating Pulmonary Tumour Embolism

Cardiac myxoma is a benign primary cardiac tumour which can present with nonspecific symptoms of right heart failure, syncope, exertional dyspnea, and pulmonary embolism. We describe a case of a right ventricular myxoma complicated with bilateral pulmonary embolism, with an incidental right coronary artery fistula but otherwise normal coronary anatomy on coronary angiogram. This case report emphasizes the importance of performing a transesophageal echo in the setting of pulmonary embolism to search for the origin of thrombus/tumour, and performing a comprehensive assessment is also necessary to rule out coronary artery disease, coronary artery fistula that may also represent a tumour blush.