Takotsubo syndrome as a cardiac manifestation of multisystem inflammatory syndrome in children.

Cases of severe heart damage in patients presenting with multisystem inflammatory syndrome in children are one of the most intriguing phenomena during the coronavirus disease of 2019 pandemic. The pathophysiology of myocardial changes in the course of this syndrome has not been fully understood yet. We present a case of a child with multisystem inflammatory syndrome in children and with cardiac changes corresponding to Takotsubo syndrome.

A milestone in congenital cardiac surgery: Four decades of the Norwood procedure.

Hypoplastic left heart syndrome (HLHS) was first described by Lev in 1952, but it was not until 1958 that it received a name from Noonan and Nadas. For the next several decades, the defect was considered untreatable. In 1979, William Norwood and his colleagues from Boston initiated a program to evaluate staged surgical management for infants with HLHS. The Norwood operation has became a milestone in the effective palliation for neonates born with HLHS. Today, the Norwood procedure is the first step of a three-stage heart surgery aimed at creating a new circulatory pathway (i.e., the Fontan pathway).

Levosimendan as a bridge to heart transplant in a 16-year-old patient with univentricular heart.

We report the successful use of levosimendan in the treatment of heart failure in a patient with the univentricular heart. The presented case was atypical because our patient had systemic right ventricle. To our knowledge, it is the first reported such case with intermittent levosimendan administration as an effective treatment and bridge to successful heart transplant in a patient with functionally univentricular heart.

Recanalisation of the left superior caval vein after Fontan procedure: not so rare complication: possibilities of percutaneous closure using various devices.

AIM: Evaluation of possibilities of percutaneous closure of recanalised left superior caval vein after total cavopulmonary connection. METHODS AND RESULTS: We analysed 19 patients after total cavopulmonary connection catheterised because of a sudden increase of desaturation. In four of them, the recanalisation of the left superior caval vein was identified. For this reason, the balloon occlusion tests of the veins were made temporarily. In all cases, the haemodynamic status of patients did not change, and arterial oxygen saturation increased significantly. Thus, using different types of implants, these veins were closed effectively in all patients. During the short-term follow-up, the effectiveness of treatments and constantly maintaining a high level of saturation were confirmed. CONCLUSIONS: Meticulous investigation of unclear causes of desaturation in cyanotic patients after Fontan completion is necessary. Almost all causes of desaturation, including recanalised additional left superior caval vein, can be effectively treated percutaneously.

Infective Endocarditis in Children as an Increasing Clinical Problem-A Case Series.

In September 2023, the European Society of Cardiology (ESC) published new guidelines for the management of endocarditis. Infective endocarditis (IE) remains a significant life-threatening disease, concerning an increasingly younger age group, especially children with congenital heart disease (CHD) and young adults after multiple cardiac surgeries. This study's aim was to alert the medical community to the problem of increasing IE case numbers and IE course complexity. Of the eight patients who suffered from IE treated in 2023 in our department, we describe the four whose course was the most extraordinary. Afterward, we compared the number of IE patients treated over the years in our department. All cases described children with congenital heart disease suffering from IE. The IE clinical presentation in all patients was extremely diverse, necessitating the utilization of all available diagnostic methods. Each child underwent specialized treatment and subsequently qualified for cardiac surgery. While the etiology and treatment of IE are well established, it remains a formidable challenge for physicians. Pediatric patients who have undergone multiple cardiac surgeries constitute a steadily expanding group and are especially susceptible to IE throughout their lives. Currently, no recommendations exist for the management of endocarditis in pediatric patients. This gap compels pediatricians to adapt existing guidelines designed for adult patients and to rely on scientific reports, such as case studies.

Efficacy and safety of zero-fluoroscopy approach for ablation of atrioventricular nodal reentry tachycardia: experience from more than 1000 cases.

BACKGROUND: Radiofrequency catheter ablation (RFCA) of the slow pathway in atrioventricular nodal reentry tachycardia (AVNRT) is associated with high efficacy and low risk of total perioperative or late atrioventricular block. This study aimed to evaluate the efficacy, safety, and feasibility of slow-pathway RFCA for AVNRT using a zero-fluoroscopy approach. METHODS: Data were obtained from a prospective multicenter registry of catheter ablation from January 2012 to February 2018. Consecutive unselected patients with the final diagnosis of AVNRT were recruited. Electrophysiological and 3-dimensional (3D) electroanatomical mapping systems were used to create 3D maps and to navigate only 2 catheters from the femoral access. Acute procedural efficacy was evaluated using the isoproterenol and/or atropine test, with 15-min observation after ablation. Each case of recurrence or complication was consulted at an outpatient clinic during long-term follow-up. RESULTS: Of the 1032 procedures, 1007 (97.5%) were completed without fluoroscopy. Conversion to fluoroscopy was required in 25 patients (2.5%), mainly due to an atypical location of the coronary sinus (n = 7) and catheter instability (n = 7). The mean radiation exposure time was 1.95 ± 1.3 min for these cases. The mean fluoroscopy time for the entire study cohort was 0.05 ± 0.4 min. The mean total procedure time was 44.8 ± 18.6 min. There were no significant in-hospital complications. The total success rate was 96.1% (n = 992), and the recurrence rate was 3.9% (n = 40). CONCLUSION: Slow-pathway RFCA can be safely performed without fluoroscopy, with a minimal risk of complications and a high success rate.

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.

Riociguat in children with pulmonary arterial hypertension: The PATENT-CHILD study.

Riociguat, a soluble guanylate cyclase stimulator, is approved for treatment of adults with pulmonary arterial hypertension (PAH). The safety, tolerability, and pharmacokinetics (PK) of oral riociguat in a pediatric population with PAH was assessed in PATENT-CHILD (NCT02562235), a multicenter, single-arm, 24-week, open-label, Phase 3 study. Patients aged 6-17 years in World Health Organization functional class (WHO-FC) I-III treated with stable endothelin receptor antagonists and/or prostacyclin analogs received riociguat equivalent to 0.5-2.5 mg three times daily in adults, as either oral pediatric suspension or tablets, based on bodyweight. Primary outcomes were safety, tolerability, and PK of riociguat. Twenty-four patients (mean age 12.8 years), 18 of whom were in WHO-FC II, were enrolled. Adverse events (AEs), mostly mild or moderate, were reported in 20 patients (83%). Four patients (17%) experienced a serious AE; all resolved by study end and two (8%) were considered study-drug related. Hypotension was reported in three patients and hemoptysis in one (all mild/moderate intensity). Riociguat plasma concentrations in pediatric patients were consistent with those published in adult patients. From baseline to Week 24, mean ± standard deviation increase in 6-minute walking distance was 23 ± 69 m (n = 19), and mean decrease in NT-proBNP was -66 ± 585 pg/ml (n = 14). There was no change in WHO-FC. Two patients experienced clinical worsening events of hospitalization for right heart failure. PK results confirmed a suitable riociguat dosing strategy for pediatric patients with PAH. The data suggest an acceptable safety profile with potential efficacy signals.

Distinct characteristics of multisystem inflammatory syndrome in children in Poland.

During the winter months of 2020/2021 a wave of multisystem inflammatory syndrome in children (MIS-C) emerged in Poland. We present the results of a nationwide register aiming to capture and characterise MIS-C with a focus on severity determinants. The first MIS-C wave in Poland was notably high, hence our analysis involved 274 children. The group was 62.8% boys, with a median age of 8.8 years. Besides one Asian, all were White. Overall, the disease course was not as severe as in previous reports, however. Pediatric intensive care treatment was required for merely 23 (8.4%) of children, who were older and exhibited a distinguished clinical picture at hospital admission. We have also identified sex-dependent differences; teenage boys more often had cardiac involvement (decreased ejection fraction in 25.9% vs. 14.7%) and fulfilled macrophage activation syndrome definition (31.0% vs. 15.2%). Among all boys, those hospitalized in pediatric intensive care unit were significantly older (median 11.2 vs. 9.1 years). Henceforth, while ethnicity and sex may affect MIS-C phenotype, management protocols might be not universally applicable, and should rather be adjusted to the specific population.

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.

Percutaneous closure of atrial septal defect: a consensus document of the joint group of experts from the Association of Cardiovascular Interventions and the Grown-Up Congenital Heart Disease Section of the Polish Cardiac Society.

Atrial septal defect is the most common congenital heart lesion in adults. Although atrial septal defect closure is recommended in those with right heart enlargement or paradoxical embolism, data informing such indications in adults are quite limited. This population has many unique characteristics and needs. In recent years, significant progress has been made with regard to diagnostic modalities that facilitate the diagnostic workup of these patients. However, the decision­making process, especially in selected adult patients population (advanced age, various concomitant diseases, poor LV function [both systolic and diastolic], pulmonary hypertension, concomitant arrhythmias or multiple defects, or deficient rims) is still not easy. Available data are predominantly focused on imaging endpoints and short­term morbidity and mortality rather than long­­term. The evidence base for outcomes with or without defect closure comes from various studies with different observation periods. Moreover, the clinical experience in diagnosing and treating that subgroup of patients is inhomogeneous between individual physicians (cardiologists, imaging specialists, operators) and between small and large experience centers. In the view of the above, the joint group of experts from the Association of Cardiovascular Interventions and the Grown­Up Congenital Heart Disease Section of the Polish Cardiac Society developed the following consensus opinion in order to standardize the principles of diagnosis, indications for treatment, methods of performing procedures, and tenets of postoperative care in Poland.

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.

[Transcatheter closure of aorto-venous fistulae--is it always necessary?]. / Przezcewnikowe zamykanie przetok aortalno-zylnych--czy zawsze jest konieczne?

We present 2 cases (4 and 17-year-old children) with aorta-vena azygos fistulae. In the fist child spontaneous closure was confirmed by clinical symptoms and aortography, in the second (with significant left-right shunt) successful closure with 10 x 8 mm Amplatzer Duct Occluder was obtained. These case reports show that the strategy of treatment of aorto-venous fistulae must be individualised.

[Transcatheter implantation of pulmonary valve - own experience]. / Pierwsze doswiadczenia w przezcewnikowym wszczepieniu zastawki plucnej w leczeniu zlozonych wrodzonych wad serca.

Transcatheter implantation of pulmonary valve became a big step forward in the field of interventional cardiology. It is especially important in the patients with defects of the right ventricular outlet tract, because they were usually candidates for multiple surgical operations. We present first transcatheter replacement of pulmonary valve in 23-years-old man. The 'Melody' valve was implanted successfully. There were no complications and the patient was discharged in good condition.

[POL-PAVTI--Polish report on transcatheter pulmonary artery valve implantation of Melody-Medtronic prosthesis in the first 14 patients in Poland]. / Ogólnopolski raport POL-PAVTI z nieoperacyjnego wszczepienia biologicznej zastawki plucnej Melody-Medtronic u pierwszych w Polsce 14 chorych.

AIM: To assess the early results of the pulmonary artery valve transcatheter implantation (PAVTI) in pts included into POL-PAVTI registry. Detailed medical and economic analyses were performed. METHODS: Pulmonary artery valve implantation was performed in 14 pts (9 men), aged 16-31 (mean 24.6 +/- 4.8) years, with pulmonary homograft dysfunction after total repair of tetralogy of Fallot (4 pts), pulmonary atresia (2 pts), pulmonary stenosis (1 pt), common arterial trunk type I (1 pt), Ross procedure (3 pts) and TGA - Rastelli operation (3 pts). Eleven pts underwent in the past 2-5 surgical or/and catheter interventions. Indication for PAVTI was based on clinical evaluation and echocardiographic studies. Assessment of morphological and functional features of the right ventricle (RV) and homograft with the use of cardiac magnetic resonance (CMR) was performed in 10 cases. Pulmonary stenosis (max. pulmonary gradient 32-119, mean 72 +/- 28 mmHg) was observed in 13 pts and/or significant pulmonary regurgitation in 10 pts. The procedure was performed in general anesthesia. The deployment of a valved stent in the pulmonary valve position was preceded by a metal stent implantation. Results were evaluated by echocardiography two days after the procedure and one month later. Four patients were evaluated 6 months after procedure. RESULTS: Time of the procedure varied 60-190 (mean 127 +/- 35) min, time of fluoroscopy ranged 12-31 (mean 21 +/- 11) min. PAVTI was successfully performed in all pts without serious complications. Patients were discharged from the hospital 48-293 (mean 120 +/- 71) h after procedure. Significant reduction of pulmonary gradient after the procedure assessed by echocardiography was observed on the second day (20-60, mean 38 +/- 12 mmHg, p < 0.0001) and one month (19-52, mean 34 +/- 9 mmHg, p < 0.0001). Mild pulmonary regurgitation was observed in 2 pts. In 5 pts evaluated 6 months after procedure haemodynamic parameters were unchanged; no late complications were observed. Average cost of the procedure including a price of the valve (82 000 PLN) was 98 000 PLN. CONCLUSIONS: Pulmonary artery valve transvascular implantation is an effective and safe method of non-surgical treatment for patients with homograft dysfunction. Cost-effectiveness is approvable.

Adam Christian Thebesius' Channels into the Human Heart: the Thebesian Veins and the Thebesian Valve.

In 1708, Adam Christian Thebesius, a 22-year-old student at Leiden University, presented his graduate thesis, Disputatio medica inauguralis de circulo sanguinis in corde. More than a doctoral dissertation, this groundbreaking work opened new channels into the study of the human coronary venous system. Thebesius' theory about the vascular communication between the coronary arteries and the chambers of the heart helped to advance understanding of hemodynamic principles and to clarify the physiologic pathways of the coronary circulation. The following article-the third in a trilogy about Lower Silesian scientists-provides an overview of the life story and achievements of this Silesian physician and innovator, whose name was immortalized in 2 cardiac eponyms: the Thebesian veins and the Thebesian valve.

The use of two Amplatzer "Cribriform" Septal Occluders to close multiple postinfarction ventricular septal defects.

Rupture of the interventricular septum is an uncommon and often fatal complication of myocardial infarction. Herein, we report the successful deployment of the Amplatzer Multi-Fenestrated Septal Occluder-"Cribriform" (AGA Medical Corporation; Plymouth, Minn) during 2 procedures to close multiple postinfarction ventricular septal defects in a severely ill patient. We show that, in selected patients who have multi-fenestrated multiple postinfarction ventricular septal defects, transcatheter implantation of the Amplatzer Cribriform Occluder can be a good therapeutic option.

Percutaneous closure of perimembranous ventricular septal defects with Amplatzer occluders--a single centre experience.

BACKGROUND: Perimembranous ventricular septal defect (VSD) is the most common congenital heart defect. Percutaneous transcatheter closure of VSD is one of the greatest challenges in interventional cardiology. AIM: Presentation of our experience in transcatheter closure of perimembranous VSD. METHODS: Eighteen patients were treated. Nine patients (group I) had VSD closed with implant Amplatzer Perimembranous VSD Occluder (PMVSDO) whereas the other nine had VSD closed with Muscular VSD Occluder (MVSDO). In the second group the presence of at least 4 mm rim from aortic valve was mandatory to undergo the procedure. Average patients age was 17.1 (3.2-40) years, defect diameter--4.7 (4-8) mm and Qp/Qs ratio--1.84 (1.5-4.6). Perimembranous interventricular septum aneurysm was noted in 5 cases. Only patients who had hemodynamically important defect (Qp/Qs ratio >1.5) were selected for interventional VSD closure. Patients with subarterial VSDs, pulmonary hypertension or/and aortic regurgitation were excluded. Ventricular septal defect closure was performed with standard techniques. RESULTS: Procedures were completed successfully in 16 of 18 patients. There was no early or late implant embolisation. After the procedure in every case complete closure or important reduction of the shunt was observed. In the group I there was a trend towards more frequent occurrence of rhythm disturbances (p=0,08), including two cases with severe arrhythmias occurring during VSD closure requiring abandoning of procedure. In other 2 cases (patients age 12 and 14 years) in the second week after PMVSDO placement complete atrio-ventricular block occurred. In one patient sinus rhythm was restored after steroid treatment whereas another patient required pacemaker implantation. In group II mild nonprogressive tricuspid regurgitation was noted in 3 patients. CONCLUSIONS: Percutaneous perimembranous VSD closure is an interesting alternative to surgical treatment. In selected cases closure of the defect with muscular VSD implant is effective and safe.

Percutaneous closure of post-traumatic and congenital muscular ventricular septal defects with the Amplatzer Muscular VSD Occluder.

BACKGROUND: Muscular ventricular septal defects (VSD) are an important and difficult surgical problem. In the last few years a new alternative has emerged - possibility of VSD closure using percutaneous approach. AIM: To present our experience in percutaneous closure of congenital muscular and one posttraumatic VSD. METHODS: We treated 10 patients - 7 children (age 0.8-7 years) and 2 adults (43 and 46 years) with congenital VSD, and one 18-year-old patient with posttraumatic VSD (knife stab). All the patients had a large haemodynamic shunt (Qp:Qs 1.9) and in all cases percutaneous closure attempt with an Amplatzer Muscular VSD Occluder (MVSDO) implant was undertaken. Five of 6 children with multiple muscular VSDs had in infancy previous pulmonary artery banding and one patient had complex heart disease: transposition of great arteries (dTGA), pulmonary stenosis (PS) and perimembranous VSD. All procedures were performed using the standard technique. RESULTS: Eleven procedures were performed in 10 patients (one child had 2 attempts). Seven procedures were successful. In all cases a considerable reduction in flow or complete closure was achived. In one case, despite multiple attempts, VSD caniulation was ineffective and the procedure was abandoned. The patient had oblique VSD - morphology confirmed was later by the operating surgeon. The reason for the other 3 failures was early embolisation to the left ventricle and aorta. This complication was noted in 2 adult patients - one with congenital and one with post-traumatic VSD. In both cases the interventricular septum was thick (10 and 11 mm) and implants were removed with a bioptome or vascular lasso. Another embolisation occurred in a child with TGA - in this case the cardiac surgeon removed the implant from the aortic arch during Rastelli operation. CONCLUSION: Our experience acquired during muscular VSD closure with MVSDO indicates that the method is useful in children with isolated defects. Adult patients and children with a complex form of congenital defects should have morphology of MVSDO carefully evaluated and width of the interventricular septum measured to avoid potential implant embolisation.