Mirta Koželj

Cardiac granulocytic sarcoma diagnosed by intracardiac echocardiography-guided biopsy

A 52-year-old man presented with clinical and echocardiographic signs of cardiac tamponade. A transthoracic echocardiogram revealed a large right atrial mass that obstructed the superior vena cava flow. Cardiac magnetic resonance imaging and computed tomography demonstrated extracardiac tumour invasion of the free atrial wall extending to the right pulmonary hilus. Intracardiac echocardiography-guided biopsy of the tumour revealed the tissue diagnosis-granulocytic sarcoma of the heart. The patient was effectively treated with radiotherapy, chemotherapy and allogeneic haematopoietic stem cell transplantation. He has remained free of the disease for 12 months after treatment.

Pulmonary hypertension in patients with advanced heart failure is associated with increased levels of interleukin-6.

Abstract Context: Inflammatory, endothelial and neurohormonal biomarkers are involved in heart failure (HF) and pulmonary hypertension (PH) pathogenesis. Objective: To study these biomarkers in PH due to advanced HF. Materials and methods: Thirty adults with HF were included. Interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), high-sensitivity C-reactive protein (hsCRP), endothelin-1 and N-terminal prohormone of brain natriuretic peptide (NT-proBNP) were measured in peripheral vein and pulmonary artery during right heart catheterisation. Results: IL-6, TNF-α, hsCRP and NT-proBNP correlated with pulmonary pressures independent of ventricular function, HF etiology and vascular bed. IL-6 was independent predictor of systolic pulmonary artery pressure (sPAP). Discussion and conclusion: Inflammatory biomarkers correlate to PH severity. IL-6 predicts sPAP in advanced HF.

A 6-year follow-up study of adult patients with congenitally corrected transposition.

The aims of this study were to assess the development of heart failure in patients with congenitally corrected transposition of the great arteries in a medium-term follow-up, to identify the impact of tricuspid regurgitation on the development of heart failure, and to determine the most reliable marker for its identification. The prospective 6-year follow-up study included 19 adult patients. All patients were evaluated clinically by the determination of N-terminal pro-hormone brain natriuretic peptide levels, exercise stress testing, echocardiography magnetic resonance, or CT. Among them, two patients died of heart failure. There was a decline in exercise capacity and systolic systemic ventricular function (p=0.011). Systemic ventricular ejection fraction decreased (48.3±13.7 versus 42.7±12.7%, p=0.001). Tissue Doppler imaging showed a decline in peak tricuspid systolic annular velocity (10.3±2.0 versus 8.3±2.5 cm/second, p=0.032) and peak tricuspid early diastolic annular velocity (14.6±4.3 versus 12.0±4.5 cm/second, p=0.048). The tricuspid regurgitation did not increase significantly. N-terminal pro-hormone brain natriuretic peptide levels increased (127.0 ng/L(82.3-305.8) versus 226.0 ng/L(112.5-753.0), p=0.022). Progressive exercise intolerance in congenitally corrected transposition of the great arteries appears to be driven mainly by a progression in systemic right ventricular dysfunction. Tricuspid regurgitation is likely to play a role, especially in patients with structural abnormalities of the tricuspid valve - Ebstein anomaly. The N-terminal pro-hormone brain natriuretic peptide levels and tissue Doppler parameters appear sensitive in detecting changes over time and may guide management.

Heart transplantation due to advanced chronic heart failure after complete correction of tetralogy of Fallot.

A 52-year-old patient decades after complete correction of tetralogy of Fallot is presented. The main late complication was left ventricular failure with consequential congestive heart failure. Heart transplantation was the only therapeutic option.

The commonest mistakes in the treatment of adult patients with congenital heart disease

Background: General practitioners and medical specialists are seeing adults with congenital heart disease in their everyday practice more frequently than ever. The lack of experience and knowledge in this field of cardiology often leads to mistakes in the treatment of these patients. The purpose of this article is to point out the commonest problems and mistakes in dealing with this group of patients. Conclusions: In the following article we pointed out the commonest problems and mistakes in dealing with adults with congenital heart disease, such as differing various types of atrial septal defects and differing atrial septal defect from patent foramen ovale. We also pointed out the commonest mistakes in dealing with patients with cyanotic heart disease, especially those with Eisenmenger syndrome.

Non-compaction cardiomyopathy - an unusual cause of heart failure.

Introduction: Non-compaction cardiomyopathy is a rare inborn anomaly caused by disorder of endomyocardial morphogenesis. The diagnosis is based on echocardiographic criteria. The prevalence in the adult population is not known. The symptoms are atypical. Three main groups of clinical signs exist: heart failure, thromobembolic events and arrhythmias. In the group of patients with reduced left ventricular function the prognosis is poor and the treatment options are limited. Patients and methods: In the recent 10 years, 7 patients with non-compaction cardiomyopathy were diagnosed at the Department of Cardiology of the University Medical Centre Ljubljana. Results: All seven patients were males, their mean age at the last follow-up being 39 ± 20.3 years (range 20 to 70 years). Five patients were diagnosed in adulthood. All of them fulfilled the echocardiographic diagnostic criteria of noncompaction cardiomyopathy. Five patients had depressed function of both ventricles, two patients had isolated left ventricular dysfunction. Three patients had decreased left ventricular ejection fraction, six patients showed left ventricular diastolic dysfunction. Only three patients had normal physical capacity. Two patients presented with clinical signs of overt heart failure. During follow-up, one patient died from heart failure. We observed thromboembolic events in one patient. Three patients suffered from nonsustained ventricular tachycardias and two patients had rhythm conduction abnormalities. Conclusions: Non-compaction cardiomyopathy is a rare disorder. We observed all common complications in our group of patients. The majority of patients displayed dysfunction of the affected ventricle and the dysfunction was more pronounced in older patients. Treatment of complications is an important factor in long-term survival of these patients.

Percutaneous balloon aortic valvuloplasty

Background: As the population ages there is an increasing number of elderly patients with severe symptomatic aortic stenosis and numerous co-morbidities. Decision for aortic valve replacement–a gold standard for the treatment of aortic stenosis–might be dii - cult in this group of patients,in whom open heart procedure may be associated with an unacceptably high risk. Less invasive percutaneous methods are being developed as alternative options for improving the quality of life and prolonging survival. Balloon aortic valvuloplasty is a percutaneous catheterbased method for dilation of the aortic valve, which was in the past almost abandoned due to a high incidence of restenosis and the lack of impact on long-term survival. Twenty years at er its introduction in the elderly patients, together with the development of percutaneous aortic valve implantation methods, balloon aortic valvuloplasty is regaining its role. Case report: We report a case of a 88-yearold woman with severe symptomatic aortic stenosis and a high perioperative risk, who was considered unsuitable for surgical aortic valve replacement. Owing to worsening of her symptoms, she had balloon aortic valvuloplasty performed. At er the procedure she improved clinically and objectively. Conclusions: Our case points out that there are additional therapeutic options for highrisk patients with severe aortic stenosis. Many share the opinion that balloon aortic valvuloplasty will play an important role as a palliative therapy or a bridge to surgical or percutaneous prosthetic valve implantation

Ebstein's anomaly and a rare coagulation disorder - case report and review of literature.

Backgrounds: Ebstein’s anomaly of tricuspid valve is a rare congenital heart anomaly. Surgical implantation of artefi cial tricuspid valve is a standard treatment modality. Common late complication of such surgical procedure is artefi cial valve thrombosis. Th is is a life threatening condition, which demands surgical intervention promptly. Conclusions: In following article, we describe a case of a patient with Ebstein’s anomaly and late artefi cial tricuspid valve thrombosis. Th e case was complicated with a mild form of haemophilia C, a rare coagulation disorder. Genetic analysis was also performed.