Jan Głowacki

Malignant tumors of the heart

Primary malignant cardiac tumors are rare, and mostly manifest as sarcomas in various types. As non-invasive diagnostic modalities, e.g. echocardiography and magnetic resonance imaging, have become more sensitive, there is a marked increase in the number of patients diagnosed. Nevertheless, most patients die within one year of initial diagnosis, either because of the often asymptomatic presentation of cardiac tumors until advanced disease, or a low index of suspicion on the part of the physician. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumors anatomic location. Cardiac sarcomas may present with a variety of symptoms and are known to be great mimickers. A quick diagnosis facilitates the initiation of a proper treatment (surgical resection, adjuvant chemotherapy), which may in turn improve the prognosis. Metastases to the heart are far more common, unfortunately, clinical manifestations are mainly dominated by generalized tumor spread. The article summarizes epidemiology, symptoms, diagnostic modalities, and possible treatment options.

Transcatheter closure of atrial septal defect in children up to 10 kg of body weight with Amplatzer device

BACKGROUND Transcatheter closure of atrial septal defect (ASD) in older children and adults is currently considered the first-choice therapeutic option. This approach remains challenging in younger children. The aim of the study was to evaluate feasibility, safety and midterm efficacy of percutaneous ASD closure in symptomatic infants ≤10 kg body weight in our institution. METHODS There were 28 children up to 10 kg of body weight, who were qualified for transcatheter closure of ASD. All patients but one showed overload of right atrium and right ventricle. Mean weight of patients who underwent transcatheter closure was 9.2 ± 0.88 kg and age 1.59 ± 0.58 years, respectively. Transcatheter closure of ASD was conducted using Amplatzer occluders (ASO). RESULTS The devices were implanted successfully in 26/28 patients (93%). In 2 (7%) children the device repeatedly straddled the septum in relatively big ASD and the procedure was abandoned. Mean ASD diameter in patients, who underwent transcatheter closure, was 9.08 ± 2.9 mm (transthoracic echocardiography) and mean implant size/weight ratio was 1.07 ± 0.31. In the child with right-left shunt through ASD normalization of saturation occurred. Mean fluoroscopy time was 4.16 min. In 3 children minor complications occurred: transient arrhythmias (n = 1), fever after procedure (n = 2). The follow-up time was 6.1 (range 1.2-11) years. At follow-up, clinical condition and/or growth improved in all patients except 4 children with coexisting comorbidity. No arrhythmia nor conduction disturbances were observed during follow-up. CONCLUSIONS In selected patients weighing less or equal to 10 kg, percutaneous closure of ASD is a safe and effective procedure.

Not at random location of atherosclerotic lesions in thoracic aorta and their prognostic significance in relation to the risk of cardiovascular events.

Summary Thoracic aortic calcium deposits are frequently detected on tomography of the chest, and in other imaging modalities. Numerous studies indicated the correlation of hemodynamic parameters such as wall shear stress in relation to distribution aortic calcifications. This publication discusses similarities and differences of two distinct pathomechanisms of arterial calcifications: intimal associated with atherosclerosis and medial knows as Mönckeberg’s arteriosclerosis. This review also analyzes the frequent coexistence of aortic calcification and coronary artery disease in terms of risk of cardiovascular events.

Comparison of early and long-term results of percutaneous coronary interventions in patients with ST elevation myocardial infarction, complicated or not by cardiogenic shock.

ObjectivesCardiogenic shock (CS) still remains one of the most important factors affecting the mortality rate of patients with ST segment elevation myocardial infarction (STEMI). However, the data with follow-up longer than 1 year are limited. The aim of this study was to evaluate the early and long-term treatment results of patients with STEMI, complicated or not by CS, who underwent percutaneus coronary interventions. MethodsA retrospective registry included data of all patients with STEMI admitted to our centre from January 1999 to December 2001. ResultsOne thousand three hundred and eighty-five patients with STEMI were hospitalized and 1237 of them were treated with immediate percutaneus coronary interventions. Among this subpopulation, 117 (9.5%) patients were with STEMI complicated with CS on admission (group I) and 1120 (90.5%) patients were with STEMI without complications from CS on admission (group II). The groups differed significantly with regard to baseline clinical characteristics, angiographic picture, and in-hospital course. A total of 38.5% of patients with myocardial infarction complicated by CS and 2.5% of patients without shock (P<0.001) died during hospitalization. At the 5-year follow-up, 58.1% of group I patients and 14.8% of group II patients (P<0.001) died. A significant difference in the 5-year mortality rate was also observed in patients who survived the in-hospital period (31.9 vs. 12.6%; P<0.001). ConclusionCS continues to be closely connected with a very high mortality rate both in the hospital and in the long-term, also among patients who survived the in-hospital period.

Predominant location of coronary artery atherosclerosis in the left anterior descending artery. The impact of septal perforators and the myocardial bridging effect

Introduction Coronary artery atherosclerosis presents characteristic patterns of plaque distribution despite systemic exposure to risk factors. We hypothesized that local hemodynamic forces induced by the systolic compression of intramuscular septal perforators could be involved in atherosclerotic processes in the left anterior descending artery (LAD) adjacent to the septal perforators’ origin. Therefore we studied the spatial distribution of atherosclerosis in coronary arteries, especially in relation to the septal perforators’ origin. Material and methods 64-slice computed tomography angiography was performed in 309 consecutive patients (92 male and 217 female) with a mean age of 59.9 years. Spatial plaque distribution in the LAD was analyzed in relation to the septal perforators’ origin. Additionally, plaque distribution throughout the coronary artery tree is discussed. Results The coronary calcium score (CCS) was positive in 164 patients (53.1%). In subjects with a CCS > 0, calcifications were more frequent in the LAD (n = 150, 91.5%) compared with the right coronary artery (RCA) (n = 94, 57.3%), circumflex branch (CX) (n = 76, 46.3%) or the left main stem (n = 42, 25.6%) (p < 0.001). Total CCS was higher in the LAD at 46.1 (IQR: 104.2) and RCA at 34.1 (IQR: 90.7) than in the CX at 16.8 (IQR: 61.3) (p = 0.007). In patients with calcifications restricted to a single vessel (n = 54), the most frequently affected artery was the LAD (n = 42, 77.8%). In patients with lesions limited to the LAD, the plaque was located mostly (n = 37, 88.1%) adjacent to the septal perforators’ origin. Conclusions We demonstrated that coronary calcifications are most frequently located in the LAD in proximity to the septal branch origin. A possible explanation for this phenomenon could be the dynamic compression of the tunneled septal branches, which may result in disturbed blood flow in the adjacent LAD segment (milking effect).

Symetis Acurate Transapical Aortic Valve: the initial experience with a second generation of transcatheter aortic valve replacement device

BACKGROUND Transcatheter aortic valve replacement (TAVR) has proven to be a valuable alternative to conventional surgical aortic valve replacement in high risk and surgically in operable patients who suffer from severe symptomatic aortic stenosis. However, a significant number of complications, associated with both the learning curve and device specificity, have required attention and subsequent improvement. The Symetis transapical TAVR system is a self-positioning bioprosthesis composed of a non-coronary leaflet of surgical quality porcine tissue valve sewn into a self-expanding nitinol stent that iscovered with a PET-skirt. METHODS From June to September 2013 six patients have been operated on severe aortic stenosis using the new TAVR device. All patients have undergone critical assessment of a local Heart Team and have been disqualified from conventional AVR. Five were woman. Mean age was 82.3 ± 2.0 (mean LogEuroScore 23.9 ± 14.3). Four patients suffered from coronary artery disease - two had history of previous percutaneous coronary intervention with intracoronary stents, while the next two had history of coronary artery bypass grafting. Diabetes was frequent (n = 3) as well as chronic obstructive pulmonary disease (n = 4). Carotid artery disease was encountered in three patients similarly to atrial fibrillation. Mean left ventricular ejection fraction (LVEF) was 51.5 ± 11.8%, but one patient had suffered from low-flow-low-gradient aortic stenosis with LVEF of 29%. RESULTS The procedure was carried out successfully in all six cases. Two patients have received the valve sized L, three - M and one - S. Mean procedure time was 180 ± 19 min, mean cine 7.2 ± 1.2 min. Mean X-ray dose 930 ± 439 mGy, while mean volume of contrast given was 135 ± 61 mL. In all patients but one perivalvular leak (PVL) was not present. One patient had trace of PVL. Also, good LVEF was noted in all patients. Similar findings were obtained 30 days post procedure. No strokes, transient ischaemic attack or other cerebrovascular incidents were observed. CONCLUSIONS This brief clinical communication reports the first Polish experience with the second generation of TAVR device - the Symetis Acurate Transapical Aortic Valve. While it lacks large patient population and longer follow-up, it reveals that TAVR procedure can be performed safely, with minimal X-ray exposure time and contrast given and successfully - with almost nonexistent PVL and no cerebrovascular incidents or heart rhythm disturbances. Heart Team approach is vital, and transapical access should not be treated inferiorly, but rather as an equally appealing TAVR option.

Novel Genetic Triggers and Genotype–Phenotype Correlations in Patients With Left Ventricular NoncompactionCLINICAL PERSPECTIVE

Background— Left ventricular noncompaction (LVNC) is a genetically and phenotypically heterogeneous disease and, although increasingly recognized in clinical practice, there is a lack of widely accepted diagnostic criteria. We sought to identify novel genetic causes of LVNC and describe genotype–phenotype correlations. Methods and Results— A total of 190 patients from 174 families with left ventricular hypertrabeculation (LVHT) or LVNC were referred for cardiac magnetic resonance and whole-exome sequencing. A total of 425 control individuals were included to identify variants of interest (VOIs). We found an excess of 138 VOIs in 102 (59%) unrelated patients in 54 previously identified LVNC or other known cardiomyopathy genes. VOIs were found in 68 of 90 probands with LVNC and 34 of 84 probands with LVHT (76% and 40%, respectively; P<0.001). We identified 0, 1, and ≥2 VOIs in 72, 74, and 28 probands, respectively. We found increasing number of VOIs in a patient strongly correlated with several markers of disease severity, including ratio of noncompacted to compacted myocardium (P<0.001) and left ventricular ejection fraction (P=0.01). The presence of sarcomeric gene mutations was associated with increased occurrence of late gadolinium enhancement (P=0.004). Conclusions— LVHT and LVNC likely represent a continuum of genotypic disease with differences in severity and variable phenotype explained, in part, by the number of VOIs and whether mutations are present in sarcomeric or nonsarcomeric genes. Presence of VOIs is common in patients with LVHT. Our findings expand the current clinical and genetic diagnostic approaches for patients with LVHT and LVNC.

Lipomatous hypertrophy of the interatrial septum: a rare cause of right ventricular impairment.

Abstract  We report a case of a 70‐year‐old woman admitted with symptoms of decompensated heart failure. Magnetic resonance imaging revealed lipomatous hypertrophy of the interatrial septum with partial upper right atrial inflow obstruction, partial obstruction of the right ventricular outflow tract, and excessive accumulation of adipose tissue in the pericardial space. The patient underwent excision of the septal lipomatous mass, which relieved the right ventricular outflow obstruction. (J Card Surg 2010;25:171‐174)

Cierre transcatéter de rotura de aneurismas del seno de Valsalva con oclusores Amplatzer

El objetivo fue evaluar el poder predictivo para diagnosticar hipertrofia del ventriculo izquierdo (HVI) de la concentracion urinaria del fragmento N-terminal del propeptido natriuretico tipo B (NT-proBNP) en 160 pacientes asintomaticos diagnosticados de hipertension esencial (HT). Las concentraciones urinarias de NT-proBNP/creatinina estuvieron incrementadas en pacientes con HVI al compararlos con no hipertroficos (p odds ratio = 4,074; p = 0,009). En conclusion, la concentracion de NT-proBNP en orina es un nuevo marcador que puede ser de utilidad para detectar HVI en sujetos con HT esencial.

The Role of Septal Perforators and “Myocardial Bridging Effect” in Atherosclerotic Plaque Distribution in the Coronary Artery Disease

Summary The distribution of atherosclerotic plaque burden in the human coronary arteries is not uniform. Plaques are located mostly in the left anterior descending artery (LAD), then in the right coronary artery (RCA), circumflex branch (LCx) and the left main coronary artery (LM) in a decreasing order of frequency. In the LAD and LCx, plaques tend to cluster within the proximal segment, while in the RCA their distribution is more uniform. Several factors have been involved in this phenomenon, particularly flow patterns in the left and right coronary artery. Nevertheless, it does not explain the difference in lesion frequency between the LAD and the LCx as these are both parts of the left coronary artery. Branching points are considered to be the risk points of atherosclerosis. In the LCx, the number of side branches is lower than in the LAD or RCA and there are no septal perforators with intramuscular courses like in the proximal third of the LAD and the posterior descending artery (PDA). We hypothesized that septal branches generate disturbed flow in the LAD and PDA in a similar fashion to the myocardial bridge (myocardial bridging effect). This coronary architecture determines the non-uniform plaque distribution in coronary arteries and LAD predisposition to plaque formation.