Objectives: To evaluate the association between histologically verified left ventricular (LV) myocardial fibrosis (MF) and its bio- and functional markers with pulmonary hypertension (PH) in severe aortic stenosis (AS). Methods: About 34 patients with isolated severe AS underwent 2D echocardiography, cardiac magnetic resonance (CMR) imaging, and plasma NT-proBNP evaluation before aortic valve replacement (AVR). LV measurements were analyzed by CMR and LV strain using feature tracking software (Medis Suite QStrain 2.0). Myocardial biopsy sampled at the time of AVR was assessed by a histomorphometric analysis. PH was defined as pulmonary artery systolic pressure (PASP) ⩾ 45 mm Hg. Results: Patients with severe AS and PH (mean PASP 53 ± 3.7 mm Hg) had higher extent of diffuse MF versus patients without PH (12 (10.4–12.7)% vs 6.6 (4.6–8.2)% (p = 0.00)). The extent of diffuse MF correlated with LV dilatation ( r = 0.7, p = 0.02), indices of LV dysfunction (lower ejection fraction ( r = −0.6, p < 0.001), global longitudinal ( r = −0.5, p = 0.02) and circumferential strain ( r = −0.5, p = 0.05), elevated NT-proBNP ( r = 0.5, p = 0.005) and elevated PASP ( r = 0.6, p < 0.001)). Histological MF > 10% (AUC 94.9%), LV global longitudinal strain > −15.5% (AUC 86.3%), and NT-proBNP > 2090 ng/l (AUC 85.1%) were independent predictors of PH in severe AS. Conclusions: The extent of diffuse myocardial fibrosis in combination with reduced longitudinal left ventricular strain and increased plasma levels of NT-proBNP relates to pulmonary hypertension in severe aortic stenosis.

Patient presentation A 33-year-old man was referred to our hospital suffering fever with shivers, dyspnea, dry cough, and weakness. Initial work up Symptoms appeared three weeks until hospitalization. Laboratory results showed leukocytosis and high levels of inflammatory markers. Haematological diseases were excluded, a chest x-ray showed infiltration in the lower lobe of the right lung, antibiotics were prescribed. Diagnosis and management When clinical and laboratory results showed no signs of improvement, chest computed tomography (CT) scan was performed and bilateral pulmonary artery thromboembolism with right lung infarct-pneumonia was diagnosed (Figure 1, 2). Treatment was supplemented with intravenous anticoagulants. In the course of treatment patient’s status worsened, respiratory failure progressed, so CT scan was performed again and showed massive pulmonary artery thromboembolism without the effect of treatment. Transthoracic 2D echocardiography showed dilated right heart chambers, relative severe tricuspid valve regurgitation, increased systolic pulmonary artery pressure (Video 1, figure 3), pulmonary artery valve masses in the stem which caused obstruction with maximum gradient about 50 mmHg(Video 2, figure 4). For a detailed workup, magnetic resonance imaging (MRI) was done, that revealed tumorous masses from pulmonary artery valve to the pulmonary trunk and right pulmonary branch (Figure 5, 6, 7). The patient underwent pulmoangiography and biopsy was taken out of the masses that suspected sarcoma. The multidisciplinary team came to a conclusion to perform surgery considering worsening patient’s status. The patient underwent pulmonary artery prosthesis implantation and right pneumonectomy (Figure 8). Masses from pulmonary artery valve were examined by pathologists, surgical specimen results showed high grade poorly differentiated pulmonary artery intimal sarcoma (Figure 9, 10). Follow up Early postoperative period was complicated with cardiogenic shock and respiratory failure. Despite extracorporeal membrane oxygenation (ECMO) and high doses of vasopressors patient did not survive the 2nd postoperative day. Conclusions Pulmonary artery sarcoma (PAS) frequently can be misdiagnosed as thromboembolism. Multimodality imaging should be considered pulmonary artery filling defects persist despite proper anticoagulation therapy for early diagnosis and better survival. Abstract P1450 Figure 3.

Širdies amiloidozė – tai reta, tačiau vis dažniau diagnozuojama patologija, kuriai bū­ dinga nenormalus baltymo – amiloido – kaupimasis širdies, nervų sistemos ir kituose audiniuose. Liga dažniausiai nustatoma vėlyvoje stadijoje, kai gydymas neveiksmingas. Tikslas. Pateikti pagrindinius širdies amiloidozės tipus, jų klinikinius požymius bei apibendrinti mokslininkų atliktų studijų duomenis apie ankstyvos ligos diagnostikos svarbą ir galimybes, atliekant sinovijos histologinį tyrimą chirurginio riešo tunelinio sindromo (RTS) gydymo metu. Metodai. Iš 19 publikacijų atrinkti ir apibendrinti pastarųjų penkerių metų mokslinėje literatūroje pateiktų 10 studijų duomenys. Išvados. Sergantiesiems abipusiu riešo tuneliniu sindromu, histologinis sinovijos tyrimas chirurginio gydymo metu leidžia anksti – 5–10 metų iki negrįžtamos organų pažaidos – diagnozuoti širdies amiloidozę, tokius ligonius siųsti tikslingam tyrimui ir, anksti pradėjus specifinį ligos gydymą, pasiekti geresnės ligos prognozės.

Background. In the end stage heart failure (HF) patients with moderate to severe tricuspid regurgitation (TR), the surgical decision for tricuspid valve (TV) repair during left ventricular assist device (LVAD) implantation surgery is not clear. Methods. We included 31 patient with end-stage heart failure and moderate to severe TVR (≥II degree) to our retrospective study analysis. 19 patients received TV annuloplasty during LVAD implantation (TVR+ group), whereas 12 patients didn’t (TVR- group). Demographic, clinical and echocardiographic parameters were assessed in both groups preoperatively and 1 month after surgery. Overall 12 months survival was analysed with Kaplan – Meier method and compared between groups. Results. Inhospital or 30 days mortality rate in TVR+ and TVR- groups was 21.1% and 8.3%, respectively (p=0.624). The overall survival rates up to 12 months did not differ between groups (75% vs 57.89%, p=0.452). TV regurgitation grade was reduced significantly after LVAD implantation with (p=0.001) and without TV repair (p=0.008). Dimensions of RV and right atrium decreased in TVR+ group (p&amp;lt;0.001). The size of RV did not change significantly in TVRgroup postoperatively, however, the diameter of right atrium reduced significantly in this group (p=0.01). Systolic and mean pulmonary artery pressures decreased significantly in both groups of patients. The indices of RV longitudinal function were reduced preoperatively and did not change after LVAD implantation with and without TV repair. Conclusions. Pulmonary artery pressures decreased in all patients after LVAD implantation, while the diameter of RV reduced significantly in patients after concomitant TV repair. Early postoperative mortality and 1 year survival rates did not differ between end stage heart failure patients after LVAD implantation with or without TV annuloplasty.

Fabry disease is a genetic lysosomal storage disease (X-linked inheritance), also known as Anderson Fabry disease. It is a lifelong progressive disease, and it develops in patients with the lack of lysosomal enzyme alpha-galactosidase (a-Gal A) and for that reason the globotriaosylceramide (GL-3) accumulates in the endothelium of blood vessels and internal organs, then injury of various organ systems occurs, as renal insufficiency, gastrointestinal disorders, specific changes of the eye fundus, injury of the coronary arteries, hypertrophy of the left ventricle, cardiomyopathy, disorders of the nervous system, specific skin rashes (known as angioceratoma), etc. Therefore, patients with Fabry disease may occur in the practice of each speciality physician. A minor disease course, that occurs in older patients, mainly affects the cardiovascular system and may be undiagnosed. Case presentationA 41-year-old man was hospitalized in the department of cardiology in the clinics of Lithuanian University of Health Sciences (LUHS) for coronary angiography in planning to enroll the patient in the waiting list for a kidney transplant.