Pál Sármán

Severe mitral regurgitation and heart failure due to caseous calcification of the mitral annulus

Caseous calcification is a rare form of mitral annular calcification. Echocardiography reveals an echodense mass in the inferior mitral annulus with smooth borders and an echolucent inner core. We present a case where caseous calcification of the mitral annulus caused severe mitral regurgitation, atrial fibrillation and heart failure. Transthoracic echocardiography, transesophageal echocardiography, cardiac mag- netic resonance and computed tomography were performed and ensured the diagnosis. The mass was surgically removed and a prosthetic valve was implanted. We conclude that caseous calcification of the mitral annulus should be considered not only in the differential diagnosis of cardiac masses but also in the background of mitral regurgitation, atrial fibrillation and heart failure. This case also represents the usefulness of multimodal imaging in identifying cardiac masses.

Endothelins: a possible mechanism of cytostatics-induced cardiomyopathy

Endothelium responds to physical and chemical stimuli by synthesis and release of a variety of vasoactive and signal molecules. Cardiac performance is regulated by cardiac endothelial cells in a paracrine manner, analogous to vascular endothelial control of vascular tone. Endothelin-1 (ET-1), one of the most potent vasoconstrictor peptides, which is synthetized and released by endothelial cells. The role of ET-1 in some special pathological state is still unclear. Authors have investigated the effect of anthracyclines (maximal dose: 450 mg/bodysurface m2) on left ventricular systolic and diastolic function and on the level of plasma ET-1, in 31 (13 male, aged 19 – 70 years, mean: 38.9) patients suffered from Hodgkin (24) and Non-Hodgkin (7) lymphomas. They have also studied the association between plasma ET-1 concentration and echocardiographic parameters. Serum ET-1 was measured by ELISA method. Left ventricular function analyzed by echocardiography: ejection fraction (EF), time velocity integral (VTI), E and A waves, E/A ratio, deceleration time (DT), Doppler index were assessed. Statistical analysis was made by the Wilcoxon rank test. ET-1 plasma level decreased significantly after therapy (5.6 ± 3.5 vs. 3.1 ± 0.9 pg/ml, P < 0.0006). EF (56.4 ± 5.0% vs. 48.7 ± 5.1%, P < 0.0001) decreased, and DT (168.1 ± 36.8 ms vs. 206.5 ± 58.8 ms, P < 0.0073) increased significantly after administration of anthracycline, showing that both systolic and diastolic left ventricular performance was deteriorated. There was no difference in other echocardiographic parameters before and after therapy. In conclusion, decrease of serum ET-1 concentration might be a result of anthracyclins direct cytotoxic effect and the decreasing level of ET-1 may play a role in the reduction of the EF. More studies are needed to evaluate the presence and severity of endothelial damage, and long-term follow-up may reveal the importance of low ET-1 level and may show the time is needed for the restoration of the ET-1 concentration to the basic level after cessation of cytostatic therapy.

Role of endothelin-1 in the development of a special type of cardiomyopathy

The role of endothelin-1 (ET-1) in certain pathological states is still unclear. We have investigated the effect of anthracyclines (maximum dose, 450 mg/m(2) of body surface) on left ventricular systolic and diastolic function and how it influences the level of plasma ET-1 in 21 patients (12 female and nine male) with Hodgkin and non-Hodgkin lymphoma. We also studied the association between plasma ET-1 concentration and echocardiographic parameters. Serum ET-1 was measured by ELISA. Left ventricular function was analysed by echocardiography: ejection fraction (EF), velocity-time integral, E- and A-waves, E:A ratio, deceleration time (DT) and Doppler index were all measured. Statistical analysis was made by the Wilcoxon rank test. EF and serum ET-1 level decreased significantly (EF, 56.29+/-5.0% to 48.57+/-5.9%, P<0.0001; ET-1, 6.45+/-4.0 pg/ml to 2.9+/-1.0 pg/ml, P<0.0001). DT increased significantly (179.8+/-47.8 ms to 215.5+/-66.7 ms, P<0.01) after anthracycline therapy. There was no difference in other echocardiographic parameters before and after therapy. The decrease in serum ET-1 concentration might be a result of anthracyclines direct cytotoxic effect and the decreasing level of ET-1 can play a role in the reduction of EF. However, more studies are needed to evaluate the presence and severity of endothelial damage.

Intracardiac echocardiography guided cardiac tumormass biopsy

Primary cardiac tumors are rare and often occur without major symptoms. The appropriate therapy for cardiac tumors without metastasis is surgical resection and chemotherapy. However, for certain patients, when the tumor cannot be safely removed, biopsy is recommended since it allows histology examination and further therapeutical considerations. The aim of our case presentation is to describe the case of a 56-year-old woman, in whom cardiac tumor-mass was revealed because of recurrent pericardial fluids. The mass appeared to be non-resectable, therefore biopsy with an eventual histological examination was planned. Intracardiac echocardiography guided percutaneous biopsy was performed. Intracardiac ultrasound ensured accurate localization of the tumor, the catheter-based grasping device and the excision could be instantly monitored. This case demonstrates that an intracardiac ultrasound-guided tumor mass biopsy is a feasible method, which increases accuracy and ensures safety.

[A rare form of adrenal Cushing's syndrome].

The authors present the case history of a 74-year-old male suffering from aortic coarctation. His endocrine evaluation was initiated because of severe hypokalemia. The diagnostic procedures revealed the presence of Cushings syndrome caused by bilateral macronodular adrenal hyperplasia. Because of the high risk of surgical treatment due to his cardiac condition, the patient was treated with the steroid biosynthesis inhibitor ketoconazole, which resulted in a clinical and biochemical regression of hypercortisolism. After interventional treatment of the aortic coarctation the physical and cardiac condition of the patient showed a significant improvement, indicating that despite an old age, surgery offered a valuable tool for management of the disease. To the best knowledge of the authors, the coexistence of aortic coarctation and bilateral macronodular adrenal hyperplasia has not been previously reported. Orv. Hetil., 154(50), 1999-2002.