Case Report: Heart aneurysm of unknown origin in a two-year-old child diagnosed in the course of Multisystem Inflammatory Syndrome in Children.

We present a case of a 22-month-old boy with a hypokinetic and thin-walled aneurysm of the left ventricle apex. The lesion was diagnosed during routine echocardiography examination in the course of MIS-C, and its occurrence due to MIS-C is plausible. Cardiac magnetic resonance imaging revealed an akinetic aneurysm of the LV apex with a full-wall ischemic scar. Aortography confirmed a normal course of coronary arteries, with adequate perfusion of essential branches and no evidence of stenosis or aneurysms. The boy underwent consultation with the heart team and was deemed eligible for surgery. The aneurysm was excised up to the margin of healthy tissues, and both the surgery and the periprocedural period were uneventful. Determining the origin of the aneurysm is challenging. The most probable etiology appears to be a congenital lesion. Another consideration is an ischemic lesion that may have resulted from impaired coronary circulation during the complicated course of MIS-C. It is possible that this disturbance resolved spontaneously before aortography was performed. Additionally, a complication of pericarditis cannot be entirely ruled out.

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.

Endothelin-1 (ET-1), N-terminal fragment of pro-atrial natriuretic peptide (NTpro-ANP), and tumour necrosis factor alpha (TNF-α) in children with primary hypertension and hypertension of renal origin.

INTRODUCTION: Hypertension is regarded as a condition of mild inflammation and endothelial imbalance. The aim of the study was to evaluate serum concentrations of biomarkers of inflammation and endothelial function: tumour necrosis factor alpha (TNF-α), endothelin-1 (ET-1), and N-terminal fragment of pro-atrial natriuretic peptide (NTpro-ANP) in hypertensive and normotensive children. MATERIAL AND METHODS: We studied 63 children aged 13.56 ± 3.73 years, divided into two groups: a group with primary hypertension (n = 50) and a group with renal hypertension (n = 13). The control group consisted of 34 normotensive children aged 12.76 ± 3.96 years. Biomarkers were measured with ELISA tests. RESULTS: ET-1 levels were significantly higher in primary hypertension (9.93 ± 1.73 pg/ml) and renal hypertension (10.77 ± 1.50 pg/ml) in comparison to controls (4.03 ± 0.97 pg/ml), (p < 0.001, p < 0.001, respectively). NT-pro ANP concentrations in primary hypertension (71.03 ± 10.02 pg/ml), and renal hypertension (84.78 ± 6.44 pg/ml) were significantly higher than in the control group (29.62 ± 5.56 pg/ml) (p < 0.001, p < 0.001, respectively). TNF-α concentrations in primary hypertension (8.36 ± 1.60 pg/ml) and renal hypertension (7.35 ± 0.93 pg/ml) significantly exceeded concentrations in controls (4.49 ± 0.93 pg/ml), (p < 0.001, p < 0.001, respectively). ET-1 and NT-pro ANP concentrations in renal hypertension significantly exceeded those in primary hypertension (p = 0.049, p < 0.001, respectively) while TNF-α levels in renal hypertension were significantly lower than in primary hypertension (p = 0.046). CONCLUSIONS: The results of our study show that ET-1, NT-pro ANP, and TNF-a concentrations are increased in hypertension in children. Our investigation indicates significant importance of inflammation and endothelial involvement in hypertension in youth.

Incomplete endothelialisation of an Amplatzer Septal Occluder device followed by meningitis and late acute bacterial endocarditis.

A 19-year-old woman with atrial septal defect treated percutaneously with an Amplatzer Septal Occluder 24 months earlier, who presented with a history of bacterial meningitis, was admitted with a diagnosis of endocarditis. After 6 weeks of treatment with antibiotics, the incompletely endothelialised occluder was surgically removed. The present report illustrates the need for long-term follow-up of patients who have received nitinol wire mesh occluders.

Transcatheter closure of ventricular septal defects with nitinol wire occluders of type patent ductus arteriosus.

Ventricular septal defects closure (VSD) depending on the anatomy and clinical setting can be performed surgically or by a hybrid and transcatheter approach. Two cases of children with VSD will be presented. Patients' defects were closed with various types of occluders made of nitinol wire mesh occluder, patent ductus arteriosus (PDA) type. The first case was a 2.5-year-old boy after cardiosurgical correction of tetralogy of Fallot (TOF). After the procedure, a significant haemodynamic residual VSD was observed, which was not successfully closed during the subsequent reoperation. Despite pharmacological treatment, symptoms of heart failure were observed in this patient. In echocardiographic images the residual VSD was presented as a tunnel-like dissection of the ventricular septum (length 6 mm and diameter 3.4 mm). The defect was closed via arterial access with an Amplatzer Duct Occluder II (ADO II). The procedure was successfully performed without any medical complications. In this child, a significant shunt reduction and a noticeable improvement in the patient's clinical status and diminished symptoms of heart failure were noticed. The second patient was a 4-year-old girl suffering from a multi-perforated perimembranous VSD accompanied by a ventricular septal defect with aneurysm. The defect was closed by a venous approach with a PDA Cardio-O-Fix occluder (very similar to ADO I). No short-term or long-term complications were visible during or after the procedure. Only a mild residual shunt through the VSD was observed 6 months afterwards. Transcatheter VSD closure with a proper morphology, with occluders of type Amplatzer Duct Occluder ADO I or ADO II, constitutes a safe and effective therapeutic alternative.

The increasing incidence of initial steroid resistance in childhood nephrotic syndrome.

BACKGROUND: Recently, a number of reports have highlighted changes in the histopathology and response to corticosteroid treatment in childhood nephrotic syndrome; however, these involved ethnically mixed populations. For comparison, the purpose of our research was to search for changes in the characteristics of nephrotic syndrome in a homogeneous population of Caucasian children over two consecutive decades. METHODS: Chart analysis was performed to identify children with new-onset nephrotic syndrome. The children were admitted to the Division of Pediatric Nephrology, Zabrze, during two periods: 1986-1995 (76 patients) and 1996-2005 (102 patients). Specifically, a comparison of clinical characteristics and morphology of nephrotic syndrome between the two groups was performed. Steroid resistance was defined as no remission within 8 weeks of corticosteroid treatment. Histopathology was available in 36.8% and 43.1% of patients respectively. RESULTS: There was a significant increase in primary steroid resistance in the latter decade: 15.8% vs 31.4% (P = 0.017). Changes in the histopathology did not reach the level of statistical significance: minimal change nephrotic syndrome 25% vs 9% (P = 0.095), mesangial proliferative glomerulonephritis 46.4% vs 61.3% (P = 0.21), focal segmental glomerulosclerosis 17.9% vs 20.4% (P = 0.78), membranoproliferative glomerulonephritis 7.1% vs 6.8% (P = 1.0), membranous glomerulonephritis 3.6% vs 0% (P = 0.39). CONCLUSIONS: Our results show the increasing incidence of primary steroid resistance in childhood nephrotic syndrome.

Application of Cardio-O-Fix occluders for transcatheter closure of patent ductus arteriosus and interatrial communications: Preliminary experience.

BACKGROUND: Transcatheter treatment has become the method of choice for treating many heart defects. Recently, Cardio-O-Fix occluder (COF) - a new, self-expandable nitinol wire-mesh device very similar to the Amplatzer device - has been introduced into clinical practice. To the best of our knowledge, this is the first publication related to its application. METHODS: Five patients aged from six months to 69 years were included in the study: two with atrial septal defect (ASD), one with patent foramen ovale (PFO) after cryptogenic stroke, and two with patent ductus arteriosus (PDA). These latter two comprised one six month old infant with co-existent hypertrophied cardiomyopathy, and a 53 year-old woman with recanalized PDA after previous ligation. All were treated percutaneously with COF. There was no preliminary patient selection. The only limitation was the size of the devices in our possession (16 and 22 mm ASD COF, 25 PFO COF, 4/6 and 6/8 PDA COF). The implantation technique was the same as previously described for Amplatzer occluders. RESULTS: All procedures were finished successfully with complete closure of the shunt. No complications were observed during a six month follow-up. In the child with PDA, we observed decrease of gradient from 80 to 60 mm Hg in hypertrophied left ventricular outflow tract, although a small protrusion of PDA-COF device was noted in the descending aorta (8 mm Hg gradient in ECHO). In the patient with recanalized PDA, the procedure was performed after arterio-venous loop creation. Mean fluoroscopy time was 4.4 (range from 1.6 to 11) minutes. CONCLUSIONS: Our preliminary experience indicates that the application of Cardio-O-Fix devices is safe and effective.

Utility of the dobutamine stress echocardiography in the evaluation of the effects of a surgical repair of aortic coarctation in children.

BACKGROUND: Exercise-induced hypertension following repair of the coarctation of the aorta (CoA) is a well known phenomenon. The most important functional parameters in the assessment of the effects of a surgical repair of CoA are the maximal pressure gradient in the descending aorta (GRAD) and systolic blood pressure (SBP). Results of treadmill exercise test using the Bruce protocol (treadmill test) and dobutamine stress echocardiography (DSE) were compared to determine utility of the DSE in the evaluation of the effects of surgical treatment of CoA in children. METHODS: The study population comprised of 29 patients, including 20 males and 9 females (mean age 12 years) who underwent a surgical repair of CoA. Changes of the cardiovascular parameters including SBP, GRAD and heart rate (HR) during the treadmill test and DSE were compared. RESULTS: During the treadmill test, SBP at peak exercise ranged from 120 to 230 (mean 163.7) mm Hg, GRAD ranged from 29 to 109 (mean 59.8) mm Hg, and HR ranged from 140 to 188 (mean 169) bpm. At the end of DSE, SBP ranged from 123 to 215 (mean 164.7) mm Hg, GRAD ranged from 29 to 113 (mean 55.4) mm Hg, and HR ranged from 76 to 155 (mean 111) bpm. We found positive correlations of SBP (r = 0.68, p < 0.001) and GRAD (r = 0.82, p < 0.001) values during both tests but no significant correlation for HR (r = 0.42, p = NS). CONCLUSIONS: Dobutamine stress echocardiography is useful in the evaluation of the effects of surgical repair of CoA in children.

[Transcatheter closure of postinfarction ventricular septal defects using Amplatzer devices]. / Cierre percutáneo de comunicaciones interventriculares postinfarto mediante los dispositivos de Amplatzer.

We carried out transcatheter procedures to close postinfarction ventricular septal defects (PIVSDs) in 19 patients: two had recanalization after surgical closure, and 17 had a primary PIVSD. In three of the latter patients, who had acute PIVSDs, the procedure was carried out in the first 3 weeks after infarction; in the 13 patients with subacute PIVSD, it was carried out 3.5-12 weeks after infarction. There was another procedure in one patient with chronic PIVSD. In total, 22 procedures were completed: 17 using an Amplatzer atrial septal occluder, two using an Amplatzer postinfarction ventricular septal defect occluder, and two using an Amplatzer muscular ventricular septal defect occluder. The procedure was successful in 14 patients: in 11 with subacute PIVSD, one with chronic PIVSD, and two with postsurgical PIVSD. Transcatheter closure of PIVSDs using an Amplatzer atrial septal occluder is probably the treatment of choice in patients undergoing surgery more than 3.5 weeks after myocardial infarction and in those with recanalization after previous surgical closure.

Cierre percutáneo de comunicaciones interventriculares postinfarto mediante los dispositivos de Amplatzer / Transcatheter closure of postinfarction ventricular septal defects using amplatzer devices

Se intentó el cierre percutáneo de la comunicación interventricular postinfarto (CIVPI) en 19 pacientes: 2 con recanalización tras el cierre quirúrgico y 17 con CIVPI primaria. En estos últimos, el procedimiento se realizó en 3 pacientes con CIVPI aguda dentro de las primeras 3 semanas postinfarto, en 13 con CIVPI subaguda 3,5-12 semanas postinfarto y en uno con CIVPI crónica. Se llevaron a cabo 22 intervenciones mediante la utilización de 17 oclusores Amplatzer auriculares, 2 ventriculares postinfarto y 2 ventriculares musculares. El procedimiento fue satisfactorio en 14 pacientes: 11 con CIVPI subaguda, uno con CIVPI crónica y 2 posquirúrgicos. El cierre percutáneo de CIVPI con el Amplatzer Atrial Occlusor es probablemente el tratamiento de elección en pacientes con más de 3,5 semanas después del infarto de miocardio y en casos con recanalización tras el cierre quirúrgico (AU)

We carried out transcatheter procedures to close postinfarction ventricular septal defects (PIVSDs) in 19 patients: two had recanalization after surgical closure, and 17 had a primary PIVSD. In three of the latter patients, who had acute PIVSDs, the procedure was carried out in the first 3 weeks after infarction; in the 13 patients with subacute PIVSD, it was carried out 3.5­12 weeks after infarction. There was another procedure in one patient with chronic PIVSD. In total, 22 procedures were completed: 17 using an Amplatzer atrial septal occluder, two using an Amplatzer postinfarction ventricular septal defect occluder, and two using an Amplatzer muscular ventricular septal defect occluder. The procedure was successful in 14 patients: in 11 with subacute PIVSD, one with chronic PIVSD, and two with postsurgical PIVSD. Transcatheter closure of PIVSDs using an Amplatzer atrial septal occluder is probably the treatment of choice in patients undergoing surgery more than 3.5 weeks after myocardial infarction and in those with recanalization after previous surgical closure (AU)

Closure of atrial septal defects in children: surgery versus Amplatzer device implantation.

We prospectively compared closure and complication rates in 91 children with secundum atrial septal defects: 44 (mean age, 8.1 +/- 4.7 years) were treated surgically and 47 (mean age, 10.1 +/- 4.9 years) were treated by percutaneous Amplatzer septal occluder Complications were classified as mild, moderate, or severe. The closure rate was similar in the 2 groups: 42/44 children (95.5%) in the surgical group versus 46/47 patients in the device group (97.5%). Mild complications were observed in 17/44 patients in the surgical group vs 2/47 in the device group; moderate, 11/44 in the surgical vs 1/47 in the device group; and severe, 2/44 in the surgical group vs none in the device group. Blood products were administered to 18 patients in the surgical group and to 1 patient in the device group (P < 0.001). Transcatheter closure of secundum atrial septal defects with the Amplatzer device has the advantage of fewer complications, shorter hospitalization, and reduced need of blood products. Nonetheless, the surgeon's ability to close any atrial septal defect regardless of its size or location remains an important advantage of surgery.

Transcatheter closure of double atrial septal defects with a single Amplatzer device.

Transcatheter closure of single secundum atrial defects has become the standard of treatment. The purpose of our study was to analyze the results of using a single Amplatzer device for closure of double atrial septal defects. Such defects were diagnosed in 41 out of 363 patients with atrial septal defects (ASDs) closed by transcatheter method. In 39, a single Amplatzer device was used. The size of the larger defect ranged from 5 to 18 mm, the smaller defect from 2 to 7 mm, with the distance between the borders of the communications ranging from 2 to 12 mm. We performed sizing and closure of only the larger defect. The mean size of implanted devices was 16.5 +/- 5.5 mm, equal to the stretched diameter of the main defect or 1-4 mm larger. The closure rate assessed by color Doppler flow examination was 61% after 24 hr, 78% after 1 month, 83% after 3 months, 86% after 1 year, and 95% after 2 years. If the distance between the two defects exceeded 7 mm, residual leaks were observed but tended to decrease and disappear with time. Selected patients with double atrial septal defects can be effectively treated with implantation of a single Amplatzer device.

[Percutaneous catheter closure of atrial septal defect. Short-term and mid-term results]. / Cierre percutáneo de la comunicación interauricular. Resultados a corto y mediano plazo.

INTRODUCTION: Percutaneous closure of atrial septal defects (ASD) is becoming more frequent. PATIENTS AND METHOD: From October 1997 to October 2002, 209 patients, age 0.4-70 (mean 19.5) years, were catheterized to close an ASD or patent foramen ovale (PFO). Transesophageal echocardiography was performed simultaneously in all patients. Two hundred and six patients had ASD (25 multiple ASDs) and 3 had PFO. Three devices were used, the Amplatzer Atrial Septal Occluder (ASO), CardioSeal (CS), and Starflex (SF). RESULTS: Device implantation was achieved in 181 patients (87%) but had to be abandoned in 28 patients, generally because the ASD was too large. One hundred and seventy-four ASOs were implanted in 172 patients with ASD (2 ASOs were implanted in 2 patients with double ASD) and CS/SF in 9 patients (3 patients with PFO and 6 with ASD). The procedure was effective in 166/172 (96%) ASO implantations and in 8/9 (89%) CS/SF implantations. The procedure was unsuccessful in 7 patients and the device had to be removed (6 ASO and 1 SF). The occlusion rate with ASO was 88% after 24 hours, 91% after 1 month, 95% after 1 year, 97% after 2 years, and 100% after 4 and 5 years. All defects treated with SF/CS were closed successfully after 24 hours. In one case the ASO device was embolized to the aorta. In the first month after ASO implantation, supraventricular tachycardia appeared in 2 patients and transient left ventricular failure in 2 patients. No late complications were observed. CONCLUSION: Percutaneous catheter closure of selected types of ASD using the Amplatzer Atrial Septal Occluder, CardioSeal, or Starflex should be offered to patients as non-surgical alternative. The type of device used depends on the defect size and morphology as well as the surgeon's experience. The presence of multiple defects does not exclude the possibility of a successful percutaneous catheter closure.

Cierre percutáneo de la comunicación interauricular. Resultados a corto y medio plazo / Percutaneous catheter closure of atrial septal defect. Short-term and mid-term results

Introducción. El cierre percutáneo de la comunicación interauricular (CIA) es una alternativa de tratamiento cada vez más utilizada. Pacientes y método. Entre octubre de 1997 y de 2002 se realizó cateterismo cardíaco, con la intención de cierre de una comunicación interatrial (CIA) o foramen oval permeable (FOP), en 209 pacientes con edades comprendidas entre 0,4 y 70 años (media, 19,5 años). En todos los casos se realizó ecocardiografia transesofágica (ETE) simultánea. En 206 casos existía CIA (25 con defectos múltiples) y en tres un FOP. Se utilizaron 3 dispositivos: Amplatzer Atrial Septal Occluder (ASO), CardioSeal (CS) y Starflex (SF).Resultados. Se aceptó para cierre percutáneo a 181 pacientes (87 por ciento). Se rechazaron 28 casos, en su gran mayoría por el excesivo tamaño del defecto. En 172 pacientes con CIA se utilizaron 174 ASO (en 2 casos con 2 defectos alejados se implantaron 2 ASO) y en 9 pacientes (seis con CIA y tres con FOP) los dispositivos CS/SF. El implante percutáneo del ASO fue efectivo en 166/172 (96 por ciento) pacientes, y el de CS/SF en 8/9 (89 por ciento) pacientes. En 6 casos de ASO y uno de SF el cierre no se pudo realizar por posicionamiento incorrecto del dispositivo. El porcentaje de cierre completo de la CIA con ASO fue del 88 por ciento después de 24 h, del 91 por ciento al mes, del 95 por ciento a un año, del 97 por ciento a los 2 años y del 100 por ciento a los 4 y 5 años de su implante. Todos los defectos tratados con dispositivos CS/SF se cerraron completamente después de 24 h. Como complicaciones destaca un caso de embolización de ASO en la aorta, extrayéndose el dispositivo con cirugía, 2 casos con episodios de taquicardia supraventricular y otros dos con insuficiencia cardíaca izquierda transitoria durante el primer mes tras la colocación de un ASO. Conclusión. El cierre percutáneo en los pacientes portadores de CIA o FOP es una atractiva alternativa al tratamiento quirúrgico. La elección del implante depende de la anatomía del defecto y la experiencia del médico. La existencia de múltiples defectos no excluye la posibilidad de su cierre percutáneo eficaz (AU)