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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.

Ključne besede:gastroezofagealna refluksna bolezen; otrok; inhibitorji protonske črpalke; H2-blokatorji; eozinofilni ezofagitis; na inhibitor protonske črpalke odzivna ezofagealna eozinofilija Development of urinary incontinence in a 7-year old boy after therapy with proton pump inhibitors and complete resolution of his clinicopathologic features of eosinophilic esophagitis after H2-receptor antagonist treatment: A case report Klinični primer razvoja urinske inkontinence pri 7-letnem fantu med zdravljenjem z inhibitorjem protonske črpalke in izginotje kliničnopatoloških znakov eozinofilnega ezofagitisa po zdravljenju z antagonisti H2 receptorjev AbstractBackground: Several diseases result in profound infiltration of esophageal mucosa by eosinophilic granulocites, with gastroesophageal reflux disease (GERD), eosinophilic esophagitis (EoE) and proton-pump-inhibitor-responsive esophageal eosinophilia (PPI-REE) being the most prevalent. Proton-pump-inhibitor-responsive esophageal eosinophilia (PPI-REE) is a newly recognized entity that must be differentiated from eosinophilic esophagitis (EoE).Case presentation: A 7-year old Slovenian male presented with a few-month history of chest pain, regurgitation and heartburn. First endoscopy was performed and revealed pronounced longitudinal furrows, and on hystology examination > 70 eosinophils per high power field were found through the entire thickness of epithelium and in the submucosis with eosinophilic microabscess formation. Results of 24-hour pH-monitoring (without impedance monitoring) excluded pathologic acid reflux. All allergy tests were negative. The patient started treatment with proton pump inhibitors (PPIs) for three times, twice with pantoprazole before the endoscopy and once with esomeprasole after it to exclude the diagnosis of GERD and PPI-REE. Urinary incontinence reappeared each time just few days after starting treatment and disapeared few days after stopping it. Therefore, urinary incontinence was considered as a plausible adverse effect of therapy with PPIs. As treatment with PPIs was not tolerated, a therapy with H2-receptor antagonists ranitidine was applied for more than 2 months followed by a second endoscopy. Both symptoms and esophageal eosinophilia completely resolved with ranitidine. The resolution of esophageal eosinophilia in PPI-REE has been atributed to proton pump independent antiinflammatory effects of PPIs. No such effects have been described in H2-receptor antagonists. Conclusions:Two unique phenomena were observed in the pediatric patient with profound esophageal eosinophilia: urinary incontinence as an adverse effect of therapy with PPIs, and complete resolution of esophageal eosinophilic inflammation with typical features of EoE after treatment with H2-receptor antagonists. IzvlečekIzhodišča: Mnoge bolezni lahko privedejo do obsežne infiltracije sluznice požiralnika z eozinofilnimi granulociti. Med njimi so najbolj pogoste gastroezofagealna refluksna bolezen, eozinofilni ezofagitis in na inhibitor protonske črpalke odzivna ezofag...

Background Antimalarial drugs chloroquine and hydroxychloroquine represent an important therapeutic option in autoimmune diseases. Rare cases of potentially lethal antimalarial-induced cardiomyopathy (AMIC) have been described. Pattern of minimal changes with wall hypertrophy, followed by restrictive and end-stage dilative cardiomyopathy was proposed in AMIC. Purpose Our aim was to identify patients with definite AMIC and characterize their clinical and imaging findings. Methods Retrospective analysis of admissions to Cardiology department from January 2010 to January 2019 was done to identify patients with definite diagnosis of AMIC based on positive endomyocardial biopsy. Clinical, echocardiographic, and cardiac magnetic resonance (CMR) imaging findings were reviewed if available. Results Three patients with biopsy proven diagnosis of AMIC were identified. Two patients, 62 and 76-year-old females, presented with signs of congestive heart failure, while the third, a 41-year-old man presented with chest pain and cardiac conduction abnormalities necessitating pacemaker implantation. All were treated with chloroquine phosphate. Both females had a history of pacemaker due to atrioventricular block. All had positive troponin and coronary artery disease was excluded. Echocardiography in the first case showed a severely dilated left ventricle (LV) with global hypokinesia and severe systolic dysfunction (LVEF 30%). CMR confirmed dilative cardiomyopathy, furthermore subepicardial late gadolinium enhancement (LGE) of the inferolateral LV wall and of the right ventricle free wall was present. In the second case a restrictive cardiomyopathy pattern was observed with mild biventricular systolic dysfunction and biatrial enlargement. CMR of this case was not available. Echocardiography in the third case showed a normal sized LV with mild diastolic and systolic dysfunction (LVEF 50%), along with septal hypokinesia. CMR showed patchy subepicardial and mid-wall LGE of the septum that was falsely attributed to myocarditis. Additionally, a chemical shift artefact suggestive of myocardial fatty infiltration (Image 1) was visible in the apical septum. After discontinuation of chloroquine two patients improved, while the first patient deteriorated and eventually succumbed to refractory heart failure. Conclusions Our three cases confirm high diversity of cardiac imaging findings in AMIC. Even mild non-specific findings such as LV systolic dysfunction or wall motion abnormalities in patients on antimalarial drugs should therefore be thoroughly investigated. CMR with non-ischaemic LGE pattern may aid in diagnosis, however definite diagnosis is currently possible only with endomyocardial biopsy. Novel imaging techniques, such as T1 mapping, have a potential to increase the diagnostic yield given the known patohistological similarities between AMIC and Anderson-Fabry disease. Abstract P362 Figure. Chemical shift artefact (arrow) in AMIC

Cardiac tumors are rare, and their treatment differs interindividually regarding the histopathological proprieties and the stage of disease. Authors present a case of symptomatic cardiac melanoma metastasis that expressed an <i>ERBB2</i> (<i>HER2</i>) gene amplification in a course of the disease that has not yet been reported. The frail patient with a history of pulmonary and renal carcinoma, was admitted to the hospital due to a symptomatic left atrial tumor mass. The patient underwent a tumor-resecting cardiac surgery. At first mistaken for myxoma on echocardiography, the histopathological examination of the tumor revealed a melanoma of acral or mucosal origin. The melanoma metastasis was negative for common genetic mutations in <i>BRAF</i>, <i>NRAS</i> or <i>KIT</i> genes, and for the presence of <i>NTRK</i> genes fusions, but carried <i>ERBB2</i> (<i>HER2</i>) gene amplification. The absence of standard gene mutations rendered it unresponsive to treatment with BRAF and MEK inhibitors. This molecular finding is rare in melanomas and represented a therapeutic target for off-label systemic treatment with drugs, primarily aimed at ERBB2 positive breast, gastric, and gastroesophageal junction cancers. A rare finding like this justifies molecular genetic analysis of unusual tumor specimen and guarantees optimal treatment for uncommon types of cardiac metastatic tumors.