Right atrial myxoma as the first manifestation of granulomatosis with polyangiitis, and a possible association with vascular endothelial growth factor (VEGF) and interleukin 6 (IL-6): a case report and review of the literature

Background Granulomatosis with polyangiitis and myxomas are rare conditions previously described to co-exist. Cardiac masses are often presumed to be myxomas rather than lesions of granulomatosis with polyangiitis. Case presentation We present a review of the symptoms for the two diagnoses along with the first verified case. Conclusions Two possible risk factors for developing myxomas (VEGF and IL-6) are explored and discussed.

Echocardiographic characteristics of cardiac myxoma

Background Accurate imaging differentiation of various cardiac masses is pivotal due to differences in clinical management and treatment. The most common primary cardiac tumor is cardiac myxoma (CM), which is typically located in the left atrium attached to the interatrial septum. Although benign, serious clinical manifestations may occur and surgical treatment of CM is warranted in most cases. Echocardiography is the most common imaging modality, with a reported sensitivity of 90–96%, however accurate diagnosis can be challenging due to the heterogeneous morphological presentation of CM. Purpose The aim of this study was to determine the utility of echocardiography in CM diagnosis. Methods We retrospectively analyzed the echocardiographic and pathohistological findings of all consecutive patients admitted to our cardiology department for possible CM between 2005 and 2020. Results During the 15-year period, 73 patients were admitted for diagnostic evaluation of a possible CM. Subsequently, 54 patients (74%) were diagnosed with CM or another non-myxomal (NM) cardiac tumor, while in others cardiac masses of other etiologies (thrombus, infective endocarditis, etc.) were diagnosed (Figure 1). All but one patient with CM or NM cardiac tumor were treated surgically at our institution and pathohistological specimens were obtained from the resected tumor. There was a significant female preponderance (n=34, 63%) and the mean age at the time of surgery was 64±14 years. Based on the preoperative echocardiographic findings, 45 (85%) tumors were diagnosed as CM and 8 as NM cardiac tumors (Figure 1). Evaluation of pathohistological specimens revealed CM in 39 of 53 (74%) operated patients. Patohistiologically, a NM cardiac tumor was diagnosed in 7 patients who were preoperatively classified as CM. The sensitivity and specificity of preoperative echocardiography for the detection of CM were 97% and 50%, respectively (Figure 1). The echocardiographic characteristics of pathohistologically confirmed CM were compared to cases of NM cardiac tumors. The comparison between the two groups revealed statistically significant differences in localization and tumor size. All NM cardiac tumors were located in an atypical position and 72% of CM were found in a typical position within the left atrium (p<0.001). NM cardiac tumors were also significantly smaller than CM (25.1±12.6 mm vs. 37.5±18.5 mm, p=0.029). Conclusion Our single-center study confirms the excellent sensitivity of echocardiography for CM diagnosis. The specificity of echocardiography was modest, thus caution is warranted due to a wide differential diagnosis of CM. The diagnosis of CM seems to be less likely with atypical tumor location and small tumor size. Other non-invasive imaging modalities such as cardiac computed tomography or magnetic resonance imaging should be considered in such cases. FUNDunding Acknowledgement Type of funding sources: None. Figure 1. Flow chart showing the number of patients diagnosed with CM on preoperative echocardiography.

Left Ventricular Chord Masquerading As An Aortic Valve Papillary Fibroelastoma

Background Characterization of cardiac masses and anomalies is challenging and usually requires multimodality imaging. Herein, we present the case of a left ventricular chord masquerading as an aortic valve papillary fibroelastoma. Case summary In this case, a 62-year-old woman presented with acute right foot weakness. Brain imaging showed multiple areas of acute infarction concerning for a cardioembolic stroke. Transesophageal echocardiography revealed a mass, which was thought to be a papillary fibroelastoma, attached to the non-coronary cusp of the aortic valve. The patient underwent surgical excision of the mass which turned out to be a left ventricular chord crossing the aortic valve and attaching to the left aortic sinus of Valsalva. Conclusion This case underlines the importance of close examination of the aortic subvalvular apparatus when assessing aortic valve masses.

A case of undifferentiated pleomorphic cardiac sarcoma

Primary cardiac sarcoma is known to have a poor prognosis due to late presentation, multiple metastases, and complications, challenging treatment options. We present a case of a 78-year-old female patient who was admitted to our hospital with progressive dyspnea and chest pain. Echocardiography revealed multiple masses of different shape and size in the left atrium, the left and right ventricles. Cardiac magnetic resonance confirmed multiple cardiac masses with signs of invasion in the myocardium and heterogeneous late gadolinium enhancement in all these masses. A biopsy of the abdominal mass revealed an undifferentiated pleomorphic sarcoma. We planned chemotherapy and supportive measures. Before the initiation of the chemotherapy, the clinical situation worsened dramatically. Despite the pericardiocentesis, the patient died due to cardiac tamponade. An autopsy revealed a massive tumor in the interatrial septum and multiple metastases in both ventricles, lungs, and abdomen.