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Objectives: The coarctation of aorta is commonly related to bicuspid aortic valve. The aim of the study was to assess arterial stiffness in adults after aortic coarctation repair and to evaluate an impact of bicuspid aortic valve concomitance on arterial stiffness results. Methods: Fifty-eight patients after coarctation of aorta repair, 36 male, median age of 27.46 ± 10.57, were compared to 30 with bicuspid aortic valve and to 30 healthy, sex, age and BMI matched volunteers. Physical examination, laboratory analysis and non-invasive assessment of arterial stiffness were performed. Results: CoA patients and BAV patients have higher central arterial stiffness parameters in comparison to healthy controls: AP (7.86 ± 6.56 vs 7.68 ± 5.96 vs 1.41 ± 3.82 mmHg, p  < 0.001, p  = 0.011, respectively) and AIx (18.81 ± 14.94 vs 18.06 ± 13.38 vs 4.41 ± 10.82%, p  < 0.001, p  = 0.006, respectively). There were no differences of PWV between CoA patients, BAV patients and healthy controls (6.07 ± 1.20 vs 5.95 ± 1.20 vs 5.67 ± 0.73 m/s, p  = 0.099, p  = 0.278, respectively). In CoA group, there was correlation of PWV with age ( r  = 0.55 p  < 0.001), BMI ( r  = 0.29, p  = 0.025), fibrinogen ( r  = 0.31, p  = 0.039), glucose ( r  = 0.58, p  < 0.001), ascending aorta diameter ( r  = 0.29, p  = 0.026) and age at operation ( r  = 0.27, p  = 0.041). Among group of BAV, there was a correlation of PWV with age ( r  = 0.58, p  < 0.001), central AP with total cholesterol ( r  = 0.38, p  = 0.036) and fibrinogen ( r  = 0.41, p  = 0.024). Conclusions: The increased arterial stiffness occurs in both groups: patients after aortic coarctation repair and patients with bicuspid aortic valve. Concomitance of coarctation of the aorta and bicuspid aortic valve has no influence on arterial stiffness augmentation.

Objective Hypertrophic cardiomyopathy (HCM) is associated with a risk of sudden cardiac death (SCD). Several models have been developed to estimate SCD risk and guide preventive therapy. The comparison of the previous 2003 with novel 2014 SCD risk models have never been studied. Methods Over a year we included 103 consecutive HCM patients without previous cardiac arrest and/or ICD implanted (65% males; aged 53.3 ± 13.9 years; mean EF 62.3 ± 18%). The SCD risk was calculated for each patient. Results Based on the 2003 model, patients had following scores: 0 points -15 (15%) patients, 1-28 (27%), 2-34 (33%), 3-18 (17%), ≥ 4-8 (8%). According to the 2014 model 83 (80%) had low risk, 12 (12%) intermediate risk, and only 8 (8%) high risk. Patients who had an intermediate or high risk in the 2003 model but a low risk in 2014 model were the oldest, experienced less frequently syncope and nsVT, but had more often abnormal blood pressure response to exercise and an intermediate LVOT gradient in comparison to the patients who had consistently low or high risk in both models. Conclusions Calculation of SCD risk using two models provides completely different risk estimates and consequently different guidance on SCD primary prevention. According to the 2003 model up to 85% of patients have indications for ICD, whereas based on the 2014 risk model only 20% of the patients had non-negligible SCD risk and are candidates for ICD therapy. SCD risk markers, used in the 2003 and 2014 models, have various discriminating power.

Background Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly which requires surgical treatment, which improves left ventricular function and clinical outcomes. The definitive diagnosis of ALCAPA is based on coronary angiography, however, noninvasive visualization of the origin of the coronary artery is often helpful in the diagnosis of ALCAPA. Objectives The purpose of this study was to evaluate noninvasive techniques for the diagnosis of ALCAPA, and present the clinical outcomes in adults after surgical correction of ALCAPA. Methods Medical charts of five patients with ALCAPA treated at the John Paul II Hospital in Cracow between 2004 and 2012 were analyzed retrospectively. Noninvasive imaging techniques were used to visualize coronary vessels preoperatively. Patients were followed one year after the operation with echocardiograms and assessment of the New York Heart Association functional class. Results Computed tomography showed ALCAPA in four patients. Magnetic resonance imaging showed abnormal left ventricle remodeling suggestive of ischemia of the anterior cardiac wall. Myocardial perfusion imaging revealed ischemia of the middle and periapical segments of the anterior and anterolateral wall of the left ventricle in two patients. Postoperatively, patients had symptomatic improvement, but there was no statistically significant difference in mean ejection fraction. Conclusions Noninvasive techniques can be used to visualize the origin of the coronary arteries and characterize their morphology. Long-term follow-up of adult patients after surgical correction of ALCAPA is not well documented in the literature, but surgical treatment revealed beneficial short-term effects and symptoms relief.

Background: Patients who have undergone a Fontan operation (FO) may suffer from both systolic and diastolic single ventricle (SV) dysfunction., Aim: The aim of the study was to quantify non-invasively the systolic and diastolic single ventricle function in adult FO patients and to assess its effect on exercise tolerance., Methods: We investigated 21 patients (12F; 9M) after FO with morphological left ventricle with a mean age of 26 +/- 6 years, and 17 age-matched, healthy people (control group). Pulse-wave Doppler signals from the mitral inflow, aortic flow and tissue Doppler imaging of the mitral annulus were obtained. A cardiopulmonary exercise test (CPET) was performed., Results: The Fontan patients, as compared to the controls, had a lower value of SV ejection fraction (SVEF), E velocity, E/A ratio, aortic valve velocity time integral, exercise time, SatO2, VO(2peak) and significantly higher E/E' ratio, VE/VCO(2peak) and VE/VO(2peak'). We found negative correlations between E/E' and exercise time and VO(2peak). Positive correlations were found between E/E' and VE/VCO(2peak) and age at surgery. S' velocity correlated positively with SVEF, VO(2peak) and negatively with E/E' ratio. Patients after FO with E/E' ratio > or = 12 had a significantly lower oxygen uptake when compared to those with E/E' ratio < 12., Conclusion: Adult patients after Fontan operations are characterized by both systolic and diastolic dysfunction of the single ventricle, associated with severe exercise intolerance reflected by shorter duration of exercise with decreased oxygen uptake and increased ventilatory response. E/E' ratio assessed by Tissue Doppler echocardiography can be a powerful predictor of oxygen uptake and ventilatory response.

Objective: Heart pathology strongly influences the course and prognosis of patients with generalized autoimmune diseases. In spite of autoimmunity being a common denominator of these diseases, systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and dermato/polymyositis (DPM) differ significantly in the pathogenesis of organ damage. The aim of the study was to compare pathologic changes in heart structure and function in these diseases by means of standard echocardiography and tissue Doppler (TDE)., Material and Methods: Four groups were examined: 60 SSc, 60 SLE and 15 DPM patients in stable clinical conditions and 30 healthy control subjects. Echocardiography with TDE was performed with the assessment of systolic (S) and diastolic (E) velocities of mitral and tricuspid annuli., Results: Heart in SSc was characterized by significant diastolic left ventricular dysfunction (mitral E 8.61 +/- 2.3 cm/s vs. 12.4 +/- 3.5 cm/s in the control group; P < 0.01) with preserved systolic function (mitral S 7.85 +/- 1.5 cm/s vs. 7.95 +/- 0.9 cm/s in control group; ns). SLE and DPM resulted mainly in pathologic thickening of valvular leaflets and/or pericardium [mitral or aortic leaflets thickened in 38 (63.3%) of SLE patients, 7 (46.7%) of DPM patients; pericardium thickened in 36 (60%) of SLE patients]. Pulmonary capillary wedge pressure was elevated in SSc (13.8 +/- 3.5 mmHg) and DPM (13.2 +/- 2.5 mmHg) patients as compared to the control group (9.2 +/- 3.7 mmHg, P < 0.01). Right ventricular systolic and diastolic dysfunction was frequent irrespective of the presence or absence of pulmonary hypertension., Conclusions: Echocardiography with TDE reveals characteristic pathology in different forms of generalized autoimmune diseases reflecting their different pathogenetic mechanisms. Overproduction of collagen in SSc results in diastolic left ventricular dysfunction, while generalized inflammation in SLE and DPM leads mainly to pathologic changes on valvular leaflets and/or pericardium. Interestingly, right ventricular dysfunction is common in all diseases analyzed, regardless of the presence of pulmonary hypertension. Echocardiography, preferably with TDE, could add valuable information about usually asymptomatic heart pathology in an individual patient with generalized autoimmune disease.

Objective: Examination of exercise function of Fontan patients and comparison with healthy control subjects., Design: Fourteen patients (6 males, 8 females; age: 5.7-17 years, mean 8.1 years) after Fontan repair in New York Heart Association (NYHA) class I with rest O(2)sat > 85% requiring no cardiovascular medications performed graded exercises on a treadmill 0.5-3.2 years postoperatively (mean 1.8 years). During the tests the heart and respiratory rate, blood pressure, oxygen uptake, carbon dioxide production, minute ventilation, tidal volume and O(2)sat were recorded. Spirometry was performed before and during exercise., Results: The peak VO(2)max in Fontan patients was significantly reduced compared with controls (p = 0.0002). Other parameters: anaerobic threshold (p = 0.0001); pulsO(2) (p = 0.00005); peak minute ventilation (p = 0.0014); physiological dead space to tidal volume ratio at peak exercise (p = 0.0004); maximal work rate (p = 0.00008); exercise time (p = 0.00003) were significantly reduced in univentricular patients. The heart rate at peak exercise was lower in the patients (p = 0.0003) and O(2)sat dropped significantly (p = 0.003)., Conclusion: The aerobic capacity, work and ventilatory parameters in Fontan patients are markedly reduced compared with controls. The anaerobic threshold was significantly lower. The decreased O(2)sat at peak exercise may suggest intrapulmonary shunting.