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.

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children.

Successful transcatheter closure of unusual giant and symptomatic right ventricle to right atrium fistula.

We report on a patient with a significantly enlarged right heart caused by a rare right ventricle to right atrium fistula. Cardiac magnetic resonance revealed diagnosis and delineated detailed anatomy of the defect. The shunt was successfully closed with an implantation of Amplatzer Vascular Plug II. We discuss possible causes of this anomaly and its treatment.

[Hybrid treatment of interrupted aortic arch in a newborn with contraindications for extracorporeal circulation: case report including 1.5 year follow-up]. / Leczenie hybrydowe noworodka z przerwanym lukiem aorty i przeciwwskazaniami do krazenia pozaustrojowego: opis przypadku z uwzglednieniem 1,5-rocznej obserwacji.

Despite marked improvement in the cardiosurgery, total repair of interrupted aortic arch with coexisting risk factors in neonatal or early infancy is associated with high mortality. We present a patient treated by an alternative hybrid procedure without exposing the critical ill neonate to the risk of cardiopulmonary bypass. At the 1.5 year of life a successful arch reconstruction, repair of associated anomalies and de-banding of pulmonary arteries with a stent cut out was done.

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.

[Radiofrequency ablation of accessory pathway from noncoronary cusp of aorta after failed cryoablation]. / Zabieg ablacji pradem o wysokiej czestotliwoscidrogi dodatkowej przednio−przegrodowejw niewiencowym platku zastawki aortalnejpo nieskutecznym zabiegu krioablacji.

Successful ablation of atrial or ventricular arrhythmia from aortic sinuses of Valsalva has been well documented with low complication rate. Radiofrequency (RF) ablation of anteroseptal pathway is always challenging for the risk of atrioventricular block. Thus cryoablation is widely accepted approach. We present a 27 year-old patient with WPW syndrome and frequent palpitations referred for cryoablation of anteroseptal pathway. Extensive mapping from high anterseptal region and from noncoronary aortic cusp of aorta revealed a sharp pathway potential but several applications of cryomapping were unsuccessful. Finally, the pathway was permanently ablated with RF energy from noncoronary cusp of aorta. The mapping of aortic valve should be performed during difficult anteroseptal pathway ablation and unsuccessful cryomapping dose not preclude successful RF ablation at the same location.

[Is atrial septal defect always a simple defect?]. / Czy ubytek przegrody miedzyprzedsionkowej jest zawsze prosta wada?

A case of an asymptomatic 4-year-old girl with hemodynamically significant type II atrial septal defect is described. The patient underwent TEE in order to assess the eligibility for interventional device closure. Due to unfavorable anatomy and suspicion of the sinus venous type of the defect the child was selected for surgical treatment. No abnormalities were detected during surgery but a superfluous left atrial return of the venous blood during the cardiopulmonary bypass was noticed. The postoperative catheterisation was performed to elucidate this finding since the intraoperative TEE was normal. An anomalous venous drainage from superior and medial lobes of the right lung to the inferior vena cava was revealed. The 'scimitar' syndrome was diagnosed. Next, the patient underwent a successful surgical correction of this abnormality. The study presents the epidemiology, symptomatology and treatment modalities of the syndrome.

Difficult to treat recurrent stenosis of the aorta.

The risk associated with repeated treatment of aortic stenosis is as high as 5% and increases to as much as 25% in complex heart diseases. Among the methods that are commonly accepted and used in the treatment of recurrent aortic stenosis are balloon dilatation and stent implantation. In this study we describe five patients with recurrent stenosis of the aorta treated with stent implantation. The short-term results of such treatment are promising. However, in some cases it is only palliative in character and does not completely resolve the problems arising from congenital heart disease. (Cardiol J 2007; 14: 186-192).

Is device closure for direct access valved stent implantation safe?

OBJECTIVE: Despite the progress made in the development of valved stents for trans-apical valve replacement, a reliable closure of the access orifice remains a major issue. The present study was designed to evaluate if device closure of the ventricular wall is safe. MATERIALS AND METHODS: Transventricular access for pulmonary valve replacement was simulated with a 26F sheath and the resulting orifice was closed with an Amplatzer Muscular VSD Occluder (AMuscVSDO) in chronic sheep experiments (body weight 45-48 kg). Mean procedure time, blood loss, and standard hemo-dynamics were recorded. The animals were sacrificed electively and the histopathological changes in and around AMuscVSDO in the right ventricular wall were systematically studied by semi-quantitative analysis of collagenisation, inflammatory response and 'resorptive' process. RESULTS: Mean procedure time was 31+/-10.7 min, blood loss was 22.5+/-8.7 ml, heart rate was 123+/-22.6 bits/min before and 128+/-28.7 bits/min after, mean arterial blood pressure was 88+/-16.7 mm Hg before and 82.6+/-18.3 mm Hg after the procedure. Mean survival was 5.3 weeks. The collagen and scar formation studies revealed three different periods: (1) initial fibrosis (0-3 weeks); (2) so-called 'capsulation' (3-9 weeks after the implantation of the Occluder); and (3) final remodelling and differentiation (9 weeks). The fabric inside the Occluder played the role of a collagenisation promoter, active from the 3rd week till it vanishes. Inflammation plays a role as a temporary reaction (0-3 weeks) during the healing process, with no signs of any active, focal or circumscribed, myocardial damage. CONCLUSIONS: (1) The closure of the free ventricular wall perforation with AMuscVSDO is safe due to the scar tissue resulting from the healing process around and in the device. (2) The myocardial healing around and inside an implanted AMuscVSDO represents two processes: extensive fibrosis ensues around metallic wires with the progression towards the inside of the myocardium, whereas inside AMuscVSDO the loose connective tissue fills the myocardial lesion. During cicatrisation, the fabric elements of AMuscVSDO act as the ground for collagen formation and fibroblast proliferation. (3) The cicatrisation processes after ventricular AMuscVSDO implantation show remodelling, with rearrangement of collagen fibres architecture and distribution.

Comparison of cardiac function in children after surgical and Amplatzer occluder closure of secundum atrial septal defects.

OBJECTIVE: The aim of the retrospective study was to examine the changes in the left and right ventricular size as well as the systolic and diastolic function of the left ventricle after surgical and transcatheter treatment of atrial septal defects with Amplatzer atrial septal defect occluder (ASDO). METHODS: Two groups of patients were examined using transthoracic cross-sectional echocardiography before and after the treatment of atrial septal defect: Group A--Amplatzer ASD occluder--38 children and Group S treated surgically--20 children. The following parameters were assessed: left and right ventricular end-diastolic diameter indexes, ejection fraction, mitral E wave to A wave velocity ratio, deceleration time (DCT), isovolumetric relaxation time (IVRT) and heart rate. RESULTS: There was a significant decrease in right ventricular and an increase in left ventricular size in both groups during the follow-up observation. The long-term ECHO examination revealed smaller right ventricle (RV) (Group A: RVEDI=1.00+/-0.20 vs Group S RVEDI: 1.18+/-0.20 (p=0.001)) and bigger left ventricle (Group A: LVEDI=1.04+/-0.08 vs Group S: LVEDI=0.99+/-0.07 (p=0.022)) in Group A in comparison to Group S. Children undergoing operation had significantly shorter IVRT (Group A: IVRT=50.00+/- 9.65 vs Group S: IVRT=42.5+/- 8.95 (p=0.02)) than patients after ASDO device application. CONCLUSIONS: (1) During the follow-up period, the diastolic function of the left ventricle is better in children with device closure of ASD compared with those patients treated surgically. (2) Postoperative changes of the left and right ventricular size indexes are more favourable in patients after the device closure of ASD compared with children undergoing the surgical procedure.

Closure of perimembranous ventricular septal defect using transcatheter technique versus surgical repair.

INTRODUCTION: Since the Membr VSD occluders have been introduced into clinical practice it is now possible to compare two treatment methods -- surgical and percutaneous. AIM: Assessment of the effectiveness and risk of complications in patients with perimembranous ventricular septal defect (VSD) treated with the Membr VSD occluder or surgery as well as comparison of postprocedural left and right ventricular systolic functions. METHODS: The study involved 11 children with perimembranous VSD treated with occluder implantation (Group A) and 12 children with surgical repair (Group C). Groups A and C differed slightly in terms of age of patients (p=0.026), but had similar mean weights (p=0.103), pulmonary to systemic flow ratios (Qp: Qs, p=0.929) and follow-up duration after the procedure. No significant differences were observed between the two groups in terms of left ventricular systolic function [left ventricular ejection fraction (EF, p=0.567)], diastolic function [mitral flow E/A ratio (p=0.975)], E deceleration time (DCT, p=0.346), isovolumetric relaxation time (IVRT, p=0.606), heart rate (HR, p=0.133) or left ventricular diameter (LV) on transthoracic echocardiography (TTE) before VSD closure. TEE was performed in all patients and the parameters listed above were measured within 3 to 16 months after the procedure, and then intra- and inter-group comparisons were carried out. Additionally, mitral (MV), tricuspid (TV) and aortic (Ao) valve regurgitations were evaluated. RESULTS: Tricuspid (TR, p=0.028) and mitral regurgitation (MR, p=0.043) decreased significantly after closure of VSD with the Membr VSDO. MR was significantly smaller in patients treated with the occluder than in those after surgery (p=0.026). Postprocedural TR (p=0.486) and aortic regurgitation (AR, 0.607) did not differ significantly between the two groups. Left ventricular EF was significantly (p=0.004) lower and HR higher (0.043) after surgery than in children treated with the occluder. No significant differences of the diastolic function were found - E/A (p=0.88), DCT (p=0.413), IVRT (p=0.09). CONCLUSIONS: 1. During the mid-term follow-up left ventricular EF was higher in patients after Membr VSDO occluder implantation than after surgical repair of VSD. 2. Higher EF and reduction of the left ventricular diameter after closure of perimembranous VSD with the occluder may result in a lower incidence and degree of MR in comparison to the subjects operated on.

[Percutaneous procedures for the repair of iatrogenic occlusion or stenosis of peripheral vessels in children--report of 5 cases]. / Przezskórne zabiegi naprawcze zwezonych jatrogennie naczyn obwodowych. Opis pieciu przypadków.

Invasive percutaneous diagnostic or therapeutic procedures are associated with the risk of thrombosis and occlusion of peripheral vessels which are used for vascular access. Data on the transcatheter therapy of vascular complications in children are scarce. We described five children in four of whom percutaneous transluminal balloon angioplasty of occluded peripheral vessels was successfully performed. Technical aspects of this treatment and indications are discussed.

Transcatheter closure of perimembranous ventricular septal defect using an Amplatzer occluder--early results.

BACKGROUND: Transcatheter closure of perimembranous ventricular septal defect with the use of an Amplatzer occluder was introduced in 1999, however, clinical experience with this method remains limited. AIM: To assess short-term safety and efficacy of this technique in children. METHODS: Since 2001, nine children underwent transcatheter closure of perimembranous ventricular septal defect in the cardiac centre in Bratislava (Slovak Republic) or in our centre. Patients were selected for the procedure based on the results of transesophageal echocardiography (TEE). Transcatheter closure using the Amplatzer occluder was guided by both TEE and angiography. The follow-up duration ranged from 3 to 22 months, mean 11.5 months. Control angiography was performed in order to detect any leakage through the aortic valve. The end-diastolic left ventricular diameter before and after the procedure as well as the function of the aortic, tricuspid and mitral valves were also assessed. RESULTS: The procedure was effective in all patients. No damage to atrio-ventricular nor aortic valves was observed. Tricuspid regurgitation, present prior to the procedure, markedly decreased following the closure of the defect. The left ventricular end-diastolic diameter significantly (p=0.001) decreased after the procedure. CONCLUSIONS: (1) Perimembranous ventricular septal defect can be safely and effectively closed using a transcatheter Amplatzer occluder. (2) Appropriate selection for the procedure as well as TEE and angiographic guidance during the procedure enable the avoidance of complications. (3) Tricuspid regurgitation is not an absolute contraindication to perform this procedure.