Lidia Tomkiewicz-Pająk

Markers of thrombogenesis and fibrinolysis and their relation to inflammation and endothelial activation in patients with idiopathic pulmonary arterial hypertension.

Background Chronic anticoagulation is a standard of care in idiopathic pulmonary arterial hypertension (IPAH). However, hemostatic abnormalities in this disease remain poorly understood. Therefore, we aimed to study markers of thrombogenesis and fibrinolysis in patients with IPAH. Methods We studied 27 consecutive patients (67% female) with IPAH aged 50.0 years (IQR: 41.0 - 65.0) and 16 controls without pulmonary hypertension. Prothrombin fragment 1+2 (F1+2) and thrombin-antithrombin (TAT) complexes were measured to assess thrombogenesis; tissue-type plasminogen activator (tPA) antigen and plasmin-anti-plasmin complex to characterize activation of fibrinolysis; plasminogen activator inhibitor 1 (PAI-1) to measure inhibition of fibrinolysis; and endothelin-1 (ET-1) and interleukin-6 (IL-6) to assess endothelial activation and systemic inflammation, respectively. In addition, in treatment-naive IPAH patients these markers were assessed after 3 months of PAH-specific therapies. Results TPA (10.1[6.8-15.8] vs 5.2[3.3-7.3] ng/ml, p<0.001), plasmin-anti-plasmin (91.5[60.3-94.2] vs 55.8[51.1-64.9] ng/ml, p<0.001), IL-6 (4.9[2.5-7.9] vs 2.1[1.3-3.8] pg/ml, p=0.001) and ET-1 (3.7 [3.3-4.5] vs 3.4[3.1-3.5], p= 0.03) were higher in patients with IPAH than in controls. In IPAH patients plasmin-anti-plasmin and tPA correlated positively with IL-6 (r=0.39, p=0.04 and r=0.63, p<0.001, respectively) and ET-1 (r=0.55, p=0.003 and r=0.59, p=0.001, respectively). No correlation was found between tPA or plasmin-anti-plasmin and markers of thrombogenesis. Plasmin-anti-plasmin decreased after 3 months of PAH specific therapy while the other markers remained unchanged. Conclusions In the present study we showed that markers of fibrynolysis were elevated in patients with IPAH however we did not find a clear evidence for increased thrombogenesis in this group of patients. Fibrinolysis, inflammation, and endothelial activation were closely interrelated in IPAH.

Is it worth closing the atrial septal defect in patients with insignificant shunt

Introduction Closure of the atrial septal defect in patients with insignificant shunt is controversial. Aim To evaluate the outcomes of transcatheter closure of atrial septal defect (ASD) in symptomatic patients with borderline shunt. Material and methods One hundred and sixty patients (120 female, 40 male) with a mean age of 30.1 ±16.2 (20–52) years with a small ASD who underwent transcatheter closure were analyzed. All patients had a small ASD with Qp: Qs ratio ≤ 1.5, mean 1.2 ±0.9 (1.1–1.5) in echo examination. Cardiopulmonary exercise tests, clinical study, transthoracic echocardiographic study as well as quality of life (QoL) (measured using the SF36 questionnaire (SF36q)) were repeated in all patients before and after the procedure. Results The devices were successfully implanted in all patients. After 12 months of ASD closure, all the patients showed a significant improvement of exercise capacity (oxygen consumption – 21.9 ±3.1 vs. 30.4 ±7.7, p > 0.001). The QoL improved in 7 parameters at 12-month follow-up. The mean SF36q scale increased significantly in 141 (88.1%) patients of mean 43.2 ±20.1 (7–69). A significant decrease of the right ventricular area (20.3 ±1.3 cm2 vs. 18.3 ±1.2 cm2, p < 0.001) and the right atrial area (15.2 ±1.9 cm2 vs. 12.0 ±1.6 cm2, p < 0.001) was observed at 12-month follow-up. Conclusions Closure of ASD in the patients with insignificant shunt resulted in significant durable clinical and hemodynamic improvement after percutaneous treatment.

Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome

Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1–84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4–5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24–1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02–2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43–0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32–0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59–3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

Noninvasive techniques for the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery in adult patients

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.

What determine the quality of life of adult patients after Fontan procedure

BACKGROUND Despite the low early mortality of Fontan procedures, Fontan patients are prone to vari-ous cardiac and extra-cardiac complications in the long term. This may influence patient perception of their health and outcome. The aim of the study was to assess the relationship of multi-organ compli-cations and physical efficiency with self-reported health-related quality of life (QOL) in adult Fontan patients. METHODS Quality of life was assessed with the Short Form-36 questionnaire. Laboratory tests were done together with echocardiography, plethysmography, and cardiopulmonary exercise test. RESULTS The QOL was poorer in patients than in control subjects. The physical characteristics of patients correlated with dynamic ventilatory parameters, heart rate at the peak of exercise, alanine aminotransferase and albumin level. CONCLUSIONS Liver impairment and chronotropic incompetence during exercise are associated with poor QOL in patients after Fontan procedure. In these patient, hepatic, pulmonary and cardiac functions should be carefully monitored. (Cardiol J 2018; 25, 1: 72-80).

Cardiac magnetic resonance imaging in a woman suspected of hypertrophic cardiomyopathy based on genotyping

617 Electrocardiography (FIGURE 1B) showed no signs typical of HCM (according to the European Soci‐ ety of Cardiology guidelines).1 On two 24 ‐hour Holter examinations, 3 consecutive ventricular beats were recorded (a short episode of nonsus‐ tained ventricular tachycardia with a heart rate of 138 bpm). No sudden death was reported in family history. To improve the quality of imag‐ ing, we used cardiac magnetic resonance (CMR) imaging with gadolinium contrast. It demon‐ strated left ventricular hypertrophy suggestive of HCM: diastolic maximum wall thickness in the inferior wall of 15 mm and late gadolinium hyperenhancement in the segment with maxi‐ mum thickness (FIGURE 1C and 1D). Left ventricu‐ lar ejection fraction calculated from high ‐quality CMR images was normal (79%). On a treadmill exercise test, the patient achieved a workload of 7 metabolic equivalents at a maximum heart rate of 86%. Maximum oxygen uptake was normal (96% of the predicted value). The test was ter‐ minated due to fatigue. The asymptomatic sta‐ tus corresponded with normal results of exercise Hypertrophic cardiomyopathy (HCM) is a com‐ mon genetic cardiac disease with a diverse clin‐ ical phenotype and imaging results; therefore, genotyping is of key importance in establishing a proper diagnosis.1 To illustrate these diagnos‐ tic challenges, we report a case of a 61 ‐year ‐old woman referred for family (including genetic) screening after diagnosing her daughter with a fully developed phenotype of HCM and typical echocardiographic findings at the age of 30 years. An MYBPC3 c.2541 C>G, p.(Tyr847*) mutation in the myosin ‐binding protein C gene was detect‐ ed in the proband (FIGURE 1A). The MYBPC3 p.Tyr847* mutation was discov‐ ered in 2004. In the ClinVar database, it has been reported several times in HCM patients from dif‐ ferent countries as pathogenic or likely pathogen‐ ic. In the mother of the proband, echocardiog‐ raphy failed to show typical abnormalities, part‐ ly due to problems with proper visualization and measurements (the maximum wall thickness was about 12–13 mm without left ventricular outflow tract obstruction). The patient was asymptomatic. CLINICAL IMAGE

Early results of aortic arch reconstruction and bilateral pulmonary artery banding: modification of the Norwood operation for treatment of hypoplastic left heart syndrome

BACKGROUND In the period from 2003 to the end of 2015, 96 Norwood I procedures were performed in the Paediatric Heart Surgery Department in Katowice, Poland, in children with hypoplastic left heart syndrome (HLHS). AIM This paper presents a retrospective analysis of early surgical results. METHODS The patients consisted of two groups: group I included 59 children operated on in the years 2003-2012, in whom the stage I Norwood procedure with the Sano modification was performed with the aortic arch reconstructed by use of a ho-mogenous pulmonary artery patch or a bovine pericardial patch. Group II included 37 children after our modification of the Norwood I procedure, in which the aortic arch was reconstructed with an extracellular matrix patch and bilateral pulmonary artery banding was done. RESULTS Aortic cross-clamping time was significantly shorter in group II (mean 52; range 38-62 min) than in group I (mean 57; range 39-72 min; p < 0.009). Eighteen (30.5%) children in group I and six (16.2%) in group II died. Although this dif-ference did not reach statistical significance (p = 0.12), it suggested that improved outcomes with the modified procedure are possible. The cause of death in group I was significantly more frequently due to massive postoperative bleeding (n = 6; 33.3%) than in group II (n = 1; 16.7%; p = 0.046). CONCLUSIONS The introduction of this new surgical technique reduced postoperative bleeding rates, shortened the operation time, and might improve the mortality rate in the first-stage surgical treatment of children with HLHS.

Quality of life in adults with repaired tetralogy of Fallot

Introduction Observations of patients after repair of tetralogy of Fallot (ToF) indicate good correction results and long-term survival. Few papers have been published in which the quality of life (QoL) of this population has been assessed. Aim To evaluate QoL in adults with repaired ToF.
 Material and methods We included 39 patients with repaired ToF and 40 age- and sex-matched healthy volunteers. Information recorded included echocardiography, cardiac magnetic resonance, cardiopulmonary exercise test, and self-reported health-related QoL questionnaire (SF-36).
 Results The perceived physical and mental domains of health were signi cantly poorer in ToF patients than in controls. A positive correlation between VO2 peak and physical domains was observed: (VO2 peak vs. physical domains (r = 0.6, p ≤ 0.001), general health (r = 0.36, p = 0.03), and physical complex status (r = 0.51, p = 0.001). VO2 peak % correlated with physical functioning (r = 0.43, p = 0.007), general health (r = 0.39, p = 0.015) and physical complex status (r = 0.49, p = 0.002). Right ventricle ejection fraction, determined with cardiac magnetic resonance, positively correlated with role physical (r = 0.38, p = 0.04). In echocardiography, pressure half time was posi- tively correlated with physical functioning (r = 0.48, p = 0.004) and role physical (r = 0.4, p = 0.02).
 Conclusions The QoL in adults after repair of ToF and healthy control subjects was compared directly. The self-perceived physical and mental domains of health were significantly poorer in ToF patients than in controls. Strong associations were found between objective exercise capacity and physical aspects of quality of life. Complex assessment and quality of life instruments should be used together to obtain an accurate view of health status of patients with repaired ToF.

Opioidergic conditioning of the human heart muscle in nitric oxide-dependent mechanism

BACKGROUND Opioidergic conditioning is well documented to trigger cardioprotection against ischemia/ reperfusion (I/R) injury. Previous studies on animal models have suggested that nitric oxide (NO) mediates the beneficial effect of opioids, but the role of NO in humans seems to be controversial. OBJECTIVES The aim of the study was to assess the influence of NO modulators on opioid-induced cardioprotection in the human myocardium. MATERIAL AND METHODS Trabeculae of the human right atria were electrically driven in an organ bath and subjected to simulated I/R injury. The non-selective inhibitor of nitric oxide synthase (NOS) - N-methyl-l-arginine (LNMMA), the donor of NO - S-Nitroso-N-acetylpenicillamine (SNAP) or morphine (in the amount of 10-4 M) were used at the time of re-oxygenation. The additional trabecula was subjected to the hypoxia protocol only (control). The contractility of the myocardium was assessed as the maximal force of a contraction (Amax), the rate of rise of the force of a contraction (Slope L) and the cardiac muscle relaxation - as the rate of decay of the force of a contraction (Slope T). RESULTS The application of 100 μM LNMMA resulted in the decrease of Amax, Slope L and Slope T during the re-oxygenation period as compared to control. The application of 10-4 M morphine and/or 100 μM SNAP resulted in a partial reversal of the detrimental influence of LNMMA. CONCLUSIONS At the re-oxygenation period, the blockade of NO synthesis has a deleterious effect on the systolic and diastolic function of the human myocardium as well as attenuates the beneficial effect of morphine conditioning.

Pregnancy in women with complete transposition of the great arteries following the atrial switch procedure. A study from three of the largest Adult Congenital Heart Disease centers in Poland

Abstract Objective: We sought to identify maternal/neonatal and cardiovascular complications in pregnant women with complete transposition of great arteries (D-TGA) following atrial switch. Methods: Clinical records of all women with D-TGA after the Mustard/Senning (M/S) operation who were followed at the three largest Adult Congenital Heart Disease (ACHD) centers in Poland were reviewed. Results: Fifteen of the fifty-nine women followed had a total of 24 pregnancies, including two spontaneous miscarriages. Twenty-two pregnancies (92%) resulted in a live birth, 91% were by cesarean section. During 5 (23%) of the 22 completed pregnancies obstetric complications were observed (one gestational diabetes, one hypertension in pregnancy, one placenta increta and two preterm labors). The mean pregnancy duration was 37.2 weeks (range: 26–41 weeks).We observed one neonatal death due to extreme prematurity. Six (25%) children had a birth weight of ≤2500 g. None of women had severe cardiac complications during pregnancy nor in the postpartum period. Conclusions: In our study, we demonstrated a large number of obstetric complications and low birth weight in the presence of a systemic right ventricle. However, from a cardiologist’s point of view pregnancy after the M/S operation was well-tolerated and relatively safe.