Percutaneous pulmonary valve implantation in patients after Ross procedure: role of intravascular ultrasound.

Coronary compression exclusion during right ventricle outflow tract stenting is recommended and potential oversight may be fatal. Balloon inflation in right ventricle outflow tract with simultaneous aortography can be inconclusive or falsely negative. We present a case of 27-year-old male post Ross operation qualified for percutaneous pulmonary valve implantation. Neither of the conventional views obtained provided a definite exclusion of coronary compression, therefore an intravascular ultrasound of the left coronary artery before and during balloon inflation in right ventricle outflow tract was performed. Acquired images allowed excluding potential constriction, thus a covered stent and pulmonary valve were implanted and the procedure was concluded. Two hours later, the patient complained of chest pain. Transthoracic echocardiography demonstrated a significant pericardial effusion. Retrospective analysis of the final angiogram revealed a possibility of subtle extravasation at the distal part of the homograft. A hybrid procedure consisting of additional covered stent implantation, pericardial drainage, and second pulmonary valve implantation was performed with an acceptable result. To conclude, in case of doubtful or unconvincing images obtained from aortography or selective angiography during balloon inflation in right ventricle outflow tract, intravascular ultrasound might be a feasible and useful technique. Signs of homograft rupture may be subtle, whereas symptoms of cardiac tamponade delayed. In selected patients percutaneous treatment of homograft rupture is achievable and beneficial.

Cardiovascular risk factors in patients with bicuspid aortic valve / Czynniki ryzyka chorób sercowo-naczyniowych u chorych z dwuplatkowa zastawka aorty

The bicuspid aortic valve (BAV) is a congenital heart disease. AIM: The aim of study was to evaluate the assessment of atherosclerosis risk factors in adult patients with bicuspid aortic valve compared against 30 healthy, age and sex matched volunteers. MATERIALS AND METHODS: Thirty patients (22 males, 8 females) at an average age of 27.00 ±10.6 years were compared against thirty healthy, age and sex matched volunteers. The arterial blood pressure, lipid profile, fasting glucose, inflammation markers and the lifestyle factors were analysed. RESULTS: Patients with bicuspid aortic valve have a higher level of glucose (4.95 vs 4.7 mmol / l, p = 0.026) and fibrinogen (2.25 vs. 1.98 g / l, p = 0.001) compared to the control group. In the group of patients with bicuspid aortic valve there was no hypertension, diabetes, tachycardia, stress exposure, alcohol abuse and a positive family history of cardiovascular diseases. CONCLUSIONS: The patients with bicuspid aortic valve have higher glucose and fibrinogen levels and they need systematic checking to reduce cardiovascular risk factors.

Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study.

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.

Long-Term Effects of Percutaneous Fenestration Following the Fontan Procedure in Adult Patients with Congenital Univentricular Heart.

BACKGROUND The Fontan procedure, performed for univentricular heart, may also include the technique of percutaneous fenestration to create a small atrial septal defect (ASD) and a right-to-left shunt. The aim of this study was to evaluate the long-term effects of fenestration in adult patients who had a Fontan procedure for univentricular heart. MATERIAL AND METHODS Fontan surgery was performed in 39 patients, including 19 (49%) patients with fenestration (Group I), and 20 (51%) patients without the fenestration procedure (Group II). Laboratory tests in both groups included echocardiography, plethysmography, cardiopulmonary exercise testing, and 24-hour Holter monitoring. RESULTS Compared with patients in Group I, patients in Group II had a significantly increased level of N-terminal pro-brain natriuretic peptide (NT-proBNP) (p=0.04), alkaline phosphatase (ALP) (p=0.01) and a significant increase in frequency of atrial fibrillation (p=0.04). Patients in Group I had a significantly increased systemic ventricular ejection fraction (SVEF) (p=0.05) and increased heart rate (HR) (p=0.006), heart rate reserve (HRR) (p=0.02), ventilatory equivalent (VE) (p=0.01), and VO2 peak (p=0.05) on cardiopulmonary exercise testing (CPET). Renal, hematologic, and ventilatory parameters, and incidence of thromboembolism showed no significant differences between the groups. CONCLUSIONS Long-term follow-up of patients who underwent Fontan procedures with percutaneous fenestration had improved single ventricular function, lower NT-proBNP levels, improved exercise capacity, and reduced ALP levels. These findings indicate that percutaneous fenestration closure should be considered for adult patients who have undergone Fontan procedure for univentricular heart.

Long-term follow-up in adults after tetralogy of Fallot repair.

BACKGROUND: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Adults with repaired ToF develop late complications. The aim of this study was to describe and analyze long-term follow-up of patients with repaired ToF. METHODS: This is a retrospective cohort study. Consecutive 83 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 30.5 ± 10.7. There were 49 (59%) male. Patients were divided into two groups according to the time since the repair (< 25 years and ≥ 25 years). The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively. RESULTS: In CPET values were not significantly different in the two groups. In CMR volumes of left and right ventricles were not significantly different in the two groups. There were no differences between the groups in ventricular ejection fraction, mass of ventricles, or pulmonary regurgitation fraction. Among all the patients, ejection fraction and left and right ventricle mass, indexed pulmonary regurgitation volume measured by CMR did not correlate with the time since repair. In ECG among all the patients, ejection fraction of the RV, measured in CMR, negatively correlated with QRS duration (r = - 0.43; p < 0.001). There was a positive correlation between QRS duration and end diastolic volume of the RV (r = 0.30; p < 0.02), indexed end diastolic volume of the RV (r = 0.29; p = 0.04), RV mass (r = 0.36; p < 0.001) and left ventricle mass (r = 0.26; p = 0.04). CONCLUSION: Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF - operated patients could be excellent up to 25 years after the repair. QRS duration could be an utility and easy factor to assessment of right ventricular function. TRIAL REGISTRATION: The study protocol was approved by the local Ethics Committee. Each participant provided informed consent to participate in the study (license number 122.6120.88.2016 from 28.04.2016).

Past and current cause-specific mortality in Eisenmenger syndrome.

AIMS: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. METHODS AND RESULTS: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). CONCLUSION: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

[Type and frequency of cardiac symptoms in patients with pulmonary sarcoidosis]. / Rodzaj i czestosc wystepowania objawów kardiologicznych u chorych na sarkoidoze pluc.

AIM: The aim of the study was to assess the type and frequency of prevalence of cardiac symptoms in patients with pulmonary sarcoidosis. MATERIALS AND METHODS: The study group consisted of 54 patients (21 female, 33 male), with biopsy-proven pulmonary sarcoidosis. Mean age was 45.85 +/-11.77 years. According to time passed from diagnosis of sarcoidosis patients were divided into 2 subgroups. Additionally, age, sex, left ventricular ejection fraction, cigarette smoking and comorbidities were analyzed. Course and stage of sarcoidosis were also included in the analysis. RESULTS: The frequency of cardiovascular symptoms in the study group was 87.04%. 59.26% of patients reported chest pain, the same subjects reported dyspnoea. 48.15% of respondents reported heart palpitations, 33.33% pre-syncope states, 12.96% syncope, and 37.04% edema of lower limbs. There were no statistically significant differences in the incidence of analyzed symptoms, depending on the duration of lung sarcoidosis. CONCLUSIONS: Patients with pulmonary sarcoidosis who report cardiovascular symptoms require further diagnostics.

[Cardiovascular risk factors in patients after coarctation of aorta repair]. / Czynniki ryzyka chorób sercowo-naczyniowych u chorych po leczeniu interwencyjnym koarktacji aorty.

The coarctation of the aorta (CoA) is a congenital condition of the thoracic aorta. AIM: The aim of the study was assessment of atherosclerosis risk factors in adult patients after surgical treatment of aortic coarctation. MATERIALS AND METHODS: 58 patients (36 male, 22 female) at median age of 27.46 ±10.57 were compared with 30 healthy, age and sex matched volunteers. The arterial blood pressure, lipid profile, fasting glucose, inflammation markers and the lifestyle factors were analyzed. RESULTS: CoA patients have higher systolic blood pressure 136.55±16.27 vs 123.47±10.34 mmHg, p<0.001, fasting glucose 4.95±0.5 vs 4.65±0.46 mmol/l p=0.002, hsCRP 1.03±0.12 vs 0.89±0.14 mg/l p=0.025 and fibrinogen 2.55±0.34 vs 1.98±0.28 g/l p<0.001. Hyperlipidemia is more common 44.8% vs 23.3% p=0.048, treated with statin. When comparing hypertensive patients (N=28) with normotensive ones (N=30), the patients with arterial hypertension are older 33.5±12.23 vs 25.73±7.12 p=0.004, have higher body weight 78.03±14.58 vs 68.7±14.29 p=0.017, in this group more common are: hypoplastic aortic arch 28.6% vs 6.7% p=0.027, recoarctation 39.3% vs 13.3% p=0.024, cardiovascular disease 14.3% vs 0% p=0.032 and family history 21.4% vs 3.3% p=0.034. CONCLUSIONS: The coarctation of aorta is related to higher cardiovascular risk due to arterial hypertension, hyperlipidemia, higher glucose and inflammation markers levels when comparing with healthy population.

Preoperative single ventricle function determines early outcome after second-stage palliation of single ventricle heart.

BACKGROUND: Second-stage palliation with hemi-Fontan or bidirectional Glenn procedures has improved the outcomes of patients treated for single-ventricle heart disease. The aim of this study was to retrospectively analyze risk factors for death after second-stage palliation of single-ventricle heart and to compare therapeutic results achieved with the hemi-Fontan and bidirectional Glenn procedures. MATERIAL AND METHODS: We analyzed 60 patients who had undergone second-stage palliation for single-ventricle heart. Group HF consisted of 23 (38.3%) children who had been operated with the hemi-Fontan method; Group BDG consisted of 37 (61.7%) who had been operated with the bidirectional Glenn method. The analysis focused on 30-day postoperative mortality rates, clinical and echocardiographic data, and early complications. RESULTS: The patients' ages at the time of repair was 33 ± 11.2 weeks; weight was 6.7 ± 1.2 kg. The most common anatomic subtype was hypoplastic left heart syndrome, in 36 (60%) patients. The early mortality rate was 13.3%. Significant preoperative atrioventricular valve regurgitation, single-ventricle heart dysfunction, pneumonia/sepsis, and arrhythmias were associated with higher mortality rates after second-stage palliation. Multivariate analysis identified significant preoperative single-ventricle heart dysfunction as an independent predictor of early death after second-stage palliation. No differences were found in the analyzed variables after bidirectional Glenn compared with hemi-Fontan procedures. CONCLUSION: Significant preoperative atrioventricular valve regurgitation, arrhythmias and pneumonia/sepsis are closely correlated with mortality in patients with single-ventricle heart after second-stage palliation. Preoperative significant single-ventricle heart dysfunction is an independent mortality predictor in this group of patients. There are no differences in clinical, echocardiographic data, or outcomes in patients treated with the hemi-Fontan compared with bidirectional Glenn procedures.

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.

[Exercise capacity in patients with pulmonary sarcoidosis]. / Wydolnosc fizyczna u chorych na sarkoidoze pluc.

Exertional dyspnea is a common manifestation of sarcoidosis. Cardiopulmonary exercise testing (CPET) is a useful tool to evaluate exercise tolerance of sarcoid patients. AIM: The aim of the study was to evaluate of exercise capacity in patients with pulmonary sarcoidosis with regard to duration of the disease. Analysis of differences in physical tolerance between patients with cardiac sarcoidosis and without cardiac sarcoidosis. MATERIALS AND METHODS: 39 patients diagnosed with pulmonary sarcoidosis were enrolled to our study. Cardiopulmonary exercise test was used to assess exercise capacity. According to time passed from diagnosis of sarcoidosis patients were grouped into 2 groups. Cardiac sarcoidosis was diagnosed in 9 subjects (23.07%). The control group consisted of 33 healthy volunteers. RESULTS: The results of the ergospirometry test in patients with lung sarcoidosis showed statistically significant differences in comparison to the control group. The age of patients with pulmonary sarcoidosis correlated negatively with maximal heart rate, oxygen consumption at peak exercise and at anaerobic threshold, breathing frequency at peak exercise, minute ventilation at peak exercise and metabolic equivalent. There was a negative, statistically significant correlation between the oxygen pulse at the peak of exercise and the treatment of steroids in the past. CONCLUSIONS: Exercise limitation in patients with pulmonary sarcoidosis is a consequence of both ventilatory and cardiocirculatory impairment. Patients with longer disease history of sarcoidosis achieved worse results at cardiopulmonary exercise test than patients with shorter disease history. Trend towards worse exercise tolerance in patients with cardiac sarcoidosis compared to patients without cardiac sarcoidosis was observed.

[Multiorgan malfunction after Fontan operation in adult patients]. / Powiklania narzadowe u doroslych chorych po operacji Fontana.

Although 85% of patients with univentricular heart after Fontan procedure survive twenty years after operation, the procedure alone seems to be an inadequate treatment as a permanent clinical solution. Patients with a "Fontan physiology" additionally have to face a various extra-cardiac complications, including thyroid, liver and kidney dysfunction, which are not only potentially life-threatening, but also can potentiate the circulatory insufficiency. AIM: The aim of the study was to assess a multiorgan dysfunction in adult patients after Fontan operation in long term follow-up and compare to healthy controls. MATERIALS AND METHODS: 54 patients after Fontan procedure (age=25.1±7 years, time after operation =19.8±6.3 years, age at the time of the procedure =5.3±4.3 years) and 30 controls (26.2±5.8 years) were included to the study. Clinical and laboratory examinations were performed including: hematologic, hepatic, renal and thyroid function tests. The following laboratory tests were performed: red blood cell counts (RBC), hematocrit (Ht), hemoglobin (Hb), platelets count, red blood cell distribution width (RDW), iron level; Btype natriuretic peptide (NT-proBNP), proteinogram blood test, alanine aminotransferase (ALT), aspartate aminotransferase (AST), gammaglutamyl transpeptidase (GGTP), alkaline phosphatase (ALP), total bilirubin, alpha fetoprotein (AFP) level. Furthermore creatinine level; cystatin C, urine albumin to creatynine ratio (ACR) and urinalysis were assessed. To assess a thyroid function free triiodothyronine (fT3), free thyroxine (fT4) and thyroid stimulating hormone (TSH) were measured. Furthermore an abdomen ultrasonography was performed. RESULTS: In terms of the hematological disorders Fontan patients had a statistically significant higher level of RBC (5.6±0,8 vs 4.8±0.4 109/µl; p<0,001), Hb (16.5±2.8 vs 14.2±1.2 g/dl; p<0,001), HCT (48.7±8.1 vs 42.1±3 %; p<0,001), RDW (14±2.6 vs 12.8±0.5 %; p=0.001), as compared to control group, while PLT level was statistically lower in Fontan group (156.2±61.4 vs 224.2±48 103/µl; p<0,0001). Hepatic parameters in Fontan patients were statistically significant higher, as depicted by the level of: ALT (28.5±10.5 vs 21.5±6 U/l, p<0,001), GGTP (85.6±48.8 vs 19.3±9 U/ l, p<0,001), total bilirubin (26.6±24.8 vs 8.9±4.7 µmol/l; p<0,001), ALP (82.4±31.5 vs 51.2±16 U/l; p<0,001) and INR (1.21±0.3 vs 0.98±0.2; p<0,001). Other parameters such as: AFP, total protein and albumin level did not statistically significantly differ. Additionally five patients (9%) according to clinical symptoms and serum albumin level were diagnosed protein-losing enteropathy (PLE). There was no difference in serum creatinine level between Fontan patients and control group (79.6±23.6 vs 75.9±18.9 µmol/l; p=0.8) as well as in uric acid level (342.7±102 vs 303±105 µmol/l; p=0.2). Cystatin C level was significantly higher in Fontan group in comparison to controls (1.1±0.6 vs 0.8±0.1 ng/ml; p=0,05). Furthermore four Fontan patients (7%) had abnormal microalbumin/creatynine ratio. Urine testing preformed in 30 Fontan patients pointed to 7 cases (23%) where hematuria was observed, while proteinuria was detected in 4 cases (13%) and urobilinogen in 2 (7%). Dysfunction of the thyroid gland was found in 30% of patients after Fontan operation (7% in the control group, p=0,034). Among disorders of the thyroid gland: 13% of patients were diagnosed with hyperthyroidism, 54% diagnosed with subclinical hypothyroidism, and the remaining 33% with hypothyroidism. CONCLUSIONS: Our study show that adult patients after Fontan procedure in long-term follow are exposed to multiorgan complications including hematological, liver, kidney and thyroid dysfunction.

Fibrosis of extracellular matrix is related to the duration of the disease but is unrelated to the dynamics of collagen metabolism in dilated cardiomyopathy.

BACKGROUND: Fibrosis of extracellular matrix (ECM) in dilated cardiomyopathy (DCM) corresponds to the myocardial over-production of various types of collagens. However, mechanism of this process is poorly understood. OBJECTIVE: To investigate whether enhanced metabolism of ECM occur in DCM. METHODS: Seventy consecutive DCM patients (pts) (48 ± 12.1 years, EF 24.4 ± 7.4 %) and 20 healthy volunteers were studied. Based on symptoms duration, pts were divided into new-onset (n = 35, 6 months) and chronic DCM (n = 35, >6 months). Markers of collagen type I and III synthesis-procollagen type I carboxy- and amino-terminal peptides (PICP and PINP) and procollagen type III carboxy- and amino-terminal peptides (PIIICP and PIIINP), collagen 1 (col-1), ECM metabolism controlling factors-tumor growth factor beta-1 (TGF1-ß), connective tissue growth factor (CTGF), and ECM degradation enzymes-matrix metalloproteinases (MMP-2, MMP-9) and their tissue inhibitor (TIMP-1) were measured in serum. All pts underwent right ventricular endomyocardial biopsy to study ECM fibrosis. RESULTS: The presence of fibrosis was detected in 24 (34.3 %) pts and was more prevalent in chronic DCM [17 (48.6 %) vs. 7 (20 %), p < 0.01]. The levels of PIIINP [4.41 (2.17-6.08) vs. 3.32 (1.69-5.02) ng/ml, p < 0.001], CTGF [3.82 (0.48-23.87) vs. 2.37 (0.51-25.32) ng/ml, p < 0.01], MMP-2 [6.06 (2.72-14.8) vs. 4.43 (2.27-7.4) ng/ml, p < 0.001], MMP-9 [1.98 (0.28-9.25) vs. 1.01 (0.29-3.59) ng/ml, p < 0.002)], and TIMP-1 [15.29 (1.8-36.17) vs. 2.61 (1.65-24.09) ng/ml, p < 0.004] were significantly higher in DCM, whereas levels of col-1 [57.7 (23.1-233.4) vs. 159.4 (31.2-512.9) pg/ml, p < 0.001] were significantly lower in DCM compared to controls. There were no differences in all measured serum markers of ECM metabolism between newonset and chronic DCM and as well as fibrosis positive and negative pts. Fibrosis was weakly correlated only with the duration of DCM (r = 0.23, p < 0.05), however, not a single serum marker of fibrosis correlated with fibrosis. Neither unadjusted nor adjusted models, constructed from serum markers of ECM metabolism, predicted the probability of myocardial fibrosis. CONCLUSIONS: Dynamics of ECM turnover in DCM is high, which is reflected by the increased levels CTGF and degradation enzymes. Synthesis of collagen type III prevailed over collagen type I. ECM metabolism was not different in DCM regardless of the duration of the disease and status of myocardial fibrosis. Serum markers of ECM metabolism were found not to be useful for the prediction of myocardial fibrosis in DCM.

Conduction abnormality and arrhythmia after transcatheter closure of atrial septal defect.

BACKGROUND: The aim of this study was to prospectively perform ambulatory 24-h ECG monitoring to assess the effects of transcatheter closure of atrial septal defect (ASD). METHODS AND RESULTS: A total of 235 consecutive subjects (female, n=163; male, n=72; age, 44.6±14.4 years) were enrolled in the study, who were due undergo ASD closure. Holter monitoring was performed before procedure and at 1, 6 and 12 months of follow-up. During the procedure transient supraventricular arrhythmia occurred in 8 patients (3.4%), and bradycardia in 3 (1.3%). In 3 patients (1.3%) an episode of atrial fibrillation occurred in the first hour after the procedure. In 8 patients (3.4%) transient first-degree atrioventricular block was noted. A significant increase in number of supraventricular extrasystoles (SVES)/24 h was noted 1 month after the procedure (P<0.001). On multiple forward stepwise regression analysis, device size and fluoroscopy time had an influence on increase in number of SVES seen 1 month after the procedure (P<0.001). CONCLUSIONS: Transcatheter closure of ASD is associated with a transient increase in supraventricular premature beats and a small risk of conduction abnormalities and paroxysmal atrial fibrillation in early follow-up. Transcatheter closure of ASD does not reduce arrhythmia that appears prior to ASD closure. Larger device size and longer procedure time are associated with increased risk of supraventricular arrhythmia on early follow-up.

Conduction Abnormality and Arrhythmia After Transcatheter Closure of Atrial Septal Defect.

Background:The aim of this study was to prospectively perform ambulatory 24-h ECG monitoring to assess the effects of transcatheter closure of atrial septal defect (ASD).Methods and Results:A total of 235 consecutive subjects (female, n=163; male, n=72; age, 44.6±14.4 years) were enrolled in the study, who were due undergo ASD closure. Holter monitoring was performed before procedure and at 1, 6 and 12 months of follow-up. During the procedure transient supraventricular arrhythmia occurred in 8 patients (3.4%), and bradycardia in 3 (1.3%). In 3 patients (1.3%) an episode of atrial fibrillation occurred in the first hour after the procedure. In 8 patients (3.4%) transient first-degree atrioventricular block was noted. A significant increase in number of supraventricular extrasystoles (SVES)/24 h was noted 1 month after the procedure (P<0.001). On multiple forward stepwise regression analysis, device size and fluoroscopy time had an influence on increase in number of SVES seen 1 month after the procedure (P<0.001).Conclusions:Transcatheter closure of ASD is associated with a transient increase in supraventricular premature beats and a small risk of conduction abnormalities and paroxysmal atrial fibrillation in early follow-up. Transcatheter closure of ASD does not reduce arrhythmia that appears prior to ASD closure. Larger device size and longer procedure time are associated with increased risk of supraventricular arrhythmia on early follow-up.

Elevated level of plasma endothelin-1 in patients with atrial septal defect.

BACKGROUND: The study aimed to assess the level of plasma Endothelin-1 (ET-1) in patients before and after transcatheter closure of atrial septal defect (ASD) and to evaluate the usefulness of measuring ET-1 levels for the diagnosis and selection of candidates for ASD closure. METHODS: 80 patients (55 F, 25 M), mean age 42,2 ± 11,5 years were enrolled for an attempt at ASD closure. A group of 19 healthy volunteers, (12 F, 7 M) mean age 39.2 ± 9.15 served as controls. All ASD patients underwent: clinical and echocardiographic study and cardiopulmonary exercise test. ET-1 levels were measured before and after closure. Whole blood was collected from femoral artery and vein and from pulmonary artery during cardiac catheterization. RESULTS: ET-1 levels at peripheral artery and vein in ASD patients were significantly higher than in the volunteers (p < 0.0001). The ASD subjects with highest ET-1 level presented the larger area of right ventricle and right atrium and higher pulmonary artery systolic pressure(p < 0.05). The ASD subjects with lower ET-1 level demonstrated longer time of exercise and higher peak oxygen consumption (p < 0.05). There was a decrease of ET-1 at peripheral artery (5.128 ± 8.8 vs. 2.22 ± 6.2; p < 0.001) and at peripheral vein (4.401 ± 3.33 vs. 2.05 ± 1.35; p < 0.001) within 48 hours after ASD closure, as compared to the baseline data. After 6 and 12 months farther drop in ET-1 level was observed. CONCLUSIONS: 1. The level of ET-1 in ASD patients is elevated in compare to healthy subject.2. The significant reduction of ET-1 level is observed after percutaneous closure of ASD.3. Elevated level of ET-1 in patients with ASD is associated with right heart enlargement.4. Measurements of ET-1 may be a supplemental diagnostic tool and may be helpful in establishing indications for defect closure.

Arterial stiffness in adult patients after Fontan procedure.

OBJECTIVES: Increased arterial stiffness is a risk factor of atherosclerosis and cardio-vascular complications. The aim of the study was to determine whether peripheral vascular function might be an early marker of impaired health status in patients with a single ventricle after Fontan procedure. METHODS AND RESULTS: Twenty five consecutive adults (11 women and 14 men) aged 24.7 ± 6.2 years after the Fontan procedure and 25 sex, age and BMI match healthy volunteers underwent physical examination, blood analysis, transthoracic echocardiography and noninvasive assessment of aortic stiffness. Augmented pressure and Augmentation Index (AIx) were both significantly elevated in Fontan when compared to the controls (6,08 ± 0,7 vs. 2,0 ± 3,7; p = 0.002 and 17,01 ± 3,3 vs. 6,05 ± 11; p < 0.001, respectively). There were no differences in pulse wave velocity (PWV), mean blood pressure (BP), brachial pulse pressure (PP), central: systolic BP, diastolic BP and PP. In Fontan group we find negative correlation between PWV and SatO2 (r = -0.68; p = 0.04) and positive correlation with WBC (0.72; p = 0.72; p = 0.013), INR (0.81; p = 0.008), TNFα (r = 0.45; p = 0.04), and postoperative time (r = 0.77; p = 0.02). AIx correlates positively only with age at surgery (r = 0.45; p = 0.04). Bilirubin level correlates positively with brachial PP (r = 0.71; p = 0.02) and central PP (r = 0.68; p = 0.03).The multivariate model showed that SatO2 (ß = -0.44, p = 0.04) was the only independent predictor of PWV (R² = 0.32, p = 0.03). CONCLUSION: Adult Fontan patients have an increased arterial stiffness assessed by a noninvasive technique. Low arterial oxygen saturation postoperative time, age at surgery, white blood cells, TNFα and bilirubin level are associated with arterial stiffening in these patients. The combination of blood parameters of the hepatic function and noninvasive measurements of arterial stiffness could be helpful in comprehensive care of patients with Fontan circulation.

Transcranial Doppler ultrasonography should it be the first choice for persistent foramen ovale screening?

BACKGROUND: Persistent foramen ovale (PFO) is considered a cause of cryptogenic stroke and a risk factor for neurological events in young patients. The reference standard for identifying a PFO is contrast-enhanced transesophageal echocardiography (TEE).The goal of this study was to evaluate the feasibility of transcranial color Doppler (TCD) and its diagnostic sensitivity compared with TEE. METHODS: We investigated 420 patients admitted to our department with cryptogenic stroke, transient ischemic attacks or other neurological symptoms. All patients underwent TCD and TEE evaluation. TCD and TEE examinations were performed according to a standardized procedure: air-mixed saline was injected into the right antecubital vein three times, while the Doppler signal was recorded during the Valsalva maneuver. During TCD the passage of contrast into the right-middle cerebral artery was recorded 25 seconds following the Valsalva maneuver. RESULTS: We detected a right-to-left shunt in 220 patients (52.3%) and no-shunts in 159 patients (37.9%) with both TCD and TEE. In 20 (4.8%) patients TEE did not reveal contrast passage which was then detected by TCD. In 21 (5.0%) patients only TEE revealed a PFO. The feasibility of both methods was 100%. TCD had a sensitivity of 95% and a specificity of 92% in the diagnosis of PFO. CONCLUSIONS: TCD has a relatively good sensitivity and specificity. TCD and TEE are complementary diagnostic tests for PFO, but TCD should be recommended as the first choice for screening because of its simplicity, non-invasive character, low cost and high feasibility.

Single ventricle function and exercise tolerance in adult patients after Fontan operation.

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.

Pregnancy Outcomes in Women after the Fontan Procedure.

Women with single ventricle physiology after the Fontan procedure, despite numerous possible complications, can reach adulthood and give birth. Pregnancy poses a hemodynamic burden for distorted physiology of Fontan circulation, but according to the literature, it is usually well tolerated unless the patient is a "failing" Fontan. Our study aimed to assess maternal and fetal outcomes in patients after the Fontan procedure followed up in two tertiary Polish medical centers. We retrospectively evaluated all pregnancies in women after the Fontan procedure who were followed up between 1995-2022. During the study period, 15 women after the Fontan procedure had 26 pregnancies. Among 26 pregnancies, eleven ended with miscarriages, and 15 pregnancies resulted in 16 live births. Fetal complications were observed in 9 (56.3%) live births, with prematurity being the most common complication (n = 7, 43.8%). We recorded 3 (18.8%) neonatal deaths. Obstetrical complications were present in 6 (40%) out of 15 completed pregnancies-two (13.3%) cases of abruptio placentae, two (13.3%) pregnancies with premature rupture of membranes, and two (13.3%) patients with antepartum hemorrhage. There was neither maternal death nor heart failure decompensation during pregnancy. In two (13.3%) women, atrial arrhythmia developed. One (6.7%) patient in the second trimester developed ventricular arrhythmia. None of the patients suffered from systemic thromboembolism during pregnancy. Pregnancy in women after the Fontan procedure is well tolerated. However, it is burdened by a high risk of miscarriage and multiple obstetrical complications. These women require specialized care provided by both experienced cardiologists and obstetricians.