Current practice of care for adolescent and adult patients after Fontan surgery in Poland.

BACKGROUND: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers. AIMS: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey. METHODS: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. RESULTS: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy). CONCLUSIONS: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.

Thromboembolic Complications in Adult Patients Following Fontan Procedure-A Multicenter Study.

BACKGROUND: Morbidity and mortality following Fontan (FO) surgery are primarily thromboembolic in nature. However, follow-up data regarding thromboembolic complications (TECs) in adult patients after FO procedure are inconsistent. In this multicenter study, we investigated the incidence of TECs in FO patients. METHODS: We studied 91 patients who underwent FO procedure. Clinical data, laboratory, and imaging investigations were collected prospectively during the scheduled medical appointments in 3 Adult Congenital Heart Disease Departments in Poland. TECs were recorded during a median follow-up of 31 months. RESULTS: Four patients (4.4%) were lost to follow-up. The mean age of patients was 25.3 (±6.0) years at enrollment, and the mean time between FO operation and investigation was 22.1 (±5.1) years. A total of 21 out of 91 patients (23.1%) had a history of 24 TECs since an FO procedure, mainly pulmonary embolism (PE; n = 12, 13.2%), including 4 (33.3%) silent PE. The mean time since FO operation to the first TEC was 17.8 (±5.1) years. During follow-up, we documented 9 TECs in 7 (8.0%) patients, mainly PE (n = 5, 5.5%). Most patients with TEC had a left type of systemic ventricle (57.1%). Three patients (42.9%) were treated with aspirin, 3 (3.4%) with Vitamin K antagonists or novel oral anticoagulants, and 1 patient had no antithrombotic treatment at the time of TEC occurrence. Supraventricular tachyarrhythmias were present in 3 patients (42.9%). CONCLUSIONS: This prospective study shows that TECs are common in FO patients, and a significant number of these events occur during adolescence and young adulthood. We also indicated how much TECs are underestimated in the growing adult FO population. The complexity of the problem requires more studies, especially to standardize the prevention of TECs in the whole FO population.

Dissimilarity in coagulation system in adults after Fontan surgery based on thrombin generations.

OBJECTIVES: The Fontan procedure is the treatment of choice in congenital cardiac malformations defined as the single ventricle. Fontan patients are at high risk of thromboembolism, but the exact mechanism of this is poorly understood. The aim of this study was to evaluate an involvement of thrombin generations and microparticles (MPs) in prothrombotic state in adults with Fontan circulation. METHODS: This study included hospitalized patients after Fontan procedure and healthy volunteers. We assessed laboratory tests including thrombin generation by calibrated automated thrombography in three variants [platelet-poor plasma (impact of coagulation factors), platelet-rich plasma (PRP) (influence of platelets) and related with MPs]. The technique allows for a comprehensive evaluation of the coagulation system. RESULTS: The study groups consisted of 81 adult Fontan patients [41 females (50.6%); median age 22 interquartile range [20-27] years] and 54 control subjects. In patients with Fontan circulation, higher values of endogenous thrombin potential and peak values were observed for both platelet-poor plasma (+17% and +33%) and MPs (+29% and 41%) compared to controls (all P < 0.05). Moreover, in the Fontan group, we found a 64.9% shorter lag time and a 70.4% time to peak for MP variant (both P < 0.001). Contrarily, analysis in the PRP showed 17.1% of reduced endogenous thrombin potential in Fontan. Furthermore, there were no differences in thrombin synthesis in PRP in Fontan patients receiving aspirin or those with thrombocytopaenia (all P > 0.05). CONCLUSIONS: This study for the first time showed that thrombin generation associated with MPs may be an important contributor to the prothrombotic state in the Fontan population.

Properties of Plasma Clots in Adult Patients Following Fontan Procedure: Relation to Clot Permeability and Lysis Time-Multicenter Study.

OBJECTIVES: thromboembolic complications are a major cause of morbidity and mortality following Fontan (FO) surgery. It is also well established that altered FO circulation results in systemic complications, including liver and endothelium damage. We sought to evaluate whether dysfunctions of these sources of hemostatic factors may result in changes of fibrin clot properties. METHODS: a permeation coefficient (Ks) and clot lysis time (CLT) were assessed in 66 FO patients, aged 23.0 years [IQR 19.3-27.0], and 59 controls, aged 24.0 years [IQR 19.0-29.0]. Ks was determined using a pressure-driven system. CLT value was measured according to assay described by Pieters et al. Endothelium and liver-derived hemostatic factors along with liver function parameters were evaluated. The median time between FO operation and investigation was 20.5 years [IQR 16.3-22.0]. RESULTS: FO patients had lower Ks (p = 0.005) and prolonged CLT (p < 0.001) compared to that of controls. Ks correlated with CLT (r = -0.28), FVIII (r = -0.30), FIX (r = -0.38), fibrinogen (r = -0.41), ALT (r = -0.25), AST (r = -0.26), GGTP (r = -0.27) and vWF antigen (r = -0.30), (all p < 0.05). CLT correlated with the time between FO operation and investigation (r = 0.29) and FIX (r = 0.25), (all p < 0.05). After adjustment for potential cofounders, TAFI antigen and GGTP were independent predictors of reduced Ks (OR 1.041 per 1% increase, 95% CI 1.009-1.081, p = 0.011 and OR 1.025 per 1 U/L increase, 95% CI 1.005-1.053, p = 0.033, respectively). Protein C and LDL cholesterol predicted prolonged CLT (OR 1.078 per 1% increase, 95% CI 1.027-1.153, p = 0.001 and OR 6.360 per 1 µmol/L increase, 95% CI 1.492-39.894, p = 0.011, respectively). Whereas elevated tPA was associated with lower risk of prolonged CLT (OR 0.550 per 1 ng/mL, 95% CI 0.314-0.854, p = 0.004). GGTP correlated positively with time between FO surgery and investigation (r = 0.25, p = 0.045) and patients with abnormal elevated GGTP activity (n = 28, 42.4%) had decreased Ks, compared to that of the others (5.9 × 10-9 cm2 vs. 6.8 × 10-9 cm2, p = 0.042). CONCLUSION: our study shows that cellular liver damage and endothelial injury were associated with prothrombotic clot phenotype reflected by Ks and CLT.

The Impact of COVID-19 Pandemic on Children with Pulmonary Arterial Hypertension. Parental Anxiety and Attitudes. Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

The COVID-19 pandemic has impacted healthcare systems worldwide. Little is known about the impact of the pandemic on medical and psycho-social aspects of children with rare diseases such as pulmonary arterial hypertension and their parents. The study is based on children registered in The Database of Pulmonary Hypertension in the Polish Population and a parent-reported survey deployed during the first 6 months of the pandemic. The questionnaire consisted of six question panels: demographic data, fear of COVID-19, General Anxiety Disorder-7 (GAD-7), social impact of pandemic, patients' medical status, and alarming symptoms (appearance or exacerbation). Out of 80 children registered, we collected 58 responses (72.5% response rate). Responders (parents) were mostly female (n = 55; 94.8%) at a mean age of 40.6 ± 6.9 years. Patients (children) were both females (n = 32; 55%) and males with a mean age of 10.0 ± 5.1 years. Eleven (19%) children had symptoms of potential disease exacerbation. Eight parents (72.7%) decided for watchful waiting while others contacted their GPs or cardiologists (n = 6; 54.5%). Three children had to be hospitalized (27.3%). Most planned hospitalizations (27/48; 56.2%) and out-patient visits (20/35; 57.1%) were cancelled, delayed, or substituted by telehealth services. Among the participating parents, the study shows very high levels of anxiety (n = 20; 34.5%) and concern (n = 55; 94.8%) and the need for detailed information (52; 89.6%) regarding COVID-19 and medical service preparedness during the pandemic. The COVID-19 pandemic has influenced child healthcare and caused high levels of anxiety among parents.

Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS).

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is an inborn complex heart malformation. A multi-stage treatment is initiated in a neonatal period with a Norwood surgery. The next step is Glenn surgery - a bidirectional superior cavo-pulmonary anastomosis. At the last stage anastomosis of inferior vena cava (IVC) with the right pulmonary artery (RPA) is formed as a result of a Fontan surgery. The aim of this study was to assess lung perfusion in patients with HLHS after completion of a surgical therapy, using a scintigraphic method. MATERIAL AND METHODS: In 92 patients with HLHS a planar lung scintigraphy in anterior and posterior projections after administration of 99mTc-macroaggregates in activity 18-111MBq was carried out twice (in several day intervals). At first, a radiopharmaceutical was administered to the right extremity in order to assess the lung distribution of blood flowing through the anastomosis of superior vena cava (SVC) with RPA. In the next study, after administration of the tracer to the right lower extremity, the distribution of blood flowing through the anastomosis of IVC with RPA was assessed. The relative percentage of each lung in the total lung perfusion was calculated on a Xeleris workstation using the "Lung perfusion analysis" program. Lung perfusion was considered close to symmetrical when the proportion was in the range of 40-60%. RESULTS: In spite of the fact that mean relative values of distribution of blood flowing through the anastomosis of SVC with RPA to the left lung (LL) and right lung (RL) in the entire study group did not differ significantly: LLmean = 47%; RLmean = 53%, p = 0.14, relative values of perfusion of both lungs were differentiated - in 26% of patients LL was better perfused, in 38% RL was better perfused and in 36% a perfusion of both lungs was similar. The analysis of blood distribution by anastomosis of IVC with RPA showed that the mean relative perfusion of RL was significantly higher than that of LL (70% vs. 30%, p < 0.0000001). No signs of pulmonary emboli were detected. In 13% of studied patients, uptake of the radiopharmaceutical in kidneys was shown indicating the presence of shunt "from right to left". CONCLUSIONS: After completion of surgical treatment of patients with HLHS, differentiated blood supply of the lungs was observed through SVC with RPA anastomosis and a tendency to higher blood supply of RL than LL by anastomosis of IVC with RPA. No signs of pulmonary embolism were detected. The study revealed a "right to left" shunt in some patients.

Fenestration closure with Amplatzer Duct Occluder II in patients after total cavo-pulmonary connection.

INTRODUCTION: Creation of a fenestration during completion of a total cavopulmonary connection (TCPC) has been associated with a reduction in early mortality and morbidity. However, the long-term benefits are negated by an associated limitation in exercise tolerance and the potential risks of thrombo-embolic complications. We sought to describe the safety and efficacy of an Amplatzer Duct Occluder II (ADO II) for transcatheter fenestration closure following TCPC. MATERIAL AND METHODS: Between January 2000 and July 2014, 102 patients underwent percutaneous closure of extra-cardiac TCPC fenestrations with a range of devices. Patients in whom fenestration closure was performed with an ADO II and who had at least 6 months of follow-up were included in this study. RESULTS: Forty-seven patients had successful fenestration occlusion with an ADO II. The mean oxygen saturation and mean systemic venous pressures increased from 84.8 ±6.1% before to 97.6 ±2.9% (p < 0.001) after and from 14.2 ±2.15 mm Hg before to 15.6 ±2.2 mm Hg after closure (p < 0.001). Eight patients developed heart failure symptoms, managed by optimization of medical therapy, with 1 patient requiring device removal to reopen the fenestration. Color Doppler transthoracic echocardiography demonstrated residual flow across the device in 18 (38%), 10 (22%), 5 (11%) and 4 (9%) patients before discharge, at 1 and 6 months, and at the latest outpatient visit, respectively. CONCLUSIONS: The ADO II can be safely and effectively used to close fenestrations in extra-cardiac type Fontan completions. Many of the design features of this device confer potential benefit in this population.

A 10-year single-centre experience in percutaneous interventions for multi-stage treatment of hypoplastic left heart syndrome.

OBJECTIVES: The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications. BACKGROUND: Constant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment. METHODS: Between January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation. RESULTS: Percutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries. CONCLUSIONS: Percutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.

Stent implantation into the interatrial septum in patients with univentricular heart and a secondary restriction of interatrial communication.

BACKGROUND: Presence of a restrictive interatrial communication in patients with univentricular anatomy significantly affects surgical outcomes. In patients with univentricular hearts, wide open atrial communication leads to lower pulmonary artery pressure, which is one of the most important factors influencing the success of bidirectional Glenn and Fontan operations. In some patients, recurrence of restricted interatrial communication can be observed despite initially successful interventional or surgical creation of unrestrictive interatrial communication. AIM: To evaluate efficacy of stent implantation into the interatrial septum in patients with univentricular heart and a secondary restriction of interatrial communication. METHODS: In 2006-2010, we created unrestrictive interatrial communication by stent implantation into the interatrial septum in 7 children with univentricular anatomy with systemic right ventricle (4 patients with hypoplastic left heart syndrome and 3 patients with mitral atresia). In all patients we diagnosed recurrent restriction of interatrial communication despite prior surgical or interventional creation of unrestrictive interatrial communication. Patient age at stent implantation was 3 to 30 months. Maximal systolic pressure gradient between the left and the right atrium was 6-29 mm Hg and left atrial pressure ranged from 20/17/19 mm Hg to 40/29/32 mm Hg. In all patients, we implanted a Palmaz-Genesis stent (length 18-29 mm) with subsequent balloon redilatation. RESULTS: In all 7 patients, we created unrestrictive interatrial communication with mean pressure gradient reduction from 13.14 mm Hg to 0.86 mm Hg (p < 0.006). Mean interatrial communication diameter increased from 4.14 mm to 10.57 mm (p < 0.0001). CONCLUSIONS: Percutaneous stent implantation into the interatrial septum in children with univentricular heart and secondary restriction of interatrial communication is a safe and effective method. Kardiol Pol 2011; 69, 11: 1137-1141.

Prenatal diagnosis of major aortopulmonary collateral arteries.

BACKGROUND: The presence of foetal major aortopulmonary collateral arteries (MAPCAs) is associated with adverse outcome, therefore early diagnosis is essential. AIM: To evaluate the usefulness of foetal echocardiography in the diagnosis and evaluation of MAPCAs in foetuses with pulmonary atresia, as well as to assess the effects of prenatal diagnosis on the management of neonates with pulmonary atresia. METHODS: From 11,678 examined foetuses, we retrieved 15 cases of patients with MAPCAs and congenital heart defects which had been diagnosed by foetal echocardiography (1994-2008), using 2D echocardiography + color-Doppler (CD) + pulsed Doppler (2DD) and spatio-temporal image correlation (STIC) techniques. In 13 patients, MAPCAs were confirmed after birth based on angiography. RESULTS: In all cases, vessels corresponding to MAPCAs were visible in longitudinal view with CD, and in three cases were additionally confirmed by STIC technique. In nine cases one, in four cases two, and in two cases three MAPCAs were suspected. In two cases, MAPCAs were not confirmed after birth; one due to misdiagnosis secondary to aberrant right subclavian artery, and one because of abnormal ductus arteriosus course coexistent with right aortic arch. CONCLUSIONS: In foetuses with pulmonary atresia, it is possible to find MAPCAs with current technology (both 2D + CD, power angiography and real time-3D echocardiograophy [4D]). The differential diagnosis (MAPCAs or other vessels) should be included. Although prenatal diagnosis does not change the obstetrical management, it is important information for a paediatric cardiologist. Early neonatal angiography might be of great value not only in confirming MAPCAs, but also in performing cardiac intervention and in some cases preventing future heart failure.

Late emergency arterial duct stenting in a patient with tetralogy of Fallot and occluded Blalock-Taussig shunt.

Over the last decade or so, stent implantation to the arterial duct has achieved acceptance as an alternative to aortopulmonary shunt procedure. We present the case of a patient with tetralogy of Fallot with diminished pulmonary flow and complete proximal occlusion of a right-sided Blalock-Taussig shunt. Surgical repair was not possible because of relative contraindications, and the interventional shunt recanalisation attempt was unsuccessful. The coronary stent was implanted into arterial duct. This resulted in oxygen saturation increase with normal contrast flow to the left pulmonary artery and right pulmonary artery (prior to the procedure we suspected non-confluent pulmonary artery). This brief report evaluates the feasibility of this new therapeutical option in such special patients with duct-dependent pulmonary blood flow.

Right ventricle outflow tract stenting as a method of palliative treatment of severe tetralogy of Fallot.

We present the case of a 3-month-old infant with tetralogy of Fallot (ToF) with pulmonary artery hypoplasia, critical right ventricle outflow tract (RVOT) obstruction and the presence of major aortopulmonary collateral arteries (MAPCA) and CATCH 22 syndrome. Due to anatomical conditions (severe pulmonary artery hypoplasia), the patient was not qualified for palliative operative treatment Blalock-Taussig shunt. We conducted catheterization with an attempt of balloon plasty and stent implantation into the right ventricle outflow tract and main pulmonary artery. Successful stent implantation into the right ventricle outflow tract was performed. The stent created a 4.1 mm diameter channel and allowed for unrestricted blood flow from the right ventricle to the pulmonary arteries. After the procedure we observed an increase in blood saturation of up to 89%. Control echocardiography revealed blood flow through the stent to the pulmonary arteries with a pressure gradient of 45 mm Hg. There were neither rhythm nor conduction disturbances in the control ECG after the procedure. After 6 days of observation the patient was discharged from our department. We conclude that successful stent implantation into the RVOT in patients with ToF and hypoplastic pulmonary arteries improves their clinical condition, increases pulmonary blood flow by physiological means and leads to an improvement of pulmonary artery development before surgical treatment.