Coexistence of neurofibromatosis type 1 with multiple malignant neoplasia.

Neurofibromatosis type 1 (NF1, von Recklinghausen disease) is inherited in autosomal dominant way genetic disorder, with an incidence at birth 1:3000. It is one of the most common congenital disorders. It is characterized by café-au-lait spots, neurofibromas, and less common MPTST and gliomas of the optic nerve. It is caused by germline mutations of the NF1 gene, which acts as tumor suppressor. Inactivation of the gene leads to increased activation of the kinase pathways, and in consequence, uncontrolled proliferation of cells. The disease predisposes to the development of both benign and malignant tumors. Malignant tumors, but not related to the nervous system occur in neurofibromatosis quite rare. The aim of the study is a literature review of NF1, with presentation of a patient with NF1 and coexisting numerous tumors: synchronous somatostatinoma and gastrointestinal stromal tumor with metachronous prostate adenocarcinoma and non-small cell lung carcinoma. And attempt to answer the question if there is a common pathway for oncogenesis of these four tumors.

[A comparison between two investigator's difference of results of left ventricular parameters using echocardiography and cardio MR in hypertensive patients]. / Analiza badanych parametrów lewej komory serca u pacjentów z pierwotnym nadcisnieniem pod wzgledem zgodnosci wyników pomiedzy dwoma badajacymi osobami w echokardiografii i kardio MR.

The aim of the study is estimation of compatibility between morphological and functional results obtained from two independent evaluations used nowadays in cardiology procedures: echocardiography and Cardio MR. 48 patients were examined (24 fema les and 24 males) from 37 to 75 years of age (mean age was 56 years) with diagnosed cardiac hypertrophy in the course of 2nd and 3rd degree primary hypertension. All patients from this gro up underwent echocardiography exami nation obtained from two independent evaluations, using 2.5 M Hz transducer. Also all the patients underwent Cardio MR examination using MR 1.5 T Signa Excite (GE) system, obtained from two independent evaluations. Dedicated software was used for post-processing (MASS). The same left ventricular mor phological and functional parameters. were assessed in both procedures. Analysis of differences between results obtainedfromtwo independent doctors of the same parameter for each method separately was performed. It was assu med, that the method there were less differences between results obtained from two examiners foreach parameter, was more objective. Statistically significant differences in measurements between different examiners in echocardiography was demonstrated of the following left ven tricular parameters: LV end-diastolic diameter (LVDd), LV end-systolic dia meter (LVSd), end-diastolic long axis (LD), end-systolic long axis (LS), end diastolic volume (EDV), end-systolic volume (ESV), ejection fraction (EF) and left ventricular mass (LVM). No statistically significant differences in measurements between both exami ners in Cardio MR for all measured LV parameters was demonstrated. Cardio MR examination is the procedure, where the result not depends on the operator.

[The assessment of left ventricular hypertrophy in patients with the primary hypertension with the use of Cardiac Magnetic Resonance]. / Ocena przerostu miesnia lewej komory serca u pacjentów z pierwotnym nadcisnieniem tetniczym przy pomocy badania Kardio MR.

The aim of the study is to present usefulness of Cardiac Magnetic Resonance (CMR) to assess left ventricular hypertrophy in hypertensive patients. 48 patients were examined (24 females and 24 males) from 37 to 75 years of age (mean age was 56 years) with diagnosed cardiac hypertrophy in the course of 2nd and 3rd degree primary hypertension. All patients from this group underwent Cardio MR examination using MR 1.5 T Signa Excite (GE) system. Dedicated software was used for post-processing (MASS, Medis). We assessed left ventricular functional parameters such as: Ejection Fraction (EF), End-Diastolic Volume(EDV) and End-Systolic Volume (ESV). We also assessed left ventricular diameters as well as left ventricular posterior wall (PWD) and interventricular septum (IVSD) thickness. We also calculated left ventricular mass (LVM) and left ventricular mass index (LVMI). In most cases (66.7%) patients had significant increase of LVM. On the base of LVMI hypertrophy of the left ventricle was found in 6.2% patients. We affirmed statistically significant changes of left ventricular's morphological and functional parameters. The significant correlation was found between posterior wall diameter (PWD) and LVM, as well as between interventricular septum (IVSD) and LVM. The significant correlation was found between posterior wall diameter (PWD) and left ventricular Ejection Fraction (EF) and End-Systolic Volume (ESV). Cardio MR is an effective method to detect left ventricular hypertrophy (LVH) in hypertensive patients.

A rare case of aggressive, hereditary paraganglioma associated with a pathogenic variant in SDHD.

Not required for Clinical Vignette.

Angiographic perfusion score assessed in patients with acute myocardial infarction is correlated with cardiac magnetic resonance infarct size and N-terminal pro-brain natriuretic peptide in 6-month follow-up.

UNLABELLED: Angiographic Perfusion Score (APS) proposed as a simple, angiographic score linking epicardial and myocardial perfusion parameters before and after percutaneous coronary intervention (PCI) is a predictor of short-term outcome in patients with ST-segment elevation myocardial infarction (STEMI) treated with PCI. Aim of the study was to analyze the correlation between APS and both infarct size and left ventricular function in long-term follow-up. In a cohort of 68 patients with STEMI treated with PCI APS was calculated for infarct-related artery based on angiographic parameters and was defined as the sum of the Thrombolysis in Myocardial Infarction (TIMI) flow grade (0-3 points) and the TIMI myocardial perfusion grade (0-3 points) before and after PCI (range of points from 0 to 12). Full perfusion was defined as APS ≥ 10. Cardiac magnetic resonance (CMR) parameters and N-terminal pro-brain natriuretic peptide (NT pro-BNP) were assessed at 6 months. RESULTS: Median APS was 7.5 points. APS ≥ 10 was present in 42% of patients. The significant correlation was found between APS and: CMR infarct size (r = - 0.48; P = 0.0001), CMR left ventricular (LV) ejection fraction (r = 0.5; P = 0.002), LV end-diastolic volume index (r = - 0.37; P = 0.004), LV end-systolic volume index (r = -0.41; P = 0.001), NT pro-BNP (r = - 0.5; P = 0.02). Patients with APS ≥ 10 had significantly lower infarct size, LV volumes, higher EF and lower NT pro-BNP. APS assessed in patients with STEMI treated with PCI is a good predictor of infarct size and left ventricular function in 6-month follow-up.

[The role of MRI in the assessment of the cardiac anatomy and function]. / Przydatnosc badania MR w ocenie budowy i czynnosci serca.

This paper discusses the role of MRI in the assessment of the cardiac anatomy and function. Cardiac MRI offers the possibility of precise quantitative heart evaluation (also in measuring the volume and mass of the heart ventricles). It is characterised by high reproducibility compared with that of echocardiography. For the assessment of the heart and the great vessels the Spin Echo and Fast Spin Echo sequences are used. The observation of the heart function is possible in Gradient Echo sequences, in the cine mode. Contrast enhancement sequences allow the assessment of the heart perfusion, the detection and scope of ischaemic area and post-infarction scar as well as the differentiation between necrotic tissue from stunned muscle. In many cardiac conditions MRI may play an important role. They include ischaemic heart disease, cardiomyopathies, cardiac defects and less common pathologies such as tumours, sarcoidosis, amyloidosis and haemochromatosis. The use of cardiac MRI in the diagnostics of those conditions is discussed.

ST-segment resolution assessed immediately after primary percutaneous coronary intervention correlates with infarct size and left ventricular function in cardiac magnetic resonance at 1-year follow-up.

BACKGROUND: Little is known about the predictive value of electrocardiographic ST-segment resolution (STR) assessed immediately after primary percutaneous coronary intervention (PCI). The aim of the study was to analyze the value of STR and maximum single-lead ST-segment elevation assessed immediately after primary PCI in prediction of infarct size and left ventricular function in cardiac magnetic resonance (CMR) at 1-year follow-up. METHODS AND RESULTS: A total of 28 patients with anterior wall ST-segment elevation myocardial infarction treated with primary PCI entered the study. There was a significant correlation of STR and maximum single-lead ST-segment elevation assessed immediately after primary PCI and CMR infarct size and left ventricular function after 1 year. When analyzed according to standard optimal reperfusion cutoff (70% for STR and 1 mm for single-lead elevation), both electrocardiographic parameters were also good predictors of CMR infarct size and left ventricular function after 1 year. CONCLUSIONS: ST-segment resolution and the single-lead maximum ST-segment elevation assessed immediately after primary PCI for ST-segment elevation myocardial infarction are good predictors of infarct size and left ventricular function in 1-year follow-up.

Effects of early abciximab administration before primary percutaneous coronary intervention on left ventricular function assessed by cardiac magnetic resonance.

BACKGROUND: It has been shown that early abciximab administration before primary percutaneous coronary intervention (pPCI) for ST-segment elevation myocardial infarction (STEMI) improves efficacy of treatment. However, there are no data on the impact of this strategy on left ventricular (LV) function during long-term follow-up. AIM: To analyse the effects of early abciximab administration in patients with first anterior STEMI treated with pPCI on infarct size and LV function assessed by cardiac magnetic resonance. METHODS: A total of 59 patients with STEMI, <12 hours from the chest pain onset, without cardiogenic shock, admitted to local hospitals without interventional facilities, with anticipated delay to pPCI <90 min were randomly assigned to two study groups: 27 patients received abciximab before transfer to the catheterisation laboratory (early abciximab group), and 32 patients received abciximab in the catheterisation laboratory just before pPCI (late abciximab group). All patients received aspirin and heparin (70 U/kg) before transfer to the cath lab. Clopidogrel loading dose was administered in the cath lab before angiography. RESULTS: Cardiac magnetic resonance was performed in 14 patients from each study group 1 year after pPCI and revealed a significantly lower LV end-systolic volume index (p=0.003), end-diastolic volume index (p=0.009) and better ejection fraction (p <0.05) in patients who received abciximab early. CONCLUSIONS: Early abciximab administration prior to transfer for pPCI in patients with first anterior STEMI results in a lower degree of LV remodelling and better LV ejection fraction at 1-year follow-up compared to late abciximab administration in the cath lab during pPCI.

[Effect of cardiac rehabilitation on left ventricular function and exertion capacity in patients with chronic congestive heart failure]. / Wplyw rehabilitacji kardiologicznej na funkcje lewej komory serca i wydolnosc wysilkowa u chorych z przewlekla niewydolnoscia serca.

UNLABELLED: Cardiac rehabilitation is proven to be an effective method of treatment in congestive heart failure (CHF) and it has a positive influence on exercise capacity. There are controversies concerning the influence of physical rehabilitation on morphological and functional parameters of the left ventricle. Cardiac magnetic resonance (CMR) is a very precise noninvasive method of cardiac examination. The aim of this study was to assess changes in exercise capacity and in morphological parameters of the left ventricle in patients with CHF (NYHA class II and III, ejection fraction less than 35%) undergoing 6-months of physical rehabilitation. 16 patients were divided into two groups: A (7 pts) trained with progressive workload and group B (9 pts) did not trained. Groups were not different in terms of age, gender and NYHA class. In all patients, CMR and cardiopulmonary exercise test were performed at baseline and after 6 months. RESULTS: At baseline, both groups were similar with respect to the LV dimensions, thickness of LV walls and exercise capacity. At follow up, we observed an increase in exercise capacity in only the trained group (A). Exercise time increased from 357.1 +/- 30 sec. to 830.1 +/- 59 sec. (p < 0.05); max. workload from 2.7 +/- 0.36 MET to 6.21 +/- 0.8 MET (p < 0.05) and VO2 peak from 14.3 +/- 1.8 ml/kg/min to 18.8 +/- 2.1 ml/kg/min (p < 0.05). In group B (not trained) there were no changes after 6 months in these parameters. Morphological LV parameters were similar in both groups at baseline and after 6-months of rehabilitation.

[Role of computed tomography in diagnosis of the vascular system]. / Rola tomografii komputerowej w diagnostyce ukladu naczyniowego.

CT Angiography (CTA) is becoming a popular method used in the diagnosis of the vascular system. The authors present own experience acquired from 163 CTA examinations. In 98 cases, the CT angiography images were the elprimary indications for surgery or intervention procedures. Follow-up CTA examinations were performed in 65 of the cases. CT angiography is a reliable method in vascular diagnostics. Further progress in the development of this technique will lead to the replacement conventional angiography in most clinical diagnostic indications.

Creatine kinase-MB assessed in patients with acute myocardial infarction correlates with cardiac magnetic resonance infarct size at 6-month follow up.

INTRODUCTION: There are still only limited data concerning the use of creatine kinase-MB (CKMB) values for predicting infarct size in long-term follow up in patients with ST-segment elevation myocardial infarction (STEMI) who have undergone primary percutaneous coronary intervention (PCI). The aim of this study was to analyze the correlation between CKMB and both infarct size and left ventricular function during a 6-month follow up. METHODS: In a cohort of 68 patients with STEMI treated with PCI, serial CKMB assessment was performed at baseline and at 6, 12, 18, 24 and 48 hours after PCI. The area under the curve (AUC) of CKMB was calculated. Cardiac magnetic resonance (CMR) parameters were assessed at 6 months. RESULTS: All CKMB single time-point values, AUC CKMB, and CKMB maximal value after primary PCI were correlated with CMR infarct size and left ventricular function, but a high correlation (r>0.7) was found only for CKMB at 6 hours, CKMB at 12 hours, CKMB AUC, CKMB maximal value, and CMR infarct size (r=0.71, r=0.73, r=0.72, r=0.75, respectively, p<0.001 for all). CONCLUSIONS: CKMB assessment is a good predictor of infarct size at 6 months in patients with STEMI treated with PCI. The CKMB value at a single time point 12 hours after PCI is a good predictor of infarct size at 6 months, comparable to serial assessment parameters such as AUC CKMB and CKMB maximal value.

Predictive utility of NT-pro BNP for infarct size and left ventricle function after acute myocardial infarction in long-term follow-up.

PURPOSE: The aim of the study was to evaluate the utility of N-terminal pro-B-type natriuretic peptide (NT-pro BNP, pg/ml) assessment to predict infarct size and left ventricle function after ST-segment elevation myocardial infarction (STEMI) at long-term follow-up. METHODS: In 45 patients with first STEMI less than 3 hours from symptom onset treated with mechanical reperfusion NT-pro BNP was assessed early (at admission) and at 6 months. Cardiac magnetic resonance (CMR) parameters (delayed enhancement infarct size (IS, %), left ventricular end-diastolic (LVEDVI, ml/m2) and end-systolic (LVESVI, ml/m2) volume indexes) were assessed at 6 months. RESULTS: No significant correlation was found between baseline NT-pro BNP assessment and IS and left ventricle function after 6 months. There was a significant correlation between 6-month NT-pro BNP and IS (r=0.65, p<0.001) and left ventricle remodeling at 6 months (LVEDVI, r=0.53, p=0.001; LVESVI, r=0.51, p=0.002). CONCLUSIONS: Assessment of NT-pro BNP level 6 months after STEMI remains a good indicator of infarct size and left ventricle function at long-term follow-up.

Lipoma of the aortic valve in a patient with acute myocardial infarction.

We hereby report the first--to the best of our knowledge--case of primary lipoma of the aortic valve. The tumor has been diagnosed by echocardiography supported by magnetic resonance imaging in a 63-year-old man with acute inferior ST-elevation myocardial infarction (STEMI) and one-vessel coronary artery disease. Five weeks from the onset of STEMI, direct implantation of a bare metal stent into the right coronary artery was successfully undertaken and 6 weeks later aortic valve with an encapsulated mass was excised with subsequent artificial valve implantation. Histological examination revealed typical features of lipoma. Three months after the operation the patient was asymptomatic and exhibited a good function of the artificial valve.